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Pulmonary Hypertension

Kelly Latimer, MD, MPH
Michael Layne, MD
Maya Payne, MD

American Family Physician. 2024;110(2):183-191.

Author disclosure: No relevant financial relationships.

Pulmonary hypertension includes a diverse set of conditions defined by a mean pulmonary artery pressure greater than 20 mm Hg found during right heart catheterization that can lead to right-sided heart failure and death if untreated. The most common cause of pulmonary hypertension is left-sided heart failure, followed by chronic obstructive lung disease. Pulmonary hypertension presents as unexplained dyspnea on exertion and possible findings of right-sided heart failure. The diagnosis is commonly delayed because the symptoms are often attributed to underlying heart or lung disease. Echocardiography is the initial study of choice, and findings can suggest a low, intermediate, or high risk of pulmonary hypertension. Right heart catheterization is the standard of care for diagnosing and classifying pulmonary hypertension, and the results may inform treatment. Patients with pulmonary hypertension should be referred to a center specializing in treatment. Patients with pulmonary hypertension have a high risk of perioperative complications, and detailed specialty preoperative evaluation is recommended. Physicians should counsel patients of childbearing age with pulmonary hypertension to prevent pregnancy to avoid worsening the severity of pulmonary hypertension and fetal loss. Pulmonary hypertension is severe, chronic, progressive, and challenging to treat; therefore, family physicians should update the patient’s immunization status, screen for and address mental health conditions, and discuss goals of care and advance directives with patients.

Pulmonary hypertension includes conditions that result in elevated pressures in the lung vasculature. Pulmonary hypertension is defined as a mean pulmonary artery pressure of greater than 20 mm Hg at rest found during right heart catheterization.1 Pulmonary hypertension affects an estimated 1% of the global population and approximately 10% of people older than 65 years.1,2 Pulmonary arterial hypertension, a subset of pulmonary hypertension, is a relatively rare diagnosis with a high mortality rate if untreated.1 Family physicians most often encounter pulmonary hypertension as a downstream consequence of cardiac or pulmonary disease.

WHAT’S NEW ON THIS TOPIC

Pulmonary Hypertension
The presence of pulmonary hypertension is a poor prognostic sign, with a 5-year estimated survival rate of 67% or less. The prognosis is slightly worse when pulmonary hypertension is secondary to lung disease and slightly better in chronic thromboembolic pulmonary hypertension.
A 2020 systematic review demonstrated a 12% risk of maternal mortality and 23% risk of pregnancy loss in patients with pulmonary hypertension.
Patients with persistent dyspnea after recovery from COVID-19 are at increased risk of pulmonary hypertension. In one meta-analysis, 22% of patients developed pulmonary hypertension after intensive care unit admission due to COVID-19.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendation Evidence rating Comments
Patients with suspected pulmonary hypertension should undergo echocardiography as the initial test.1,10 C Expert consensus
Right heart catheterization is the test of choice for confirmation of pulmonary hypertension (especially if pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension is suspected) and can guide treatment decisions.1 C Expert consensus
Patients with pulmonary arterial hypertension should be referred to a specialty center.1 C Expert consensus
Patients with pulmonary arterial hypertension whose arterial blood oxygen pressure is less than 60 mm Hg should be on long-term oxygen therapy with some recommendations suggesting oxygen use to maintain oxygen saturation greater than 90% at sea level.1,20 C Expert consensus; no data to demonstrate improved patient-oriented outcomes
Patients with chronic thromboembolic pulmonary hypertension (group 4) should be on lifelong therapeutic doses of anticoagulation.1 C Expert consensus and inconsistent randomized controlled trials showing improved survival
Patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension who are optimized and stable on medical therapy should participate in a supervised exercise training program.1,19,25,26 A Consistent evidence from randomized controlled trials showing improvement in a 6-minute walking test and quality-of-life scores
Patients of childbearing potential who have pulmonary hypertension should be counseled to avoid pregnancy and consider two forms of contraception, particularly those treated with bosentan.1,28 C Expert consensus and clinical review article

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

KELLY LATIMER, MD, MPH, is an associate professor of family and community medicine at Eastern Virginia Medical School, Norfolk, Va.

MICHAEL LAYNE, MD, is an assistant professor of family and community medicine at Eastern Virginia Medical School.

MAYA PAYNE, MD, is a physician at MidAtlantic Long Term Care, Norfolk, Va. At the time this article was written, she was the ship physician for the USS John C. Stennis (CVN 74).

Address correspondence to Kelly Latimer, MD, MPH, at latimekm@evms.edu.

Author disclosure: No relevant financial relationships.

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