Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan

Following the Norwood procedure for hypoplastic left heart syndrome (HLHS), pulmonary artery distortion and hypoplasia are common and may negatively impact late outcome. The hemi-Fontan procedure (HFP) augments the central pulmonary arteries and establishes a connection between the right atrial/superior vena cava junction and the pulmonary arteries, while excluding the inferior vena cava.

The hospital records of all 114 patients undergoing a HFP for HLHS between August 1993 and April 1998 were reviewed to assess patient, procedural, and morphologic determinations of outcome. The results of cardiac catheterization, Doppler/echocardiography, 12 lead electrocardiograms, hospital and subsequent course, as well as suitability and outcome for the Fontan procedure were analyzed.

Mean age was 5.4 months (range 1.5 to 15 months). Right ventricular function was normal in 95 patients, moderately depressed in 14, and severely depressed in five. Tricuspid regurgitation was absent or mild in 91 patients, moderate in 13, and severe in 10. Concomitant procedures included left superior vena cava to pulmonary artery anastomosis (12), tricuspid valve repair (10), pulmonary artery stent placement (3), coarctation repair (2), and aortic pseudoaneurysm repair (1). Hospital survival was 112/114, 98% (95% confidence interval [CI]: 95% to 100%). There were two late deaths, one noncardiac. Sinus rhythm is present in 105 patients (92%, 95% CI: 87% to 97%). To date, 79 of these patients have undergone the Fontan procedure with 74 survivors (94%, 95% CI: 89% to 99%).

The HFP may be performed with excellent results for HLHS. It effectively augments the central pulmonary arteries while preserving sinus rhythm in the majority. In addition, the HFP facilitates the subsequent Fontan procedure and has significantly improved the overall outcome.