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The incidence of congenital heart disease

Julien I. E. Hoffman, MD, FACC^*,* and Samuel Kaplan, MD, FACC

^* Department of Pediatrics and the Cardiovascular Research Institute, University of California, San Francisco, California, USA
Ahmanson/UCLA Adult Congenital Heart Disease Center, Departments of Medicine, Pediatrics and Surgery, UCLA School of Medicine, Los Angeles, California, USASupported in part by Program Project Grant HL 25847 from the National Heart, Lung and Blood Institute.

View larger version (14K): [in a new window]   Figure 1 Histogram of the incidence of congenital heart disease per 1,000 live births in 62 reports. The six highest values all came from echocardiographic studies of infants in the newborn nursery.

View larger version (20K): [in a new window]   Figure 2 The incidence of congenital heart disease (CHD) per 1,000 live births is plotted against the final year of the study (left) and against the percentage of ventricular septal defects (VSDs) (right). In the center, the percentage of VSDs is plotted against the final year of the study. The horizontal lines are drawn arbitrarily at an incidence of 10/1,000 live births and 40% of all CHD, and the vertical lines are drawn arbitrarily at 1985 and 40%, respectively. An increasing proportion of the high incidences are beyond 1985 and for series that have over 40% VSDs.

View larger version (15K): [in a new window]   Figure 3 The incidence of ventricular septal defects (VSDs) on the horizontal axis is plotted against the total incidence of all congenital heart disease (CHD) in the left panel, against the incidence of the remainder of CHD (that is, all CHD except for VSD) in the center panel, and against the incidence of all cyanotic CHDs in the right panel (all incidences/million live births).

View larger version (22K): [in a new window]   Figure 4 Modified box plots to show variability of the incidence per million live births of total congenital heart disease (CHD), ventricular septal defects (VSDs), the remainder of CHD (total minus VSD) and all cyanotic CHD. The plots were made with StatView 5 (SAS Institute Inc., Cary, North Carolina). In them, the rectangle shows the upper and lower quartiles and the horizontal line in the middle is the median. The two short horizontal lines connected to the rectangle by thin vertical lines indicate the 90th and 10th percentiles, and data points outside these limits are shown individually.

View larger version (23K): [in a new window]   Figure 5 Modified box plots made in StatView 5; description as for Figure 4. Each panel has a different vertical scale, that on the top panel being 10 times larger than that on the bottom panel. (Top) The four major left-to-right shunts (ventricular septal defect [VSD]; patent ductus arteriosus [PDA]; atrial septal defect [ASD]; atrioventricular septal defect [AVSD]) and the three major obstructive lesions (pulmonic stenosis [PS]; aortic stenosis [AS]; coarctation of the aorta [Coarctation]). (Bottom) Tetralogy of Fallot (Tetralogy); complete transposition of the great arteries (d-TGA); hypoplastic right heart (HRH), which includes tricuspid atresia, Ebstein’s anomaly, and pulmonary atresia with an intact ventricular septum (pul atresia IVS); hypoplastic left heart (HLH); truncus arteriosus (Truncus); double outlet right ventricle (DORV); single ventricle (SV); total anomalous pulmonary venous connection (TAPVC). The three components of the HRH syndrome do not add up to the total, because not all reports included all three.