Decades ago, people with congenital heart disease had lower odds of survival beyond childhood, so the idea prevailed that surviving to adulthood meant patients did not need ongoing treatment.
Congenital heart defects occur in approximately 1% of all live births, or about 40,000 infants, each year in the US, amounting to one child born with congenital heart disease every 15 minutes. About half of these cases are ventricular septal defects.
Among infants born with congenital heart disease, about 1 in 4 have a severe defect requiring surgery or other interventions in the first year. The neonatal period and first year of life are critical times for survival. Between 1999 and 2017, about 1 in every 814 deaths occurring in the US were attributable to heart defects, and 48% of these occurred in children younger than 1 year.
With continued improvements, we now know that 97% of patients with congenital heart disease will reach adulthood, and for those alive at age 18, 75% may live into their sixties. However, complex defects like tricuspid atresia or pulmonary atresia with intact ventricular septum are associated with higher infant mortality and shorter average lifespans.
The recommendations also include several new and updated sections for managing adults with congenital heart disease.
Specifically, the guidelines advocate an individualized approach for authorizing physical activity, which represents a shift away from more blanket restrictions.
The guideline authors noted that many patients with adult congenital heart disease “tolerate the hemodynamic changes of pregnancy without major adverse events,” but emphasize that problems can arise. The involvement of clinicians with expertise in adult congenital heart disease, maternal-fetal medicine, and anesthesiology is paramount.
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