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Fifteen or 20 years ago, pulmonary arterial hypertension had almost 100% mortality. While there is still no cure, there are many treatments that significantly improve life expectancy and the quality of life in patients with this disease.
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Pulmonary hypertension means high pressure inside blood vessels of the lung. This is different than the high blood pressure measured in your arm, and is diagnosed with very specific tests (see evaluation below). As the lung blood vessels narrow and the pressure inside them rises, the right ventricle (the right lower chamber of the heart that pumps blood toward the lung via the pulmonary artery) has to work hard to overcome the resistance to blood flow. As a result, the right side of the heart becomes enlarged and weak. Over time, the weakened right ventricle becomes less efficient and fails, a process called right ventricular failure.
There are many causes for pulmonary hypertension. Pulmonary arterial hypertension refers to elevated pressure inside the pulmonary arteries, and usually affects young adults. This used to be called primary pulmonary hypertension, but now it is classified as World Health Organization Group 1. The most common causes include connective tissue or rheumatologic diseases such as scleroderma or lupus, use of drugs or medications such as methamphetamines and fenfluramine/phentermine (Fen-Phen), liver cirrhosis, infection with HIV, congenital heart defects, or idiopathic (which means we don’t know why this happens).
Other conditions can cause secondary pulmonary hypertension. Congestive heart failure and valvular disease; lung diseases such as chronic obstructive pulmonary disease, interstitial lung disease (pulmonary fibrosis), and obstructive sleep apnea; pulmonary emboli which are blood clots in the pulmonary arteries; and miscellaneous causes need to be considered.
Symptoms of pulmonary hypertension include shortness of breath, chest pressure, leg swelling, fatigue, dizziness, and fainting spells. These symptoms can be a sign of many other conditions, which makes pulmonary hypertension difficult to diagnose.
Fifteen or 20 years ago, pulmonary arterial hypertension had almost 100% mortality. While there is still no cure, there are many treatments that significantly improve life expectancy and the quality of life in patients with this disease. It is very important to promptly diagnose and treat pulmonary hypertension to have the best chance at survival.
Your doctor will talk with you at length to find out about your symptoms and any exposures that increase your risk for pulmonary hypertension. Your doctor will perform a physical examination and order a variety of tests, some of which are described below.
Blood tests – these tests are used to evaluate kidney and liver function, check for anemia, and check for rheumatologic conditions such as scleroderma.
Echocardiogram - this is an ultrasound of the heart that is an excellent screening tool for pulmonary hypertension
Pulmonary function tests – these are breathing tests that can diagnose asthma, emphysema, or other lung conditions
Six minute walk test – this is a simple exercise test that requires you to walk on a flat surface for 6 minutes; the respiratory therapist performing this test will measure your oxygen saturation and the distance walked
Polysomnography – this is a sleep study that can diagnose obstructive sleep apnea
Ventilation - Perfusion (V/Q) scan – this test diagnoses blood clots in the lungs
CT chest – this test provides a detailed picture of the lung tissues and surrounding structures, and can diagnose certain lung conditions such as pulmonary fibrosis
Right heart catheterization - this is the gold standard procedure to measure the pressures inside the blood vessels of the lung and the efficiency of the heart pump, and is required to diagnose pulmonary arterial hypertension. It is performed in the cardiac catheterization lab under local anesthesia (which means you do not need to be asleep for the procedure). Usually, patients go home the same day.
Most patients with pulmonary hypertension require a diuretic to prevent water retention in the body and to improve the leg swelling. Some people may need supplemental oxygen. Depending on the type and severity of pulmonary hypertension, your doctor may discuss with you pulmonary vasodilator therapies. These medications include pills, specialized inhalations, and intravenous infusions that work to lower the pressure inside the arteries of the lung.
Olga M. Fortenko, M.D.
Dr. Olga Fortenko specializes in the diagnosis and management of pulmonary vascular disease, including pulmonary arterial hypertension and pulmonary emboli. Dr. Fortenko attended medical school at Saint Louis University School of Medicine. She completed Internal Medicine residency training at UC Davis. Dr. Fortenko completed fellowships in Pulmonary and Critical Care Medicine and Pulmonary Vascular Disease at Stanford Medical Center. She has been practicing since 2014.
While Dr. Fortenko is the lead physician for the Pulmonary Hypertension Program, she works closely with a group of her colleagues who assist in co-managing hospitalized patients with pulmonary hypertension. These physicians include Melissa Lim, MD, Mojdeh Talebian, MD, and Sigal Tene, MD.