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EMGuideWire's Radiology Reading Room: Peripartum Cardiomyopathy

S
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Sean M. FoxProfessor of Emergency Medicine & Pediatrics

The Department of Emergency Medicine at Carolinas Medical Center is passionate about education! Dr. Michael Gibbs is a world-renowned clinician and educator and has helped guide numerous young clinicians on the long path of Mastery of Emergency Medical Care. With his oversight, the EMGuideWire team aim to help augment our understanding of emergent imaging. You can follow along with the EMGuideWire.com team as they post these educational, self-guided radiology slides or you can also use this section to learn more in-depth about specific conditions and diseases. This Radiology Reading Room pertains to Peripartum Cardiomyopathy and is brought to you by Kaley El-Arab, MD, Blaire Langa, NP, Claire Lawson, NP, and Ashley Moore Gibbs, DNP. It is has the special guest editors: Richard Musialowski, MD and Laszlo Littmann, MD.

EMGuideWire's Radiology Reading Room: Peripartum Cardiomyopathy

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Peripartum Cardiomyopathy
Kaley El-Arab, MD, Blaire Langa, NP, Claire Lawson, NP,
Ashley Moore Gibbs, DNP
Departments of Emergency Medicine and Internal Medicine
and the Sanger Heart & Vascular Institute
Carolinas Medical Center
Michael A. Gibbs, MD, Lead Editor
Richard Musialowski, MD, Cardiology Editor
Laszlo Littmann, MD, ECG Subject Matter Expert
Carolinas Medical Center Imaging Mastery Project: Cardiology
Visit Our Educational Website
www.EMGuidewire.com
Amyloid
Dilated
• Ischemic
• Peripartum
• Hypertensive
• Iron overload
Genetic
• Hypertrophic
• LV Noncompaction
• ARVC1
Inflammatory (Myocarditis)
• Viral
• Giant cell
• Eosinophilic
• Chagas
• COVID-19
Metabolic
• Diabetic
• Hypothyroid
• Acromegalic
• Cardiac Sarcoid
Stress-Induced (Takotsubo)
Tachycardia-Induced
Toxic
• Alcoholic
• Chemotherapy-induced
• Cocaine-induced
• Other drug related
1Arrhythmogenic Right Ventricular Cardiomyopathy
Classification Of Cardiomyopathies
Embedded References
Davis MB. Peripartum cardiomyopathy. JACC State-Of-The-Art Review. Journal Of The American
College Of Cardiology. 2020. Volume 75, Number 2.
Honinberg M. Peripartum Cardiomyopathy: State-Of-The-Art Review. British Medical Journal.
January 2019. 10.1036/bmj/k5287
Corazón Irizarry O. Comparison of Clinical Characteristics and Outcomes of Peripartum
Cardiomyopathy between African American and Non-African American Women. New England
Journal of Medicine. 2017. Volume 2, Number 11.
Peripartum Cardiomyopathy
Cases From Carolinas Medical Center
32-Year-Old With Peripartum Cardiomyopathy.
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EMGuideWire's Radiology Reading Room: Peripartum Cardiomyopathy

  • 1. Peripartum Cardiomyopathy Kaley El-Arab, MD, Blaire Langa, NP, Claire Lawson, NP, Ashley Moore Gibbs, DNP Departments of Emergency Medicine and Internal Medicine and the Sanger Heart & Vascular Institute Carolinas Medical Center Michael A. Gibbs, MD, Lead Editor Richard Musialowski, MD, Cardiology Editor Laszlo Littmann, MD, ECG Subject Matter Expert Carolinas Medical Center Imaging Mastery Project: Cardiology
  • 2. Visit Our Educational Website www.EMGuidewire.com
  • 3. Amyloid Dilated • Ischemic • Peripartum • Hypertensive • Iron overload Genetic • Hypertrophic • LV Noncompaction • ARVC1 Inflammatory (Myocarditis) • Viral • Giant cell • Eosinophilic • Chagas • COVID-19 Metabolic • Diabetic • Hypothyroid • Acromegalic • Cardiac Sarcoid Stress-Induced (Takotsubo) Tachycardia-Induced Toxic • Alcoholic • Chemotherapy-induced • Cocaine-induced • Other drug related 1Arrhythmogenic Right Ventricular Cardiomyopathy Classification Of Cardiomyopathies
  • 4. Embedded References Davis MB. Peripartum cardiomyopathy. JACC State-Of-The-Art Review. Journal Of The American College Of Cardiology. 2020. Volume 75, Number 2. Honinberg M. Peripartum Cardiomyopathy: State-Of-The-Art Review. British Medical Journal. January 2019. 10.1036/bmj/k5287 Corazón Irizarry O. Comparison of Clinical Characteristics and Outcomes of Peripartum Cardiomyopathy between African American and Non-African American Women. New England Journal of Medicine. 2017. Volume 2, Number 11.
  • 5. Peripartum Cardiomyopathy Cases From Carolinas Medical Center
  • 6. 32-Year-Old With Peripartum Cardiomyopathy.
  • 7. Two Years Later: Worsening Heart Failure Despite Medical Therapy
  • 8. Successfully Implanted HeartMate II™ LVAD As A Bridge To Transplant
  • 9. 18-Year-Old Female Transferred To CMC In Respiratory Failure After An Emergent C- Section. Chest X-Ray 1 Year Ago Hospital Day #1
  • 10. Hospital Day #2: Worsening Respiratory Failure And Shock 18-Year-Old Female Transferred To CMC In Respiratory Failure After An Emergent C- Section.
  • 11. Hospital Day #5: Successfully Implanted HeartMate III™ LVAD As A Bridge To Transplant 18-Year-Old Female Transferred To CMC In Respiratory Failure After An Emergent C- Section.
  • 12. 21-Year-Old Female With A History Of Post-Partum Cardiomyopathy In 2016. Chest X-Ray Before 1st 2016
  • 13. 21-Year-Old Female With A History Of Post-Partum Cardiomyopathy In 2016. Note: Severely Dilated Left Atrium & Ventricle End Systole
  • 14. 21-Year-Old Female With A History Of Post-Partum Cardiomyopathy In 2016. End Diastole End Systole Note: Minimal Chamber Contractility
  • 15. 21-Year-Old Female With A History Of Post-Partum Cardiomyopathy In 2016. AICD Placed In 2016
  • 16. 21-Year-Old Female With A History Of Post-Partum Cardiomyopathy In 2016. 2020: Most Recent CXR
  • 17. 37-Year-Old Develops Acute Respiratory Distress Within Two Hours Of A Cesarian Delivery. Intubated, EF 35% BNP 1250
  • 18. 37-Year-Old With Postpartum Cardiomyopathy. She Improves Clinically After Three Days Of Ventilator Support With Nitroglycerin And Diuretics Therapy. Discharged Home Much Improved On An ACE Inhibitor.
  • 20. Definition 2010 Heart Failure Association of the European Society of Cardiology Working Group: “Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of heart failure is found.” Left ventricular ejection fraction (LVEF) is ≤45%.
  • 21. Epidemiology Global estimates vary by regions: Incidence Per Delivery Japan 1/20,000 United States 1/4,000 Haiti 1/300 Nigeria 1/100
  • 22. EPUB: British Medical Journal. January 2019. 10.1036/bmj/k5287.
  • 23. EPUB: British Medical Journal. January 2019. 10.1036/bmj/k5287.
  • 24. Risk Factors • The incidence is highest in women of African ancestry. • In the United States. the incidence is 3 to 16 times higher in African- American women compared with Caucasian women. Other Risk Factors • Pre-eclampsia • Hypertension • Multi-gestational pregnancies • Older maternal age
  • 28. Pathophysiology • The etiology of PPCM in not fully understood and is likely multifactorial. • Suggested but no proven mechanisms include: • Vascular/hormonal alterations • Pregnancy-related hemodynamic stress • Autoimmune processes • Nutritional deficiencies • Viral myocarditis
  • 29. EPUB: British Medical Journal. January 2019. 10.1036/bmj/k5287.
  • 30. Diagnosis Most women with PPCM are diagnosed after delivery, typically during the first postpartum month. Symptoms1 Signs1  Dyspnea  Chest tightness  Orthopnea  PND2  Fatigue  Tachycardia  Tachypnea  Pulmonary rales  Elevated JVP3  Peripheral edema 1A minority will present with shock, dysrhythmias, or thromboembolic complications 2Paroxysmal nocturnal dyspnea 3Jugular venous pressure
  • 31. Diagnostic Studies During Acute Care Chest X-Ray Pulmonary vascular congestion Serum BNP Markedly elevated1 ECG Non-specific; a normal ECG does not rule out PPCM ECHO LVEF <45% (by definition), may also reveal  Multi-chamber dilatation  Function mitral regurgitation  Intracardiac thrombus2 1Serum BNP not elevated during pregnancy; minimally elevated in pre-eclampsia 2Ensure that the left ventricular apex is well-visualized to rule-out a thrombus
  • 33. Management: Heart Failure Therapies • Treatment of heart failure during pregnancy requires special modifications to ensure fetal safety. • Following delivery most heart failure medications are compatible with lactation
  • 34. Management: Anticoagulation Rationale: • Increased incidence of LV thrombi and systemic thromboembolism • Hypercoagulability of pregnancy Recommendation: • Anticoagulation is recommended in the setting of severely decreased LVEF during late pregnancy and 6 to 8 weeks postpartum • American Heart Association cutoff: when LVEF is <30%
  • 35. Management: Anticoagulation • Warfarin crosses the placenta and is avoided during pregnancy • Low-molecular-weight heparin does not cross the placenta • Both warfarin and low-molecular-weight heparin are considered safe during lactation
  • 40. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;75:207-221. JACC: Executive Summary • PPCM is an idiopathic condition with left ventricular (LV) systolic dysfunction (ejection fraction [EF] <45%) towards the end of pregnancy or following delivery, when no other cause of heart failure (HF) is found. • In the United States, incidence varies from 1 in 1,000 to 1 in 4,000. Risk factors for PPCM include African ancestry, pre-eclampsia and hypertension, multifetal pregnancies, and older maternal age. • Etiology of PPCM is poorly understood. Existing studies suggest that PPCM is a vascular disease, triggered by peripartum hormonal changes with prolactin breakdown products having vasculotoxic effects. Genetic studies have identified pathogenic variants in the titin gene in afflicted patients supporting a two-hit hypothesis. APPENDIX 1
  • 41. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;75:207-221. JACC: Executive Summary • Diagnosis is often delayed, as symptoms overlap those of normal pregnancy. Echocardiography is needed for diagnosis, and intracardiac thrombi should be ruled out when EF is severely reduced due to high risk for thromboembolism. • Predictors of adverse outcomes include EF at the time of diagnosis (EF <30% is associated with worse outcomes), African ancestry, obesity, pre-eclampsia, LV dilatation, right ventricular systolic dysfunction, and LV thrombus. PPCM is associated with higher recovery rates compared to other forms of HF, occurring as late as 2 years. • Management must ensure fetal safety. During pregnancy and lactation, loop diuretics, beta-blockers, digoxin, and hydralazine/nitrates can be used. Renin-angiotensin- aldosterone inhibitors are contraindicated during pregnancy, but during lactation, enalapril, captopril, and spironolactone can be used. APPENDIX 1
  • 42. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;75:207-221. JACC: Executive Summary • Early delivery or termination of pregnancy should be considered in case of hemodynamic instability. Stable patients are delivered vaginally unless there are obstetric reasons for a cesarean section. Postpartum risk of decompensation should be anticipated. • Since prolactin has been implicated in the pathogenesis of PPCM, 2010 European guidelines on PPCM advise against breastfeeding. However, a small study in United States and data from an observational registry suggest that breastfeeding is safe. • Data on risk for arrhythmias are conflicted. It is reasonable to consider wearable defibrillators for women with new-onset PPCM and severe LV dysfunction until recovery or until an implantable cardioverter-defibrillator is indicated. APPENDIX 1
  • 43. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;75:207-221. JACC: Executive Summary • Anticoagulation is endorsed by the American Heart Association if EF is <30% during late pregnancy and up to 8 weeks postpartum. Warfarin is contraindicated during pregnancy, but low molecular weight heparin can be used. Both of these agents are safe during lactation. Novel oral anticoagulants have not been studied during pregnancy and are best avoided. • PPCM is the most common cause of cardiogenic shock during or shortly after pregnancy. Nitroprusside should be avoided due to possible cyanide toxicity, and outcomes may be worse with dobutamine. Temporary mechanical support has been used successfully and should be considered early. Cardiac transplant in this population has been associated with higher rejection rates and lower survival. APPENDIX 1
  • 44. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;75:207-221. JACC: Executive Summary • Contraception should be discussed as soon as feasible. Progesterone-releasing subcutaneous implants or Mirena intrauterine devices are first-line choices and estrogen should be avoided. • If LV dysfunction persists, medications should be continued indefinitely. In those with LV recovery, available observational data support continued therapy indefinitely. If HF medications are stopped, they should be weaned in a stepwise manner with frequent clinical and echocardiographic assessments. • Appropriate counseling should be provided for patients considering additional pregnancies. If LV dysfunction persists, women must be counseled about worse maternal and fetal outcomes. Women who recover to EF >50% still have an increased risk for HF, which may persist after pregnancy. APPENDIX 1
  • 45. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2020;75:207-221. JACC: Executive Summary • During subsequent pregnancies, women with PPCM should be closely followed with serial clinical assessments, echocardiograms, and B-type natriuretic peptide levels from prior to contraception until after delivery. Angiotensin-converting enzyme inhibitors/angiotensin-receptor blockers and aldosterone antagonists should be discontinued prior to conception and restarted after delivery. APPENDIX 1
  • 48. EPUB: British Medical Journal. January 2019. 10.1036/bmj/k5287. APPENDIX 3
  • 49. If You Have Interesting Cases Of Peripartum Cardiomyopathy We Invite You To Send A Set Of Digital PDF Images And A Brief Descriptive Clinical History To: michael.gibbs@atriumhealth.org Your De-Identified Case(s) Will Be Posted On Our Education Website And You And Your Institution Will Be Recognized!