Abstract
This patient underwent a modified Norwood operation with a right ventricle–pulmonary artery shunt at the age of 7 days following the diagnosis of hypoplastic left heart syndrome with mitral and aortic atresia; this procedure was followed by bidirectional Glenn operation at the age of 5 months. The girl subsequently developed congestive heart failure despite the administration of angiotensin-converting enzyme inhibitors and β-blockers after the initial operation. At the age of 11 months, she required hospitalization and intravenous inotropic support. Coil embolization and surgical ligation of the aortopulmonary collaterals were performed at the age of 1 year but could not control the heart failure. The maximum plasma brain natriuretic peptide level after the second palliative operation was 3823 pg/mL. Cardiac catheterization performed at the age of 1 year and 4 months revealed ventricular dyssynchrony, ventricular ejection fraction of 36.2%, right atrial pressure of 7 mm Hg, superior vena cava pressure of 16 mm Hg, and grade 2 tricuspid regurgitation. QRS duration was 184 ms (Figure 1), and arterial oxygen saturation was 80%. To detect the most delayed and earliest …
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