Peripartum Cardiomyopathy
– A Familial Disease

A collaborative project between University of the Witwatersrand, Republic of South Africa & the University of Cape Town, Republic of South Africa.

Research Team

Kemi Tibazarwa
  • Dr. Kemi Tibazarwa (PhD project – Peripartum Cardiomyopathy and idiopathic dilated cardiomyopathy – a genetic disorder?). See poster on diagnosis.
  • Prof. Karen Sliwa
  • Prof. Bongani Mayosi, Groote Schuur Hospital, University of Cape Town
  • Bridget Phooko, research assistant
  • Dr Kemi Tibazarwa is receiving a fellowship from the World Heart Federation for 2008!

Background

Peripartum cardiomyopathy (PCM) is defined as unexplained heart failure associated with left ventricular (LV) dysfunction occurring between the last month of pregnancy and the first 5 months postpartum.

Although some studies have suggested most cases will have a good prognosis, the evidence is questionable, and it remains a poorly understood disease.

Some studies have suggested a familial predisposition from siblings of PCM patients who demonstrate left ventricular dilatation and/or left ventricular dysfunction, leading us to further examine the similarities between PCM and familial dilated cardiomyopathy (DCM).

The study, which began in 2006, comprises components in both Cape Town and Johannesburg. It involves a cross-sectional examination of all cases and their adult relatives, including clinical examination and relevant investigations.

A typical case of peripartum cardiomyopathy is described below.

Peripartum Cardiomyopathy: a typical case

1Fig.1: 2-dimensional transthoracic echocardiography (TTE) with 2-dimensional transthoracic echocardiography (TTE) with long axis parasternal view and M-Mode dilated chambers and poor LV systolic function.

A female patient presented at Chris Hani Baragwannath Hospital with a 5 week history of shortness of breath equivalent to New York Heart Association Functional Class II, orthopnoea associated with cough, bilateral leg swelling, and mild dizziness. The patient presented one week after she underwent spontaneous vaginal delivery; the symptoms thus having begun almost 4 weeks prior to term delivery. On further interrogation, there was positive family history of sudden “unexplained” death and this patient was neither a smoker nor did she consume alcohol.

Clinical examination revealed bilateral pitting pre-tibial oedema, and raised jugulo-venous pressure. The pulse rate was 92 per minute, occasionally irregular, weak, and blood pressure 97/72mmHg. On palpation the apex beat was displaced laterally, and the abdominal exam proved there to be tender hepatomegaly. Positive findings on cardiac auscultation included a loud split second heart sound, and systolic murmur best heard over mitral and tricuspid areas. The arrhythmia was suspected to be one of ventricular extrasystoles. Chest auscultation proved there to be bilateral basal crepitations.

2Fig.2: 2-dimensional TTE in long axis 4-chamber view showing dilated cardiac chambers and functional mitral regurgitation.

Chest x-ray demonstrated 4-chamber cardiomegaly and pulmonary congestion, while her ECG showed right axis deviation, with diffuse T-wave inversion, and non-sustained ventricular ectopics. Relevant positive 2-dimensional echocardiography findings have been illustrated below, including dilatated cardiac chambers, poor systolic left ventricular function, functional mitral regurgitation, tricuspid regurgitation, and a small clear pericardial effusion. The ejection fraction, as estimated from the fractional shortening of 21%, was 42%. Tissue-doppler imaging revealed no evidence of diastolic function.

Further tests permitted the exclusion of other commom causes of dilated cardiomyopathy, as well as important differential diagnoses. The working diagnosis remained that of peripartum cardiomyopathy.

Funding:

  • University of the Witwatersrand
  • University of Cape Town