[Anatomic and functional hypoplastic left heart syndrome and its surgical Norwood and Fontan treatment]. / Das anatomische und funktionelle hypoplastische Linksherz-Syndrom und seine chirurgische Behandlung nach Norwood und Fontan.

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.

Early rhythm disorders after arterial switch and intraatrial repair in infants with simple transposition of the great arteries.

Between 1986 and 1990 68 infants with transposition of the great arteries (TGA) have been repaired within their first six months of life. From the 51 simple TGA, forming the study group, 20 underwent intraatrial repair according to Mustard and Brom (group I) and 31 received the arterial switch procedure (group II). We compared the observed rhythm disorders after both surgical methods. There were no deaths in group I and 3 deaths in group II. Group I: 7 cases (35%) had early postoperative arrhythmias, 6 patients received transient therapy. There were 3 cases of supraventricular tachycardia (SVT), 2 cases of frequent supraventricular premature beats (SVPB), 1 infant with temporary AV junctional rhythm and 1 with temporary total AV block. Both needed external pacemaker support for maximal two days. Group II: 14 cases (50%) presented rhythm disorders, 13 patients received transient therapy. There were 4 SVT and 3 SVPB. The time of occurrence was between the first and the sixth postoperative day. 2 cases of temporary AV junctional rhythm and 2 infants with transient total AV block needed external pacemaker support for maximal two days. 3 cases with relative sinus bradycardia (less than 130 beats/min) improved clinically with temporary external pacemaker support for several hours. Finally all patients of both groups were in sinus rhythm and after two months all antiarrhythmic drugs could be stopped. There have not been any ventricular arrhythmias in the early postoperative period. During the whole follow up (mean 28 months, range 4-60 m) no arrhythmias reappeared with two (4%) exceptions.

Primary repair of complete atrioventricular septal defect in infancy.

Forty infants with complete atrioventricular septal defect have undergone primary repair within their 1st year of life. The mean age at time of surgery was 4 months (range 1-12 months) and the mean weight 4.2 kg (range 2.9-7.0 kg). Either the one- or the two-patch method was used. Four patients died (10%). There were no late deaths. Three patients needed reoperation due to mitral valve insufficiency. Postoperative complications were: 1 total atrioventricular block in an infant with an absent ventricular septum 3 weeks after surgery, 9 complete right bundle branch blocks, 3 small residual ventricular septal defects. Mean follow-up of the 36 survivors is 22 months (3-46 months). Thirty (83%) are in NYHA functional class I, 4 (11%) in NYHA class II and 2 (6%) in NYHA class III. No atrioventricular valve regurgitation is detectable by the color Doppler technique in 19 (53%) patients. Mitral incompetence is mild in 13 cases (36%) and moderate in 4 (11%). In ten recatheterized patients the average systolic pulmonary to systemic artery pressure ratio dropped from 1.0 to 0.42 and the average pulmonary arteriolar resistance was reduced from 5.5 to 3.9 U/m2. All but one patient gained weight and underwent adequate statomotoric development. Primary repair should be performed at the age of 5-6 months or even earlier, if pulmonary arteriolar resistance exceeds 5 U/m2.