About Pulmonary Hypertension

Pulmonary hypertension (PH) is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels. The small arteries of the lung narrow throughout the lungs. Pulmonary hypertension is the result of greater resistance to blood flow. As a result of the increased workload caused by this resistance, the right side of the heart becomes enlarged. Eventually, progressive right heart failure may develop.

When pulmonary hypertension occurs without a known cause, it is know as Idiopathic Pulmonary Arterial Hypertension (IPAH)---meaning that there are no other diseases of the heart or lungs causing the high blood pressure.

The cause of IPAH is unknown. Some cases are inherited and that is known as familial PAH.

This disease is rare, but affects people of all ages and ethnic backgrounds. It affects more women than men.

There are other forms of PAH where the cause is known. A common cause is collagen vascular diseseas such as scleroderma, lupus or Crest Syndrome. Congenital heart diseases such as ventricular and atrial septal defects, HIV infection, and liver disease are also possible causes of pulmonary hypertension.

Symptoms of Pulmonary Hypertension

Early on, many patients may have subtle or few symptoms, making the diagnosis quite challenging. Symptoms may include:

Physical examination might show enlargement of the veins in the neck, normal lungs, a heart murmur, enlargement of the liver, and swelling due to fluid retention in the tissues.

Screening Tests

To establish a diagnosis a series of tests are performed that show how well a person's heart and lungs are working. Tests may include:

Medical and Surgical Treatments

PH is a chronic condition that can become life threatening. It has only one known cure -- a surgical treatment known as pulmonary thromboendarterectomy, or PTE. The University of Maryland Medical Center is one of the few medical centers in the country offering this procedure.

In addition, several non-surgical treatments are available and patients now have more options and improved life expectancy. Medical treatment is aimed at slowing the progression of the disease and improving symptoms. Some possible medications include:

  • Anticoagulants
  • Calcium Channel Blockers
  • Diuretics - reduces fluid in the system
  • Inhaled oxygen
  • Bosentan - an oral endothelin receptor antagonist. It works by helping to reduce elevated blood pressure in the lungs, thereby helping the heart to pump blood more effectively.
  • Epoprostenol - a prostacyclin analogue that helps reduce constriction of lung blood vessels and reducing the high blood pressure in the lungs.
  • Treprostinil is also a prostacyclin analogue used to reduce constricted blood vessels.
  • Sildenafil - an oral PDE-5 inhibitor. It works by reducing the pressure in the lungs.
  • Iloprost - a prostacyclin analogue that is inhaled.

If these treatments are not sufficient, and the patient is not a candidate for PTE, a lung transplant evaluation may be recommended.

This page was last updated: July 1, 2013

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