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Background: Although progress in perioperative management and surgical treatment has improved the survival rate of patients with hypoplastic left heart syndrome (HLHS), the Fontan completion rate remains low and prognosis is poor. Analysis of prognostic factors is challenging because HLHS complicates hemodynamics in patients with typical HLHS without ventricular septal defect and nontypical HLHS. This study aimed to categorize typical HLHS into four groups with combinations of stenosis/atresia of the mitral valve/aortic valve and to investigate survival rates and mortality risk factors to improve Fontan completion and prognosis. Methods: We enrolled 119 patients with HLHS [mitral valve atresia (MA)/aortic valve atresia (AA): 61 patients; MA/aortic valve stenosis (AS): 4; mitral valve stenosis (MS)/AA: 24; and MS/AS: 30)]. After excluding the MA/AS group because of a small number of patients, we investigated the survival rate, number of mortalities, and cause of death in the MA/AA, MS/AA, and MS/AS groups by using prenatal diagnosis, MS/AA, low birth weight, atrioventricular valvular insufficiency, noncardiac complications, restricted foramen ovale, and length of intensive care unit stay following initial surgery as variables. Results: The overall 5-year survival rate for typical HLHS was 58%, which significantly varied by subtype (MS/ AS: 72%; MA/AA: 59%; and MS/AA: 33%; p=0.002). Multivariate analysis identified MS/AA (p=0.002) and positive prenatal diagnosis (p=0.03) as significant prognostic risk factors. Conclusion: In typical HLHS, MS/AA was found to be an adverse prognostic factor. Because prognosis after total cavopulmonary connection was similar to those of other groups, preoperative diagnosis and treatment strategies before right heart bypass must be improved.

Comparison of Childhood Outcome by Mitral Valve and Aortic Valve Subtypes of Typical Hypoplastic Left Heart Syndrome