Pulmonary Arterial Hypertension (PAH) Clinic

Pulmonary Arterial Hypertension (PAH) is an uncommon, albeit progressive disease of the blood vessels of the lungs. One of the greatest risks of PAH, it that is asymptomatic in its earliest stages. As the disease progresses, pressure inside the pulmonary arteries increases. This pressure increase is coupled with a blood flow decrease through the vessels. This strain causes an eventual failure of the right ventricle, with symptoms such as fluid retention, marked fatigue and fainting episodes. Without treatment, most patients with PAH will not survive five years.

In recent years there has been a substantial amount of progress in research into the causes and treatments for PAH.

Causes

Many causes are known to contribute to PAH. These include:

  • A familial form of the disease that may be associated with connective tissue diseases such as scleroderma or lupus.
  • Blood clots.
  • The use of certain diet pills and stimulants such as methamphetamine.
  • Congenital heart disease.

At times, there is no identifiable cause of the PAH disease. These patients are labeled as having Idiopathic PAH (IPAH).

Diagnosis

CT scans, echocardiograms, laboratory tests, six minute walk tests, breathing tests and a right heart catheterization are all used to assist physicians in diagnosing PAH. Patients with existing disease are followed with a carefully crafted protocol of testing.

Treatment

Treatment and medicines to treat PAH have progressed greatly in the last 20 years. Epoprostenol (Flolan) was approved in 1995, which is a leading PAH medication. There are now nine medications that are approved to aid in the treatment of the disease. Although there is no cure for PAH, the prognosis has improved substantially.

If medical regimens do not produce the desired outcome, lung transplantation provides an additional channel of treatment.

PAH is a complex disease that requires dedicated expertise in diagnosis and management. The physicians at Midwest Cardiovascular are experts in the diagnosis and management of pulmonary arterial hypertension.