The objective of this study was to ascertain the prevalence and survival rate of children born with a heart defect. A total of 816,569 children live-born between 1980 and 1990 in Bohemia (52,478 km 2 , population 6.314 million, western Czech Republic) were followed up and those with suspected heart disease referred to a center. Echocardiography was done in all of them. All dead children were autopsied. Congenital heart disease was found in 5030 of 816,569 children (6.16 per 1000 live births). The most frequent conditions were ventricular septal defect (41.59%), atrial septal defect (8.67%), aortic (7.77%) and pulmonary (5.81%) stenoses, transposition of the great arteries (5.39%), coarctation of the aorta (5.29%) and persistent ductus arteriosus (5.07%). The first week was survived by 92.46%, the first month by 89.14%, 6 months by 82.42%, and the first year of life by 80.02%, and 77.11% (95% CI 75.91-78.31%) survived to age 15 years. The best prognosis was found in pulmonary stenosis (15-year survival 95.55%), atrial septal defect (92.04%), persistent ductus arteriosus (90.59%), ventricular septal defect (89.37%) and aortic stenosis (88.39%). The worst results were attained in hypoplastic left heart, truncus arteriosus and pulmonary atresia with intact ventricular septum. In conclusion, the prevalence of congenital heart disease was 6.16 per 1000 live births; 77.11% of patients survived to age 15 years.

All 91,823 children born in 1980 in Bohemia (population 6.314 million; area 52,478 square kilometers) were examined at least four times during infancy and at the age of three and four years. All children who died were autopsied and those with heart disease were selected. A total of 779 children (8.223/1000 live births) were suspected by provincial pediatric cardiologists of having a heart disease. All of these were examined at the age of four years at our Center of Pediatric Cardiology. At this age heart disease was proved in 613 alive or deceased children (6.676/1000 live births), congenital cardiac malformations in 589 (6.415/1000 live births), and cardiomyopathies in 24. The most frequent congenital heart defects (CHD) were ventricular septal defect (VSD) (31.41%), atrial septal defect (ASD) (11.37%), aortic stenosis (AS) (7.64%), pulmonary stenosis (PS) (7.13%), coarctation of the aorta (CoA) (5.77%), and transposition of the great arteries (TGA) (5.43%), followed by persistent ductus arteriosus (PDA) (4.75%), atrioventricular septal defect (AVSD) and hypoplastic left heart syndrome (HLHS) (4.07% each), tetralogy of Fallot (TF) (3.56%), and pulmonary atresia (PA) (2.38%). A prevalence of less than 0.1/1000 live births was found for the remaining cardiovascular defects. One hundred fifty-nine (25.9%) patients were admitted to our highly specialized center, 116 (19.7%) catheterized and 85 (13.9%) treated surgically, during the first four years of life. A total of 440 (71.8%) patients survived the fourth year of life. The percentage of deaths was 25.6% among those with congenital heart diseases and 71% with cardiomyopathies. The overall mortality rate was 27% in surgically and 26% in medically treated patients.

Static lung volumes, lung elasticity, and airway patency indices were measured in 47 children operated on for ventricular septal defect (VSD). Open-heart surgery was performed at the age of 0.6-12.0 years (median 4.1 years). In the first group (34 subjects), after primary repair of the VSD, there was an increase in lung recoil pressure at 100% of total lung capacity (TLC) (128% of the predicted value) and a reduction in specific airway conductance (sGaw) (75% of the predicted value). In the second group (13 patients), who had had previous pulmonary artery banding at 0.2-4.0 years (median 0.7 year) there was an increased functional residual capacity/TLC ratio (111% of predicted value), reduced sGaw (69% of predicted value), and reduced maximum expiratory flow at 25% of vital capacity (79% of predicted value). Lung volumes were insignificantly reduced in both groups. The frequency of lung function disturbances was similar in the two groups (71% of patients in the former group and 77% of patients in the latter group). A linear positive correlation between specific airway conductance and the mean pulmonary artery pressure (mPAP) (r = 0.793, p < 0.006) was observed in children with an mPAP <30 mmHg prior to open-heart surgery. A positive correlation between static recoil pressure at full inflation and mPAP (r = 0.545, p < 0.03) was found in children with an mPAP > 30 mmHg. The severity of congenital heart disease prior to surgery and the influence of the timing of the surgical procedures may cause the differences in lung function tests between the groups.

Anastomosis of the superior vena cava and right pulmonary artery (Glenn and Patiino, 1954;Edwards and Bargeron, 1968) is indicated in some patients with congenital heart disease associated with diminished pulmonary blood flow. The procedure represents a major interference with pulmonary haemodynamics. The blood supply to the right lung then comes from the superior vena cava and from the bronchial collaterals. The blood to the left lung comes from the inferior vena cava and from the systemic vascular bed via anastomoses. A part of the venous return from the inferior vena cava passes through interatrial or interventricular communications directly into the systemic circulation without having passed through the lungs. The total pulmonary blood flow after cavopulmonary anastomosis increases. The ratio between the left and right pulmonary blood flow varies according to the magnitude of the intracardiac shunt.Another important feature of the haemodynamics after Glenn's operation is the difference between the character of the left and right pulmonary blood flow. This difference is due to the participation of myocardial contraction on the ejection of blood only to the left lung.Evaluation of all these haemodynamic abnormalities created by Glenn's operation stimulated our present study. We expected to find the changes in the distribution of pulmonary blood flow. Great differences between the right and left lung appeared to be of special interest. It was possible to study the effect of pulsatile or steady blood flow on the distribution of blood in the lungs for the first time in conscious human beings. Finally, we tried to evaluate the contributions of lung scanning for assessing the patency of the anastomosis, as shown

A retrospective review of 11 children, aged 2 months to 15 years, demonstrates the feasibility of surgical treatment for infective endocarditis in childhood. Except for one case of perinatal infection, in all instances the infective endocarditis was a complication of a congenital heart defect. As medical treatment was not successful, surgery was indicated. Debridement of infected tissue and repair of the congenital heart defect was performed. There were no septic complications postoperatively although 8 patients were operated upon during the active stage of infection. One 2-month-old child did not survive excision of an infected tricuspid valve. The follow-up period of 8 years to 5 months (median 39 months) showed a good haemodynamic result (NYHA class I) in the remaining 10 patients. This included 4 patients with prosthetic valves.