Massively Hypertrophied Right-Sided Heart with Hypoplastic Left-Sided Heart in a Neonate (A Rare Type of Hypertrophic Cardiomyopathy)

Am J Cardiol. 2023 Mar 1:190:126-130. doi: 10.1016/j.amjcard.2022.11.024. Epub 2022 Dec 16.

Abstract

Described herein is a newborn boy with likely right-sided hypertrophic cardiomyopathy (HC), who survived for 18 hours after birth. At necropsy, he had a severely thickened right ventricular free wall, ventricular septum, right atrial wall and a hypoplastic left-sided heart. There was a large fossa ovale type atrial septal defect and also a patent ductus arteriosus. During peak systole, the right ventricular outflow tract was obstructed, and its contents were pushed into the thick-walled right atrium, then rapidly into the thin-walled left atrium via a large fossa ovale atrial septal defect. The contents were then pushed into the thin-walled left ventricle and finally into the small ascending aorta and into the lungs via a large patent ductus arteriosus. We were unable to find a similar published case.

Keywords: Hypertrophic cardiomyopathy; atrial septal defect; hypoplastic left-sided heart; patent ductus arteriosus.

Publication types

  • Case Reports

MeSH terms

  • Cardiomyopathy, Hypertrophic* / complications
  • Cardiomyopathy, Hypertrophic* / diagnosis
  • Ductus Arteriosus, Patent*
  • Heart Atria / diagnostic imaging
  • Heart Septal Defects, Atrial*
  • Heart Ventricles
  • Humans
  • Infant, Newborn
  • Male