A. Characteristics and Etiology
- ~1:2000-4000 live births
- More common in Older, multiparous, black patients
- Toxemia and Twin Pregnancies have increased risk
- Chronic Hypertension
- ? Diffuse Myocarditis
- Usually within 3 months of delivery
- Thromboembolic stroke and brain hemorrhage incidence also increased peripartum [2]
B. Pathology
- Dilatation of all cardiac chambers
- Pale appearing myocardium
- Ventricular Thrombi
- Disintegration of sarcoplasm and heart muscle fibers
- Myocarditis
- 30-80% of patients with PPCM have evidence of myocarditis
- Endomyocardial Biopsy: Viral, Autoimmune (possible cocksackievirus), ? Toxin
- Molecular marker sof inflammation are found in most patients
C. Symptoms and Signs
- Dyspnea, Orthopnea
- Cough, Hemoptysis
- Fatigue, Chest Pain
- Biventricular Failure (cardiomegaly)
- Tachycardia
- Hypertension (early on)
- Pulmonary Congestion with rales
- Echocardiography critical to evaluating left ventricular (LV) dysfunction
- May also present with symptoms related to thromboembolism
D. Management
- Outcomes
- About 50% improve without therapy
- About 25% have recurrent CHF
- Overall better prognosis than other forms of dilated cardiomyopathy [3]
- Increased risk of LV dysfunction, heart failure, and death with subsequent pregnancies
- Women whose LV function does not return to normal after initial pregnancy are at highest risk for death or severe heart failure with next pregnancy [4]
- Concern for systemic embolization
- Bedrest, Sodium Restriction (1.5-2gm/day)
- Management of the CHF
- Furosemide
- Potassium and magnesium supplements to maintain serum levels
- ß-adrenergic blockers are used early - reduce heart rate, arrhythmias
- Hydralazine ± nitrates for afterload reduction during pregnancy
- ACE inhibitors preferred afterload reduction but are contraindicated during pregnancy
- Digoxin - slow load may be helpful but caution as complications are high
- Intravenous inotropic support as needed
- Heparin is given to reduce risk of thromboembolism
- Pulmonary artery catheterization during and post delivery may be helpful
- Management of Myocarditis
- Prednisone 40-60mg po qd
- Consider azathioprine as a glucocorticoid sparing agent
- The evidence for efficacy of these agents is sparse
References
- Silwa K, Fett J, Elkayam U. 2006. Lancet. 368(9536):687
- Kittner SJ, Stern BJ, Feeser BR, et al. 1996. NEJM. 335(11):768
- Felker GM, Thompson RE, Hare JM, et al. 2000. NEJM. 342(15):1077
- Elkayam U, Tummala PP, Rao K, et al. 2001. NEJM. 344(21):1567