Five Things You Need to Know about Pulmonary Hypertension

March 29, 2016 Brigham and Women's Hospital

The hallmark symptom of pulmonary hypertension is shortness of breath (dyspnea).

The hallmark symptom of pulmonary hypertension is shortness of breath (dyspnea).

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). 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. Today’s post provides some key information about pulmonary hypertension from Dr. Aaron Waxman, Director of the Brigham and Women’s Hospital (BWH) Pulmonary Vascular Disease Program – the only program in Massachusetts to be accredited by the Pulmonary Hypertension Association.

Pulmonary hypertension can be caused by other conditions.

Scleroderma (a connective tissue disease); certain liver conditions; 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.

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.

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.

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.

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.  BWH is one of few hospitals in the nation to offer this procedure.

– Jessica F.

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