Pulmonary arterial hypertension (PAH) affects just over 3% of individuals with congenital heart disease (CHD), reveal nationwide data from the Netherlands.
This equates to an incidence rate of 100 cases in every million of the general population, reports the research team, led by Berto Bouma from the University of Amsterdam, in the International Journal of Cardiology.
The study findings show that of the four clinical classifications of PAH-CHD (Eisenmenger syndrome [group A], PAH with left-to-right shunts [group B], PAH with coincidental CHD [group C] and postoperative PAH [group D]), postoperative PAH was the most common.
“[W]e would like to emphasize that the awareness of PAH in patients after shunt closure should be increased to guarantee optimal treatment and medical care and lifelong cardiac follow-up is recommended in these patients”, say the researchers.
The team drew data from the Dutch Congenital Corvitia registry in 2011, which included 12,624 adults with CHD. A total of 5487 individuals were considered at risk of PAH on account of having an initial diagnosis of systemic-to-pulmonary shunt, and 1814 of these patients were randomly selected for study.
In all, 135 patients had possible PAH – a systolic pulmonary arterial pressure of at least 40 mmHg – giving an estimated PAH prevalence of 3.2% for the whole CHD population. Recent estimates of CHD prevalence at 3000 per million adults gives a CHD-related PAH incidence rate of 100 per million of the general population, say Bouma and colleagues.
Fewer cases of possible PAH were classified as group A, B or C than group D, at 51, 15 and five compared with 64, respectively.
The researchers calculated a “concerning” PAH rate of 5.7% among all patients in the sample who underwent corrective cardiac surgery or intervention and suggest that this could from delayed correction of the defect, “especially if pulmonary vascular disease had already developed”.
They add that “[f]uture research should aim to provide more insight into which patients are at highest risk to develop post-operative PAH, to be able to provide tailored care to patients born with systemic-to-pulmonary shunts”, noting that not every patient will necessarily benefit from closure of a defect.
Finally, PAH prevalence increased with age, at a prevalence of around 2.5% in under-30-year-olds compared with 35.0% in those aged 80 years or older.
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