Background: Within the spectrum of what is considered to be hypoplastic left heart syndrome (HLHS) the left ventricle may be of varying size and morphology. We attempted to quantify left ventricular (LV) morphology and function to determine if it affected outcome following the Norwood operation.
Methods and results: Echocardiograms were reviewed from 108 patients who underwent Norwood palliation at our institution over the past 11 years. Total cardiac diameter, thickness of the interventricular septum (IVS), LV area, and LV myocardial area were calculated. Competing risk analysis was performed for each outcome and potential predictors were sought via a logistic multivariable model. Form the Norwood operation up to stage-2 operation, mortality was predicted by IVS thickness (p=0.02), while the absence of ventricular dysfunction was predictive of survival to stage 2 operation (p=0.002). For the complete pathway, form Norwood to Fontan operation, mortality was predicted by IVS thickness (p=0.04), a lower right ventricular fractional area change (p=0.02), and the presence of significant tricuspid regurgitation (p=0.01). Cardiac transplantation during this period was predicted by a lower right ventricular fractional area change (p=0.02) and a larger LV area in diastole (p=0.002).
Conclusions: These results indicate that left ventricular hypertrophy and decreased right ventricular function adversely effect survival following the Norwood operation. Patients with severe LV hypertrophy and decreased right ventricular function might be better served by considering primary transplantation rather than staged palliation.
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