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The disease

Ashort description of the disease 'Peripartum Cardiomyopathy' by Prof. Karen Sliwa and Prof. Denise Hilfiker-Kleiner.

Heart Failure (HF)

Peripartum cardiomyopathy (PPCM) is a rare but potentially life-threatening disorder of unknown aetiology and pathophysiology. Because of its low incidence, geographical variations and heterogeneous presentation, PPCM continues to be incompletely characterized and understood.

Diagnosis of PPCM is based on four primary criteria, as outlined by the workshop recommendations of the National Heart Lung and Blood Institute and the Office of Rare Diseases (Pearson et al. 2000), as follows:

  • the development of the disease in the last month of pregnancy or within 5 months of delivery;
  • absence of an identifiable cause of heart failure;
  • absence of recognizable heart disease prior to the last month of pregnancy;
  • LV systolic dysfunction demonstrated by classical echocardiographic criteria.

It is essential to remove the possibility of it being another cardiocascular disease. At present PPCM is considered a form of dilated cardiomyopathy and is treated according the guidelines for dilated cardiomyopathy with no specific therapy (Sliwa et al. 2006a). Treatment includes pharmacotherapy for heart failure with ACE-inhibitors, beta-blockers, vasodilators and diuretics. (standard therapy for heart failure) (Sliwa et al. 2006a).

Peripartum Cardiomyopathy with left ventricular thrombus. K. Sliwa, Soweto Cardiovascular Research Unit.
Peripartum Cardiomyopathy with left ventricular thrombus. K. Sliwa, Soweto Cardiovascular Research Unit.

Nevertheless the prognosis of affected women is poor with deterioration reported in up to 50% of cases despite optimal medical treatment and a mortality rate as high as 15%.  Only 23% of patients are said to recover fully.  (Brar et al. 2007, Elkayam et al. 2005, Reimold et al. 2001, Sliwa et al. 2000, 2002, 2006a).