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Pulmonary hypertension in adults with congenital heart disease

Heidi M Connolly, MD, FASE
Robert P Frantz, MD
Section Editors
Candice Silversides, MD, MS, FRCPC
Jess Mandel, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC


Approximately 3 to 10 percent of patients with congenital heart disease (CHD) develop pulmonary arterial hypertension (henceforth described as pulmonary hypertension-congenital heart disease [PH-CHD]) [1,2]. Early diagnosis and repair of CHD has decreased the number of patients with Eisenmenger syndrome; however, the overall number of PH-CHD patients is increasing because more patients with complex and palliated CHD survive to adulthood [3]. PH-CHD is more common in women, patients with shunt lesions, and older CHD patients, and is associated with excess mortality and increase in health care costs [1].  

The clinical manifestations, diagnosis, and management of PH-CHD in adults are discussed here. The evaluation and prognosis of Eisenmenger syndrome are discussed separately. (See "Evaluation and prognosis of Eisenmenger syndrome" and "Management of Eisenmenger syndrome".)


Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure ≥25 mmHg at rest. PH can be purely postcapillary (secondary to elevated pulmonary venous pressure with normal pulmonary vascular resistance [PVR]), purely precapillary (elevated PVR with normal pulmonary venous pressure), or a combination of the two (mixed PH). PH-congenital heart disease (PH-CHD) can also be secondary to increased flow through the pulmonary vasculature; this type of PH is not typically observed in non-CHD populations, aside from high output states such as severe anemia, thyrotoxicosis, cirrhosis, and large dialysis fistulae. (See "Classification and prognosis of pulmonary hypertension in adults".)


Patients with pulmonary hypertension-congenital heart disease (PH-CHD) represent a heterogeneous patient population, predominantly in group 1 (precapillary or pulmonary arterial hypertension [PAH]) PH clinical classification [4] (table 1). (See "Clinical features and diagnosis of pulmonary hypertension in adults", section on 'Diagnostic criteria'.)

In the past, a classification system based on cause of PH-CHD was used and patients were classified into the following groups:

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Literature review current through: Nov 2017. | This topic last updated: Aug 24, 2017.
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