78. Case Report: Severe Functional Mitral Regurgitation treated with MitraClip – University of Mississippi Medical Center

CardioNerds (Amit Goyal) join University University of Mississippi Medical Center cardiology fellows (Catherine Lowe, Chris Latour and Adi Sabharwal) as they sit at the reservoir enjoying a great view of the water at the Pelican Cove Grill in Jackson, MS. They discuss and educational case of decompensated heart failure and shock in the setting of severe functional mitral regurgitation treated with MitraClip. Dr. Kellan Ashley provides the E-CPR and program director Dr. Trey Clark 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 mediaCase teachingReferences

CardioNerds (Amit Goyal) join University University of Mississippi Medical Center cardiology fellows (Catherine Lowe, Chris Latour and Adi Sabharwal) as they sit at the reservoir enjoying a great view of the water at the Pelican Cove Grill in Jackson, MS. They discuss and educational case of decompensated heart failure and shock in the setting of severe functional mitral regurgitation treated with MitraClip. Dr. Kellan Ashley provides the E-CPR and program director Dr. Trey Clark 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

76 year-old male with a history of non-ischemic heart failure and CRT-D placement, A-Fib, and chronic kidney disease presents with persistent shortness of breath. He had experienced a number of recent hospitalizations for heart failure exacerbations (7 in the last 9 months at a number of hospitals). Symptoms were primarily dyspnea on limited exertion, orthopnea, paroxysmal nocturnal dyspnea, and lower extremity edema. Patient was on guideline-directed medical therapy including salcubitril-valsartan, metoprolol, spironolactone, and apixaban for A-Fib. Physical exam was positive for S3, IV/VI holosystolic murmur at the apex with radiation to the axilla, bibasilar rales, and bilateral 3+ lower extremity edema. ECG with a-sensed/v-paced rhythm at 72 beats per minute and evidence of left atrial dilation.  Echo exhibited LV ejection fraction of 30-35% with global hypokinesis, RVSP 56mmHg, moderately thickened mitral leaflets with posterior leaflet restriction and 4+ eccentric posteriorally-directed mitral regurgitation, estimated regurgitant orifice 0.43cm2, regurgitant volume 57mL, mean gradient across the valve 3mmHg. Subsequently admitted to the hospital with decompensated heart failure, labs notable for Cr 2.43, proBNP 2417, lactate 2.6.  The patient acutely received diuresis, and given repeat hospitalizations with severe functional mitral regurgitation was considered fro MitraClip placement. This was performed one month following hospitalization without complication, and at two months following MitraClip placement the patient noted improvement in dyspnea, orthopnea, and PND, had not required repeat hospitalization.  


Case Media

A. Right atrial pressure
B. Right ventricular pressure
C. Pulmonary artery pressure
D. Wedge pressure
C. Pre and post MitraClip left atrial pressure


Episode Schematics & Teaching

Coming soon!


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

Coming soon!


References

Coming soon!


CardioNerds Case Reports: Recruitment Edition Series Production Team

73. Case Report: Wet Beriberi & Stiff Left Atrial Syndrome – Scripps Clinic

CardioNerds (Amit Goyal & Daniel Ambinder) join Scripps cardiology fellows (Christine Shen and Andrew Cheng) for some Cardiology and California Burritos in San Diego! They discuss an informative case of Wet Beriberi and Stiff Left Atrial Syndrome. Dr. Thomas Heywood provides the E-CPR and program director Dr. Malhar Patel provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join Scripps cardiology fellows (Christine Shen and Andrew Cheng) for some Cardiology and California Burritos in San Diego! They discuss an informative case of Stiff Left Atrial Syndrome and Wet Beri Beri. Dr. Thomas Heywood provides the E-CPR and program director Dr. Malhar Patel provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das 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 mid-60s with history of rheumatic mitral stenosis s/p mechanical mitral valve replacement, HFpEF, and paroxysmal atrial fibrillation s/p ablation presents with subacute worsening dyspnea despite escalating diuretic doses. TTE shows an EF of 62%, normal gradients across the mitral valve without mitral regurgitation, and a dilated IVC. She is admitted with a presumed diagnosis of decompensated heart failure, and started given IV furosemide. Her symptoms slightly improve though do not resolve, and her creatinine increases from 1.4 to 2.1.  

In light of the unclear hemodynamic picture, a RHC is done, showing a RA pressure 9, RV pressure of 80/10, PAP 70/25 with mPAP 40, PCWP 30, SVR 872, CO 11 (by thermodilution), and CI 5.2. Notably, large V waves are noted on the RHC. Given concern for mitral regurgitation in the setting of large V waves, a TEE was pursued, which confirmed the lack of MR seen on TTE. Thus, her large V waves were felt to be due to stiff left atrial syndrome, and a cardiac CT showed a severely calcified “coconut left atrium”. Labwork revealed a profoundly low thiamine level (21, with LLN of 70), raising concern for wet beri beri syndrome.  

The patient’s unifying diagnosis was indolent left atrial syndrome that was exacerbated by high outout heart failure due to Wet Beri Beri syndrome. The patient received thiamine supplementation, and was diuresed to euvolemia with dramatic improvement in symptoms. A repeat RHC after thiamine replacement showed a CO of 5.7 and CI of 2.74 by thermodilution, demonstrating resolution of her high output heart failure.  


Case Media

A. CXR
B. ECG
C. RHC: large V waves are noted on the RHC
D. CO 11 and CI 5.2 by thermodilution pre-treatment
E. Cardiac CT showed a severely calcified “coconut left atrium”
F. Repeat CO of 5.7 and CI of 2.74 by thermodilution after thiamine replacement

TTE 1
TTE 2
TEE 1 – Mitral Valve
TEE 2 – Mitral Valve
Cardiac CT

Episode Schematics & Teaching


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

1) This case featured a patient with Stiff Left Atrial Syndrome! Cardionerds, what the heck is that?  

  • Stiff Left Atrial Syndrome (SLAS) is fundamentally a disorder of atrial compliance, wherein a non-compliant left atrium (LA) leads to abnormal atrial diastole. During LV systole (atrial diastole), the LA receives blood from the low-resistance pulmonary veins. Under normal conditions, the LA pressures initially fall (x-descent). Then, as the atrium fills from both RV contraction and passive filling from the pulmonary veins, there is a steady and modest rise in LA pressure (v-wave). In patients with decreased LA compliance, the V-wave may be accentuated.  
  • In SLAS, left atrial compliance is significantly decreased, leading to very large v-waves that reflect the inability to accommodate LA filling and the steepened slope of the pressure-volume curve (see the below diagram from Urey et al). This leads to dramatically increased LA pressures during LV systole (especially in late LV systole), contributing to post-capillary pulmonary hypertension over time and symptoms of dyspnea on exertion.  
s 
repeated ablation 
VOLUME 
Figure 2. Hypothetical pressure—volume 
relationship as seen in stiff left atrial (LA) 
syndrome. The blue line represents a nor- 
mal LA pressure—volume relationship with 
a compliant LA. With a stiff, noncompliant 
LA, as can be seen after atrial ablations, 
the pressure—volume relationship steep- 
ens, accounting for large V waves and 
subsequently the symptoms of pulmonary 
congestion.

2) Which patients are at risk of developing SLAS, how is it diagnosed, and how is it managed?  

  • Stiff Left Atrial Syndrome was first described in the late 1980s as a complication of mitral valve surgery, and has been increasingly recognized as a complication of left atrial ablation procedures leading to atrial fibrosis. While the condition is relatively rare (occurring in ~1.4% of patients following ablation), significant heart failure symptoms and pulmonary hypertension can develop.  
  • While no diagnostic criteria exist, SLAS should be considered in patients with HFpEF, a small or calcified LA on imaging, and risk factors including mitral valve surgery and/or prior left atrial ablations. Invasive hemodynamics will show large v-waves in the absence of mitral regurgitation (or disproportionate to the degree of MR) and an elevated PCWP out of proportion to the LVEDP. It is important to exclude pulmonary vein stenosis, another potential complication of ablation.  
  • Management consists primarily of diuretics and reducing ventricular afterload as tolerated, though an intra-atrial septostomy could be considered in refractory cases.  
  • Notably, SLAS may be asymptomatic in many patients due to the compliance of the pulmonary venous vascular system, which can store blood volume without significant increases in pressure. However, this compliance could become overwhelmed in certain stressed states or exercise. 

3) Our patient experienced a stressor in the form of high output heart failure; what is the pathophysiology of high output heart failure, and what is your differential for high output heart failure? 

  • While a number of causes for high output heart failure exist, they share an underlying pathophysiology of excessively decreased systemic vascular resistance and increased metabolic demand. The persistently low SVR leads to decreased ventricular afterload, increased LV emptying and thus increased stroke volume and cardiac output. This subsequently leads to increased preload and symptoms of congestive heart failure. Furthermore, increased oxygen demands requires increased cardiac output. Additionally, the persistently low SVR causes low renal perfusion pressure (renal hypoperfusion) which leads to RAAS activation and volume expansion 
  • Diagnosis is based on echocardiographic evaluation, RHC hemodynamics, and an identified cause of a high output state. TTE may show normal or reduced ejection fraction; additional findings may include a dilated IVC, RV enlargement or dysfunction, elevated estimated pulmonary artery pressures, and/or LV enlargement. RHC typically shows a CO > 8 L/min or a CI > 4 L/min/m2, though these cutoffs are not absolute.  
  • The differential for high output heart failure includes etiologies secondary to predominantly low SVR (e.g., obesity, cirrhosis, AV fistula) versus those secondary to increased metabolic drive (e.g., hyperthyroidism, myeloproliferative disorders). See the CNCR episode from the Johns Hopkins Hospital for more details!  

4) How does thiamine deficiency lead to high output heart failure?  

  • Thiamine is vital to aerobic metabolism in the Krebs cycle and the Pentose Phosphate Pathway. In states of thiamine deficiency, anaerobic metabolism is favored over aerobic metabolism, leading to increased levels of lactate and pyruvate. This leads to a decrease in adenosine triphosphate (ATP) and increase in adenosine monophosphate (AMP), which is released into skeletal muscle as adenosine. This release of adenosine leads to vasodilation and decreased systemic vascular resistance through shunt physiology. 
  • Arterial hypoperfusion of the kidneys leads to activation of the RAAS and expansion of plasma volume. Increased oxygen demand lead to an increased cardiac output. 
  • Importantly, CO by thermodilution and Fick may be discrepant in Beriberi! This is because mitochondria are unable to utilize O2 by performing aerobic metabolism. Thus, less oxygen is extracted from the blood, and venous oxygen saturations will be relatively elevated. This may leads to an erroneously elevated CO by Fick’s method as compared to thermodilution! 

5) Lets bring it all together! Cardionerds, what is your illness script for Beriberi?  

  • Pathophysiology: As detailed above, thiamine deficiency causes an increase in anaerobic metabolism, increased oxygen demand and systemic vasodilation through increased adenosine levels.  
  • Epidemiology: Patient populations at risk for severe thiamine deficiency include patients with severe malnutrition, chronic alcohol use, incarceration, social isolation, refugee populations, history of bariatric surgery, or chronic loop diuretic use. Notably, 90% of patients on diuretics can develop some level of thiamine deficiency. 
  • Signs/Symptoms: “Dry” beriberi involves symmetrical peripheral neuropathy, primarily in the distal extremities. “Wet” beriberi is characterized by high output heart failure and can lead to shock in severe cases.  
  • Diagnosis: Thiamine deficiency is difficult to diagnose. Blood thiamine levels can be low in acute illness and do not reflect total body stores. Erythrocyte transketolase activity and thiamine pyrophosphate effect tests can be used, though these tests have poor specificity and sensitivity. The gold standard is high performance liquid chromatography, though access to this test is expensive and not commonly available.  
    • As a historical note, in 1945, Marion Blankenford developed diagnostic criteria for wet beriberi, which includes evidence of an enlarged heart with normal rhythm, dependent edema, elevated venous pressure, peripheral neuritis or pellagra, nonspecific alternans on ECG, no evidence of other cardiac disease, at least 3 months of thiamine deficiency, and improvement in symptoms and reduction in heart size following thiamine replacement.  
  • Treatment: The cornerstone of wet beriberi management is supportive treatment of heart failure while replacing thiamine stores. A rapid and dramatic improvement following thiamine replacement is diagnostic of wet beriberi.  

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

69. Case Report: Cardiac Allograft Vasculopathy (CAV) – UCSD

CardioNerds (Amit Goyal & Daniel Ambinder) join University of California San Diego (UCSD) cardiology fellows (Harpreet Bhatia, Dan Mangels, and Quan Bui) for a relaxing beach bonfire in the beautiful city of San Diego! They discuss a challenging case of post-transplant cardiac allograft vasculopathy. Dr. Hao (Howie) Tran provides the E-CPR and program director Dr. Daniel Blanchard 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 mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join University of California San Diego (UCSD) cardiology fellows (Harpreet Bhatia, Dan Mangels, and Quan Bui)  for a relaxing beach bonfire in the beautiful city of San Diego! They discuss a challenging case of post-transplant cardiac allograft vasculopathy. Dr. Hao (Howie) Tran provides the E-CPR and program director Dr. Daniel Blanchard 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.

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 20s with a past medical history of orthotopic heart transplant, presents with one-week of progressive lower extremity edema and dyspnea with NYHA class IV symptoms. 5 years prior, he underwent orthotopic heart transplant for arrhythmogenic right ventricular cardiomyopathy. Subsequently, he has had multiple episodes of rejection or recurrent graft dysfunction. On presentation, he was normotensive and borderline tachycardic. Exam revealed elevated JVP, decreased breath sounds, and pitting edema.  Labs demonstrated leukocytosis, acute kidney injury, and elevated pro-BNP. TTE demonstrated LVEF 35%, apical akinesis, and grade III diastolic dysfunction (all similar to prior). He was initially diuresed and RHC/EMB was performed to evaluate for rejection. Early in his course, the patient unfortunately suffered a PEA arrest with ROSC was quickly achieved after 1 minute of CPR. He was intubated and cannulated for VA ECMO. EMB demonstrated ISHLT Grade 1R cellular rejection and he was ultimately listed for re-transplant. Shortly thereafter, the patient received an OHT. His pathology demonstrated intimal thickening of all his coronaries, consistent with coronary artery vasculopathy, felt to be the major contributor to his presentation.  


Case Media


Episode Schematics & Teaching


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

1. What is CAV?  

  • CAV stands for cardiac allograft vasculopathy. Within the transplanted heart, CAV is the proliferation of vascular smooth muscle and intimal thickening in the epicardial coronary arteries and microvasculature leading to diffuse narrowing. CAV is common, present in greater than 30% of patients at 5 years post-transplant. It is a significant contributor to post-transplant mortality after the first year.  
  • CAV, in contrast to typical atherosclerotic lesions, is diffuse and concentric while atherosclerosis tends to be focal with eccentric luminal narrowing and heterogenous plaque composition. Patients s/p OHT can still develop typical coronary artery disease, likely developed from pre-existing disease in the donor heart. CAV should be high on the differential for the cause of graft dysfunction, especially after the first year post-transplant.  

2. How and Why Does CAV Occur? 

  • CAV has multiple contributing factors. There are immunologic and non-immunologic factors, but it appears the immunologic components play the larger role given that the pan-vasculopathy develops in the donor heart and not in the recipient’s vasculature. In CAV, there is chronic immune-mediated injury creating a persistent inflammatory state in the donor coronary endothelium leading to a neointimal proliferative process in the coronaries. Amongst immunologic factors, it appears the number of episodes of cellular rejection correlates with the development of CAV.  
  • CAV occurs when foreign antigens are recognized by the host immune system as “non-self,” a process termed allorecognition.  T-cells are subsequently activated, and release a number of inflammatory cytokines that leads to additional T-cell stimulation, inflammatory cell proliferation, and endothelial cell propagation.  Ultimately this inflammatory cascade leads to smooth muscle cell advancement and intimal growth into the arterial lumen.  
  • Other immunologic factors include HLA mismatch and antibody-mediated rejection. There are numerous non-immunologic factors, including older donor age, CMV infection, hyperlipidemia, insulin resistance, donor brain death secondary to intracranial hemorrhage, and prolonged ischemic time.  

3. How Do Patients with CAV Present?  

  • Donor hearts are denervated at explantation, and so post-transplant patients typically will not develop classic anginal symptoms as seen with typical atherosclerotic coronary disease. Thus, routine surveillance is necessary (see below).  
  • If not diagnosed early, the clinical presentation may include LV dysfunction (with or without symptoms), acute myocardial infarction, heart block, arrhythmias, syncope, or sudden cardiac death.  

4.  How Do We Diagnose CAV? 

  • Routine surveillance is necessary because patients are generally asymptomatic and there is a high incidence of CAV posttransplant.  
  • The most common method for screening includes coronary angiography, but its sensitivity is reduced compared to traditional atherosclerotic disease as CAV is diffuse. Intravascular ultrasound (IVUS) significantly improves sensitivity and the early the detection of disease.  
  • The timing and method of screening will be center-specific. As the patient is farther removed from their transplant date, dobutamine stress echo may be a reasonable method to screen for CAV. Myocardial perfusion imaging, specifically with PET Rest/Stress with absolute myocardial blood flow quantification, and coronary CTA may also be effective methods to diagnose CAV.  
  • The ISHLT grading of CAV by angiography is as follows: 
    1. CAV(Nonsignificant): No detectable angiographic lesion 
    2. CAV1 (mild): Angiographic LM lesion <50%; or primary vessel with maximum lesion of <70%; or any branch vessel stenosis <70% without allograft dysfunction 
    3. CAV2 (moderate): Angiographic LM <50%; or a single primary vessel ≥70% stenosis; or isolated branch stenosis in 2 systems ≥ 70% without allograft dysfunction 
    4. CAV3 (Severe): Angiographic LM ≥50%; or ≥2 primary vessel ≥70% stenosis; or isolated branch stenosis in all 3 systems ≥70%; CAV1 or CAVwith allograft dysfunction or evidence of significant restrictive physiology 

5. How Do we Treat CAV?  

  • Primary prevention remains key. Statins have been shown prospectively to reduce cardiac allograft vasculopathy and improve survival. Chronic immunosuppression is the foundation of post-transplant care. The mTOR inhibitors, everolimus and sirolimus, harbor antiproliferative properties that may prevent allograft vasculopathy. However, these are generally not first-line immunosuppressive medications in the United States, given the potential for multiple side effects including impaired wound healing in new transplant patients. In patients with documented or progressive CAV, escalation of immunosuppression to sirolimus may be considered. Revascularization for patients may be considered, given the morbidity associated with CAV, though no survival advantage has been shown. In patients with severe CAV, re-transplantation should be considered.  

References

  1. Mehra, M. R., Crespo-Leiro, M. G., Dipchand, A., et. al (2010). International Society for Heart and Lung Transplantation working formulation of a standardized nomenclature for cardiac allograft vasculopathy—2010. 
  2. Chih, S., Chong, A. Y., Mielniczuk, L. M. et. al. (2016). Allograft vasculopathy: the Achilles’ heel of heart transplantation. Journal of the American College of Cardiology68(1), 80-91. 
  3. Schmauss, D., & Weis, M. (2008). Cardiac allograft vasculopathy: recent developments. Circulation117(16), 2131-2141. 

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

50. Case Report: Hereditary Restrictive Cardiomyopathy – Duke University

CardioNerds (Amit Goyal & Dan Ambinder) discuss a case of hereditary restrictive cardiomyopathy with Duke University cardiology fellows Navid Nafissi and Sipa Yankey, and Marat Fudim, an advanced heart failure attending. E-CPR is provided by Dr. Richa Agarwal, fellowship program director of advanced heart failure and cardiac transplantation at Duke University with a final message by fellowship director Dr. Anna Lisa Crowley. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  

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CardioNerds (Amit Goyal & Dan Ambinder) discuss a case of hereditary restrictive cardiomyopathy with Duke University cardiology fellows Navid Nafissi and Sipa Yankey, and Marat Fudim, an advanced heart failure attending at Duke University. E-CPR is  provided by Dr. Richa Agarwal, fellowship program director of advanced heart failure and cardiac transplantation at Duke University with a final message by fellowship director Dr. Anna Lisa Crowley. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal 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.

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Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A 69 yo M with history of atrial fibrillation presents with 5 months of progressive HF symptoms, now NYHA class IV. He was found to be grossly volume overloaded, tachycardic in atrial fibrillation, and hypoxic. CXR demonstrated significant cardiomegaly, and labs indicated new normocytic anemia with evidence of hepatic dysfunction and an elevated NT proBNP. TTE demonstrated massive bi-atrial enlargement, preserved ejection fraction, filling pattern consistent with grade III diastolic dysfunction, and torrential TR. The echocardiogram did not have evidence of constrictive pericarditis and agitated saline study showed Right to Left shunt through a likely PFO. MRI to evaluate for infiltrative cardiomyopathy did not show late gadolinium enhancement (LGE). RHC demonstrated findings consistent with restrictive cardiomyopathy including equalization of diastolic pressures, square root sign, and concordance of RV and LV pressures. PYP scan evaluating for TTR amyloid was negative and laboratory workup did not suggest AL amyloid, Fabry’s, Hemochromatosis, or storage disease. Patient’s symptoms remained refractory and thus eventually underwent successful OHT. Genetic testing eventually revealed missense mutation in MYBPC3 – revealing an inherited cause of restrictive CM for the patient! 


Case Media


Episode Schematics & Teaching


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

  1. When thinking about the etiology of a restrictive cardiomyopathy, we can organize the causes into four major categories: 
    1. Infiltrative (e.g., amyloidosis, sarcoidosis) 
    2. Storage diseases (e.g., hemochromatosis, Fabry’s) 
    3. Non-infiltrative (e.g., idiopathic, diabetic cardiomyopathy, genetic causes) 
    4. Endomyocardial (e.g., endomyocardial fibrosis, hypereosinophilic syndrome) 
  2. On examination, patients with restrictive cardiomyopathy may have prominent right-sided symptoms, including hepatomegaly, ascites, and significant peripheral edema. On exam, neck veins may demonstrate a steep y descent, and cardiac auscultation may have a +S4, and murmurs of tricuspid and mitral regurgitation.
  3. A multimodal imaging workup is recommended for evaluating RCM.  
    1. TTE: Typically demonstrates normal LVEF, normal chamber volumes, biatrial enlargement, and restrictive diastolic filling patterns (.e.g, E/A > 2, E/e’ > 14, decreased mitral deceleration time < 150 ms). We can see increased wall thickness with infiltrative processes and storage diseases.
    2. cMRI: specific patterns of Late Gadolinium Enhancement may indicate certain pathology (e.g., amyloid may demonstrate diffuse subendocardial, heterogeneous, or transmural signal). T2 signal can identify inflammation and quantify iron. 
    3. PYP scan: It has a >99% sensitivity for cardiac ATTR amyloid. If monoclonal gammopathy excluded, PPV 100%. 
  4. Invasive hemodynamics can show physiology consistent with RCM. This includes square root sign, equalization of diastolic pressures, and concordance during respiration of LV/RV pressure changes. In contrast, constrictive physiology will yield discordant respirophasic LV/RV pressure changes. Endomyocardial biopsy may be necessary to identify etiology but the yield for patchy diseases, like sarcoid, can be low. 
  5. Patients with end-stage RCM can be difficult to manage medically, especially as they become refractory to diuretics. They poorly tolerate antihypertensive agents (due to inability to augment limited stroke volume), heart rate lowering medication (due to low SV, they are dependent on HR to maintain CO which is HR x SV), and have a very limited optimal preload window (need high filling pressures to fill the stiff ventricles but also have symptoms of fluid overload). Due to anatomic considerations, patients are rarely candidates for durable left ventricular assist devices and often require orthotopic heart transplant. However, patients with RCM have higher waitlist mortality and longer wait times, in part due to lower utilization of MCS.   

Educational Video

Produced by Dr. Karan Desai

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

37. Palliative Care in Heart Failure with Dr. Rab Razzak

Episode #37. Palliative Care in Heart Failure

The role of palliative care in the management of heart failure is discussed by palliative care expert, Dr. Rab Razzak, clinical associate professor and clinical director of palliative care at University Hospital Cleveland Medical Center. CardioNerds host Amit Goyal is joined by special guest interviewer, Dr. Arsalan Derakhshan, Assistant Program director at Case Western Internal Medicine residency program and the head of the Global Health Pathway as well as co-host of the Clinical Problem Solvers podcast!

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Dr. Rab Razzak went to medical school at Bangledesh Medical College and internal medicine residency at St. Joseph Regional Medical Center in Patterson, NJ. He worked initially as a hospitalist at Cedars Sinai and was grandfathered into palliative care.  He moved to Maryland to work at Johns Hopkins, where he practiced for 4.5 years. He is now a clinical associate professor and clinical director of palliative care at University Hospital Cleveland Medical Center. He is also a devoted husband, dedicated father, and a stand-up comedian! We could think of no one better to discuss Palliative Care in Heart Failure than Dr. Razzak.

Dr. Arsalan Derakhshan was born in Iran and moved to Atlanta, Georgia as a young child. He loves to travel and considers himself a global citizen. After graduating from Emory University, he attended the Medical College of Georgia and completed internal medicine residency at Johns Hopkins Hospital. He spends his time attending on inpatient team, staffing resident clinic, and working as the global health track director. He enjoys working with medical students and interns to help them discover their passions. His primary interests include clinical reasoning, global health,  and medical innovations. He co-hosts one of the most popular medicine podcast, The Clinical Problem Solvers and has been incredibly instrumental in the launch of The CardioNerds!

35. Heart Failure with Preserved Ejection Fraction with Dr. Kavita Sharma

Heart Failure with Preserved Ejection Fraction with Dr. Kavita Sharma

We discuss Heart Failure with Preserved Ejection Fraction (HFpEF) with Dr. Kavita Sharma, director of the Heart Failure with Preserved Ejection Fraction Program and interim director of Advanced Heart Failure Transplant section at The Johns Hopkins Hospital. CardioNerds hosts Carine Hamo and Daniel Ambinder are joined by Dr. Beth Feldman (resident at The Johns Hopkins Hospital). Topics discussed include a definitions, diagnosis, phenotypic presentations, inpatient management of acute decompensated heart failure, role of dopamine, advanced therapies of HFpEF, and the Paraglide trial.

References mentioned in this episode can be found here

On the CardioNerds Heart Failure topic page you’ll podcast episodes, references, guest experts and contributors, and so much more.

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Acute Decompensated Heart Failure Primer – Youtube

Dr. Kavita Sharma graduated from the University of Virginia School of Medicine and completed her residency and served as the assistant chief of service, cardiology fellowship and advanced heart failure fellowship at the Johns Hopkins Hospital. She is the Director of the Johns Hopkins Heart Failure with Preserved Ejection Fraction Program and is currently the interim director of Advanced Heart Failure Transplant section at Hopkins. She has a specialized interest in heart failure with preserved ejection fraction (HFpEF), and directs one of the largest programs in the country dedicated to caring for patients with this condition. She is the principal investigator of numerous clinical and translational trials in HFpEF and leads a team of nurses, research coordinators, and fellows-in-training in this multifaceted program. She is an invited speaker at national meetings in topic areas covering advanced heart failure and HFpEF.

Dr. Beth Feldman graduated from Temple University School of Medicine and is currently on the Longcope Firm on the Osler Medical Service at Johns Hopkins University Hospital. Before pursuing a career in medicine, she worked in health care consulting focusing in health systems. She is passionate about health policy and health systems research. She is hoping to pursue a career in cardiology, with a particular interest in critical care.

31. Fulminant Myocarditis with Cardiogenic Shock: Case Discussion

The CardioNerds discuss a case of fulminant myocarditis, teaching a comprehensive approach to myocarditis with just 5 foundational principles. Review the myocarditis infographic on the myocarditis topic page. The episode ends with a special message from the true heroes of this episode, Chas and Julie Miller. 

This marks our first episode after 100,000 downloads of the show – and this is a benchmark that we are celebrating together with all of you. Since launch, we have had 82 voices on the show and youtube channel. Our website which collates all the podcast episodes, youtube videos, tweetorials, and more has been accessed in 120 countries. We just cannot thank you enough! 

On the CardioNerds Myocarditis page you will find podcast episodes, infographic, youtube videos, references, tweetorials and guest experts & contributors, flutter stars and so much more.

5 points of maximal impulse in approaching myocarditis

  1. Build the clinical suspicion for myocarditis: You need a high index of suspicion given the variable presentation, and definitely need to keep a differential so you don’t miss things like acute coronary syndromes.
  2. Decide if EMBx is necessary: Most often obtained in fulminant presentations to look for pathologic findings of giant cell myocarditis or eosinophilic myocarditis, because these findings will change management.
  3. Manage the acute cardiac injury, which can range from supportive care to treatment of shock, arrhythmias, and even tamponade.
  4. Manage the chronic cardiac sequelae: Recovering from the acute phase of myocarditis doesn’t necessarily mean smooth sailing — some develop chronic heart failure, warranting GDMT — or guideline directed management and therapy, as defined by Dr. Randall Starling in Ep 13.
  5. Treat the myocarditis: Immunosuppression is often started empirically in fulminant disease, but continuation depends on what you find on pathology.

30. Myocarditis with Drs. JoAnn Lindenfeld, Javid Moslehi and Dr. Enrico Ammirati: Part 2


Cardiovascular experts, Drs. JoAnn Lindenfeld, Javid Moslehi and Richa Gupta from Vanderbilt University Medical Center and Dr. Enrico Ammirati from Milan, Italy join Amit and Dan for a two part discussion about all things to consider for myocarditis in general (part 1) and COVID-19 myocarditis and heart transplantation in the COVID-19 era (part 2). Flutter Moment by Barrie Stanton (RN).

On the CardioNerds Myocarditis page you will find podcast episodes, infographic, youtube videos, references, tweetorials and guest experts & contributors, flutter stars and so much more.

Take me to the Myocarditis Series Page 
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Dr. JoAnn Lindenfeld, is a Professor of Medicine and the Director of Heart Failure and Heart Transplantation Section at Vanderbilt Heart and Vascular Institute.  She is the past president of the Heart Failure Society of America and serves on editorial boards of numerous journals including JACC, JACC Heart Failure and JHLT.  She is also a member of the AHA/ACC/HFSA heart failure guideline writing committee and was previously chair of the HFSA practice guidelines for the 2006 and 2010 guidelines. In addition to this she’s been an investigator in multiple large-scale clinical trials including the COAPT trial and has served on numerous steering committees, end point committees and data and safety monitoring committees. She is the author of a more than 300 original papers, reviews, and book chapters in the field of heart failure and heart transplantation.

Dr. Javid Moslehi is an associate professor of medicine at Vanderbilt University Medical Center where he is the director of the cardio-oncology program.  He is a clinical cardiologist and basic/translational biologist interested in cardiovascular complications associated with novel molecular targeted cancer therapies and the implications of these on our knowledge of basic cardiovascular biology. At Vanderbilt he runs an independent basic and translational research laboratory and program with a focus on signal transduction in the myocardium and vasculature as well as establishing pre-clinical models of cardiotoxicity involving novel targeted oncologic therapies.

Dr. Enrico Ammirati is an assistant professor of cardiology and advanced heart failure and transplant cardiologist in Milan, Italy at the Niguarda Hospital with a special research interest and expertise in acute myocarditis and acute heart failure.  He is a fellow of the European Society of Cardiology and has won numerous awards, he has also published incredibly important work on the distinction between fulminant and nonfulminant myocarditis and the prognostic implication of histologic subtypes.  His research interests also include the role of adaptive immunity in heart transplantation and atherosclerosis and he is the author of well over 100+ peer reviewed publications.

27. COVID-19: ID Part 4: Virology and epidemiology with Drs. Chida and Nematollahi

Infectious disease experts from the Johns Hopkins Hospital, Drs. Natasha Chida and Saman Nematollahi join the CardioNerds for a 4 part COVID-19 infectious disease series. In this final episode, we discuss the virology and epidemiology of SARS-CoV-2 that serves as the underpinnings for the three prior episodes. Flutter Moment by Dr. Sumeet Bahl (Vascular and Interventional Radiology)

We are honored to promote the incredible efforts of #GetUsPPE, a nonprofit organization working hard to make sure every healthcare worker is protected. Dr. Seth Trueger, emergency medicine physician and digital media editor @JAMA Network Open shares an earnest request.

In light of the COVID-19 pandemic, we shifted gears to meet the educational need as we all are learning more about the cardiovascular implications of SARS-CoV-2. On the CardioNerds COVID-19 series page, you will find our collection of podcast episodes, infographic, youtube videos, curated #Tweetorials, references, guest experts & contributors, flutter stars and so much more.

Check out the COVID-19 series page!
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Click here for Youtube COVID-19 Playlist
Click here for our Youtube CV fundamentals playlist

Dr. Natasha Chida, an infectious disease expert at Johns Hopkins. Dr Chida received her MD from the University of Miami Miller School of Medicine, where she also earned an MSPH (masters of science in public health). She completed internal medicine residency at Jackson Memorial Hospital and infectious disease fellowship at Johns Hopkins, where we were lucky to keep her on as faculty. She is a truly incredible educator and mentor to all levels of trainees — she serves as assistant director of the infectious disease fellowship program, co-director of the medical education pathway for residents, director of education for the Johns Hopkins Center for Global Health Education, and course director for the ‘Topics in Interdisciplinary Medicine’ course for medical students.

Dr. Saman Nematollahi grew up in Tucson, Arizona. He completed his undergrad at the University of Arizona with dual degrees in Physiology and Spanish Literature. He spent some time after undergrad working in a neuroscience lab before starting med school at the University of Arizona. He then moved to NYC where he completed residency at Columbia. His clinical interests include management of immunocompromised hosts, and his research interest is in medical education.  More than that he is a teacher at heart and is obtaining a Master’s of Education at the Johns Hopkins School of Education and was recently awarded a grant to develop a fungal diagnostic curriculum for residents. He loves to play soccer with his wife and son. He is master educator, appeared on and has authored many important tweetorials. He has also appeared on the hit medical podcast, The Clinical Problem Solvers.