Successful surgical treatment of a neonate with prenatal diagnosis of severe Ebstein's anomaly.

We present a case of a patient with a severe form of Ebstein's anomaly diagnosed in a 36th week of gestation through prenatal echocardiographic evaluation. The emergency surgery consisted of closure of the tricuspid valve and creation of a residual atriventricular communication, enlargement of interatrial communication, closure of the pulmonary artery, and construction of an aortopulmonary shunt. Subsequently, the patient underwent the hemiFontan and Fontan operation. We conclude that prenatal echocardiographic diagnosis is crucial for the survival of children with a severe form of Ebstein's anomaly, which is a neonatal emergency and requires prompt decision mating to initiate surgical management.

Hypoplastic left heart syndrome with an anomalous origin of the left coronary artery.

Abnormal origin of the coronary artery in children with hypoplastic left heart syndrome is an extremely rare defect. We describe a newborn with concomitant hypoplastic left heart syndrome and abnormal origin of the left coronary artery arising from the right pulmonary artery. A Norwood procedure and direct reimplantation of the left coronary artery to the ascending aorta was performed.

[Results of surgical treatment of congenital heart defects in infants below 2500 grams]. / Wyniki operacyjnego leczenia wrodzonych wad serca u dzieci z masa ciala ponizej 2500 g.

We present a review of our recent experience of operating on infants below 2500 g suffering from congenital heart disease. A retrospective review was performed in 73 children who had undergone cardiac operations at our institution from 1990 to 1999. There were 43 (59%) females and 30 (41%) males; the mean age at operation was 28 days (range 4 to 92). The mean gestational age was 34.7 weeks (range 24 to 41), mean birth weight--1899 g (range 700 to 2450) and mean weight at operation--2013 g (range 640 to 2500). Cardiac diagnoses included patent ductus arteriosus (PDA) (N = 21, 28.7%), ventricular septal defect (VSD) (N = 9, 12.3%), transposition of great arteries (TGA) (N = 10, 13.7%), tetralogy of Fallot (TOF) (N = 7, 9.6%), double-outlet right ventricle (DORV) (N = 4, 5.5%), truncus arteriosus communis (TAC) (N = 4, 5.5%), hypoplastic left heart syndrome (HLHS) (N = 4, 5.5%), coarctation of aorta (CoAo) (N = 6, 8.2%), total anomalous pulmonary venous return (TAPVR) (N = 2, 2.7%), aortic stenosis (AoVS) (N = 3, 4.1%), interrupted aortic arch (IAA) (N = 1, 1.4%), pulmonary atresia (PA) (N = 1, 1.4%) and common atrioventricular canal (CAVC) (N = 1, 1.4%). Hospital mortality was 16.4%. There were 6 early deaths (8.2%) and 6 late deaths (8.2%). A higher mortality rate was noted in premature children and in children who had undergone palliative procedures. At a 1-116-month follow-up (mean 39 months), of 57 (93.4%) patients, 46 (80.7%) were in NYHA class I and 11 (19.3%) in NYHA class II. In conclusion the authors believe that early intervention can be performed in infants below 2500 g, and the associated mortality and morbidity rates are low.

Factors influencing early outcome of Norwood procedure for hypoplastic left heart syndrome.

OBJECTIVE: [corrected] The operative outcome of the Norwood procedure for hypoplastic left heart syndrome is still not satisfactory. Conflicting reports concern factors associated with early Norwood procedure mortality and the reasons for the instability after surgery are not sufficiently understood. The purpose of this study was to determine some perioperative factors influencing early (30 days) outcome. METHODS: We retrospectively analyzed a group of 30 consecutive children with hypoplastic left heart syndrome (aged 5-39 days) who underwent Norwood procedure in 1997 and 1998. The following factors were considered and statistically analyzed: operative age, birth weight, operative weight, serum level of bilirubin, aminotransferases, creatinine, urea, arterial blood gasses, anatomic subgroups, ascending aorta and arch size, systemic to pulmonary modified right Blalock-Taussig shunt size, cardiopulmonary circulatory arrest time, cardiopulmonary bypass time, and delayed chest closure. Eighteen patients underwent hemi-Fontan procedure with one late death and the modified Fontan operation was performed in 16 of them (one late death). RESULTS: The early mortality was 37%. Seven deaths (64%) occurred during the first 24 h after operation. There was a significant difference between survivals and non-survivals in: birth weight (P=0.047), operative age (P=0.016), preoperative serum level of bilirubin (P=0.044), and cardiopulmonary circulatory arrest time (P=0.006). The other assessed factors were not found to be predictors of early mortality. All 16 survivals followed up are in New York Heart Association class I or II. CONCLUSIONS: Anatomic and functional status of the patient, as well as procedural factors are related to Norwood operation early mortality. High mortality in hypoplastic left heart syndrome after stage I surgery indicates the necessity of assessing all factors which may determine further improvement in the outcome.

[Operative outcome in children with total anomalous pulmonary venous connection]. / Wyniki operacyjnego leczenia dzieci z calkowitym nieprawidlowym polaczeniem zyl plucnych.

The authors present their experience in surgical treatment of children with total anomalous pulmonary venous connection. A retrospective analysis was carried out in 42 patients operated on in the years 1979-1999. The group included 30 boys aged 7 days to 7 years (mean 170 +/- 480 days) and 12 girls aged 14 days to 7 months (mean 86 +/- 80 days). Diagnostic studies allowed to determine that 24 patients (57%) had supracardiac type (type I), nine (21%)--cardiac (type II), five (12%)--infracardiac (type III) and four (10%)--mixed (type IV). The diagnosis was based on clinical examinations and echocardiography, supplemented in 17 patients (40%) by hemodynamic studies and angiocardiography. Preoperative pulmonary hypertension was noted in 34 children (81%), including 15 patients (36%) diagnosed by hemodynamic studies and 19 children (45%) by echocardiography. All children were operated on in deep hypothermia with circulatory arrest. In the early postoperative period (30 days), seven children (17%) died, while four more (9.5%) died in the latter period. The former were characterized by significantly lower birth weight values (p = 0.023). The total mortality rate was higher in children with type II and III anomalies. No statistically significant association was found between preoperative pulmonary hypertension and early mortality. Thirty-one children (74%) remain in late follow-up. All of them are in NYHA (New York Heart Association) class I and develop normally. Total anomalous pulmonary venous connection is a rare congenital anomaly that can be fully repaired with improving early and late results.

Results of surgical treatment of congenital heart defects in children with Down's syndrome.

We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ostium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 patients total correction was performed. The total death rate was 6%. Death in the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 months) are in NYHA class I or II. There were no reoperations. The postoperative course was complicated by pulmonary infections in 38% of patients, which converted to generalized infection in 10% and was the cause of death in 8% of patients. These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to eliminate problems connected with congestive heart failure and pulmonary hypertension.