59. Case Report: Constrictive Pericarditis & Severe Mitral Regurgitation – Mayo Clinic

CardioNerds (Amit Goyal & Daniel Ambinder) join join Mayo Clinic cardiology fellows (Mays Ali, Charlie Jain, Korosh Sharain) for a scenic walk through gorgeous Rochester, Minnesota! They discuss a fascinating case of constrictive pericarditis and severe mitral regurgitation. Dr. Rick Nishimura provides the E-CPR and program director Dr. Frank Brozovich provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Bibin Varghese 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 Mayo Clinic cardiology fellows (Mays Ali, Charlie Jain, Korosh Sharain) for a scenic walk through gorgeous Rochester, Minnesota! They discuss a fascinating case of constrictive pericarditis and severe mitral regurgitation. Dr. Rick Nishimura provides the E-CPR and program director Dr. Frank Brozovich provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Bibin Varghese 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
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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

Constrictive Pericarditis & Severe Mitral Regurgitation – Patient Summary

A woman in her late 40s with a history of lupus and hypertension presented with worsening dyspnea on exertion and orthopnea over a year. She reported intermittent pleuritic chest discomfort that had persisted since an episode of acute pericarditis years prior. A TTE suggested severe mitral regurgitation, and she was referred to the Mayo Clinic for mitral valve intervention.   

The official TTE report from the OSH suggested non-dilated LV, EF 55-60%, normal RV function, severe MR with thickened leaflets and sub-valvular apparatus, moderate to severe TR and a dilated IVC. Furthermore, the CXR showed pericardial calcifications. Upon evaluation by the Mayo Clinic fellows, the JVP was elevated to about 10-12 cm with rapid x and y descents, a positive Kussmaul’s sign, and the murmurs of MR and TR. Her lungs were clear to auscultation and extremities did not demonstrate edema. Re-review of the TTE images revealed posterior pericardial thickening, no septal shift on respiration, but suggestion of annulus reversus where medial mitral annulus tissue doppler (9 cm/s) was greater than lateral (8 cm/s). Further, there was evidence of expiratory hepatic vein diastolic flow reversal. 

For the team, there was discordance between the apparent severity of her MR reported by echocardiogram and her clinical symptoms. In addition, the echocardiogram was suggestive of specific signs of constrictive pericarditis. Thus, simultaneous RHC/LHC was obtained. There was equalization of RV/LV pressures during diastole, demonstration of a “square root sign” and importantly discordance between LV and RV pressures with respiration. Thus, discordant clinical findings led to a suspicion for constrictive pericarditis and was corroborated by discordance on invasive hemodynamics! Further, the V-waves were not prominent on wedge pressure tracing and to investigate the mitral regurgitation further, an LV ventriculogram was done. This demonstrated 3+ to 4+ MR.  Based on all the findings, the patient was diagnosed with constrictive pericarditis, severe MR and moderate to severe TR. She underwent pericardiectomy, mitral valve replacement (given that repair was not feasible due to the sub-valvular thickening) and given that TR has been shown to worsen after pericardiectomy and is a poor prognostic factor, she additionally underwent tricuspid valve repair. Her symptoms improved markedly following intervention. 


Case Media

A. CXR: Heart size was borderline enlarged with biatrial enlargement.  LV does not appear very enlarged.
B. Mitral Regurgitation by CW Doppler
C. Tricuspid regurgitation by CW Doppler. TR Max 2.43
D. Tissue Doppler of the mitral valve annulus: Medial e’ = 9 cm/s, Lateral e’ 8 cm/s
E. Hepatic Vein PW Doppler
F. Right atrial pressure tracing
G. RV and LV simultaneous pressure tracings
H. Wedge pressure and LV simultaneous pressure tracings

Neck Vein Exam
TTE
TTE: Color Doppler across mitral valve
TTE: Short Axis
TTE: Short Axis, TV
TTE: Hepatic Vein Doppler
Left ventriculogram with severe mitral regurgitation


Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case- Constrictive Pericarditis & Severe Mitral Regurgitation

  1. One of the early clues to the etiology of this patient’s dyspnea was a positive Kussmaul’s sign. What is the mechanism and differential of Kussmaul’s Sign?  
  • Typically the JVP decreases with inspiration as the negative intrathoracic pressure “sucks in” blood from the vena cavae. When there is a lack of decrease or an increase in JVP with inspiration, this finding is called Kussmaul’s sign. Kussmaul’s sign reflects conditions where there is right ventricular dysfunction, impaired RV filling and elevated right atrial pressure   
  • Kussmaul’s sign is classically associated with constrictive pericarditis. Remember, during normal inspiration, the diaphragm contracts and increases abdominal pressure with variable effect on venous return. However, in constriction, the rise in abdominal pressure increases venous return from the congested hepato-splanchnic vasculature. And since RV filling is constrained by a non-compliant pericardium, there is a rise in JVP during inspiration.   
  • Other conditions where we may see a positive Kussmaul’s sign include restrictive cardiomyopathy, RV predominant infarction, massive pulmonary embolism, tricuspid stenosis, and severe tricuspid regurgitation. 

2. A decision was made to pursue invasive hemodynamic evaluation to differentiate between restrictive and constrictive physiology. How do we differentiate between the two on echocardiogram?  

  • Differentiating between the two diagnoses requires an understanding of the pathophysiologic differences between constriction and restriction.  Enjoy Ep #58 and CN5 for understanding constrictive physiology and echocardiographic findings! In restriction we do not have intrathoracic-intracardiac disassociation as intrathoracic pressures are transmitted normally to the cardiac chambers during respiration. Further, there is no exaggerated interventricular dependence.   
  • We may see increased myocardial thickness in restriction (secondary to infiltrative or storage disorders – enjoy Ep #50 – restrictive cardiomyopathy) and increased pericardial thickness/calcification in constriction.   
  • Unlike constrictive pericarditis, restrictive cardiomyopathies should not demonstrate respirophasic septal shift or variation across the atrioventricular valves. Both conditions will demonstrate hepatic vein diastolic flow reversal. However, in constriction, the exaggerated ventricular interdependence and greater LV filling during expiration causes septal bowing into the RV and results in greater hepatic vein diastolic flow reversal during expiration. In restriction, hepatic vein flow reversal is more prominent in inspiration as the stiff myocardium is unable to tolerate the increased RV preload that occurs during inspiration.   
  • The estimated pulmonary artery systolic pressures tend to be elevated (>55-60 mmHg) in restrictive cardiomyopathy and are often normal in constriction. In constriction, the annular tissue velocity is preserved except at the lateral annulus, where tethering of the lateral wall results in decreased velocities and annulus reversus (See Ep#58 and CN5). Meanwhile, annular tissue velocity (e’) is significantly reduced in restrictive cardiomyopathy.   
  • Enjoy Ep #58 and CN5 for differences on invasive hemodynamics! 

3. Given that the patient had subvavlular thickening, the patient was not a candidate for mitral valve repair and the patient underwent mitral valve replacement. What are the indications for mitral valve surgery in patients with severe chronic MR? Why is mitral valve repair preferred for primary MR?  

  • Class I indications for mitral valve surgery include (1) symptomatic patients in the absence of severe LV dysfunction (defined as EF <30%; Stage D MR); (2) asymptomatic patients with chronic severe MR and mild to moderate LV dysfunction (EF 30% to 60% and/or LV end-systolic dimension ≥ 40 mm; Stage C); (3) patients with chronic severe primary MR undergoing cardiac surgery for other indications. 
  • Class IIa indications include (1) asymptomatic patients with LVEF > 60% and LVESD < 40 mm (Stage C1) in whom the likelihood of successful repair is >95% and expected mortality is <1%; (2) asymptomatic patients with new-onset atrial fibrillation and preserved LVEF (Stage C1); (3) asymptomatic patients with pulmonary hypertension (defined as PASP > 50 mmHg) and preserved LVEF. 
  • For primary mitral regurgitation involving an abnormality of the valve apparatus (e.g., leaflets, chordae, annulus, papillary muscles) – MV repair is preferred over MV replacement in most patients as it is associated with lower operative mortality and improved long term survival. Feasibility of valve repair will generally depend on valve anatomy and surgical experience. Class I indications for MV repair over mitral valve replacement (MVR) include chronic severe primary MR limited to the posterior leaflet and chronic severe primary MR involving the anterior leaflet or both leaflets when a successful and durable repair appears feasible. 

4. The patient underwent TV repair. What is the reason to potentially pursue TV intervention in patients undergoing pericardiectomy?  

  • Tricuspid regurgitation (TR) is often a co-morbid condition in patients with constrictive pericarditis. After pericardiectomy, TR can worsen after a thickened, calcified pericardium is removed allowing RV dilation and subsequent dilation of the TV annulus leading to worsening functional TR. Tricuspid regurgitation is associated with increased short and long-term mortality following pericardiectomy, and the Mayo Clinic in particular has been integral in demonstrating that even mild TR is a marker of increased operative and long-term mortality and worsening symptoms. Thus, in patients with moderate to severe TR at the time of pericardiectomy, consideration should be given for tricuspid valve repair (if feasible). Of note, most data to date suggests that this approach provides symptomatic improvement but no clear mortality benefit has been demonstrated. 

5. Finally, the Mayo CardioNerds taught us to think about our thinking! What are some cognitive errors to be aware of when evaluating patients?  

  • The team at the Mayo Clinic masterfully demonstrated how to avoid the common cognitive errors of the anchoring heuristic and premature closure while diagnosing a case of constrictive pericarditis and severe mitral regurgitation. 
  • Anchoring heuristic is the tendency to rely on selected signs or simple investigations, often fixating on early data and not incorporating new information. In other words, one “anchors” onto the initial data and impressions. Ways to avoid anchoring include: attention to base rates of disease, knowledge of test characteristics of diagnostics, deliberately seeking more information to support or refute the initial impression, and following data-driven diagnostic algorithms.  
  • Premature closure is the acceptance of a diagnosis before it has been fully verified. To avoid this, consider alternative diagnoses and search for data that challenge the provisional diagnosis. 

References for episode 59: Constrictive Pericarditis & Severe Mitral Regurgitation

  1. Geske, J. B., Anavekar, N. S., Nishimura, R. A., Oh, J. K. & Gersh, B. J. Differentiation of Constriction and Restriction: Complex Cardiovascular Hemodynamics. J. Am. Coll. Cardiol. 68, 2329–2347 (2016). 
  2. Garcia, M. J. Constrictive Pericarditis Versus Restrictive Cardiomyopathy? J. Am. Coll. Cardiol. 67, 2061–2076 (2016). 
  3. Chiabrando, J. G. et al. Management of Acute and Recurrent Pericarditis: JACC State-of-the-Art Review. J. Am. Coll. Cardiol. 75, 76–92 (2020). 
  4. Scott, I. A. Errors in clinical reasoning: causes and remedial strategies. BMJ 338, (2009). 
  5. Nishimura, R. A. et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J. Am. Coll. Cardiol. 70, 252–289 (2017). 
  6. Bilchick, K. C. & Wise, R. A. Paradoxical physical findings described by Kussmaul: pulsus paradoxus and Kussmaul’s sign. Lancet Lond. Engl. 359, 1940–1942 (2002). 
  7. Tariq, M. U., Aman, W., Karwa, A., Benatti, R. & Klein, A. Right Ventricular Dilatation Post Pericardiectomy Causes Tricuspid Regurgitation. J. Am. Coll. Cardiol. 65, A1315 (2015). 
  8. Lazam, S. et al. Twenty-Year Outcome After Mitral Repair Versus Replacement for Severe Degenerative Mitral Regurgitation: Analysis of a Large, Prospective, Multicenter, International Registry. Circulation 135, 410–422 (2017). 

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

57. Case Report: Peripartum Cardiomyopathy with Cardiogenic Shock – University of Pennsylvania

CardioNerds (Amit Goyal & Daniel Ambinder) join Penn cardiology fellows (Brian McCauley, Norrisa Haynes, and Mahesh Vidula) for a rooftop picnic in sunny Philadelphia! They discuss an informative case of peripartum cardiomyopathy with cardiogenic shock. Program director Dr. Frank Silvestry provides the E-CPR segment and a message to applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summaryCase figures & mediaCase teachingReferencesProduction team

CardioNerds (Amit Goyal & Daniel Ambinder) join Penn cardiology fellows (Brian McCauley, Norrisa Haynes, and Mahesh Vidula) for a rooftop picnic in sunny Philadelphia! They discuss an informative case of peripartum cardiomyopathy with cardiogenic shock. Program director Dr. Frank Silvestry provides the E-CPR segment and a message to applicants. 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.

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

Two weeks postpartum, a woman in her mid 20s, G1P1, with no past medical history presented following a tonic-clonic seizure. Prior to this, she had been experiencing 1 week of worsening dyspnea and lower extremity edema. Initial work-up revealed a left MCA stroke and she underwent thrombectomy. Limited TTE found LVEF <20% and a LV apical thrombus; she was started on milrinone due to concern for cardiogenic shock and transferred to the University of Pennsylvania. 

Upon arrival, she was found to be hypotensive and tachycardic. Exam was notable for elevated JVP, +S3, LE edema and R sided hemiparesis. Labs showed multiorgan injury, elevated NT-proBNP and elevated lactate. EKG demonstrated sinus tachycardia with no ST-T changes. Formal TTE showed severely dilated LV with EF 10%, diffuse LV hypokinesis, and confirmed a large LV apical thrombus. A pulmonary artery catheter was placed for tailored therapy and found elevated L-sided > R-side filling pressures with low cardiac index despite inotropes. Cardiac power output (CPO) was severely decreased with borderline pulmonary artery pulsatility index (PAPI), corroborating left > right heart failure. Patient ultimately required a durable left ventricular assist device (LVAD). Over the course of 9 months her guideline directed medical therapy (GDMT) was titrated and her intrinsic cardiac function and symptoms improved. Her EF improved to 35-40% and she tolerated an LVAD weaning protocol, so her LVAD was ultimately explanted! She is currently doing well on GDMT alone!  


Case Media


TTE 1
TTE 2

Episode Schematics & Teaching


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

1. How do we define Peripartum Cardiomyopathy?  

  • Diagnosis is made by the development of heart failure towards the end of pregnancy or in the months following delivery (~5 months postpartum), no other identifiable cause of HF, and demonstration of LV systolic dysfunction with LVEF typically less than 45% with or without dilation. 
  • Risk factors include history of pre-eclampsia, hypertension, cocaine use, multifetal pregnancies, older maternal age, and African descent.    
  • Keep a broad differential diagnosis for new onset heart failure in the peripartum period. The differential includes pre-existing cardiomyopathy, valvular disease or congenital cardiomyopathy unmasked by the hemodynamic changes of pregnancy (see CNCR episode 48 for more on the hemodynamic changes of pregnancy!). Other differentials should include ischemia/spontaneous coronary artery dissection (SCAD), stress-induced cardiomyopathy, CM due to systemic disease (e.g. sepsis, rheumatologic disease), myocarditis and tachycardia-induced CM. 

2. The team used invasive hemodynamics to guide shock management. Why is a PAC helpful?  

  • Despite data from the ESCAPE trial, a pulmonary artery catheter (PAC) can be very useful in the diagnosis and management of cardiogenic shock. Remember, the ESCAPE trial included chronic HF patients in whom there was clinical uncertainty on whether a PAC may be useful. Further, any patient on milrinone was excluded. Cardiac power output (CPO) and pulmonary artery pulsatility index (PAPi) are some of the parameters obtained from a PAC that can guide cardiogenic shock therapy and need for mechanical circulatory support (MCS). 
  • Resting CPO = CO x MAP / 451. CPO measures the “pumping power” of the LV and correlates with end-organ perfusion. In the SHOCK registry, CPO <0.53 watts highly correlated with ↑ in-hospital mortality. CPO can also help identify patients ready for LVAD weaning trials and/or explantation. As with most cardiac parameters, the trends matter as much as the absolute values!    
  • PAPi: PA(Systolic) – PA(diastolic) / RA(mean). PAPi was initially developed as a marker severe RV dysfunction in acute inferior wall myocardial infarction and those undergoing LVAD placement. It measures the ratio of the PA pulse pressure to a given preload (RA pressure). It is hard to apply PAPi thresholds across different clinical situations, but we can generally use less than 1-1.75 as a predictor of worsening right-sided function.   

3. How should we approach “typical HF” management in peripartum cardiomyopathy?  

  • Though many aspects of the acute management of peripartum cardiomyopathy are similar to typical heart failure and/or cardiogenic shock management, there are a few key differences.  
  • Remember, ACEI/ARBs/ARNI and spironolactone are contraindicated during pregnancy. ACE-I have demonstrated safety data while breastfeeding, but ARNIs and ARBs have not been studied robustly.   
  • The decision to pursue an ICD in patients with PPCM needs special attention to the natural history of PPCM. Many of these patients will have recovery of LV function in 3-12 months, thus deferring primary prevention ICD while titrating GDMT for ~6 months may be appropriate. There may also be a role for a wearable cardioverter/defibrillators in those with severe LV dysfunction as a bridge to recovery or until an implantable ICD is indicated.  
  • Consider early mechanical support in patients deteriorating on medical therapy (including inotropes) alone.  

4. What are some considerations unique to peripartum cardiomyopathy management?  

  • The prothrombotic state of pregnancy and stasis within a weak ventricle both increase the risk of LV thrombus and a subsequent cardio-embolic event. Therefore, anticoagulation should be considered in PPCM patients with a severely reduced LVEF (EF < 30% by AHA vs ≤35% by ESC) during late pregnancy and 6 to 8 weeks postpartum. LMWH does not cross the placenta and is preferred during pregnancy. Both warfarin and LMWH may be used during lactation, while the direct oral anticoagulants have not been studied either during pregnancy or lactation and should be avoided.  
  • Bromocriptine, a dopamine agonist, reduces prolactin levels (a possible culprit in the development of PPCM). However, data is conflicting on benefit, and thus this therapy should be considered investigative. It is pro-thrombotic, and so it is recommended for patients to be on anticoagulation if bromocriptine is used.  
  • Prior to delivery, multidisciplinary discussions should occur around timing and method of delivery for patients diagnosed with PPCM during pregnancy. During delivery, remember that there are several hemodynamic changes (See Ep #48 – AS Complicating Pregnancy!). These include increased preload from placental auto-transfusion and relief of IVC compression.   
  • The 2018 ESC guidelines suggest avoiding breastfeeding in patients with severe HF because of the high metabolic demands of lactation and the induced prolactin may contribute to worsening HF. However, some small studies suggest in clinically stable patients, breastfeeding does not worsen HF symptoms or LVEF. Considerations regarding possible risk must be counterbalanced with the known benefits of breastfeeding for infants and mothers.   

5. How do we approach long-term management in patients with peripartum cardiomyopathy?  

  • Providers should engage in shared decision-making about future pregnancies in patients with recovered EF (>50%) as they are at higher risk of future recurrence. The 2018 ESC guidelines advise against pregnancy if EF has not recovered to >50-55%.   
  • Contraception counseling should be done early: before discharge or at the time of diagnosis. Patients should avoid estrogen-containing methods especially in the early postpartum period as thromboembolism risk is high.  
  • If a patient plans on another pregnancy, teratogenic HF medications, like ACE/ARB/ARNI and spironolactone, must be stopped prior to stopping contraception. Following cessation of these GDMT medication, follow-up echocardiography to ensure LV functional stability should be performed after at least 3 months off therapy. The patient should be monitored very closely during a subsequent pregnancy for changes in symptoms and followed with serial echocardiograms and NT-proBNP levels during pregnancy and thereafter.   

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

55. Case Report: Suicide LV post-TAVR – The University of Texas at Austin, Dell Medical School

CardioNerds (Amit Goyal & Daniel Ambinder) join UT-Austin cardiology fellows (Priya Kothapali, Sergio Montano, Travis Benzing, and Michael Grzeskowiak) for a speedboat adventure on Lake Travis! They discuss a fascinating case of Suicide LV post-TAVR. Dr. Mark Pirwitz provides the E-CPR and program director Dr. Clay Cauthen 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 figures & mediaCase teachingReferencesProduction team

CardioNerds (Amit Goyal & Daniel Ambinder) join UT-Austin cardiology fellows (Priya Kothapali, Sergio Montano, Travis Benzing, and Michael Grzeskowiak) for a speedboat adventure on Lake Travis! They discuss a fascinating case of Suicide LV post-TAVR. Dr. Mark Pirwitz provides the E-CPR and program director Dr. Clay Cauthen 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 early 70s, with a history of CAD s/p PCI to LAD & RCA with DES six months prior, to presentation, paroxysmal atrial fibrillation s/p ablation, type 2 diabetes mellitus, hypertension, prior TIA, and severe symptomatic AS was admitted for elective TAVR. She underwent successful implantation of a 29mm Medtronic Evolut Pro valve via left common femoral artery access. Post-valve deployment and following protamine administration for heparin reversal, course was complicated by hypotension with PEA arrest requiring CPR for 4 minutes. Intra-op TEE and angiogram showed a well-seated prosthetic valve with trace paravalvular leak and no evidence of acute aortic regurgitation, significant paravalvular leak, pericardial effusion, coronary obstruction, aortic dissection, or access site complications. She was treated for suspected Protamine reaction with high dose steroids & epinephrine. However, she remained hypotensive with MAP in the 50s on high dose Epinephrine, Norepinephrine, and Vasopressin. Hemodynamics by pulmonary artery catheter demonstrated CVP 7, mPA 26, PCWP 18 mmHg and CO/CI 2.8 L/min and 1.3 L/min/m2. Her lactate was elevated at 5.92 mmol/L and EKG demonstrated normal sinus rhythm. Bedside TTE in the ICU showed hyperdynamic LV function with LVEF 70% and near-complete mid to distal cavity obliteration with significant intracavitary gradient. She was diagnosed with post-TAVR suicide LV and managed with aggressive volume resuscitation and rapid wean of Epinephrine/Norepinephrine with improvement in her hemodynamics. She was eventually extubated and discharged on beta-blocker therapy.


Case Media

A. ECG: Normal sinus rhythm, no evidence of AV block, no ST segment elevation or depression.
B. CXR: Pulmonary vascular congestion, no pneumothorax, ETT at level of carina, PAC in appropriate position

Pre-Aortogram
Implantation
Post-Dilation
Post-Aortogram
TEE: Mid-Esophageal Three-Chamber View
TEE: Mid-Esophageal Short Axis View
Abdominal aortography showed no evidence of vascular access site complication or contrast extravasation.
Aortic root angiography showed no evidence of AI, coronary obstruction, or dissection.
TTE: PLAX and A4C


Episode Schematics & Teaching


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

  1. Remind all CardioNerds – how do we define Severe Aortic Stenosis? 
  • By TTE, severe AS is defined as a mean gradient >40 mmHg, a peak velocity >4 m/s, an aortic valve area <1 cm2 and dimensionless index < 0.25. But remember, in HF patients decreased trans-aortic valve flow secondary to LV disease can make the diagnosis challenging! 
  • In HFrEF, patients with severe AS by aortic valve area (AVA) may not meet velocity or gradient criteria in one of four contexts: AVA measurement error, pseudo-severe AS, poor contractile reserve, or true severe Low Flow, Low Gradient AS (LFLG AS). 
  • Similarly, in HFpEF patients, disproportionately low flow or gradient AS may occur due to AVA measurement error or from true severe “paradoxical” LFLG due to decrease stroke volume (stroke volume index <35 mL/m2). 
  • Low gradient AS is a critical subset of patients with generally with a comparatively worse clinical trajectory marked by increased heart failure hospitalizations and mortality compared with high gradient AS. 
  • Enjoy the aortic stenosis series (Ep #1-2) and related figure for a deeper dive! 

2.     This patient received a TAVR for her severe AS. What are the major complications? 

  • Generally there are five relatively important post-TAVR complications – termed the “Big 5” – that contribute to long-term morbidity. These include disabling stroke, acute kidney injury, moderate/severe paravalvular leak, vascular and bleeding complications, and conduction abnormalities with possible need for PPM. 
  • Modern TAVR valves include mechanisms to reduce paravalvular leak (i.e., an outer skirt or a porcine pericardial tissue wrap or cuff), but may lead to more conduction abnormalities 

3.     This case covered Suicide LV. What is the pathophysiology behind this? 

  • Severe AS leads to LV hypertrophy and chronic pressure overload. When the fixed obstruction is relieved, the increased contractility of the LV is “unmasked” and can lead to dynamic LV cavity obstruction or obliteration.  
  • When this compromises cardiac output, these labile hemodynamics are termed “suicide LV”.   

4.     What are risk factors for developing Suicide LV post-AVR? 

  • The major risk factors for Suicide LV with dynamic intracavitary gradient include small LVOT and LV size, hyperdynamic and elevated LVEF, asymmetric septal hypertrophy with an elevated interventricular septal to posterior wall thickness ratio, high valve gradients prior to AVR and small LV wall mass. 

5.     What should be my basic approach to Suicide LV management? 

  • The management of suicide LV is similar to that of Hypertrophic Obstructive Cardiomyopathy (see Ep#3) and related figure for a refresher! 
  • The basics include increasing preload with intravenous fluids, increasing afterload to reduce intra-cavitary gradients (phenylephrine tends to be the vasoactive of choice), avoiding inotropes (e.g., digoxin or beta-agonist infusions) while considering negative inotropes (e.g., beta blockers), and maintaining AV synchrony to ensure adequate LV preload 

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

54. Case Report: Wild type aTTR Cardiac Amyloid – Washington University in St. Louis

CardioNerd (Amit Goyal) join Washington University in St. Louis cardiology fellows (Adam Lick, Manny Rivera Maza, and Sam Lindner) for some amazing local St. Louis craft brews! They discuss a fascinating case of  wild-type aTTR cardiac amyloid. Prior to meeting up with the group, Amit bumps into Rachita Navara: a Wash U #FIT, aspiring electrophysiologist, & a rock star of the band “The Pacemakers” (be sure to check out their performance at the end of the episode!) who shares thoughts about the program and her cutting edge contributions to the field of EP. Dr. Katie Zhang provides the E-CPR and program director Dr. Andy Kates provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase figures & mediaCase teachingReferencesProduction team

CardioNerd (Amit Goyal) join Washington University in St. Louis cardiology fellows (Adam Lick, Manny Rivera Maza, and Sam Lindner) for some amazing local St. Louis craft brews! They discuss a fascinating case of  wild-type aTTR cardiac amyloid. Prior to meeting up with the group, Amit bumps into Rachita Navara: a Wash U #FIT, aspiring electrophysiologist, & a rock star of the band "The Pacemakers" (be sure to check out their performance at the end of the episode!) who shares thoughts about the program and her cutting edge contributions to the field of EP.  Dr. Katie Zhang provides the E-CPR and program director Dr. Andy Kates provides a message for applicants. 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.

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 early 70s, with a history of hypertension, a bicuspid aortic valve, chronic kidney disease and carpal tunnel syndrome presents with two weeks of worsening dyspnea on exertion. At baseline, he is an avid cyclist and noticed he can now only bike ½ mile when before he could bike extended distances. In addition, he noted abdominal swelling and palpitations. Vitals signs showed mild tachycardia, irregularly irregular rhythm, and no clear evidence of volume overload. Labs demonstrated acute on chronic kidney disease, an elevated NT-proBNP and elevated troponin. ECG demonstrated atrial flutter with variable conduction block. TTE demonstrated marked concentric left ventricular hypertrophy with preserved ejection fraction, biatrial enlargement, reduced global longitudinal strain with apical sparing, and bicuspid aortic valve with moderate aortic stenosis. Further diagnostics revealed normal serum kappa/lambda light chains and PYP scan was positive. Patient underwent EMB which demonstrated ATTR amyloid deposition and genetic screening did not show mutations commonly associated with hereditary ATTR. 


Case Media

A. CXR: Stable mild enlargement of the cardiac silhouette; mildly tortuous aorta. Lung fields are clear, no pneumonia, pleural effusions, or pneumothorax.
B. AV continuous wave Doppler
C. Strain Image
D. Tc-99 PYP Scan

TTE 1
TTE 4
TTE 2
TTE 5
TTE 3
TC-99 PYP Scan
Strain video 1
Strain video 2
Strain video 3

Episode Schematics & Teaching


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

  1. Cardiac amyloidosis can have a range of cardiac and extra-cardiac findings as amyloid fibrils can deposit in many different tissues, depending on the amyloid protein involved. The first step in diagnosis is maintaining a high index of suspicion. 
    1. The presence of prominent right-sided HF symptoms, low voltage on ECG, biatrial enlargement, ventricular hypertrophy, conduction disease, and pericardial effusion should prompt a high suspicion for cardiac amyloid. These are typical features of infiltrative or storage restrictive cardiomyopathies. PEARL: ATTR CM can cause asymmetric LVH and is an important HCM phenocopy! 
    2. Remember, amyloidosis is a systemic disease and extra-cardiac findings are common. ATTR amyloid has a predilection for the musculoskeletal system (including bilateral carpal tunnel syndrome, lumbar spinal stenosis, and biceps tendon rupture) and peripheral nerves. Conversely, AL amyloid is widely deposited outside the CNS an can result in protean manifestations, including periorbital ecchymoses from vascular fragility, macroglossia, and visceral organ involvement (including nephrotic syndrome, hepatic infiltration, and gut amyloid). 
  2. When considering cardiac amyloid, there are four primary etiologies: 
    1. Wild-Type (non-hereditary) ATTR amyloidosis is caused by deposition of misfolded transthyretin proteins. It is an under-recognized cause of HFpEF, with incidence increasing with age. 
    2. Hereditary ATTR is caused by a genetic mutation that leads to instability of the transthyretin tetramer. The most common mutation is the Val30Met variant. Specific mutations tend to have templated organ manifestations, natural history, and prognosis.  
    3. AL amyloidosis is caused by deposition of light-chains from a clonal plasma cell dyscrasia. 
    4. AA amyloidosis is a rare form of cardiac amyloid caused by deposition of the acute phase reactant serum amyloid A protein due to a chronic inflammatory process. 
  3. Evaluating for AL amyloid with appropriate lab workup is crucial. “Missing AL amyloid is like missing a STEMI” – Dr. Paul Cremer (Episode #8)! Workup should include SPEP, UPEP, serum/urine immunofixation, and serum kappa/lambda free light chains to maximize sensitivity. Note: SPEP and UPEP alone are insensitive and inadequate! 
  4. Like many cardiac diseases, multimodal diagnostics are key in the diagnosis of cardiac amyloid 
    1. ECG: Low voltage, pseudoinfarct pattern, variable conduction disease, atrial arrhythmias. PEARL: 10% of patients with cardiac amyloid may have high voltages on ECG. LVH on imaging out of proportion to EKG voltages is a red flag! 
    2. TTE: We have discussed typical features of an infiltrative or storage restrictive cardiomyopathy previously , including marked LVH with normal LV volumes and bi-atrial enlargement. Other features include: RV hypertrophy, thickened valve leaflets, thick interatrial septum, speckled appearance of the myocardium, and small pericardial effusion. Bi-atrial enlargement may lead to functional MR and TR as well as atrial arrhythmias. Mitral inflow pattern and tissue doppler will show varying degrees of diastolic dysfunction depending on stage. Systolic function may be borderline and progressively decline in “burned-out” disease. Reduced global longitudinal strain with apical sparing may help differentiate cardiac amyloid from hypertensive heart disease. There is an overlap with aortic stenosis which will frequently manifest as a low flow and/or low gradient phenotype due to reduce stroke volumes. 
    3. Cardiac MRI: There are characteristic findings of early subendocardial late gadolinium enhancement (LGE) and later transmural LGE with abnormal blood-pool-to-myocardial nulling (an MRI technique to accentuate pathology).  
    4. PYP scan: >99% sensitivity for cardiac ATTR amyloid. If monoclonal gammopathy is excluded, the positive predictive value is 100%. 
    5. RHC +/- EMBx: the hemodynamic profile is that of restrictive cardiomyopathy with elevated filling pressures, blunted x descent, and steep y descent. There may be diastolic pressure equalization. If EMBx is pursued, pathology will reveal amyloid protein as a salmon-pink color when congo red stain is applied and when placed under polarized light the amyloid proteins have an apple-green birefringence. Amyloid fibrils are seen with electron microscopic study.  Mass spec for protein identification. 
  5. Cardiac amyloid can be very difficult to treat with typical HF regimens, as beta blockers and ACE-I/ARB can lead to excessive hypotension in the setting of autonomic neuropathy and restrictive hemodynamics.  For TTR amyloid, there are treatment options like tafamidis (a transthyretin protein stabilizer) which can reduce mortality considerably in selected patients.  Investigational RNA-targeted therapies (i.e., patisiran) are increasing the options for treating TTR, but cost remains a major barrier to care. For AL amyloid, a multidisciplinary cardio-oncology team is vital to coordinate chemotherapy and cardiovascular care. Heart transplant +/- bone marrow transplant may be options in advanced stages of disease. The challenges of managing advanced disease highlight the importance of early recognition.  

References


CardioNerds Case Reports: Recruitment Edition Series Production Team