Abstract
Objectives: To review the experience with Norwood and Damus-Kaye-Stansel (DKS) staged repair in the management of hypoplastic left heart syndrome (HLHS), or functional single ventricle (FSV) with systemic outflow tract obstruction (SOTO).
Methods: A retrospective study was conducted from a single center from January 2001 to September 2007 at the Cardiac Sciences Department, King Abdulaziz Cardiac Center, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia. The cardiac departmental database was reviewed, together with the echocardiographic findings. Demographic data representing age and weight at operation, gender, cardiac anatomy, non-cardiac abnormalities, and operative details were collected.
Results: Twenty-eight patients with a diagnosis of HLHS were included in the study. The mean +/-SD for weight was 3.4 +/- 0.85 kg and 32 +/- 37 days for age. All infants at our institution who underwent a Norwood or DKS surgery for HLHS, or other forms of FSV with SOTO were included. All included patients underwent first stage palliation consisting of either a classical Norwood procedure in 6/23 (21%) patients, or a modified Norwood with right ventricle to pulmonary artery (RV-PA) conduit in 22/28 (79%) patients. After first stage palliation, 23 patients (82%) survived, and all but one underwent second stage palliation with bidirectional cavopulmonary anastomosis (BCPA). The survival rate after second stage repair was 91%. Subsequently, 4 patients completed a third stage Fontan with 100% survival. All deceased patients had HLHS.
Conclusion: The Norwood procedure is applied to a heterogeneous group of patients with variable outcomes in certain subgroups. Patients with HLHS palliated with the Norwood procedure are at a greater risk for morbidity and mortality compared to those with other forms of FSV with SOTO.