Pulmonary Hypertension (PH) is high blood pressure in the blood vessels of the lungs. It occurs when the small blood vessels that go through the lungs become thicker, constrict or become plugged. This, in turn, makes the right side of the heart work harder to pump blood through the lungs.
If PH worsens, the heart will become enlarged and weaker, pumping blood less efficiently into the lungs and throughout the body. Patients then develop progressive fatigue and shortness of breath, two typical symptoms of pulmonary hypertension.
There are more than 20 known causes of pulmonary hypertension, which can affect people of any age, race and ethnic background. It is more common in women than in men.
Pulmonary Hypertension Condition Groups
Pulmonary hypertension experts divide PH into five groups of conditions based on the many causes of this complicated disease. It is important that patients are diagnosed and treated by physicians who are experts in all components of the disease and can provide a full range of treatment options.
As the largest academic pulmonary hypertension program in the state, we offer patients comprehensive care plus access to physician specialists in the related conditions that cause PH.
Group 1: Pulmonary Artery Hypertension (PAH) Conditions
Group 1 conditions are those specifically related to pulmonary hypertension in arteries of the lungs. The group includes pulmonary artery hypertension conditions that are inherited, caused by drugs or toxins (often street drugs or certain weight-loss medications), caused by conditions that affect the veins and small blood vessels in the lungs and those that have no definitive cause.
It also includes PH caused by connective tissue diseases, HIV infection, liver disease, congenital heart disease, and sickle cell disease.
Group 2: Conditions that Affect the Left Side of the Heart
Group 2 conditions, the most common causes of pulmonary hypertension, are those that affect the left side of the heart. These conditions may include valvular disease, mitral valve disease and long-term high blood pressure. We often coordinate care with the patient’s cardiologist and primary care physician for this group of patients.
Group 3: Conditions Associated with Lung Disease
Group 3 conditions are those that relate to lung diseases, including chronic obstructive pulmonary disease (COPD) and interstitial lung diseases, which cause scarring of the lung tissue. This group also includes sleep apnea and other sleep-related breathing disorders.
Group 4: Conditions Associated with Blood Clots and Related Disorders
Group 4 is made up of conditions caused by blood clots in the lungs or by blood clotting disorders. This group includes thromboembolic diseases, such as chronic thromboembolic pulmonary hypertension (CTEPH), in which patients who have had blood clots in their veins (most commonly in leg veins) may develop pulmonary hypertension after a number of those clots migrate to the lung blood vessels (pulmonary embolism).
Group 5: Other Causes of Pulmonary Hypertension
Group 5 includes all causes of pulmonary hypertension not covered in other groups. These conditions include blood disorders, systemic disorders (which involve several organs of the body and include diseases such as sarcoidosis), metabolic disorders (such as thyroid disease and glycogen storage disease) and other conditions, such as kidney disease and tumors.
Early Diagnosis, Specialized Treatment Are Key
Since symptoms of pulmonary hypertension can be caused by other heart and lung conditions, it is important for patients to be correctly diagnosed as early as possible. PH is usually a chronic condition that can be difficult to treat. Fortunately, recent discoveries and continued research are giving specialists the tools to treat the condition more effectively, helping patients feel better and improving the quality of their lives. As a result, primary care physicians are looking for this disease sooner in patients and referring patients for specialized care in more timely ways.
Symptoms of Pulmonary Hypertension
Early in the disease, symptoms may be nonspecific and can mimic symptoms of other medical conditions. It is also possible that patients may experience only limited symptoms. Symptoms typically associated with pulmonary hypertension include:
- Progressive shortness of breath (also called dyspnea)
- Fatigue or tiredness in association to shortness of breath
- Chest pain or pressure that typically occurs during activity (angina)
- Dizzy spells that typically occur during activity or exercise
- Leg swelling (edema)
- Rapid pulse rate (tachycardia)
- Heart throbbing (palpitations)