Clinical and immunologic characteristics in peripartum cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a rare disorder of dilated cardiomyopathy and left ventricular dysfunction occurring in the last month of pregnancy or within 5 months postpartum. Outcome of PPCM is highly variable, comprising clinical improvement and rapid deterioration unresponsive to medical treatment requiring heart transplantation or even death. In this study, we report the clinicopathologic findings of 10 patients with PPCM who were retrospectively identified in our cardiomyopathy registry. During a follow-up of 69+/-27 months, no patient died or required orthotopic heart transplantation. Left ventricular ejection fraction was 38+/-7% at the time of diagnosis and 53+/-7% during follow-up. While all patients had sinus rhythm at the time of diagnosis, three patients presented with left bundle branch block. We found no evidence of viral infection in endomyocardial biopsy samples of seven patients by PCR. Histopathologic findings revealed the presence borderline myocarditis in two of seven patients (29%). Circulating autoantibodies to cardiac tissue of any kind were observed in all patients. In conclusion, in our retrospective observational study, no patient diagnosed with PPCM died or received orthotopic heart transplantation. Improvement of left ventricular ejection fraction was present in eight patients (80%), while LV dysfunction persisted in four patients. Our findings support the hypothesis of an underlying autoimmune pathomechanism in this rare disease.