Pulmonary arterial hypertension associated with congenital heart disease: classification and pathophysiology

Abstract

While the development of pulmonary arterial hypertension is not uncommon in adult congenital heart disease patients, other forms of pulmonary hypertension (PH) may also be present. A good understanding of PH classification is therefore vital for clinicians managing adult patients with congenital heart disease. This paper reviews both the general classification of PH and more detailed approaches to classifying pulmonary arterial hypertension in association with congenital heart disease.