Congenital Heart Disease Patients Have Much Higher PH Rate, Study Shows

People with congenital heart disease are seven times more likely to develop pulmonary hypertension than the general population, a long-term Danish study indicates.

In addition, congenital heart disease patients with PH are four times more likely to die than those without PH, the researchers reported.

Their article, “Incidence and Mortality of Adults With Pulmonary Hypertension and Congenital Heart Disease,” appeared in the American Journal of Cardiology.

Congenital heart disease affects nearly 1 percent of newborns, making it one of the most common birth defects. Advances in diagnosis and treatment have led to those with the disease living longer.

One of the complications of congenital heart disease is PH. It can lead to a person having less function, an increased risk of an irregular heartbeat, heart and kidney failure, liver problems, and premature death.

Few studies have been done on PH in those who are living longer with congenital heart disease. The ones that have been done have focused on pulmonary arterial hypertension and patients with shunts — surgical connections between a trunk artery and a lung artery that can help ease patients’ symptoms.

The Danish study was the first to evaluate the incidence of PH in congenital heart disease patients, and their death rates, in an entire country.

Researchers used medical registries to identify 14,860 Danes with a congenital heart disease between 1963 and 1974 and between 1977 and 2012. They dropped patients who were under 18 years old from the study, then examined the patients’ PH rates and death rates

A key finding was that 7.2 percent of congenital heart disease patients developed PH by the age of 70, versus 0.4 percent of the population as a whole.

In addition, 24 percent of people with both conditions died within one year of a PH diagnosis, 44 percent within five years, and 52 percent within 10 years. The increasing trend represented a four-fold increase in death compared with congenital hearth disease patients without PH.

“This nationwide study demonstrates that PH in the aging CHD [congenital heart disease] population is a common and morbid long-term complication,” the researchers wrote. “Our study indicates that the risk for PH in adults with CHD is broadly distributed across the entire population, and that the risk is not isolated to those with systemic-to-pulmonary shunts or severe CHD type.”