Time Trends in Simple Congenital Heart Disease Over 39 Years: A Danish Nationwide Study

Abstract

Background We describe calendar time trends of patients with simple congenital heart disease. Methods and Results Using the nationwide Danish registries, we identified individuals diagnosed with isolated ventricular septal defect, atrial septal defect, patent ductus arteriosus, or pulmonary stenosis during 1977 to 2015, who were alive at 5 years of age. We reported incidence per 1 000 000 person-years with 95% CIs, 1-year invasive cardiac procedure probability and age at time of diagnosis stratified by diagnosis age (children ≤18 years, adults >18 years), and 1-year all-cause mortality stratified by diagnosis age groups (5-30, 30-60, 60+ years). We identified 15 900 individuals with simple congenital heart disease (ventricular septal defect, 35.2%; atrial septal defect, 35.0%; patent ductus arteriosus, 25.2%; pulmonary stenosis, 4.6%), of which 75.7% were children. From 1977 to 1986 and 2007 to 2015, the incidence rates increased for atrial septal defect in adults (8.8 [95% CI, 7.1-10.5] to 31.8 [95% CI, 29.2-34.5]) and in children (26.6 [95% CI, 20.9-32.3] to 150.8 [95% CI, 126.5-175.0]). An increase was only observed in children for ventricular septal defect (72.1 [95% CI, 60.3-83.9] to 115.4 [95% CI, 109.1-121.6]), patent ductus arteriosus (49.2 [95% CI, 39.8-58.5] to 102.2 [95% CI, 86.7-117.6]) and pulmonary stenosis (5.7 [95% CI, 3.0-8.3] to 21.5 [95% CI, 17.2-25.7]) while the incidence rates remained unchanged for adults. From 1977-1986 to 2007-2015, 1-year mortality decreased for all age groups (>60 years, 30.1%-9.6%; 30-60 years, 9.5%-1.0%; 5-30 years, 1.9%-0.0%), and 1-year procedure probability decreased for children (13.8%-6.6%) but increased for adults (13.3%-29.6%) were observed. Conclusions Increasing incidence and treatment and decreasing mortality among individuals with simple congenital heart disease point toward an aging and growing population. Broader screening methods for asymptomatic congenital heart disease are needed to initiate timely treatment and follow-up.

Keywords: cardiovascular intervention; congenital heart disease; mortality; temporal trends.

Figure 1. Flowchart depicting study design and exclusion criteria.

ASD indicates atrial septal defect; CHD, congenital heart disease; PDA, patent ductus arteriosus; PS, pulmonary stenosis; and VSD, ventricular septal defect.

Figure 2. Incidence rate and age at time of diagnosis for individuals diagnosed before 18 years of age in Denmark between 1977 and 2015.

(A) Crude incidence rate per 1 million‐person years. (B) Distribution of age at time of diagnosis shown as boxplots.

Figure 3. Incidence rate and age at time of diagnosis for individuals diagnosed after 18 years of age in Denmark between 1977 and 2015.

(A) Crude incidence rate per 1 million person‐years. (B) Distribution of age at time of diagnosis shown as boxplots. ASD indicates atrial septal defect.

Figure 4. All‐cause mortality risk within 1 year after time of diagnosis, stratified by age at time of diagnosis.

CHD indicates congenital heart disease.

Figure 5. Probability of surgery within 1 year after time of diagnosis, stratified by age at time of diagnosis.

CHD indicates congenital heart disease.