Hypoplastic left heart syndrome

Case contributed by Arkadi Tadevosyan
Diagnosis certain

Presentation

Cyanosis since birth. Diagnosis of complex congenital heart disease was made during pregnancy. Parents decided not to terminate pregnancy.

Patient Data

Age: 10 days
Gender: Female

Typical features of hypoplastic left heart syndrome:

  • underdeveloped left ventricle
  • mitral valve stenosis
  • aortic valve atresia
  • hypoplastic ascending aorta

There is compensatory dilatation of the right cardiac chambers with possible signs of endocardial fibroelastosis. An atrial septal defect and patent ductus arteriosus are demonstrated. 

Case Discussion

The patient underwent Norwood procedure (constructing a neo-aorta by side-to-side anastomosis of the main pulmonary artery and ascending aorta) but unfortunately died intraoperatively.

 

Special thanks to Dr. Naina Hakobyan, "AMC" Hospital, Armenia Department of Radiology, for contributing this case.

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