Pulmonary hypertension means literally "high blood pressure within the lungs". This can range from mild to very severe. The symptoms of PH are nonspecific which can make it hard to diagnose. Symptoms include, but are not limited to, shortness of breath, (especially noticed with exercise), chest discomfort, lightheadedness, and passing out. It is usually most reliably diagnosed with a cardiac echocardiogram. With timely recognition and appropriate treatment patients with PH can experience marked improvement in their ability to do daily activities and exercise, quality of life and lengthened survival. PH is a very serious illness, but new treatments developed in the last few years hold new promise for patients with this condition.

Pulmonary vascular disease includes a spectrum of conditions or diseases in which the most serious and common complication is pulmonary hypertension. While pulmonary hypertension can occur without any obvious relationship to other diseases ("primary pulmonary hypertension") the majority of cases of pulmonary hypertension are seen in association with a variety of medical conditions and/or toxic exposures. These conditions may include blood clots in the lungs, congenital heart disease, heart and lung diseases which cause low oxygen levels, connective tissue or rheumatologic diseases, sleep apnea, liver disease, HIV infection and exposure to drugs such as amphetamines and diet pills.

There are approximately 32,000 cases new cases of congenital heart disease per year in the United States and 1.5 million new cases worldwide. A large proportion of these patients have abnormalities of the pulmonary vasculature, including pulmonary hypertension. While pulmonary hypertension is now less common with earlier diagnosis of congenital heart disease there still exists a significant population of adults with this debilitating illness. 

The estimated annual incidence of primary pulmonary hypertension in the United States and Europe is 1-3 cases per million people per year. The incidence among users of certain diet pills may be as high as 25-50 persons per million per year. Pulmonary hypertension frequently complicates the rheumatologic condition known as scleroderma (which includes patients with the CREST syndrome). In addition, up to 20% of patients with systemic lupus may develop pulmonary hypertension. Pulmonary hypertension can arise in the setting of both acute and chronic pulmonary embolism (blood clots to the lungs) and can represent a fatal complication of this condition. The annual incidence of fatal pulmonary embolism in the U.S. is estimated to be around 200,000, half of which were potentially curable with appropriate treatment. Chronic disease with pulmonary embolism is far less common, but is also often undiagnosed though potentially curable with surgery or other interventions.

The treatment for PH depends on the type of PH and its severity, as well as on other health problems each patient may have. Based on each individual's medical condition and their response to previous medications they are placed on a regimen that is best suited for them. There is no "one size fits all" approach to PH. Treatments may range from oral medications, exercise programs, inhaled medications and in some cases, medications that are delivered directly into the blood stream via a catheter or into the subcutaneous layer of skin through a small catheter. In some cases surgery or other cardiac interventions might be recommended. 

The response to PH medicines can vary from person to person. Blood tests and echocardiograms, among other tests, are used to monitor how well each person is doing. Patients at Stanford and other PH centers also have access to some of the latest drug treatment through ongoing clinical trials of new PH drugs. Stanford is also currently doing basic science research in the lab to better understand PH and help develop new therapies for treatment. Read more...

Many patients may be concerned about their physical ability to continue working following a PH diagnosis. While many people affected by PH may stop working for various reasons, there are some who feel well-enough and feel able to perform within safe physical guidelines to continue their employment. Whether or not you continue to work outside of the home is a decision that you and your health care team should make together, depending on your individual circumstances. Factors influencing this decision may include your specific symptoms and how well they are managed, your treatment needs and physical response to therapies, physical limitations and type of work you do, and personal financial and practical considerations. There are resources available to help people with disabilities to retain, acquire and train for gainful employment, including the California Department of Rehabilitation and the Social Security Administration.