ABSTRACT
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.
Subject(s)
Abnormalities, Multiple/surgery , Aorta, Thoracic/abnormalities , Aortic Diseases/surgery , Heart Bypass, Right , Hypoplastic Left Heart Syndrome/surgery , Pulmonary Atresia/surgery , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/mortality , Angiography , Aorta, Thoracic/diagnostic imaging , Aortic Diseases/congenital , Aortic Diseases/diagnosis , Aortic Diseases/mortality , Blood Vessel Prosthesis Implantation , Cardiac Surgical Procedures/methods , Cardiac Surgical Procedures/mortality , Child, Preschool , Echocardiography, Doppler , Follow-Up Studies , Heart Ventricles/abnormalities , Heart Ventricles/diagnostic imaging , Hospital Mortality , Humans , Hypoplastic Left Heart Syndrome/diagnosis , Hypoplastic Left Heart Syndrome/mortality , Infant , Infant, Newborn , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Pulmonary Atresia/diagnosis , Pulmonary Atresia/mortality , Retrospective Studies , Risk Factors , Survival Rate , Treatment OutcomeABSTRACT
UNLABELLED: The surgical therapy of newborns with hypoplastic left heart syndrome (HLHS) is still regarded with some distrust. The complete heart conserving palliation includes not only the Norwood operation during the newborn period but also the complete separation of both circuits by the Fontan operation some time later. Our experiences with each surgical step are presented. From 1989 to 1996, 43 infants with anatomical (n = 33) or functional (n = 10) HLHS underwent the Norwood operation. Functional HLHS were: Mitralatresia with double outlet right ventricle and subaortic stenosis (n = 2), atrioventricular septal defect with hypoplastic left ventricle, subaortic stenosis, and aortic coarctation (n = 1), hypoplastic, subaortic right ventricle with restrictive ventricular septal defect and aortic hypoplasia (n = 7). The median age at operation was 15 days (5 to 182 days), mean weight was 3.3 kg (3.0 to 4.9 kg). Total operative mortality was 32% (n = 14) with 16% since 1994 (3/19 patients). Five infants (12%) died 2 weeks to 6 months later, and 2 patients underwent cardiac transplantation. Up to now, 19 out of the 22 long term survivors underwent the bidirectional cavopulmonary anastomosis (Hemi-Fontan) at a median age of 7 months (2 to 14 months). Two infants died (10%). Up to now, 12 out of the remaining 17 survivors received the total cavopulmonary anastomosis after a mean period of 12 months. All children survived, and they are now completely palliated. The longest follow up after the complete Fontan operation is 6 years. CONCLUSION: With increasing experience the results of the Norwood operation improved. The following two-stage Fontan procedure bears only a low risk and leads to good quality of life.
