An infant with hypoplastic left heart syndrome showed paroxysmal episodes of bradycardia, hypotension, and hypoxemia upon crying after modified Norwood operation. Echocardiography showed decreased right ventricular ejection with grade III tricuspid regurgitation, a markedly enlarged aortic arch, and accelerated blood flow distal to the enlarged aorta. Aortography demonstrated an aneurysmal neo-aorta with an apple-shaped appearance. The pressure measurements revealed intriguing aortic hemodynamics: the diastolic pressure of the ascending aorta was lower than that of the descending aorta (42 mmHg vs. 52 mmHg) despite no systolic pressure gradient. Markedly reduced compliance in the ascending aorta relative to that in the descending aorta, which was suggested by the difference in time constant of aortic pressure decay, may explain this hemodynamics. Impaired coronary circulation caused by lowered diastolic pressure in ascending aorta was indicated by reduced subendocardial viability ratio, and may account for her symptom and lowered ventricular ejection. The patient's condition was indeed significantly improved by surgical correction of the aortic shape. This case highlights the importance of aortic shape and properties after Norwood operation.
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