BMJ Case Reports 2012; doi:10.1136/bcr-02-2012-5861
  • Rare disease

The first reported case of non-compacted cardiomyopathy in a preterm infant with Ebstein's anomaly

  1. Aymen Y Khella2
  1. 1Neonatal Intensive Care Unit, Women's Hospital, Hamad Medical Corporation, Doha, Qatar
  2. 2Paediatric Cardiology, Department of Paediatrics, Hamad Medical Corporation, Doha, Qatar
  1. Correspondence to Dr Foaud F Abou Nahia, fabounahia{at}

Non-compaction of the ventricular myocardium is a congenital dysfunction of ventricular morphogenesis caused by the arrest of normal embryogenesis of the ventricles. It is considered as a rare disease and can involve both ventricles but commonly the left ventricle. It is classified into isolated non-compaction of the ventricles and non-compaction associated with other congenital heart defects. Its association with Ebstein's anomaly is very rare. We are reporting a rare case of severe heart failure and complex cardiac anomaly including biventricular non-compaction cardiomyopathy, Ebstein's anomalies and large patent ductus arteriosus with severe persistent pulmonary hypertension in a 31-weeks-old preterm infant. The infant recovered smoothly and continued to be asymptomatic and off medications throughout his 2 years follow-up period. Up to our knowledge, this is the youngest and first patient yet reported to have this disease at this age with good recovery.


  • Competing interests None.

  • Patient consent Obtained.

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