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Children with congenital heart disease: probability of natural survival
1Center of Pediatric Cardiology and Cardiovascular Surgery, University Hospital Motol, Prague, Czechoslovakia.
Insights
Children with congenital heart disease (CHD) have a 67% survival rate through childhood. Survival varies by defect type, with pulmonary stenosis showing high survival, while hypoplastic left heart syndrome has the lowest early survival rates.
Area of Science:
- Pediatric Cardiology
- Public Health
- Medical Statistics
Background:
- Congenital heart disease (CHD) is a significant cause of mortality in children.
- Understanding survival probabilities is crucial for clinical management and public health planning.
Purpose of the Study:
- To determine the survival rates of children born with congenital heart disease (CHD).
- To analyze age-specific mortality and survival probabilities for various CHD types.
Main Methods:
- Utilized death distribution data over 27 years in Central Bohemia for children with CHD.
- Incorporated incidence data of CHD in children born in Bohemia in 1980.
- Calculated survival probabilities based on age distribution of deaths and birth incidence.
Main Results:
- Overall, 67% of children with CHD are expected to survive childhood.
- High survival rates observed for pulmonary stenosis (94%), aortic stenosis (84%), and atrial septal defect (84%).
- Lowest survival rates in the first postnatal week noted for hypoplastic left heart syndrome (39%) and double-outlet right ventricle (50%).
Conclusions:
- Survival rates for children with CHD are significantly influenced by the specific type of defect.
- Early postnatal mortality is a critical period, especially for complex CHD.
- Long-term survival estimates provide valuable data for pediatric cardiac care and research.
Abstract:
The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life--mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septal defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life--mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70-80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%. The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life.