Extreme underdevelopment of the left heart. The ultimate hypoplastic left heart syndrome.
We report a case of extreme underdevelopment of the left heart with a 12-day survival. At postmortem, neither a left atrium nor connecting pulmonary veins were identified. The left ventricle was only identified on histology. This was, effectively, a case of absence of the left heart.
Ho
S Y
SY
Department of Paediatrics, National Heart and Lung Institute, London, U.K.
Sunnegårdh
J
J
Gerlis
L
L
Michaëlsson
M
M
D001013:Aorta, Thoracic / Q000002:abnormalities / Q000473:pathology; D001017:Aortic Coarctation / Q000473:pathology; D004374:Ductus Arteriosus, Patent / Q000473:pathology; D006325:Heart Atria / Q000002:abnormalities / Q000473:pathology; D006329:Heart Conduction System / Q000002:abnormalities / Q000473:pathology; D006330:Heart Defects, Congenital / Q000473:pathology*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities / Q000473:pathology