Cardiovascular ImagingHypoplastic left heart syndrome
a group of
congenital anomalies consisting of varying degrees of hypoplasia of the ascending
aorta,
stenosis or atresia of the aortic and/or mitral valves, and hypoplasia of the left
ventricle. This syndrome is a frequent cause of severe heart failure in the first few days of life. This
lesion is fatal in the first week of life without surgical treatment. The usual surgical treatment is the Norwood procedure, Stage I, consisting of end-to-side anastomosis of the
pulmonary artery to the hypoplastic ascending
aorta with re-establishment of
pulmonary blood flow by placing a tube graft between the right subclavian and right
pulmonary artery. Stage II consists of removal of the systemic to
pulmonary arterial shunt and construction of a
Fontan procedure.
Chest X-ray usually shows severe pulmonary oedema and cardiomegaly with right-sided chamber enlargement. The diagnosis is usually established by echocardiography which displays hypoplasia of the ascending aorta and left ventricular cavity along with severe stenosis or atresia of the aortic and mitral valves. Angiography can display the same features but is infrequently required for initial diagnosis. After palliative surgery evaluation is done with echocardiography, angiography, and MRI, which shows an enlarged mid and distal ascending aorta connected by an end-to-side anastomosis with the main pulmonary artery and a tube graft from the aortic arch or subclavian artery to the right pulmonary artery (Fig.1).
CBH
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ECG gated spin echo image at the base of the heart (a ) shows the hypoplastic aorta (arrow) posterior and rightward to the pulmonary valve. Another image at a more cranial level (b) shows the anastomosis (arrowheads) of the main pulmonary artery to the aorta (Stage I Norwood procedure).
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Hypoplastic left heart syndrome, Fig.1 (a) | | Hypoplastic left heart syndrome, Fig.1 (b) | |