Peripartum Cardiomyopathy.

Peripartum cardiomyopathy20,21 is defined as a cardiomyopathy manifesting between the last month of pregnancy and 6 months post partum. The etiology is unclear, but inflammatory factors are highly implicated, and some studies reveal high incidence of lymphocytic inflammation. Peripartum cardiomyopathy is common in Africa but also is manifested in the developed world; it has an excellent long-term natural history if patients survive the initial period (see Fig. 68-4), during which time hemodynamic compromise may be severe.22 Prognosis is worse in the developing world and among indigent patients in the United States.23 It is important to differentiate peripartum cardiomyopathy from a chronic cardiomyopathy exacerbated by the volume load occurring during pregnancy.20 Women who recover are at increased risk of recurrences with subsequent pregnancies; women with full recovery are more likely to tolerate a subsequent pregnancy than are those with residual left ventricular dysfunction Peripartum Cardiomyopathy Peripartum cardiomyopathy is characterized by the onset of left ventricular dysfunction in the last month of pregnancy or within 5 months of delivery, with no pre-existing cardiac dysfunction and no recognized cause of the cardiomyopathy. There is evidence that patients submitted to endomyocardial biopsy early after presentation have a high frequency of myocarditis.29 As most patients with this disorder recover with standard therapy, biopsy is recommended only for those with persistent left ventricular dysfunction and symptoms despite heart failure management

PERIPARTUM CARDIOMYOPATHY. Peripartum cardiomyopathy (PPCM) is a dilated cardiomyopathy documented with echocardiographic left ventricular dysfunction occurring in the last month of pregnancy or within 5 months of delivery. Patients with a prior history of myocardial disease are excluded from this definition, although this makes the diagnosis challenging in many women who have had neither a chest radiograph nor an echocardiogram to confirm that they had previously normal ventricular function. The actual incidence is unknown, but it is probably approximately 1 in 3000. Risk factors include multiparity, being black, older maternal age, and preeclampsia. In a retrospective study of 123 women with PPCM,33 a history of hypertension was obtained in 43% of patients, and twin pregnancies were reported in 13%. Consistent with earlier studies, most patients (75%) presented in the first month post partum, perhaps suggesting an autoimmune cause rather than the pregnancys exacerbating a pre-existing cardiomyopathy. This theory is supported by the documentation of autoantibodies in some cases. The treatment of PPCM is the same as for other forms of congestive heart failure, except that ACE inhibitors and angiotensin receptor blocking agents are contraindicated in pregnancy. Hydralazine, beta blockers, and digoxin have been used and are safe, and diuretics may decrease preload and improve symptoms.34 Intracardiac thrombus and embolism are common, and consideration should be given to anticoagulation with unfractionated heparin in those with an ejection fraction lower than 35%.35 Early fetal delivery may be necessary in women needing hospitalization for heart failure. Myocarditis is a causative factor in some cases, with a reported incidence between 8.8% and 78%. The role of endomyocardial biopsy is controversial, but it probably should be considered and performed by those with considerable experience. Immunosuppressive treatment should be given to those with proven myocarditis. Normalization of ventricular function occurs in about 50% of patients and appears more likely if the ejection fraction is more than 30% at the time of diagnosis.33 Most physicians counsel against a second pregnancy, even if the ventricular function does return to normal, because PPCM will recur in approximately 30% of cases. This may result in significant clinical deterioration and even Death