Pulmonary hypertension in adults with congenital heart disease
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
- Robert P Frantz, MD
Robert P Frantz, MD
- Professor of Medicine
- Mayo Clinic College of Medicine
- Section Editors
- Candice Silversides, MD, MS, FRCPC
Candice Silversides, MD, MS, FRCPC
- Section Editor — Congenital Heart Disease
- Associate Professor of Medicine
- University of Toronto
- Jess Mandel, MD
Jess Mandel, MD
- Section Editor — Pulmonary Vascular Disease
- Professor of Medicine
- University of California, San Diego
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 . PH-CHD is more common in women, patients with shunt lesions, and older CHD patients, and is associated with excess mortality and increase in healthcare costs .
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 "Medical management of Eisenmenger syndrome".)
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure ≥25 mmHg at rest. PH can be secondary to elevated pulmonary venous pressure (post-capillary pulmonary hypertension), elevated pulmonary vascular resistance (PVR), defined by PVR >3 Wood units (precapillary PH), or 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 "Overview 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  (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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- CLINICAL MANIFESTATIONS
- Symptoms and signs
- Initial test findings
- - Laboratory tests
- - Electrocardiogram
- - Chest radiograph
- DIAGNOSIS AND EVALUATION
- When to suspect pulmonary hypertension-congenital heart disease
- Approach to diagnosis and evaluation
- Key diagnostic tests
- - Cardiovascular imaging
- Computed tomography or cardiovascular magnetic resonance
- - Cardiac catheterization
- - Nuclear lung scintigraphy
- - Pulmonary function tests with volumes and diffusion capacity
- Tests to evaluate patients with PH-CHD
- - Natriuretic peptide level
- - Pulse oximetry
- - Exercise test
- DIFFERENTIAL DIAGNOSIS
- General management
- Disease-specific therapy
- - Primary therapy
- - Advanced therapy
- Patient selection
- Agent selection
- Interventional treatments including cardiac surgery
- Management of pregnancy
- Participation in athletics
- SUMMARY AND RECOMMENDATIONS