Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations.

OBJECTIVE: From October 1989 to June 1998, 60 patients have undergone the Norwood procedure (stage I) at our institution. The results of the staged surgical reconstruction and risk factors were analysed. Typical hypoplastic left heart syndrome (HLHS) and complex lesions with aortic hypoplasia were compared with each other.

PATIENTS: Typical HLHS: N = 48, median age 15 days (5-190 days), median weight 3.6 kg (2.6-5.3 kg). Complex lesions (dominant left ventricle): N = 12, median age 59 days (10-884 days), median weight 3.4 kg (2.4-12 kg).

RESULTS: Typical HLHS: The stage-I hospital survival was 73% (35/48). It improved from 60% (95% confidence interval: 49-71%) during 1989-1994 to 91% (95% CI: 81-100%) during 1997-1998. Seven patients were lost late. The right ventricular end diastolic diameter (P = 0.015), shortening fraction (P = 0.027), and the presence of an obstructed pulmonary venous return (P = 0.0032) were significant risk factors. 23 children underwent stage-II operation with four (17%) deaths. All survivors experienced an improvement of their statomotoric development. Stage-III operation was performed in 13 patients with no hospital death. Follow up after stage-III procedure was 7 months to 7 years. 4 year actuarial survival, including hospital mortality and deaths at subsequent stages, improved from 28% (95% CI: 18-38%) during 1989-1994 to 58% (95% CI: 48-68%) during 1994-1997. No patient had signs of myocardial insufficiency. Complex lesions: Stage-I hospital survival was 83% (10/12) with no late death. Stage-II was performed in 8 and stage-III in 6 patients with no death respectively.

CONCLUSION: In typical HLHS the results have improved over time. Both size and function of the right ventricle determined significantly stage-I survival. An early operation prevents the natural progression of pulmonary blood flow and may weaken all three risk factors. Patients with complex lesions seemed to have better chances of surviving the early postoperative period. The multistage reconstructions have become a realistic option for patients with HLHS and similar lesions, regardless of the morphologic subtype or diminutiveness of the aorta, and lead to an acceptable quality of life.