CTICasesHypoplastic Left Heart Syndrome with a Hypoplastic Ascending Aorta
Imaging findings
In a 6-day-old with hypoplastic left heart syndrome, imaging shows a diminutive, nearly absent left ventricle with a markedly hypertrophied right ventricle, a dilated pulmonary artery, and a large patent ductus arteriosus supplying the descending aorta. The ascending aorta and aortic arch are diffusely hypoplastic, with the great vessels arising from a very small aorta and a widely patent ductus.
Key takeaways
In hypoplastic left heart syndrome, the ascending aorta and arch are diffusely small because there is minimal antegrade left ventricular outflow through them, with the ductus arteriosus instead supplying the systemic circulation retrograde; this diffuse hypoplasia should be distinguished from true coarctation, which by definition refers to a focal narrowing at the ductal/ligamentum insertion site rather than diffuse arch underdevelopment.
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