110. Case Report: Feeling Dyspneic & Rejected – University of Maryland

CardioNerds (Amit Goyal and Karan Desai) enjoy a picnic at Charm City’s Inner Harbor with Dr. Manu Mysore, Dr. Shawn Samanta, and Dr. Rawan Amir from the University of Maryland division of Cardiology as they dive into important case discussion about a patient with of non-ischemic cardiomyopathy s/p orthotopic heart transplantation who presents with dyspnea due to cell mediated rejection. Dr. Gautam Ramani Medical Director of Clinical Advanced Heart Failure at the University of Maryland, provides the e-CPR segment.

Claim free CME just for enjoying this episode!

Jump to: Patient summaryCase mediaCase teachingReferences


Patient Summary

A 58 year old woman with a history of non-ischemic cardiomyopathy s/p orthotopic heart transplantation in 2015 presented with worsening dyspnea upon exertion. Dyspnea in a post cardiac transplant brings forth a wide differential diagnosis spanning all the typical causes of dyspnea as well as causes more specific or common to the patient with a heart transplant. In this particular case, TTE showed newly reduced ejection fraction and valvular disease. Cell mediated rejection was considered highest on the differential and confirmed on endomyocardial biopsy. Given hemodynamic compromise with multiple foci of myocyte damage on biopsy, she was started on high dose steroids and anti-thymocyte globulin for treatment of rejection.  Early identification and management of cell mediated rejection is crucial to the survival of patients like ours. Final diagnosis: orthotopic heart transplantation rejection.


Case Media – Orthotopic heart transplant rejection

TTE: Short axis
TTE: Long axis
TTE: Apical 4 Chamber
Coronary angiography: RCA
Coronary angiography: LAD/LCx

Episode Education

Pearls

  1. New onset heart failure in a post cardiac transplant patient should raise concern for acute cardiac allograft rejection, as well as all the usual culprits in nontransplant patients.
  2. Younger African American women and those with elevated HLA mismatches are key risk factors for cell mediated rejection.
  3. Treatment for cell-mediated (i.e., T-Cell mediated) rejection includes steroids and antithymocyte immunoglobulin and regimens are based on the severity ofclinical and histologic features.
  4. Though infrequent as an initial presentation of acute cellular rejection, new onset arrhythmias in a post cardiac transplant patient should raise concern for rejection as a possible etiology. 
  5. Reversal of rejection should be verified with endomyocardial biopsy following treatment for rejection. The timing and frequency of biopsy will likely depend upon whether corticosteroids and/or antithymocyte therapy was utilized.

Notes – Cell mediated rejection and more!

1) What are some common complications of cardiac transplantation?

Common complications following cardiac transplantation can be divided into two major categories: graft-related complications and non-graft-related complications.

  • Graft-related complications include:
    • Early graft dysfunction (EGD) – reversible and irreversible injury related to organ procurement and reperfusion. Remember it is common for transplant patients to require inotropic and vasopressor support coming off cardiopulmonary bypass. Furthermore, LV diastolic dysfunction is also common after transplantation usually reflecting reversible ischemia or reperfusion injury and normally resolves over days to weeks, depending on the severity of reperfusion.
      • Primary graft dysfunction (PGD) is a severe form of EGD that presents as a left, right or biventricular dysfunction occurring within the first 24 hours of transplantation for which there is no identifiable secondary cause (e.g. hyperacute rejection, prolonged ischemic time from massive intra-operative bleeding. The etiology is likely multifactorial including but not limited to reperfusion injury, the effect of donor brain death, and pre-existing donor heart disease.
    • Early RV dysfunction related to pulmonary vascular resistance and fluid shifts early post-transplant may be particularly challenging. The RV is exposed to similar reperfusion injury or ischemic insults as the LV and typically RV dysfunction post-transplant includes RV dilation, subsequent poor coaptation of the tricuspid valve and tricuspid regurgitation. The “untrained” donor RV has to overcome potentially increased afterload (due to increased pulmonary vascular resistance) in the recipient, and as has been covered in previous Cardionerds episodes, the RV systolic function is highly sensitive to changes in afterload.
    • Acute allograft rejection – either cellular-mediated rejection or antibody-mediated rejection, occurring due to the recipient’s immune system reacting against graft antigens (e.g., mainly, but not only, the human leukocyte antigen (HLA) mismatches). Hyperacute rejection is rare and commonly fatal complication of cardiac transplantation. It is mediated by preformed anti-donor antibodies and can lead to diffuse hemorrhage and thrombosis in the allograft. In the current era of panel-reactive antibody screening (PRA) where we screen for preformed anti-HLA recipient antibodies to donor lymphocytes, hyperacute rejection is rare but remains a possibility (especially in highly sensitized patients and/or depending on the technique of obtaining PRAs). See more below on antibody- and cell-mediated rejection.
    • Cardiac Allograft Vasculopathy – an important cause of morbidity and mortality late following heart transplant related to both immune- and nonimmune-mediated coronary injury causing accelerated atherosclerosis and fibroproliferation with diffuse intimal hyperplasia resulting in allograft ischemia. For a detailed discussion on CAV, enjoy Ep #69.
  • Non-Graft-Related Complications
    • Infections – related both to nosocomial exposures and immunosuppression, the typical infectious agents and syndromes predictably vary according to time from transplant. Early following transplant, the recipient is particularly susceptible due to post-operative nosocomial exposures (e.g., surgical wound, vascular access, urinary catheter, etc) and high dose peri-transplant immunosuppression. As such, wound/line/urinary infections and infections involving fungal and multidrug resistant bacterial organisms are common in the early phase (<1 month). In the mid-term (1-6 months), pneumonia, UTIs, and viral infections (CMV, HSV, VZV) are common. In the late-term, after the first post-transplant year, opportunistic infections become less common, and the typical community-based pathogens predominate.
    • Acute and chronic renal injury – renal dysfunction is a common and important complication post-cardiac transplantation. Etiologies are varied and interrelated and include pre-transplant renal dysfunction, acute injury pre-operatively, calcineurin inhibitor toxicity, cardiorenal syndromes related to graft dysfunction, and chronic injury due to long-term metabolic complications (diabetes, hypertension).
    • Malignancies – major problem in transplant recipients with rising cumulative risk over time. Post-transplant cancer risk is related to both immunosuppression dulling the normal immune system’s cancer surveillance and viral triggers for carcinogenesis. Common malignancies include lung cancer (especially as a significant proportion of patients with ischemic cardiomyopathy have a history of tobacco use), skin cancer, lymphomas, and breast and colon cancer. Post-transplant lymphoproliferative disorder (PTLD) is an EBV-associated proliferation of B-lymphocytes that is typically related to the degree of immunosuppression.

2) What are acute cell mediated rejection and antibody mediated rejection?

  • Acute cell mediated rejection (ACR) is a host T cell lymphocyte response directed towards allograft tissue, leading to T-cell mediated cytotoxicity of myocardial tissue. It can be seen anywhere from weeks to months after transplantation. Risk factors include younger donor and/or recipient age, African American ethnicity, and history of significant HLA mismatches.  
  • Acute antibody mediated rejection (AMR) constitutes graft injury by circulating antibodies (immunoglobulin M or G) targeting antigens expressed by graft endothelial cells. Injury may be complement mediated or complement independent (e.g., by other inflammatory pathways within endothelial cells and/or by natural killer cells).

3) What are clinical manifestations of acute cell mediated rejection?

  • Ideally, ACR is diagnosed prior to overt clinical manifestations from surveillance endomyocardial biopsies or Allomap testing (a blood test of gene-expression profiling of peripheral blood mononuclear cells used in select patients).
  • Clinical manifestations of acute cell mediated rejection typically include symptoms of LV dysfunction including dyspnea, PND, orthopnea, palpitations, syncope or near-syncope. Signs of RV dysfunction causing right-sided congestion may include gastrointestinal symptoms such as nausea which could be a marker of hepatic congestion! Occasionally, patients can present with new onset atrial arrhythmias including atrial fibrillation or atrial flutter.
  • Ultimately, cardiac transplant rejection is a form of myocarditis and so progressively severe forms may result in any of the manifestations of fulminant myocarditis including cardiogenic shock, atrial and ventricular arrhythmias, and conduction abnormalities. Thankfully, this is rare with modern immunosuppression and with routine rejection surveillance.

4) How is acute cell mediated rejection diagnosed and what are the histologic classifications?

  • ACR is diagnosed via endomyocardial biopsy.
  • Notably, there is wide interobserver variability in severity grading between pathologists and centers.
  • Biopsy samples are graded histologically as follows:
Histological GradeInterpretation
Grade 0RIndicates no sign of cell mediated rejection.
Grade 1RRepresents mild cell mediated rejection with interstitial and/or perivascular infiltrate with up to one focus of myocyte damage.
Grade 2RRepresents moderate cell mediated rejection involving ≥2 foci of infiltrate with associated myocyte damage.
Grade 3RRepresents severe cell mediated rejection with diffuse infiltrate and multifocal myocytic damage, with or without edema, hemorrhage, or vasculitis.
Biopsy samples are graded histologically

5) How is acute cell mediated rejection treated?

  • Treatment of ACR depends on the severity of rejection, as deemed both clinically and histologically.
  • Clinical severity is determined by presence of hemodynamic instability (i.e., decrease in cardiac output, decrease in pulmonary artery oxygen saturation, elevated pulmonary capillary wedge pressure, symptoms of heart failure)
  • Histologic severity is determined by the histologic grade as above.
  • If there is ACR warranting immunosuppression escalation, serial endomyocardial biopsies are typically performed to verify resolution and guide further management.
  • Anti-rejection therapy should typically be adjusted or discontinued if there is a documented infection with resolution of histologic rejection on subsequent biopsy.
  • Antibiotic and antiviral prophylaxis is given with anti-rejection treatment (high-dose steroids +/- anti-thymocyte globulin)
  • Treatment is (generally) as follows:
    • Grade 1R without hemodynamic compromise – generally does not warrant specific treatment.
    • Grade 1R w/ hemodynamic compromise – generally treated with high-dose corticosteroids or antibody therapy depending on the severity of hemodynamic compromise. If there is severe hemodynamic compromise, some centers will pursue more aggressive therapy with Grade 1R rejection including considering plasmapharesis.
    • Grade 2R without hemodynamic compromise – generally treated with a transient increase in the corticosteroid dose with subsequent return to the prior oral steroid dose. Select patients may even be treated as an outpatient if there is no hemodynamic compromise.
    • Severe or refractory rejection (Grade 2R w/ hemodynamic compromise, Grade 3R, or rejection unresponsive to corticosteroid therapy) – generally treated with pulse dose steroids with a slow taper as well as anti-thymocyte globulin (ATG). ATG is an infusion of horse or rabbit antibodies against human T cells to deflect a high immunologic burden. Other treatment options depending on the clinical situation include plasmapheresis, extracorpeal photopheresis (an apheresis and photodyamic therapy technique that uses 8-methoxy-psolaren and UV light to modulate T-cell therapy), and total lymphoid irradiation.

6) What is the surveillance schedule post cardiac transplantation for acute cell mediated rejection?

  • Acute cell mediated rejection as mentioned above happens most frequently during the first three to six months after cardiac transplantation.
  • A typical transplant center will perform endomyocardial biopsies weekly for the first four weeks after cardiac transplantation followed by biweekly for the next six weeks or so.
  • Schedule switches slightly in that biopsies are then pursued monthly for the next three to four months before being switched to every three months until it has been a year since cardiac transplantation.
  • This schedule does vary between transplant centers and it is common practice to pursue less invasive monitoring beyond the first few years after transplantation such as through peripheral blood gene expression profiling.

References – Cell mediated rejection

  1. Hamon D, Taleski J, Vaseghi M, Shivkumar K, Boyle NG. Arrhythmias in the Heart Transplant Patient. Arrhythm Electrophysiol Rev. 2014;3(3):149-155. doi:10.15420/aer.2014.3.3.149
  1. Ramzy D, Rao V, Brahm J, Miriuka S, Delgado D, Ross HJ. Cardiac allograft vasculopathy: a review. Can J Surg. 2005;48(4):319-327.
  1. Ludhwani, Dipesh. “Heart Transplantation Rejection – StatPearls – NCBI Bookshelf.” National Center for Biotechnology Information, https://www.ncbi.nlm.nih.gov/books/NBK537057/. Accessed 26 Jan. 2021.
  1. Ingulli E. Mechanism of cellular rejection in transplantation. Pediatr Nephrol. 2010;25(1):61-74.
  1. Potena, Luciano et al. “Complications of Cardiac Transplantation.” Current cardiology reports vol. 20,9 73. 10 Jul. 2018

CardioNerds Case Report Production Team

101. Nuclear and Multimodality Imaging – Coronary Microvascular Disease

CardioNerd Amit Goyal is joined by Dr. Erika Hutt (Cleveland Clinic general cardiology fellow), Dr. Aldo Schenone (Brigham and Women’s advanced cardiovascular imaging fellow), and Dr. Wael Jaber (Cleveland Clinic cardiovascular imaging staff and co-founder of Cardiac Imaging Agora) to discuss nuclear and complimentary multimodality cardiovascular imaging for the evaluation of coronary microvascular disease.  To learn more about multimodality cardiovascular imaging, check out Cardiac Imaging Agora

Collect free CME/MOC credit just for enjoying to the episode! 



Guest Profiles

Wael Jaber, MD, is a staff cardiologist in the Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, at the Sydell and Arnold Miller Family Heart, Vascular & Thoracic Institute at Cleveland Clinic. Dr. Jaber specializes in cardiac imaging (both nuclear cardiology and echocardiography) and valvular heart disease. Dr. Jaber attended college at the American University in Beirut, graduating with a Bachelor of Science in biology. He then went on at the American University to receive his medical degree while making the Dean’s honor list. He completed his residency in internal medicine at the St. Luke’s-Roosevelt Hospital Center at Columbia University College of Physicians and Surgeons, where he also completed fellowships in cardiovascular medicine and nuclear cardiology. Dr. Jaber is currently is the Medical Director of the Nuclear Lab and of the Cardiovascular Imaging Core Laboratory in C5Research. He is fluent in English, French and Arabic. He is the author of Nuclear Cardiology review: A Self-Assessment Tool and cofounder of Cardiac Imaging Agora.

Dr. Aldo L Schenone is one of the current Chief Non-Invasive Cardiovascular Imaging Fellows at the Brigham and Women’s Hospital. He completed medical school at the University of Carabobo in Valencia, Venezuela, and then completed both his Internal Medicine residency and Cardiology fellowship at the Cleveland Clinic where he also served as a Chief Internal Medicine Resident.

Dr. Erika Hutt @erikahuttce is a cardiology fellow at the Cleveland Clinic. Erika was born and raised in Costa Rica, where she received her MD degree at Universidad de Costa Rica. She then decided to pursue further medical training in the United States, with the goal of becoming a cardiologist. She completed her residency training at Cleveland Clinic and went on to fellowship at the same institution. Her passions include infiltrative heart disease, atrial fibrillation, valvular heart disease and echocardiography among many. She is looking forward to a career in advanced cardiovascular imaging.


References and Links

  1. Kaski, J.-C., Crea, F., Gersh, B. J., & Camici, P. G. (2018). Reappraisal of Ischemic Heart Disease. Circulation. https://doi.org/10.1161/circulationaha.118.031373
  2. Jaber, W., & Gimelli, A. (n.d.). Cardiac Imaging Agora. https://www.cardiacimagingagora.com/list/
  3. Taqueti, V. R., & Di Carli, M. F. (2018). Coronary Microvascular Disease Pathogenic Mechanisms and Therapeutic Options: JACC State-of-the-Art Review. In Journal of the American College of Cardiology. https://doi.org/10.1016/j.jacc.2018.09.042

96. Hypertension part 1 with Dr. Luke Laffin

CardioNerds (Amit Goyal and Daniel Ambinder) are joined by Cleveland Clinic cardiology fellow Dr. Gregory Ogunnowo to discuss hypertension with Dr. Luke Laffin, cardiology faculty in the division of Preventive Cardiology and Rehabilitation and Medical Director of Cardiac Rehabilitation at the Cleveland Clinic. Part 1 of this discussion covers the definition of hypertension, correct measurement of blood pressure, nonpharmacologic HTN management, initial choice of BP agents, and hypertensive disorders of pregnancy. Be sure to follow-up with Part 2 to learn about evaluation for secondary causes of HTN, approach to resistant HTN, interventional anti-hypertensive procedures, and a note on cardiac rehabilitation.


Show notes

Coming soon!


Cardionerds Cardiovascular Prevention Series: by the Cardionerds Cardiology Podcast in Collaborate with the The American Society For Preventive Cardiology ASPC
Cardionerds Cardiovascular Prevention Series

The Cardionerds CV prevention series  includes in-depth deep dives on so many prevention topics including the ABCs of prevention, approach to obesity, hypertension, diabetes mellitus and anti-diabetes agents, personalized risk and genetic risk assessments, hyperlipidemia, women’s cardiovascular prevention, coronary calcium scoring and so much more!

We are truly honored to be producing the Cardionerds CVD Prevention Series in collaboration with the American Society for Preventive Cardiology! The ASPC is an incredible resource for learning, networking, and promoting the ideals of cardiovascular prevention! This series is kicked off by a message from Dr. Amit Khera, President of the American Society for Preventive Cardiology and President of the SouthWest Affiliate of the American Heart Association.


Guest Profiles

Dr. Luke Laffin, serves as cardiology faculty in the division of Preventive Cardiology and Medical Director of Cardiac Rehabilitation at the Cleveland Clinic. Dr. Laffin attended medical school at Vanderbilt University School of Medicine. He trained in internal medicine and cardiology at the University of Chicago where he completed a dedicated fellowship in hypertensive diseases. He is a clinical specialist in hypertension designated by the American Society of Hypertension – which has now merged with the AHA.

Dr. Gregory Ogunnowo is a cardiology fellow at the Cleveland Clinic. He completed medical school at the University of South Carolina School of Medicine in Columbia, South Carolina. He went on to complete internal medicine residency at Washington University School of Medicine in St. Louis where he stayed on as faculty in the Department of Hospital Medicine for a year prior to pursing fellowship. His interests include outcomes research in interventional cardiology and medical education In his spare time, Greg enjoys traveling, exercising, and experiencing new cultures through their food. When he’s not in the hospital, you can find Greg planning a trip with close friends and family.


References and Links

Coming soon!

93. Obesity for CardioNerds with Dr. Chiadi Ndumele

CardioNerds (Carine Hamo, Amit Goyal, and Daniel Ambinder) discuss the obesity epidemic and how it relates to the cardiovascular system with Dr. Chiadi Ndumele, cardiologist and epidemiologist at The Johns Hopkins Hospital and chairs the obesity subcommittee of the American Heart Association (AHA). They cover obesity definitions, epidemiology, strengths and limitations of different biometrics, including BMI, impact on myocardial structure and function, and current pharmacologic & surgical options for weight loss. They also discuss the practical approach to addressing obesity with patients. This episode was produced by Dr. Carine Hamo. Show notes & references by Dr. Daniel Ambinder.

CardioNerds (Carine Hamo, Amit Goyal, and Daniel Ambinder) discuss the obesity epidemic and how it relates to the cardiovascular system with Dr. Chiadi Ndumele, cardiologist and epidemiologist at The Johns Hopkins Hospital. They cover obesity definitions, epidemiology, strengths and limitations of different biometrics, including BMI, impact on myocardial structure and function, and current pharmacologic & surgical options for weight loss. This episode was produced by Dr. Carine Hamo. Show notes & references by Dr. Daniel Ambinder.
Episode graphic by Dr. Carine Hamo

Cardionerds Cardiovascular Prevention Page
CardioNerds Episode Page
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!


Show notes

1. What is obesity and how do we define it at the personal and population level? 

  • Obesity is when there is an excess and often dysfunctional adipose tissue that contributes to morbidity and to premature mortality 
  • The metric used to define obesity is Body Mass Index (BMI), defined as a person’s weight in kilograms divided by the square of the person’s height in meters (kg/m2) 
  • See WHO BMI classification below

2. What is the current epidemiology of obesity and are there certain populations that are affected more than others? 

  • Rates of obesity are climbing. Currently, around 70% of the population meets criteria for being either overweight or obese and ~40% are at the level of obesity. 
  • Minorities such as African Americans, Native Americans, and Latinos have higher rates of obesity. 
  • Higher rates of obesity are also seen in groups with lower socioeconomic status. 
  • Certain populations, such as Southeast Asians, tend to develop severe metabolic consequences of obesity such as insulin resistance and cardiovascular consequences with less excess weight than other populations. 
  • Adult weight is very important but weight history (long standing obesity) plays a role as well when it comes to cardiovascular risk associated with obesity.   

3. Currently the WHO classifies obesity based on BMI. What are the limitations to using BMI as a measure of obesity? Are their benefits to measuring waist circumference instead? 

  • BMI is a far from a perfect measure but it correlates nicely at the population level with cardiovascular events and premature mortality 
  • BMI is more accessible than a direct quantitative or functional measure of adipose tissue   
  • A major limitation of BMI is that it does not reflect body composition. Body composition is very important in understanding risk associated with obesity. For example, football players may fall into the category of grade 1 obesity if just using BMI to classify their weight status. 
  • Waist circumference (WC) is a good way of getting a sense of body composition. Abdominal obesity is most closely linked to insulin resistance and various metabolic consequences such as diabetes, hypertension, and inflammation. This is why WC is incorporated into the metabolic syndrome construct. 
  • Adding WC measurements to the BMI measurements, particularly for individuals in the overweight and grade 1 obesity group (BMI 25-29.9, and 30-34.9) provides significant prognostic information about the development of cardiovascular disease. 

4. How do obesity and metabolic syndrome impact myocardial structure and function? How does obesity and increased adiposity fit into the larger scheme of metabolic risk and metabolic syndrome? 

  • Obesity is independently associated with myocardial remodeling and with increased heart failure risk. This contrasts with coronary heart disease (CAD) and stroke. For CAD and stroke, most associations with obesity are largely mediated by diabetes, hypertension and dyslipidemia. However, in heart failure, there is a strong unexplained association that remains after you consider those associated conditions. 
  • The independent association of obesity with heart failure pertains almost exclusively to heart failure with preserved ejection fraction (HFpEF) and not heart failure with reduced ejection fraction (HFrEF). 
  • The mechanism for this independent association is not well understood and is an area of active research. In mice that are predisposed to obesity have several inflammatory processes that occur locally in the myocardium and systemically that likely contribute to cardiac risk.
  • At the local level, lipotoxicity occurs within the myocardium as it does in nonalcoholic fatty liver disease.
  • At the systemic level, adipose tissue releases adipokines and cytokines that are linked to myocardial damage, injury, and fibrosis. 
  • There is a spectrum of metabolic risk among individuals with excess weight. And when obesity is associated with metabolic syndrome in individuals, the risk for cardiovascular disease markedly rises.   

4. What are some core tenants of addressing obesity when working with patients when it comes to exercise and diet?         

  • A core tenant of discussing obesity with patients is to discuss it! Obesity is generally under-addressed and under-discussed. 
  • Motivation by being positive about risk reduction with a healthier lifestyle can be very effective. 
  • Help patients “take time to invest in themselves”. Having them put items on the calendar that include exercise activities, such as taking a walk or going to the gym, can be a useful strategy for patients who are particularly busy with work or school. 
  • Stress reduction is an important component to diet and exercise. 
  • Smaller activities, a brisk walk or taking the stairs at work can help reduce the activation energy required for exercise and can make exercise feel more attainable to patients. 
  • Meal planning and meal timing are both very important aspects to counseling for patients when it comes to healthy eating. 

5. What are some tips and tricks on broaching the subject of obesity with patients given the sensitivity of the subject. 

  • It is important to check biases in this space. Obesity should not be considered an individual failing when there is a systemic and societal based issue. We need to think of obesity as a multi-factorial disease that has a behavioral component but also has a more complex societal and biological contribution as well. 
  • Approaching the patient with a plan for partnership of management of obesity as a disease, like other diseases such as hypertension and diabetes can be very helpful. 
  • Patients want to lose weight, it just becomes very challenging for a variety matters. 
  • The weight of the clinician can have an impact as to the discussion of weight in the clinic. For example, clinicians with a higher weight than the patient tend to avoid discussing obesity during clinic visits. Clinicians who have healthy weight statuses can used stigmatizing language when counseling patients. 
  • Appreciate that weight management can be challenging and there’ll be stops and starts but there can be great outcomes with long-term partnerships with patients. 

6. What are the current pharmacologic options for weight loss and when should these agents be considered? 

  • Pharmacological agents should be considered once physical activity and social stressors are addressed. Pharmacological therapy can be a nice adjunct to lifestyle modification, particularly when BMI remains above 30 or when BMI remains >27 with comorbidities. 
  • There are a variety of agents such as Orlistat, Liraglutide, Phentermine, Topiramate, and Bupropion. 
  • These medications are generally underutilized due to cost and side effects. 
  • Some agents have cannot be used long term which may limit their use. 
  • The only agent that has been related to cardiovascular risk reduction is Liraglutide. 

7. What do we know about the role of bariatric surgery in cardiovascular disease prevention and does weight loss through bariatric surgery provide differential benefit over other forms of weight loss? 

  • Bariatric surgery is probably the most powerful weapon in our obesity arsenal. 
  • There are two major subtypes of bariatric surgery. There is a restrictive subtype, such as a sleeve gastrectomy, and a malabsoptive subtype, such as a gastric bypass surgery. The Roux-en-Y gastric bypass has both the malabsorptive and restrictive components. 
  • There is prospective data that shows that bariatric surgery is associated with more weight loss than lifestyle modifications. Bariatric surgery is also shown to be associated with a reduction in comorbidities like hypertension, diabetes and dyslipidemia. 
  • Bariatric surgery is also associated with a reduction in pathophysiological processes like inflammation and endothelial dysfunction. 
  • Prospective studies with matched data, such as the Swedish Obesity study cohort, bariatric surgery has been associated with a reduced risk in cardiovascular disease events and a markedly improved survival. There have been significant risk reductions in heart failure as well. 
  • Most cardiovascular disease reductions seen with bariatric surgery occur through the profound weight loss that occurs after surgery. 
  • Risk calculators such as https://riskcalc.org/BariatricSurgeryComplications/ can help guide clinicians and patients when considering bariatric surgery. 
BMIWHO Classification
Below 18.5Underweight
18.5-24.9 Normal weight
25.0-29.9Pre-obesity
30.0-34.9Obesity class I
35.0-39.9Obesity class 2
> 40.0Obesity class 3

Cardionerds Cardiovascular Prevention Series: by the Cardionerds Cardiology Podcast in Collaborate with the The American Society For Preventive Cardiology ASPC
Cardionerds Cardiovascular Prevention Series

The Cardionerds CV prevention series  includes in-depth deep dives on so many prevention topics including the ABCs of prevention, approach to obesity, hypertension, diabetes mellitus and anti-diabetes agents, personalized risk and genetic risk assessments, hyperlipidemia, women’s cardiovascular prevention, coronary calcium scoring and so much more!

We are truly honored to be producing the Cardionerds CVD Prevention Series in collaboration with the American Society for Preventive Cardiology! The ASPC is an incredible resource for learning, networking, and promoting the ideals of cardiovascular prevention! This series is kicked off by a message from Dr. Amit Khera, President of the American Society for Preventive Cardiology and President of the SouthWest Affiliate of the American Heart Association.


Guest Profiles

Dr. Chiadi Ndumele is an Assistant Professor in the Department of Medicine at Johns Hopkins University. Dr. Ndumele graduated from Harvard University School of Medicine. He completed his Internal Medicine training at Brigham and Women’s Hospital, where he also served as Chief Medical Resident. He was Chief Cardiology Fellow at Johns Hopkins University. During fellowship training, Dr. Ndumele received an MHS and Ph.D. in Epidemiology at Johns Hopkins Bloomberg School of Public Health. Dr. Ndumele’s research has been supported by career development awards from the NHLBI and Robert Wood Johnson Foundation, a Catalyst Award from Johns Hopkins, an R01 from the NHLBI and an AHA Strategically Focused Research Network Grant. He has received national recognition for his work, including a Young Physician-Scientist Award from the American Society of Clinical Investigation. He has national leadership roles including Chair of the Obesity Subcommittee of the American Heart Association (AHA) and Editorial Board membership on the journals Circulation and Circulation Research. Dr. Ndumele’s research focuses on mechanisms linking adiposity to CVD and strategies to improve prediction and prevention.


References and Links

  1. Jensen MD, Ryan DH, Apovian CM, et al. 2013 AHA/ACC/TOS guideline for the management of overweight and obesity in adults: A report of the American College of cardiology/American Heart Association task force on practice guidelines and the obesity society. Circulation. 2014;129(25 SUPPL. 1):102-138. doi:10.1161/01.cir.0000437739.71477.ee
  1. Yu Z, Grams ME, Ndumele CE, et al. Association Between Midlife Obesity and Kidney Function Trajectories: The Atherosclerosis Risk in Communities (ARIC) Study. Am J Kidney Dis. September 2020. doi:10.1053/j.ajkd.2020.07.025
  1. Kaze AD, Musani SK, Bidulescu A, et al. Plasma Adipokines and Glycemic Progression Among African Americans: Findings from the Jackson Heart Study. Diabet Med. November 2020. doi:10.1111/dme.14465
  1. Cohen LP, Vittinghoff E, Pletcher MJ, et al. Association of Midlife Cardiovascular Risk Factors with Risk of Heart Failure Subtypes Later in Life. J Card Fail. November 2020. doi:10.1016/j.cardfail.2020.11.008
  1. Khera R, Pandey A, Ayers CR, et al. Performance of the Pooled Cohort Equations to Estimate Atherosclerotic Cardiovascular Disease Risk by Body Mass Index. JAMA Netw open. 2020;3(10):e2023242. doi:10.1001/jamanetworkopen.2020.23242
  1. Fliotsos M, Zhao D, Rao VN, et al. Body Mass Index From Early-, Mid-, and Older-Adulthood and Risk of Heart Failure and Atherosclerotic Cardiovascular Disease: MESA. doi:10.1161/JAHA.118.009599
  1. Mann JFE, Nauck MA, Nissen SE, et al. Liraglutide and cardiovascular outcomes in type 2 diabetes. Drug Ther Bull. 2016;54(9):101. doi:10.1056/nejmoa1603827
  1. Schauer PR, Bhatt DL, Kirwan JP, et al. Bariatric Surgery versus Intensive Medical Therapy for Diabetes — 5-Year Outcomes. N Engl J Med. 2017;376(7):641-651. doi:10.1056/nejmoa1600869
  1. Aminian A, Zajichek A, Arterburn DE, et al. Association of Metabolic Surgery with Major Adverse Cardiovascular Outcomes in Patients with Type 2 Diabetes and Obesity. JAMA – J Am Med Assoc. 2019;322(13):1271-1282. doi:10.1001/jama.2019.14231
  1. https://riskcalc.org/BariatricSurgeryComplications/

91. Aspirin, Vitamin D, Calcium & Omega 3 Fatty Acids Supplementation with Dr. Erin Michos

The CardioNerds (Carine Hamo and Daniel Ambinder) discuss aspirin as primary prevention, Vitamin D, Calcium, and omega 3 fatty acids supplementation with Dr. Erin Michos, director of women’s cardiovascular health and the associate director of preventive cardiology with Johns Hopkins Ciccarone Center for the Prevention of Cardiovascular Disease. We are also joined by Dr. Michos’ mentees, Dr. Rick Ferraro, Dr. Andi Shahu, and student doctor Sunyoung (Sarah) Jang for a discussion about mentorship and career development. This episode was produced by Dr. Rick Ferraro and Dr. Carine Hamo. Show notes & references by Dr. Amit Goyal.

The CardioNerds (Carine Hamo and Daniel Ambinder) discuss aspirin as primary prevention, Vitamin D, Calcium, and omega 3 fatty acids supplementation with Dr. Erin Michos, director of women's cardiovascular health and the associate director of preventive cardiology with Johns Hopkins Ciccarone Center for the Prevention of Cardiovascular Disease. We are also joined by Dr. Michos' mentees, Dr. Rick Ferraro, Dr. Andi Shahu, and student doctor Sunyoung (Sarah) Jang for a discussion about mentorship and career development. This episode was produced by Dr. Rick Ferraro and Dr. Carine Hamo. Show notes & references by Dr. Amit Goyal.
Episode graphic by Dr. Carine Hamo

Cardionerds Cardiovascular Prevention Page
CardioNerds Episode Page
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!


Show notes – Aspirin, Vitamin D, Calcium & Omega 3 Fatty Acids Supplementation

What is the role of aspirin for primary ASCVD prevention? 

  • The Conundrum: ASCVD event rates are much lower in the primary prevention than in the secondary prevention population, BUT the bleeding rates are comparable. So in the primary prevention patients, the bleeding risk is just as high, but the propensity for benefit is lower. 
  • The Question: Does low dose aspirin have a place in the primary prevention of ASCVD events. 
  • The Data
    • ARRIVE Trial: in moderate risk nondiabetic patients without prior ASCVD events, there was no different in the composite ASCVD end point, but there was an increased risk of bleeding (mostly mild GI bleeding). Thus, in the moderate risk patients –> primary prevention aspirin has an unfavorable risk-benefit profile. The benefit in a higher risk (>10-20% estimated 10-yr risk) remains unclear.  
    • ASCEND Trial: In men and women age ≥ 40yrs with diabetes without prior ASCVD events, there was a modest benefit (NNT = 59 patients for 10 years to prevent 1 major ASCVD event) counterbalanced by a similar magnitude of harm (NNH = 77 patients for 10 years to cause 1 major bleeding event). Thus, in adults with diabetes –> primary prevention aspirin had a neutral risk-benefit profile. 
    • ASPREE Trial: in elderly patients (≥ 70 years; ≥ 65 years for Hispanic or Black patients) without prior ASCVD events, there was no difference in ASCVD events but there was a significant increase in bleeding events (NNH = 42 patients for 10 years to cause 1 major bleeding event). The trial was stopped early due to futility. Interestingly, there was higher all-cause mortality driven primarily by cancer. Importantly, patients had to have a life expectancy longer than 5 years and those with dementia, substantial physical disability, or high estimated bleeding risk were excluded. Thus, in elderly patients –> primary prevention aspirin led to overall harm.  
  • The Recommendations
    • There was insufficient evidence to recommend a specific risk threshold for starting primary prevention aspirin. This may be due to more widespread contemporary prevention strategies like lifestyle management, tobacco cessation, statin use, better blood pressure control, etc.  
    • Individualize the decision based on the totality of evidence for an individual’s risk of ASCVD events versus bleeding events. Notably, those with higher ASCVD risk generally also have a higher bleeding risk.           
    • Class IIB: Low-dose aspirin (75-100 mg orally daily) might be considered for the primary prevention of ASCVD among select adults 40 to 70 years of age who are at higher ASCVD risk but not at increased bleeding risk. 
      • There may be a role for primary prevention aspirin in select adults with a high estimated ASCVD risk and low bleeding risk. 
      • CAC score ≥ 100 may help identify those might benefit from primary prevention aspirin.           
      • As always, shared decision making remains           crucial. 
    • Class III: Low-dose aspirin (75-100 mg orally daily) should not be administered on a routine basis for the primary prevention of ASCVD among adults >70 years of age. 
    • Class III: Low-dose aspirin (75-100 mg orally daily) should not be administered for the primary prevention of ASCVD among adults of any age who are at increased risk of bleeding. 

What is the role of Vitamin D supplementation in preventing cardiovascular disease?            

  • The Conundrum: Low levels of Vit D is associated with increased risk of CV outcomes including myocardial infarction, stroke, heart failure, atrial fibrillation, and more. But while low Vit D seems to be a marker for bad outcomes, correlation ≠ causation. Notably, this correlation was not confirmed by Mendelian randomization studies, further refuting possible causation. Confounding factors include links between low Vit D levels and obesity and sedentary lifestyle, themselves risk factors for adverse CV outcomes. 
  • The Question: Given the association between low Vit D levels & CV disease –> can you prevent CV disease by identifying and treating low Vit D with supplementation.  
  • The Data
    • Randomized Clinical Trials –> treatment with vitamin D does not prevent CV disease. 
    • Women’s Health Initiative: Calcium & Vit D supplementation had no effect on incident coronary or cerebrovascular events. But perhaps this was due to a low Vit D dose of only 400 IU daily. Would a higher dose have benefit? 
    • ViDA Study: monthly high-dose Vit D supplementation (100,000 IU of D3) did not prevent CV disease, including within the 25% of patients who had level < 20 ng/mL. But this was an atypical supplementation regimen. 
    • Vital Trial: neither n-3 fatty acid (1g/day) nor Vit D3 (2000 IU/day) were effective for primary prevention of CV or cancer events among healthy middle-aged men and women over 5 years of follow-up. This was among the largest of the Vit D supplementation trials targeting CV outcomes and most definitively argues against the benefit of supplementation.               
  • The Recommendations:      
    • Data for Vit D supplementation to improve CV outcomes is all null.  
    • National Academy of Medicine: 
      • Age 19-70 years: 600 IU daily 
      • Age > 70: 800 IU daily 
      • Level <12 ng/mL indicates deficiency, 12-20 ng/mL is inadequate, and >20 ng/mL is adequate for bone and overall health. But the optimal level remains contested. The Endocrine Society recommends aiming for level ≥ 30 ng/mL. 

What is the role of Calcium supplementation in preventing cardiovascular disease? 

  • The Conundrum: Calcium supplementation is common and important for bone health. However there is some concern that excess calcium may worsen adverse CV outcomes.  
  • The Question: Does calcium intake cause CV harm? 
  • The Data
    • The Auckland Calcium Study: raised concern that calcium supplementation may increase cardiovascular risk (secondary analysis of a study designed to assess impact on bone health). 
    • EPIC-Heidelberg, MESA, & other observational studies: calcium supplementation is associated with adverse cardiovascular events.  In contrast, calcium intake from food sources does not seem to be associated with adverse CV events.  
    • Meta-analysis by Khan et al. 2019: calcium + Vit D was associated with an increased risk for stroke. 
    • These findings may be from the bolus effect of calcium supplementation whereby a sudden rise in serum calcium levels may result in vascular calcium deposition and interact with the coagulation cascade.  
  • The Recommendations
    • Use calcium supplementation cautiously, according to the recommended daily intake, and using food sources. 
    • Personalized approach using shared decision making considering CV risk and bone health. 
    • Avoid excess calcium supplementation. 
    • Recommended daily intake: 
      • Adults aged 19-50 years old & Men aged 51-70 years: 1000 mg/day 
      • Adults aged >70 years and Women aged 51-70: 1200 mg/day  

What is the role of Omega-3 Polyunsaturated Fatty Acids in preventing cardiovascular disease?  

  • The Conundrum: High triglyceride levels are associated with adverse CV events, but triglyceride-reducing agents like niacin and fibrates, have not been effective in reducing the risk of these events. 
  • The Question: Does intake of Omega-3 Fatty Acids improve CV health and if so, what is the appropriate formulation? 
  • The Data
    • ASCEND Omega-3 Trial & VITAL Trial: lower doses of EPA/DHA combination omega-3 fatty acids ~840mg daily are not beneficial in reducing CV events. 
    • REDUCE-IT Trial: 4g of icosapent ethyl (IPE – a pure EPA formulation) daily reduced MACE events in those with elevated triglyceride levels despite statin use.  The Japanese JELIS trial also showed a CV benefit using a lower dose of pure EPA (1.8mg daily) among statin-treated adults with hyperlipidemia.   
    • STRENGTH Trial: 4g EPA/DHA Omega 3-fatty acid formulation failed to show CV benefit among statin-treated patients with dysplipidemia. The difference from REDUCE-IT trial may be due to the drug formulations (pure EPA vs EPA/DHA combination). Notably, in REDUCE-IT, the benefit had a dose-response relationship with blood levels of EPA; the higher the EPA, the greater the benefit.  
    • Dietary fish oil supplements may include a substantial portion of harmful saturated fats vs the beneficial polyunsaturated fats. Dietary supplements have been shows to contain far less Omega-3 fatty acids than indicated on the label! Furthermore, dietary supplements may get oxidized and contain harmful contaminants.  
  • The Recommendations
    • Use IPE 4g daily for patients ≥ 45 years old with established ASCVD or ≥ 50 years old with diabetes & other risk factor(s) who are on maximally tolerated statin and continue to have elevated Triglyceride level 135-499 mg/dL. 
    • The marked CV benefit from icosapent ethyl seen in the REDUCE-IT trial should not be extrapolated to other fish oil preparations!        

The Cardionerds CV prevention series  includes in-depth deep dives on so many prevention topics including the ABCs of prevention, approach to obesity, hypertension, diabetes mellitus and anti-diabetes agents, personalized risk and genetic risk assessments, hyperlipidemia, women’s cardiovascular prevention, coronary calcium scoring and so much more!

We are truly honored to be producing the Cardionerds CVD Prevention Series in collaboration with the American Society for Preventive Cardiology! The ASPC is an incredible resource for learning, networking, and promoting the ideals of cardiovascular prevention! This series is kicked off by a message from Dr. Amit Khera, President of the American Society for Preventive Cardiology and President of the SouthWest Affiliate of the American Heart Association.

Cardionerds Cardiovascular Prevention Series: by the Cardionerds Cardiology Podcast in Collaborate with the The American Society For Preventive Cardiology ASPC
Cardionerds Cardiovascular Prevention Series

Guest Profiles

Dr. Erin Donnelly Michos is an Associate Professor of Medicine at Johns Hopkins School of Medicine, with joint appointment in the Department of Epidemiology at the Johns Hopkins Bloomberg School of Public Health. She is the Director of Women’s Cardiovascular Health and the Associate Director of Preventive Cardiology with the Johns Hopkins Ciccarone Center for the Prevention of Cardiovascular Disease. Dr. Michos completed medical school at Northwestern University in Chicago, IL, and then completed both her Internal Medicine residency and Cardiology fellowship at the Johns Hopkins Hospital in Baltimore, MD.  She also completed her MHS in Cardiovascular Epidemiology at the Johns Hopkins Bloomberg School of Public Health. She has authored or co-authored over 300 manuscripts in peer reviewed journals and is an internationally known leader in preventive cardiology and women’s health. 

Dr. Rick Ferraro is midwest raised, spent two years as a Teach For America Corps member teaching science in Milwaukee before heading to medical school at Weill Cornell Medicine in NYC. Current senior resident at the Osler Medical Residency program and will begin cardiology fellowship at Johns Hopkins Hospital in 2021. Completed intern year under the incredible leadership of Dr. Amit Goyal. Interested in cardiovascular prevention and imaging

Dr. Andi Shahu is a resident physician in the Osler Medical Residency in Internal Medicine at Johns Hopkins Hospital in Baltimore, MD. He will begin General Cardiology fellowship in July 2021 at Yale University. He is interested in the intersection between cardiovascular outcomes, health equity and health policy. You can follow him on Twitter @andishahu

Sunyoung (Sarah) Jang is a third year medical student at the Johns Hopkins School of Medicine in Baltimore, MD. Interested in public health, she was drawn to Cardiology when she learned that cardiovascular diseases were the leading cause of death globally. She aspires to pursue a career in Cardiology with continued interest in public health, preventative medicine and high value care. 


References and Links

1. Al Mheid I, Quyyumi AA. Vitamin D and Cardiovascular Disease: Controversy Unresolved. J Am Coll Cardiol. 2017. doi:10.1016/j.jacc.2017.05.031 

2. Effects of Aspirin for Primary Prevention in Persons with Diabetes Mellitus. N Engl J Med. 2018. doi:10.1056/nejmoa1804988 

3. McNeil JJ, Nelson MR, Woods RL, et al. Effect of Aspirin on All-Cause Mortality in the Healthy Elderly. N Engl J Med. 2018. doi:10.1056/nejmoa1803955 

4. Manson JE, Cook NR, Lee I-M, et al. Vitamin D Supplements and Prevention of Cancer and Cardiovascular Disease. N Engl J Med. 2019. doi:10.1056/nejmoa1809944 

5. Scragg R, Stewart AW, Waayer D, et al. Effect of monthly high-dose vitamin D supplementation on cardiovascular disease in the vitamin D assessment study: A randomized clinical trial. JAMA Cardiol. 2017. doi:10.1001/jamacardio.2017.0175 

6. Bolland MJ, Barber PA, Doughty RN, et al. Vascular events in healthy older women receiving calcium supplementation: Randomised controlled trial. BMJ. 2008. doi:10.1136/bmj.39440.525752.BE 

7. Li K, Kaaks R, Linseisen J, Rohrmann S. Associations of dietary calcium intake and calcium supplementation with myocardial infarction and stroke risk and overall cardiovascular mortality in the Heidelberg cohort of the European Prospective Investigation into Cancer and Nutrition study (EPIC-Heidelberg). Heart. 2012. doi:10.1136/heartjnl-2011-301345 

8. Khan SU, Khan MU, Riaz H, et al. Effects of nutritional supplements and dietary interventions on cardiovascular outcomes. Ann Intern Med. 2019. doi:10.7326/M19-0341 

9. Yokoyama M, Origasa H, Matsuzaki M, et al. Effects of eicosapentaenoic acid on major coronary events in hypercholesterolaemic patients (JELIS): a randomised open-label, blinded endpoint analysis. Lancet. 2007. doi:10.1016/S0140-6736(07)60527-3 

10. Orringer CE, Jacobson TA, Maki KC. National Lipid Association Scientific Statement on the use of icosapent ethyl in statin-treated patients with elevated triglycerides and high or very-high ASCVD risk. J Clin Lipidol. 2019. doi:10.1016/j.jacl.2019.10.014 

11. Gaziano JM, Brotons C, Coppolecchia R, et al. Use of aspirin to reduce risk of initial vascular events in patients at moderate risk of cardiovascular disease (ARRIVE): a randomised, double-blind, placebo-controlled trial. Lancet. 2018. doi:10.1016/S0140-6736(18)31924-X 

12. Nicholls SJ, Lincoff AM, Garcia M, et al. Effect of High-Dose Omega-3 Fatty Acids vs Corn Oil on Major Adverse Cardiovascular Events in Patients at High Cardiovascular Risk. JAMA. 2020. 

13. Arnett DK, Blumenthal RS, Albert MA, et al. 2019 ACC/AHA Guideline on the Primary Prevention of Cardiovascular Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019;74(10):1376-1414. doi:10.1016/j.jacc.2019.03.009 

Referenced work by Dr. Michos Mentees in this episode

Jang S, Ogunmoroti O, Ndumele CE, et al. Association of the Novel Inflammatory Marker GlycA and Incident Heart Failure and Its Subtypes of Preserved and Reduced Ejection Fraction: The Multi-Ethnic Study of Atherosclerosis. Circ Hear Fail. 2020;(August):251-260. doi:10.1161/CIRCHEARTFAILURE.120.007067

AHA Press release: Low-income adults less likely to receive preventive heart disease care.

ASSOCIATION BETWEEN INDIVIDUAL INCOME AND INCIDENCE OF HEART FAILURE SUBTYPES IN THE MULTI-ETHNIC STUDY OF ATHEROSCLEROSIS (MESA)

Ferraro R, Latina JM, Alfaddagh A, et al. Evaluation and Management of Patients With Stable Angina: Beyond the Ischemia Paradigm: JACC State-of-the-Art Review. J Am Coll Cardiol. 2020;76(19):2252-2266. doi:10.1016/j.jacc.2020.08.078

65. Case Report: Spontaneous Coronary Artery Dissection (SCAD) Requiring Heart Transplantation – UCLA

CardioNerds (Amit Goyal & Daniel Ambinder) join  join UCLA cardiology fellows (Jay Patel, Hillary Shapiro, and Ruth Hsiao) for some beach bonfire in Santa Monica! They discuss a challenging case of Spontaneous Coronary Artery Dissection (SCAD) requiring heart transplantation. Dr. Jonathan Tobis provides the E-CPR and program director Dr. Karol Watson provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join join UCLA cardiology fellows (Jay Patel, Hillary Shapiro, and Ruth Hsiao) for some beach bonfire in Santa Monica! They discuss a challenging case of Spontaneous Coronary Artery Dissection (SCAD). Dr. Jonathan Tobis provides the E-CPR and program director Dr. Karol Watson provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A woman in her late 40s presented with a one day history of intermittent chest pain. EKG showed anteroseptal and lateral STE with reciprocal ST depressions in the inferior leads. High-sensitivity troponin was elevated at 333 ng/mL. Emergent LHC showed a long and narrow left main with areas of additional contrast filling into a false lumen with no flow in the LAD. RCA and LCx were normal appearing (make sure you check out the angiogram videos below!). IVUS showed dissection and heavy thrombus burden in the left main artery. Shortly after the diagnostic angiogram, the patient went into V-fib arrest and received one shock with ROSC. Amiodarone was started and an Impella CP was placed for additional left ventricular support. ECMO and emergent CABG were not readily available at this time so the interventional team attempted revascularization with PCI to the left main given patient’s hemodynamic instability from ongoing ischemia. However, even after PCI to left main, flow to LAD remained poor and the LCx now also appeared occluded. The decision was made to cease further attempts at revascularization. Unfortunately, post-procedure TTE showed a reduced EF of 22% with anterior and anterolateral hypokinesis. She was transferred to CCU on maximal Impella support. Patient eventually developed acute renal and liver failure secondary to cardiogenic shock and suffered an additional V-fib arrest with ROSC. Eventually, Ronald Reagan UCLA was contacted for transfer and the mobile ECMO team was dispatched. They placed the patient on VA-ECMO in the outside facility and transferred her to Ronald Reagan UCLA. At Ronald Reagan, revascularization was attempted given persistent cardiogenic shock and 3 stents were successfully deployed in the LAD. She was eventually weaned off of both Impella and ECMO after successful PCIs to LAD. However, TTE showed persistently low EF and patient eventually underwent successful heart-kidney transplantation. 


Case Media

A. ECG: Anterior STE, STE in I/aVL but depressedions in V4-V6, inferior reciprocal ST depression
B. X-ray of explanted heart shows stents extending from LM -> dLAD
C. Cross-section of explanted heart from apex to base showing infarct in the anteroseptal area
D. Histological cross section of the explanted LAD. 

This film shows slit like LM with no LAD.  High OM/RI and LCx look ok.
BMW wire used to cross distal LM into high OM/RI
Arrested after diagnostic, got ROSC and then Impella CP inserted L femoral
Attempting wiring the true lumen, stuck in mLAD after 1st septal comes off
IVUS from RI to LM.  Shows dissection plane and lots of thrombus
LM IVUS details: 4.57 x 5.0mm proximally, 4.52mm distally
Unable to pass a wire into mid LAD
PCI of LM: Synergy 4.0 x 16mm DES deployed in LM and post-dilated with Emerge NC 4.5 x 8mm balloon
Flow remained poor at mLAD even after PCI to LM
LCx system closed after LM PCI, so no further attempts
TTE: Apical 4 Chamber
TTE: Apical 2 Chamber
TTE: Apical 3 Chamber
LCx occluded with large OM1/Ramus still patent
Post PCI of the LAD

Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. What is SCAD and what population is most at risk? 
    • SCAD stands for spontaneous coronary artery dissection. It is an acute coronary event and is defined as a spontaneous separation of the coronary artery wall that is not iatrogenic or related to trauma. It is an important cause of acute myocardial infarction. 
    • Women comprise 87%-95% of SCAD patients with a mean age of presentation between 44-53 years, just like the patient in this case. The “typical” SCAD patient is a middle-aged female with few traditional cardiovascular risk factors such as hypertension, hyperlipidemia, and tobacco use. However, our understanding of a “typical” SCAD patient is limited as the majority of patients in large series have been white and it is likely that patients of different ethnic and racial backgrounds have been under-represented in most current registries.  
    • In some studies, the prevalence of SCAD appears around 4% of all patients presenting with ACS and up to 35% in women 50 years or under presenting with ACS.  
    • Pregnancy associated SCAD is an important subset of patients. It can occur at any time during the pregnancy or post-partum, with the majority of cases occurring postpartum. SCAD associated with pregnancy tends to have a more severe clinical presentation, including left main involvement, multi-vessel dissection and cardiogenic shock. Both pregnancy and non-pregnancy associated SCAD tend to occur more frequently in multiparous women and those that report a higher prevalence of pre-eclampsia.  
  2. What are the two hypotheses that have been proposed to explain the pathophysiology of SCAD? 
    1. Before reviewing the pathophysiology, let’s briefly review the coronary arterial wall structure. The intima is the inner layer in contact with the intraluminal space. In normal vasculature, the intima is only a few cell layers thick and is separated from the media by the internal elastic lamina. The media is the middle layer and is made up of layers of smooth muscle cells which help regulate vascular tone. The media is separated from the adventitia by the external elastic lamina. Finally, the adventitia surrounds the media and through fibrous connective tissue provides support for the epicardial vessel.  
    2. In SCAD, a hematoma forms within the tunica media separating the intima or intima/media from the vessel and compressing the true lumen leading to ischemia. There are several proposed hypotheses for how this occurs: 
      1. “Inside-out” hypothesis: an endothelial-intimal disruption or “flap” develops first and then blood enters the sub-intimal space from the true lumen via this “flap” 
      2. “Outside-in” hypothesis: a hematoma forms within the media, possibly from disruption of traversing micro-vessels, and compresses and occludes the true lumen as the hematoma expands.  
    • In both hypotheses, the end result is separation of the layers of coronary artery wall, creating a false vs. true lumen. Currently, the evidence favors the “outside-in” theory because in most SCAD cases, there are no communication between the true and false lumens observed.  
  3. What are the angiographic appearances of SCAD? 
    • Left anterior descending artery is the most commonly affected vessel in SCAD. 
    • There are three classifications of SCAD based on angiographic appearance (the Yip-Saw classification).  
      1. Type 1: Contrast dye staining of the arterial wall demonstrates double or multiple radiolucent lumens separated by a radiolucent flap. There may be dye “hang-up” or slow contrast clearing.  
      2. Type 2: This is the most common subtype. It is characterized by long, diffuse, and smooth narrowing that can vary from mild stenosis to complete occlusion, often with abrupt changes in lumen diameter. 
        • Type 2a SCAD demonstrates normal arterial segments proximal and distal to a dissection and does not extend into a distal vessel.  
        • Type 2b does extend into the distal tip of a vessel.  
      3. Type 3: The angiographic appearance mimics a focal stenosis of atherosclerotic disease and typically requires intracoronary imaging to make a definitive diagnosis.  
    • There are other angiographic findings that may clue the cardiology team into a diagnosis of SCAD. SCAD tends to affect more distal segments than atherosclerotic disease. Furthermore, the left anterior descending (LAD) artery is the most commonly affected vessel in SCAD. Patients with SCAD tend to have more tortuous vessels and atherosclerotic lesions are usually absent from vessels not affected by the SCAD. Some reports have indicated the external luminal compression by the intramural hematoma (IMH) gives the appearance of a stick insect.  
  4. What is the management of SCAD in the acute setting?  
    • As Dr. Hayes et al. note in their JACC review, the focus in the acute setting is to restore perfusion and maintain myocardial function as conservatively as possible rather than on how to restore normal coronary architecture as in atherosclerotic ACS. The use of thrombolytics have resulted in extension of dissection or hematoma and should be avoided. Diagnostic left heart catheterization is recommended but outcomes of PCI in SCAD are less predictable. There are often technical challenges due to the fragility of the vessel wall, instrumentation can propagate a dissection or occlude distal branches, and long-stents may be required as SCAD lesions tend to be extensive and in small distal vessels. Temporal resolution of the IMH may lead to late stent mal-apposition as the IMH reabsorbs.  
    • CABG is typically only considered if PCI has failed or in high risk lesions. Again, the fragility of the vessels makes a successful result challenging. Identifying the true lumen (for graft anastomosis) may be difficult. Sutures may not hold and patients may be prone to anastomotic complications. Over the long-term, healing of the native coronaries may lead to bypass graft failure due to competitive flow into the native system. However, CABG can still be an effective measure to stabilize unstable patients.  
    • Among patients treated conservatively, 95% of patients usually will heal within 30 days; therefore, if there is no ongoing ischemia or hemodynamic instability, instrumentation should be avoided. 
    • In terms of medications, patients with LV dysfunction should receive GDMT (with special attention to teratogenic medications for patients that are pregnant or breastfeeding) and patients undergoing PCI should receive DAPT. In patients that do not receive PCI, the evidence and expert opinion is varying on whether DAPT should be recommended for medical management of ACS.  
  5. What’s the long-term management and outcomes for patients with SCAD? 
    • SCAD can be associated with underlying systemic arteriopathy such as fibromuscular dysplasia (FMD) in >50% cases and head/neck aneurysms in 7-11% cases. Patients diagnosed with SCAD should undergo CTA or MRA from head to pelvis to identify additional extra-coronary vascular abnormalities. 
    1. Post-SCAD chest pain is common and may occur for many months following the index episode. There can be various triggers of the chest pain, including exercise, stress, or during the menstrual period. Given the increased risk of iatrogenic catheter-induced dissection in post-SCAD patients, a multi-modal evaluation is recommended before considering repeat LHC (e.g., ECG, biomarkers, echo, coronary CTA, stress imaging). 
    • Rates of recurrent SCAD have been reported as 10 to 30%. Secondary prevention for SCAD include avoidance of potential triggers such as stress or extreme exertion and blood pressure control. However, cardiac rehabilitation and moderate exercise’s benefits likely outweigh the theoretical risk of recurrent SCAD with exertion. Beta-blockers may decrease the risk of recurrence but evidence is limited.  
    1. There are important considerations for future pregnancy and SCAD. See the JACC review for more details! Another important aspect of post-SCAD care is recognizing the high burden of psychological distress amongst SCAD patients. Clinicians must recognized this early and provide early treatment and appropriate referrals to ensure recovery.  
  1. It sounds like the benefits of E-CPR remain to be further elucidated. Are there any specific features that help predict who would benefit from ECPR?  
    • While we do not have high quality randomized data, observational data in EPCR has shown that shorter no flow times (i.e., CPR initiated within 5 minutes of arrest), total duration of CPR <60 minutes, intermittent return of spontaneous circulation, an initial shockable rhythm, and lower serum lactate concentration have been associated with increased survival with better neurologic recovery. 
    • A well-known protocol is the University of Minnesota ECPR protocol (transport with ongoing CPR to the cardiac catheterization laboratory for ECPR) for patients with refractory VT/VF arrest. The inclusion and exclusion criteria for this protocol included 
      1. Inclusion: (1) OHCA with presumed cardiac etiology cardiac arrest; (2) first presenting rhythm is shockable (VF or VT); (3) Age 18 to 75 years; (4) Received at least 3 direct current (DC) shocks without sustained ROSC; (5) received Amiodarone 300 mg; (6) Body can accommodate a Lund University Cardiac Arrest System (LUCA) automated CPR device; and (7) Transfer time from the scene to the Cardiac Catheterization Lab of < 30 minutes 
      2. Exclusion: (1) ROSC before 3 shocks were delivered; (2) Nursing home residents: (3) DNR/DNI orders; (4) known terminal illness (e.g., malignancy); (5) Traumatic arrest; (6) PEA or asystole; (7) significant bleeding; (8) manual CPR as the only option 
    • Using this strict UMN-ECPR protocol, Bartos et al. retrospectively compared 160 consecutive adult patients with refractory VT/VF arrest treated with ECPR to 654 patients treated with conventional CPR from the amiodarone arm of the ALPS study (Amiodarone, Lidocaine or Placebo study). They found ECPR had favorable survival compared with conventional CPR at each CPR duration interval <60 minutes; however, longer CPR duration was associated with worsening neurologic outcomes and survival in both groups. There remains considerable evidence gaps to define which patient populations would most benefit from this intensive resource.  

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

61. Case Report: Cardiac Arrest due to Peripartum Cardiomyopathy – Medical College of Wisconsin

CardioNerds (Amit Goyal & Daniel Ambinder) join Medical College of Wisconsin cardiology fellows (Katie Cohen, Div Mohananey, and Dave Lewandowski) for some cold brews by Lake Michigan in Cream City aka Milwaukee, WI! They discuss a case of a pregnant woman presenting cardiac arrest due to peripartum cardiomyopathy. Dr. Sarah Thordsen provides the E-CPR and program director, Dr. Gaglianello Nunzio, provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join Medical College of Wisconsin cardiology fellows (Katie Cohen, Div Mohananey, and Dave Lewandowski) for some cold brews by Lake Michigan in Cream City aka Milwaukee, WI! They discuss a case of a pregnant woman presenting cardiac arrest due to peripartum cardiomyopathy. Dr. Sarah Thordsen provides the E-CPR and program director, Dr. Gaglianello Nunzio, provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A G2P1 woman in her early 30s with a history palpitations presented after a witnessed out-of-hospital cardiac arrest while at work. She received 6 rounds of CPR and 2 shocks before ROSC was achieved. She was intubated and given fluids but continued to remain hypoxic and hypotensive. Exam demonstrated sinus tachycardia, no murmurs, gravid abdomen and cool extremities. Initial labs demonstrated leukocytosis to 14k, lactic acid at 4.3 mmol/L, troponin-I peak at 0.07 ng/dL and elevated NT-proBNP. CXR demonstrated bilateral effusions and pulmonary congestion, and post-arrest EKG showed a wide complex tachycardia, leading to suspicion of VT arrest. In sinus, there  were no ST segment elevations and TTE showed LVEF 10-20%, global hypokinesis and no valvular disease. Given the severity of her shock, she was placed on central VA-ECMO with Impella support as an LV vent. During ECMO cannulation, she underwent emergent cesarean section due to fetal distress. Coronary angiography showed non-obstructive coronaries, but with sluggish flow in the setting of her cardiogenic shock and possible coronary spasm in setting of multiple vasoactive medications. Endomyocardial biopsy was negative for giant cell myocarditis. Within 4-5 days, she was weaned off all vasoactive agents and ECMO was decannulated; repeat echocardiogram showed LV functional recovery. GDMT was slowly titrated and a subcutaneous ICD was eventually placed before discharge. She and her child have done well over the course of a year! 


Case Media

A: ECG: Initially in sustained wide complex irregular tachycardia
B: CXR: Extensive consolidative changes throughout the lungs

TTE: Parasternal Long Axis
TTE: Apical 4 Chamber

Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

1. What is the differential for cardiac arrest in pregnant patients? 

  • When thinking about a cardiac etiology of arrest, the differential should include pregnancy-induced hypertension, peripartum cardiomyopathy, myocardial infarction from acute coronary syndrome or spontaneous coronary artery dissection, pulmonary embolism, amniotic fluid embolism and aortic dissection. Non-cardiac etiologies include hemorrhagic shock, sepsis, stroke, trauma and anesthetic complications. In addition to these unique considerations, pregnant patients are also susceptible to the usual culprits! 
  • As noted in the 2015 AHA Scientific Statement, cardiac arrest in pregnancy is not common, occurring in 1:12,000 admissions for delivery. As of 2016, per the CDC the pregnancy-related mortality rate was ~17 deaths per every 100,000 live births. However, mortality data does not fully capture critical illness in pregnancy, and thus the AHA recommends considering maternal “near-miss” data.  
  • Knowledge gaps, provider unfamiliarity, and lack of medical ward or medical/cardiac ICU preparation for cardiac arrest in pregnancy may contribute to morbidity and mortality.  
  • Finally, as many of the early warnings signs of impending cardiac arrest may overlap with symptoms of pregnancy (e.g., progressive dyspnea), early interventions may be delayed. Thus, the AHA recommends using a validated obstetric early warning score to risk stratify ill pregnant patients.  

2. Remind us of some important physiologic changes in pregnancy that can affect cardiopulmonary resuscitation  

  • Hormonal and physiologic changes during pregnancy make pregnant patients more prone to hypoxia, hypotension, pulmonary edema, and difficult airway intubation. 
  • Systemic vascular resistance typically decreases due to the production of endogenous vasodilators, though there are important differences in patients with pre-eclampsia. The enlarging uterus can reduce preload by compressing the IVC and increase afterload by compressing the aorta. In the supine position, which is preferable for resuscitation, this compression can be exacerbated. 
  • Furthermore, as the uterus enlarges and limits diaphragmatic movement, functional residual capacity can decrease by 10-25%. At the same time, there is increased oxygen consumption due to metabolic and fetal demands. With limited reserve and increased oxygen demands, hypoventilation or apnea can rapidly precipitate hypoxemia.  
  • Cardiac output increases by 30-50% via increased stroke volume, and lesser extent HR, leading to increased circulating volume, making patients prone to pulmonary edema.  
  • Finally, pregnancy hormones can lead to airway edema and more friable tissue making intubation more difficult with increased risk of bleeding.  
  • For more on pregnancy physiology, enjoy:  

3. What are some aspects unique to advanced cardiac life support (ACLS) in pregnant patients? 

  • Cardiac arrest is inherently different than other cardiac arrest that we typically encounter as there are two patients: mother and the fetus.  
  • Chest compressions, delivery of shocks and medications can continue per standard adult ACLS algorithm.  
  • Importantly, while chest compressions are ongoing and patient is in the supine position, there should be continuous manual left uterine displacement (LUD) to relieve aortocaval compression. Furthermore, IVs should be established above the diaphragm so that intravenous infusions and medications are not impeded by caval compression of the uterus. 
  • Teams should be prepared for perimortem caesarean delivery (PMCD) and this should occur at the site of arrest. PMCD may facilitate return of spontaneous circulation (ROSC) after the gravid uterus is emptied. 
  • PMCD should occur within four minutes due to a rapid decline in fetal survival with longer delays to delivery.  

4. What are the considerations for post-arrest care for pregnant patients? 

  • Targeted temperature management (TTM) is not contraindicated in pregnancy, and no necessary intervention should be withheld for fear of fetal damage. The primary focus should be maternal outcomes since that best serves fetal outcomes. 
  • If TTM is pursued, there should be continuous fetal monitoring. The patient should continue to be in the left lateral decubitus position if it does not compromise other management. Routine cardiac catheterization is certainly not recommended unless post-arrest EKG demonstrates clear signs of ischemia. 
  • Remember that embryogenesis is mostly complete by 12 weeks of gestation. Thus, the AHA recommends providing all necessary medications, even teratogenic medications (e.g., corticosteroids, phenytoin) especially if the cardiac arrest occurs after the first trimester.  

5. What is the data for extra-corporeal life support (ECLS) during pregnancy and postpartum 

  • There are no consensus guidelines, however, ECLS is not contraindicated in pregnancy and should be considered for life-threatening conditions. There is lack of long-term data for maternal and fetal outcomes.  
  • Although survival varied depending on indication, one systematic review of ECLS in peripartum patients showed overall 30-day survival of 75% for mother and 64% for fetus. Interestingly, survival in the immediate post-partum group was the highest. 
  • Complications includes bleeding, deep vein thrombosis, and vascular complications similar to the non-pregnant population.  

References

Arany Zolt, and Elkayam Uri. “Peripartum Cardiomyopathy.” Circulation 133, no. 14 (April 5, 2016): 1397–1409.

Campbell, Tabitha A, and Tracy G Sanson. “Cardiac Arrest and Pregnancy.” Journal of Emergencies, Trauma and Shock2, no. 1 (2009): 34–42. 

Jeejeebhoy Farida M., Zelop Carolyn M., Lipman Steve, Carvalho Brendan, Joglar Jose, Mhyre Jill M., Katz Vern L., et al. “Cardiac Arrest in Pregnancy.” Circulation 132, no. 18 (November 3, 2015): 1747–73. 

Gilotra, Nisha A, and Gerin R Stevens. “Temporary Mechanical Circulatory Support: A Review of the Options, Indications, and Outcomes.” Clinical Medicine Insights. Cardiology 8, no. Suppl 1 (February 3, 2015): 75–85. 

Naoum Emily E., Chalupka Andrew, Haft Jonathan, MacEachern Mark, Vandeven Cosmas J. M., Easter Sarah Rae, Maile Michael, Bateman Brian T., and Bauer Melissa E. “Extracorporeal Life Support in Pregnancy: A Systematic Review.” Journal of the American Heart Association 9, no. 13 (July 7, 2020): e016072. 

Sharma, Nirmal S., Keith M. Wille, Scott C. Bellot, and Enrique Diaz-Guzman. “Modern Use of Extracorporeal Life Support in Pregnancy and Postpartum.” ASAIO Journal 61, no. 1 (February 2015): 110–114. 


CardioNerds Case Reports: Recruitment Edition Series Production Team

58. Case Report: Constrictive Pericarditis – University of Tennessee

CardioNerds (Amit Goyal & Daniel Ambinder) join join University of Tennessee cardiology fellows (Rachel Goodwin, Emmanuel Isang, and William Black) for some chocolate cake and hikes in the Smoky Mountains! They discuss a fascinating case of constrictive pericarditis. Dr. Tjuan Overly provides the E-CPR and a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase figures & mediaCase teachingReferencesProduction team

CardioNerds (Amit Goyal & Daniel Ambinder) join join University of Tennessee cardiology fellows (Rachel Goodwin, Emmanuel Isang, and William Black) for some chocolate cake and hikes in the Smoky Mountains! They discuss a fascinating case of constrictive pericarditis. Dr. Tjuan Overly provides the E-CPR and a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A man in his late 40s with a history of renal failure secondary to IgA nephropathy and now status post a kidney transplant 10-15 years ago was referred by hepatology for evaluation of recurrent ascites and LE edema. He appeared grossly volume overloaded on exam with JVP elevated past the mandible, RV heave, and 2+ pitting edema. TTE demonstrated LVEF of 55-60%, RVSP 40mmHg, abnormal septal motion with respiration, and respirophasic variation in mitral inflow across the mitral valve raising the suspicion for constrictive pericarditis. RHC pressures demonstrated a mean RA pressure of 20mmHg, RV 40/25mmHg, PA 38/30mmHg (mean 32 mmHg) and PCWP mean of 26 with V-waves at 28 mmHg. Simultaneous LV and RV pressure tracings showed ventricular discordance with respirophasic variation, consistent with constrictive physiology. Patient underwent pericardiectomy with markedly improved heart failure symptoms. Repeat TTE showed no evidence of constriction. 


Case Media

A. ECG
B. Pulsed-wave Doppler spectrum of tricuspid inflow velocities demonstrates a marked respiratory variation (In irregular rhythms, such as the atrial fibrillation seen here, respirophasic changes may still be seen but are confounded by the varying R-R interval)
C. Simultaneous LV and RV pressure tracings showing discordance with respirophasic variation

Apical 4-chamber view demonstrating abnormal septal motion due to interventricular dependence – dissociation of thoracic and cardiac chamber pressures leads to increased RV filling during inspiration
Short axis view of the LV demonstrating a D-shaped interventricular septum during inspiration.  Note the presence of a pericardial effusion as well.

Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. The initial presentation clinically seemed to be right greater than left heart failure. What are the signs and common causes of right heart failure?   
  • The signs and symptoms of RHF are often similar to left-sided CHF, but may describe more severe dyspnea on exertion, significant abdominal distension, and early satiety due to ascites or gut edema. Symptoms of pulmonary edema from elevated left-sided filling pressures (orthopnea, paroxysmal nocturnal dyspnea) may be absent.
  • On examination, there will be elevated JVP with likely prominent v-waves, possibly Kussmaul’s sign (inspiratory rise in JVP rather than fall) depending on the pathology, abdominal ascites, pulsatile hepatomegaly, and lower extremity edema. An RV heave may be discernible along with a loud P2 component and murmur of TR.
  • Broadly, RV failure may be caused by pressure overload (ex: pulmonary hypertension, pulmonic stenosis), volume overload (ex: intracardiac shunt, tricuspid regurgitation), or myocardial disease (ex: cardiomyopathies, ischemia/infarct). The most common cause of chronic right heart failure is LV failure (causing post-capillary pulmonary hypertension). Other causes of RV failure include pre-capillary pulmonary hypertension, congenital heart disease (e.g., ASD, residual RVOT obstruction in Tetralogy of Fallot patients), ARVC, RV ischemia, myocarditis, right sided valvular disease, constrictive pericarditis, and restrictive cardiomyopathy.
  1. The patient in this case was diagnosed with constrictive pericarditis (CP). What are the causes of CP?  
  • Remember that the etiology of CP can vary considerably depending on the patient’s demographics. In developed countries, the majority of cases are idiopathic or viral, post-operative, or post-radiation therapy. In developing countries, infectious etiologies are more common, with tuberculosis the most common cause. 
  • Amongst the causes, remember that acute bacterial and tuberculosis pericarditis have the highest chances of progressing into constriction. With post-radiation constrictive pericarditis, remember there can be significant delay (even up to 20 years) between radiation therapy and development of constriction and often accompanies concomitant myocardial fibrosis with restrictive physiology as well. 
  • Other etiologies include immunologic disorders (e.g., rheumatoid arthritis, lupus, sarcoidosis), malignancy (e.g., breast and lung cancers, lymphoma, mesothelioma), and myocardial infarction.  

3.  What are the TTE findings suggestive of constrictive pericarditis?  

  • To understand the basic TTE findings, we need a basic understanding of the pathophysiology. Constriction leads to a noncompliant pericardium that encases the heart. Heart failure occurs because there is impaired diastolic ventricular filling.   
  • The ventricles fill almost entirely in early diastole, because once they can no longer expand because of the non-distensible pericardium, diastolic filling abruptly stops. This pathophysiology reflects one of the key findings in CP: equalization of the end-diastolic pressures. 
  • At the same time, the thickened/fibrotic/calcified pericardium prevents the normal transmission of intrathoracic pressures to the cardiac chambers. However, structures “outside” the pericardium – such as the pulmonary vasculature – still “see” the normal changes in intrathoracic pressure. Normally, when we take a breath in: the intrathoracic pressure falls and this is transmitted equally to the pulmonary capillaries (e.g., the wedge pressure) and the cardiac chambers. The gradient for mitral valve inflow reflects the difference in wedge pressure and intra-cardiac chamber (LV) pressure. 
  • In CP, the drop in intrathoracic pressure with inspiration is transmitted to the pulmonary capillaries but not the cardiac chambers. Thus, there is now a decreased gradient for mitral valve inflow during inspiration. This is called intrathoracic-intracardiac pressure disassociation. 
  • At the same time, with inspiration right heart preload increases and to accommodate this volume the RV expands. However, RV expansion is limited by the encasing noncompliant pericardium, and thus to accommodate the volume the interventricular septum shifts to the left. This, further decreases the gradient for mitral valve inflow and the physiology is termed enhanced ventricular interdependence. The opposite occurs on expiration.   
  • Thus, on echocardiogram we may see abnormal respirophasic septal shift, reflecting enhanced ventricular interdependence. The septum moves to the left in early diastole with inspiration and then back to the right on expiration. This is one of the most sensitive echocardiographic findings for CP. 
  • Reflecting compromised diastolic filling, markedly elevated filling pressures, and equalization of end-diastolic pressures, there will be a high E-wave velocity with a decreased A-wave velocity (E/A > 1) across the mitral valve. Due to pericardial restraint, this early rapid diastolic filling (high velocity with a tall E wave) stops abruptly and so the E wave has a short deceleration time. The latter is analogous to the pericardial knock heard on physical exam and the ventricular early diastolic “square root” sign (dip and plateau) on the RHC.  
  • Reflecting intrathoracic-intracardiac pressure disassociation and enhanced ventricular interdependence, there will be increased respirophasic variation in mitral and tricuspid valve inflow. This typically is best demonstrated with the first few beats of inspiration and expiration. Specific cut-offs include a decrease in peak mitral E-wave velocity > 25% and increase in peak tricuspid E-wave velocity > 40% during inspiration (opposite during expiration). In other words, as you inspire there is increased filling of the RV with decreased filling of the LV.  
  • Expiratory hepatic vein diastolic flow reversal is one of the most specific findings of CP. Reflecting intrathoracic-intracardiac pressure disassociation and enhanced ventricular interdependence, during expiration RV filling is compromised (as the LV is filling with septal shift to the right) and thus there is “back-flow” from the right heart and we see more prominent flow reversal in the hepatic veins during expiration. In contrast, hepatic vein diastolic flow reversal occurs predominantly during inspiration with restrictive physiology.  
  • Normally, the lateral e’ (tissue doppler) velocity > medial e’ velocity. In CP, we see annulus reversus, where the peak e’ at the medial annulus > lateral annulus because in constriction the lateral free wall may be tethered to the fibrotic/calcified pericardium and restricted in movement. In addition, when considering constriction vs restriction, normal or elevated annular e’ velocities are more consistent with constriction (normal myocardial relaxation) whereas reduced annular e’ velocities are more indicative of restriction (impaired myocardial relaxation).  

4. What are the characteristics of CP on invasive hemodynamics?  

  • All the findings on invasive hemodynamics are reflecting reliance on early diastolic filling and equalization of diastolic pressures. 
  • The end-diastolic pressures in the ventricles are usually within 5 mmHg of each other. 
  • We may see the square root sign on ventricular pressure tracings. The upward deflection in early diastole reflects rapid early diastolic filling and the subsequent plateau represents the abrupt halt in filling once the non-compliant pericardium can no longer expand. This is analogous to a pericardial knock on physical exam and a tall E wave with a short deceleration time on echocardiogram.  
  • The right atrial pressure waveform may have a “W-shape” reflecting rapid x and y descents. Conversely restrictive physiology may have rapid y descents but typically not with the x descent.  
  • Finally, simultaneous RV and LV pressures tracings will show discordance in pressures with respiration – with inspiration RV pressure increases and LV pressure decreases. This is analogous to respirophasic septal shift and changes in mitral and tricuspid inflow velocities seen on echocardiogram. This should not be present in patients with restriction.  
  • Note, if patients are hypovolemic, typical features of CP may not be seen, and thus a fluid bolus may be required to unmask the findings of CP.  

5. What’s the general approach to management of CP?  

  • If active inflammation is present on labs or imaging, trial a course of anti-inflammatory medications such as colchicine and NSAIDs is recommended before surgery. Similarly, patients with effusive-constrictive pericarditis a pericardiocentesis and a trial of medical therapy initially is recommended. Refractory cases may warrant anti-inflammatory escalation including steroids, steroid-sparing agents, and biologics including anti-IL1 agents. Prolonged courses may be required with therapy tailored to symptoms, inflammatory markers (ESR, CRP), and cardiac MRI. Enjoy Ep #33 – CMR with Dr. Kwon! 
  • If patient has persistent NYHA III or IV symptoms refractory to medical therapy without severe co-morbid illnesses, pericardiectomy may be indicated. Patients with idiopathic or viral pericarditis tend to have better outcomes with pericardiectomy than those with radiation therapy. 

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

56. Case Report: Arrhythmogenic Desmoplakin Cardiomyopathy – Northwestern University Feinberg School of Medicine

CardioNerds (Amit Goyal & Daniel Ambinder) join Northwestern University cardiology fellows (Sarah Hale, Sarah Chuzi, and Graham Lohrmann) for burgers and a great case by the Chicago River! They discuss a fascinating case of arrhythmogenic desmoplakin cardiomyopathy. Dr. Lisa Wilsbacher provides the E-CPR and program director Dr. Benjamin Freed provides a message for applicants.  Episode notes were developed by Johns Hopkins internal medicine resident Richard Ferraro with mentorship from University of Maryland cardiology fellow Karan Desai

Jump to: Patient summaryCase figures & mediaCase teachingReferencesProduction team

CardioNerds (Amit Goyal & Daniel Ambinder) join Northwestern University cardiology fellows (Sarah Hale, Sarah Chuzi, and Graham Lohrmann) for burgers and a great case by the Chicago River! They discuss a fascinating case of desmoplakin dilated cardiomyopathy Desmoplakin (DSP) dilated cardiomyopathy with LMNA (lamin A/C) variant of uncertain significance. Dr. Lisa Wilsbacher provides the E-CPR and program director Dr. Benjamin Freed provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Richard Ferraro with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A male in his early 40s presented for second opinion regarding multiple ICD shocks. 10 years prior he was diagnosed with a “weak heart,” thought secondary to a viral illness and a dual-chamber ICD was placed at that time. He noted shocks occurring for the first time 5 years prior, at which time amiodarone was started. They recurred two years prior, when he was diagnosed with paroxysmal atrial fibrillation. Finally, he was hospitalized one month before presentation with multiple ICD shocks and was found to have high defibrillation thresholds (DFTs) and amiodarone was stopped. He  then presented for a second opinion from the Northwestern CardioNerds! 

The patient had been doing well on GDMT and had NYHA Class I symptoms (Enjoy Ep #13 – Approach to GDMT). He did note a family history of a cousin with “cardiac issues” and did not know his father’s family history. Physical exam demonstrated bradycardia and ECG demonstrated an a-paced, v-sensed rhythm at 50 bpm. TTE demonstrated a moderately dilated LV with LVEF 30%, globally reduced LV function and multiple wall motion abnormalities without a vascular distribution.  PET-CT was performed which showed diffuse uptake and high-intensity signal at the inferolateral and basal anterior walls. Cardiac MRI showed diffuse circumferential epicardial delayed enhancement with associated diffuse, enhancing thickening of the pericardium favoring inflammatory versus fibrotic process. Patient was initially diagnosed with cardiac sarcoid and started on prednisone and weekly methotrexate.  

On return of genetic testing, patient found to have a pathogenic variant for desmoplakin gene, and it was felt his cardiomyopathy was secondary to desmoplakin Left Dominant Arrhythmogenic Cardiomyopathy (LDAC, or left-dominant ARVC) presenting with inflammatory myocardial injury. On follow up the patient remained listed for transplant, and DFTs improved off amiodarone.  


Case Media


Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. We started the case talking about DFTs. What are DFTs?! 
    • Defibrillation Thresholds (DFTs) are the minimal amount of energy required to return a patient to sinus rhythm that is in a cardiac dysrhythmia.  
    • Most modern ICD leads have thresholds less than 15 joules and typically less than 10 joules with biphasic shocks. High DFT thresholds tend to be defined as >25J or a safety margin of <10J. 
    • DFT testing is not routinely recommended during implantation of left-sided transvenous devices; however, in patients undergoing right-sided transvenous ICD or ICD pulse generator changes, DFT testing is a reasonable approach. Patients undergoing subcutaneous ICD placement should generally have DFT testing. 
    • Contraindications to DFT testing include acute LV thrombus, atrial fibrillation/flutter without adequate anticoagulation, severe aortic stenosis, recent stroke or TIA, or hemodynamic compromise as DFT itself can cause hypotension and/or CVA.   
  2. What are the major causes of high DFTs? 
    • First there are myocardial factors. This includes pathology that affects the current density through the myocardium. Conditions like hypertrophic cardiomyopathy, inflammatory cardiomyopathy, or significant LV dilation can lead to high DFTs.    
    • The second set of factors are extra-cardiac causes that lead to increased impedance or resistance in the counter coil, such as high BMI or medications that lead to electrical imbalances such as amiodarone, which is a common cause of increased DFTs. However, the increase in DFT caused by amiodarone can be small and routine DFT testing in patients is not recommended.   
    • The last cause is a device factor, such as a lead fracture or a mal-positioned lead.  
  3. In the case, we used MRI and PET. What are their role in Cardiomyopathy? 
    • Cardiac MRI (CMR) has transformed our ability to assess cardiomyopathies, specifically by accurately defining chamber size & function, characterizing myocardial tissue, and determining ischemia & viability. The specific pattern of late gadolinium enhancement (LGE) can help us differentiate between ischemic and non-ischemic etiologies and specific cardiomyopathies have characteristic patterns on MRI. Further, MRI can reliably identify edema, inflammation, and fatty replacement. CMR can provide a wealth of information in a variety of disease processes. Enjoy Ep #33 – CMR!
    • When evaluating an unexplained cardiomyopathy, FDG-PET can be useful in identifying active myocardial inflammation. 18F-FDG is a glucose analogue that can differentiate activated macrophages in areas of inflammation from normal myocytes if there is appropriate suppression of normal physiologic myocardial glucose uptake (I.e., Ketogenic Diet). This can be especially useful in Cardiac Sarcoidosis. Note, if there is global myocardial uptake, without diffuse perfusion defects, it may be a false positive scan in the setting of inadequate prep!  
    • For more on evaluation of heart failure, enjoy Ep #12 – Eval of New Onset HF & CPS Ep #48 – HFrEF
  4. When should we consider a genetic cause to cardiomyopathy? 
    • If a family history suggests a genetic predisposition to cardiomyopathy, a cardiomyopathy seems out of proportion to an identified ischemic or non-ischemic cause, a patients presents with a cardiomyopathy at a young age, or if multi-modal imaging has not revealed a clear cause of a cardiomyopathy, genetic testing would be appropriate. 
    • Various studies have indicated that 30 to 50% of unexplained cases of (DCM) may have a genetic component. A detailed, at least three-generation family history should be obtained when initially evaluating a dilated CM, as most genetic cardiomyopathies are autosomal dominant with variable penetrance.  
    • Genetic counseling is key prior to genetic testing given complexities including interpretation of potentially confounding results and contextualizing results for relatives. 
  5. Our patient’s final diagnosis was “Arrhythmogenic Desmoplakin Cardiomyopathy”…what’s that?! 
    • Arrhythmogenic RV Cardiomyopathy is a familial cardiomyopathy which usually affects the RV via fibrous or fibro-fatty replacement of normal myocardium. This predisposes patients to sudden cardiac death (SCD), ventricular arrhythmias, and heart failure.  
    • ARVC classically displays autosomal dominant inheritance from mutations in genes encoding desmosomal proteins affecting the cell-to-cell junction: desmoplakin (DSP), plakophilin 2 (PKP2), desmoglein 2 (DSG2), and desmocollin 2 (DSC2). Rarely, genes unrelated to cell-to-cell junction may be involved.  
    • Clinically we have noted a “Left-Dominant Arrhythmogenic Cardiomyopathy” (LDAC), with similarities to classic ARVC, but affecting predominantly the LV.  
    • Genotype-phenotype studies are shedding light on these “Arrhythmogenic Cardiomyopathies”. DSP mutations affect predominantly the LV (causing LDAC) whereas PKP2 mutations affect predominantly the RV (causing ARVC); these are distinct entities with key differences in presentation, progression, and markers of SCD risk (see Smith et al., Circulation 2020 reference for more!). Pertinent to our case, a subset of patients with DSP cardiomyopathy will have evidence of myocardial inflammation on FDG-PET and will are initially misdiagnosed as a myocarditis or sarcoidosis. 

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

53. Case Report: CTEPH & May Thurner Syndrome – Temple University

CardioNerds (Amit Goyal & Daniel Ambinder) join Temple Cardiology Fellows (Anika Vaidy and Anne- Sophie LaCharite-Roberge) in Philadelphia, PA! They discuss a fascinating case of pulmonary hypertension secondary to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) associated with May Thurner syndrome and large uterine fibroids. Dr. Vaidya provides the E-CPR and message to applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summaryCase figures & mediaCase teachingEducational videoReferencesProduction team

CardioNerds (Amit Goyal & Daniel Ambinder) join Temple Cardiology Fellows (Anika Vaidy and Anne- Sophie LaCharite-Roberge) in Philadelphia, PA! They discuss a fascinating case of pulmonary hypertension secondary to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) associated with May Thurner syndrome and large uterine fibroids. Dr. Vaidya provides the E-CPR and message to applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A woman in her late 40s with history of iron deficiency anemia, uterine fibroids, and recurrent DVTs/PEs for the past 6 years despite different anticoagulation regimens, presented with syncope and progressive dyspnea on exertion. Family history was negative for DVT/PE or hypercoagulable states. On exam, she was euvolemic. Labs were significant for anemia, a normal pro-BNP, and a negative extensive hypercoagulable workup. TTE showed interventricular systolic septal flattening, right ventricular outflow tract pulse wave doppler with mid-systolic notch, and shortened acceleration time consistent with elevated pulmonary artery pressure. A VQ scan showed bilateral right greater than left mis-matched perfusion defects. CT angiogram showed right greater than left chronic mural thrombus, correlating with pulmonary angiogram which showed severe proximal and mid-vessel disease on the right and distal disease on the left. RHC corroborated the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) with mean pulmonary artery pressure of 41 and PVR of 5.2 Woods Units (WU).  

To determine the etiology of her recurrent clots, a lower extremity venogram was performed and showed 80% stenosis of her left common iliac vein by the overlying right common iliac artery confirming May-Thurner syndrome. Lower extremity venogram also showed severe proximal stenosis of right iliac vein thought to be due to large uterine fibroids. Given her severe proximal and mid-vessel clot burden, she underwent pulmonary thromboendarterectomy with a subsequent drop in pulmonary vascular resistance to 1 WU.  The etiology of DVTs and CTEPH was determined to be external compression related to both May-Thurner syndrome and uterine fibroids. To prevent future thromboembolic events, she underwent stenting of her left common iliac vein and hysterectomy. With these interventions, RV function returned to normal, and her symptoms completely resolved! 


Case Media

Figue Legend:
A. CXR
B. ECG
C. TTE: Interventricular systolic septal flattening, RVOT pulse wave Doppler with mid-systolic notch and shortened acceleration time, consistent with elevated PVR, RV:LV ratio 1.3, consistent with moderate RV enlargement, The RV is apex-sharing with an open apical angle
D. VQ Scan: Multiple b/l perfusion defects, R > L, V scan normal
E. CT Angiogram: 1) Enlarged R main PA2) large proximal chronic mural thrombus with minimal vessel count throughout R side, 3) Segmental LLL lining thrombus
F. Pulmonary angiogram significant for severe proximal and mid-vessel disease in the right segmental arteries. Patient also had severe distal disease in the left sub segmental disease (not shown).
G. Pulmonary Thromboendarterectomy (PTE)
H. LE Venogram: LEFT CIV  > 80% stenosis as a result of compression from an overlying right common iliac artery. This is consistent with May-Thurner syndrome. RIGHT EIV (not shown)– Significant proximal stenosis
I. Status Post left iliac vein stent
J. ECG: New typical atrial flutter


Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. In patients with progressive dyspnea, exercise intolerance, and persistence of symptoms of PE despite adequate anticoagulation, there should be an evaluation for Chronic Thromboembolic Pulmonary Hypertension (CTEPH).  CTEPH is a form of pre-capillary pulmonary hypertension (PH) from incomplete resolution of pulmonary thromboemboli causing chronic, fibrotic, flow limiting changes to the pulmonary vascular bed. Risk factors include recurrent PE, hypercoagulable states, and medical conditions such as splenectomy, ventriculo-atrial shunts, infected intravenous catheters/devices, malignancy, and external venous compression. NOTE: many patients will not have a documented history of DVT/PE and so a high index of suspicion is important. 
  2. The following echocardiographic findings suggest pulmonary hypertension: interventricular systolic septal flattening indicating RV pressure overload, RV outflow tract (RVOT) pulse wave doppler with mid-systolic notch, decreased RVOT acceleration time, right atrial enlargement, right ventricular dilation and hypertrophy +/- functional tricuspid regurgitation, and an elevated estimated RV systolic pressure (RVSP). 
  3. CTEPH is rare and carries a high rate of morbidity and mortality; therefore, a high index of suspicion is necessary. There are two components to diagnosis:  
    • Diagnose CTEPH: Multiple imaging modalities are involved to confirm and assess severity. V/Q scan is highly sensitive and is the initial screening modality to detect perfusion abnormalities. Chest CT with IV contrast may identify parenchymal and mediastinal pathology not otherwise seen; although not sensitive, CT may show: PA dilation, eccentric filling defects with variable degrees of occlusion, vascular webs or bands, mosaic perfusion pattern of the lung parenchyma, and RV enlargement. RHC can confirm the diagnosis of pulmonary hypertension, differentiate pre-capillary from post-capillary PH, quantify vascular resistance and cardiac output, and can be a comparative marker post-intervention. Conventional PA angiography is useful for pre-operative planning. 
    • Diagnose the underlying predisposition/etiology: Think about Virchow’s Triad: Hypercoagulability, Stasis, and Endothelial injury. Apart from ruling out hypercoagulable states, evaluating for lower extremity vascular compression can be important in select patients with imaging such as venography. May-Thurner syndrome is a condition of extrinsic venous compression of the left common iliac vein by the common iliac artery. Evidence of any compression should be addressed to prevent future venous thrombi and subsequent emboli.  
  4. Treatment for CTEPH starts with lifelong anticoagulation. Pulmonary angiography aids in surgical planning as proximal disease is more likely to be operable than distal disease. Based on severity and other comorbidities, pulmonary endarterectomy (PEA) can be a favorable option. PEA improves symptoms, survival, hemodynamics parameters, and RV remodeling. Other treatment strategies include medical management, balloon pulmonary angioplasty, and lung transplant. Pulmonary artery denervation is being considered as an experimental modality.  
  5. Supraventricular tachycardia (SVT) is common in patients with pulmonary artery hypertension and CTEPH and often indicate progression of right-sided dysfunction. PH patients rely more on active than passive RV filling, and are highly sensitive to changes in RV afterload. Supraventricular tachycardias may further precipitate decompensation given (1) the loss of atrial kick which compromises diastolic filling, and (2) tachycardias which increase RV wall tension increasing RV afterload. Therefore, restoration of sinus rhythm is strongly encouraged. 

Educational Video

Produced by Dr. Karan Desai


References


CardioNerds Case Reports: Recruitment Edition Series Production Team