Woman breathing heavily after working out

Pulmonary Hypertension: Five Things You Need to Know

Private: Aaron B. Waxman, MD, PhD
Contributor Aaron B. Waxman, MD, PhD

Pulmonary hypertension (PH), or high blood pressure in the lungs’ blood vessels, is a serious and disabling condition that can progress rapidly if not properly identified and treated.

“The hallmark symptom of PH is shortness of breath (dyspnea),” says Dr. Aaron Waxman, Director of the Brigham and Women’s Hospital Pulmonary Vascular Disease Program – the only program in Massachusetts to be accredited by the Pulmonary Hypertension Association. “As the disease progresses, other symptoms may include dizziness and fainting, chest pain during exercise, increased heart rate, cough, and swelling in the lower extremities.”

Here are five things you should know:

1. Pulmonary hypertension can be caused by other conditions.

Scleroderma (a connective tissue disease); certain liver conditions; some lung diseases, such as emphysema, pulmonary fibrosis, and cystic fibrosis; congenital heart disease; certain blood disorders; and other illnesses can cause pulmonary hypertension. In these cases, it is important to address the underlying conditions as well.

2. Accurate diagnosis is critical.

Pulmonary hypertension can fall into multiple categories, and treatments can vary depending on the type of pulmonary hypertension. Multiple tests are often required to diagnose pulmonary hypertension and determine the specific category of PH. These tests may include right heart catheterization and advanced cardiopulmonary exercise testing, which use catheters to directly measure heart and lung function and assess how the body is utilizing oxygen at rest and during exercise.

3. Specialty care is needed for patients with pulmonary hypertension.

Patients with pulmonary hypertension should receive care by physicians who specialize in PH. These typically include pulmonologists and cardiologists who have a particular interest in PH and collaborate with other specialists, such as cardiac surgeons, rheumatologists, and hematologists, to treat underlying conditions.

4. Pulmonary hypertension impacts the heart.

The right side of the heart pumps blood into the lungs to be oxygenated. In pulmonary hypertension, the blood vessels in the lungs thicken and become stiff, making it difficult for them to accommodate the varying volume of blood flow from the right side of the heart. This results in inefficient pumping of the right side of the heart, which eventually becomes enlarged, and over time, can fail.

5. Treatment options for pulmonary hypertension have increased dramatically in recent years.

Treatment for PH can lower patients’ pulmonary pressure and resistance, reduce symptoms, improve exercise capacity, and prolong life expectancy. Newer medications include oral and inhaled therapies that provide alternatives to intravenous treatments. Surgical treatments also may be appropriate. For patients with chronic thromboembolic pulmonary hypertension (CTEPH) – a rare form of pulmonary hypertension caused by recurring blood clots in the lung – pulmonary thromboendarterectomy may be performed to remove blockages in the lung. Brigham is one of few hospitals in the nation to offer this procedure.

Private: Aaron B. Waxman, MD, PhD
Aaron B. Waxman, MD, PhD

Aaron B. Waxman, MD, PhD, is a pulmonologist in the Division of Pulmonary and Critical Care Medicine at Brigham and Women’s Hospital and Associate Professor of Medicine at Harvard Medical School.

Before you go,

Learn about managing lung conditions, from asthma to COPD and lung cancer, and what treatment options can help you optimize your health. Read more articles about lung conditions.