Peripartum Cardiomyopathy - Summary
1. Definition
• Towards the end of pregnancy to 5 months postpartum
• Usually LVEF <45% with or without LV dilation
• Idiopathic LV dysfunction → exclude other causes*
2. Differential Diagnosis
• Pre-existing cardiomyopathy (e.g., familial or dilated)
• Valvular heart disease
• Congenital heart disease
• Hypertensive heart disease
• Myocardial infarction
• Stress cardiomyopathy
• Pulmonary embolus
3. Etiology
• Actual etiology remains unknown
• Final pathway likely an imbalance of angiogenic factors + oxidative stress
- Proposed Mechanism: Dysregulation of VEGF (Pro-Angiogenic) through ↑ sFLT1 levels (levels ↑ in pre-eclampsia)
- Proposed Mechanism: Altered prolactin processing with ↑ cleavage into a pro-angiogenic fragment
• Other: Myocarditis? Genetic predisposition (TTN gene) ? Hemodynamic stressors of pregnancy
4. Risk Factors and Worse Prognostic Markers
• Risk Factors: African ancestry, pre-eclampsia, hypertension, multiple pregnancy, maternal age > 30 years, cocaine use
• Worse Prognosis: LVEF < 30%, LVEDd > 6.0 cm, LV thrombus, RV systolic dysfunction, Obesity, African ancestry, LGE on MRI
5. Clinical
• Under-recognized: sx overlap with normal pregnancy
• May have typical HF sx: dyspnea on exertion, orthopnea, PND, LE edema
• Minority of Pts: cardiogenic shock and severe arrhythmias
6. Management during Pregnancy
• Avoid ARB/ACE-I/ARNI/MRA
• Avoid Warfarin and DOAC
• Planning for delivery mode and timing with Cardio-OB team
7. Management during Delivery
• Stable patients typically deliver vaginally
• Account for changes in hemodynamics (e.g., placental auto-transfusion and relief of IVC compression ↑ preload)
• A multi-disciplinary team is critical!
8. Management during Postpartum Period
• Breast-feeding: no consensus on risk vs. benefit. - Some studies show no ↓ LV function. Avoid ARBs
• ICD: Many patients will recover LVEF. Consider waiting ~6 months before 1° prevention. Possible role for wearable defibrillator as a "bridge to recovery"
• Contraception counseling should be done on diagnosis or discharge. Avoid estrogen products early post-partum
9. Other Considerations
• Thromboembolic complications are relatively common. In patients with LVEF (ESC), suggest prophylactic anticoagulation up to 8 weeks postpartum
• Consider early mechanical support for patients clinically deteriorating on medical therapy, including inotropes
• Bromocriptine, a dopamine agonist, prevents the release of prolactin. It is an investigative therapy in PPCM. If started, patients should be on a/c.
• If no LVEF recovery (e.g., <50%), ESC guidelines recommend against future pregnancy. Risk of recurrence remains even if recovery.
• During a future pregnancy, teratogenic GDMT meds (e.g., ACE/ARB) need to be stopped. Serial TTE and close follow-up with Cardio-OB team needed!
- Cardionerds - Karan Desai MD, MPH @karanpdesai
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Cardiomyopathies
Manifestations of cardiomyopathy (CM) range from microscopic alterations in cardiac myocytes to fulminant Heart Failure with inadequate perfusion, fluid accumulation, & cardiac rhythm dysfunction. Divided into 2 major groups based on predominant organ involvement.
Primary Cardiomyopathies:
• Hypertrophic Cardiomyopathy
• Arrhythmogenic Right Ventricular Cardiomyopathy
• LV Noncompaction
• Channelopathies
• Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)
• Short QT Syndrome
• Dilated Cardiomyopathy (DCM)
• Restrictive Cardiomyopathy
• Myocarditis
• Stress (Takotsubo) Cardiomyopathy
• Peripartum/Postpartum
Secondqry cardiomyopathies - Pathological myocardial involvement as part of a large number & variety of generalized systemic (multiorgan) disorders:
• Infiltrative (Amyloidosis, Gaucher, Hurler's & Hunter's).
• Storage (Hemochromatosis, Fabry's disease, Glycogen storage)
• Toxicity (Drugs. heavy metals. chemical agents)
• Endomyocardial (Fibrosis. Hypereosinophilic syndrome)
• Inflammatory (granulomatous) - Sarcoidosis
• Endocrine (DM, Hyper/hypothyroidism, ↑PTH, Pheochromocytoma. Acromegaly)
• Neuromuscular/neurological (Friedreich's ataxia. Duchenne-Becker dystrophy, Neurofibromatosis, TS)
• Nutritional deficiencies (Beriberi. pellagra. scurvy. selenium)
• Autoimmune/collagen (SLE, RA. Dermatomyositis, Scleroderma, Polyarteritis nodosa)
• Electrolyte imbalance
• Cancer Therapy (Anthracyclines. Cyclophosphamide. Radiation)
Satyendra Dhar, MD @DharSaty
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Causes of Myocarditis
Infectious Etiologies:
• Viral agents: Adenoviruses, Enteroviruses (coxsackievirus), Herpesviruses (human herpesvirus 6, Epstein—Barr virus), Hepatitis C virus, HIV, Influenza A, Parvovirus B19
• Parasitic agents: Larva migrans, Schistosomiasis
• Bacterial agents: Borrelia species, Mycobacterium species, Mycoplasma pneumoniae, Streptococcal species, Treponema pallidum
• Fungal agents: Aspergillus species, Candida species, Coccidioides species, Cryptococcus species, Histoplasma species
• Protozoal agents: Trypanosoma cruzi (Chagas disease)
Non-Infectious Etiologies:
• Toxins: Anthracyclines, Cocaine, Interleukin-2
• Hypersensitivity: Cephalosporins, Digoxin, Diuretics, Dobutamine, Sulfonamides, Tricyclic antidepressants
• Immunological Syndromes: Churg-Strauss syndrome, Diabetes mellitus, Inflammatory bowel disease, Giant cell myocarditis, Granulomatosis with polyangiitis (Wegener granulomatosis), Sarcoidosis, Systemic lupus erythematosus, Takayasu arteritis, Thyrotoxicosis
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Myocarditis
Diagnosis
• Endomyocardial biopsy (gold standard, rarely performed)
• Clinical presentation
• Cardiovascular MRI
Clinical
• Excessive fatigue, exercise intolerance
• Chest pain, pericarditis
• Unexplained sinus tachycardia
• S3, S4 or summation gallop
• Abnormal ECG, echocardiogram
• Dysrhythmia, heart block
• Heart failure
• Elevated troponin
• Cardiogenic shock, sudden cardiac death
Management
• Supportive
• Heart failure therapy as needed
• Antidysrhythmics as needed
• Antlviral (lymphocytic myocarditis)
• Immunosuppressants (chronic myocarditis)
• IVIG (Giant cell, eosinophilic)
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Myocarditis
5 Principles of
Myocarditis:
1. Build clinical suspicion
2. Decide on EMBx
3. Manage acute cardiac injury
4. Manage chronic cardiac sequelae
5. Treat myocarditis
Clinical Presentation:
- Fever, Rash, Myalgias, Fatigue, Resp/Gl sx
Fulminant:
- Acute HF, Cardiogenic shock, Arrythmias, Pericarditis, Tamponade
EKG Findings:
- AV block, Aberrancy, VT/VF, Myocardial injury (ST elevation), Low voltage
Treatment:
- Lymphocytic Myocarditis: role of steroids +/- azathioprine unclear
- Giant Cell Myocarditis: steroids, calcineurin inhibitor, anti-metabolite
- Immune Checkpoint Inhibitors: cessation of inciting therapy, steroids, ARB/sacubitril-valsartan, PPM placement
- Necrotizing Eosinophilic Myocarditis: identify precipitating agent, steroids, AC
CardioNerds Podcast @cardionerds
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