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Pulmonary hypertension in ACHD 

Pulmonary hypertension in ACHD
Pulmonary hypertension in ACHD

Sara Thorne

and Sarah Bowater

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date: 12 August 2020

Pulmonary arterial hypertension (PAH) is a common complication in ACHD patients, with a prevalence of around 10% in developed countries. It is most commonly due to large, uncorrected left-to-right shunts, although a permissive genotype might explain its development in other, seemingly lower-risk, patients. Histologically, PAH in ACHD is the same as PAH due to other aetiologies, such as idiopathic and connective tissue disease.

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