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Pulmonary Hypertension

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Updated May 16, 2007

Pulmonary hypertension is a type of high blood pressure that only affects the part of the circulatory system that connects the lungs and heart. Specifically, it is defined as elevated pressure in the pulmonary artery or pulmonary vein. To make a diagnosis, the official guidelines require an average pulmonary artery pressure of either:

  • >25mmHg at rest
  • >30mmHg during exercise

What Causes Pulmonary Hypertension?

Sometimes the hyperension is related to another disease that leads to high blood pressure, such as when a blood vessel is blocked by a clot, but not always. Instead, it is thought that some people have a genetic defect which makes them more sensitive to the changes caused by certain diseases. Some of these genetically predisposed people develop pulmonary hypertension as a side effect of an underlying disease. Research has pinpointed some genes that may be at fault.

People who may be at especially increased risk include:

  • SSc (previously called Scleroderma)
  • Chronic liver disease
  • Obstructive sleep apnea

Symptoms of Pulmonary Hypertension

Because pulmonary hypertension is usually present along with another underlying disease, the symptoms can be difficult to classify. Usually the symptoms caused by the other disease are strong enough to mask any symptoms actually being caused by the pulmonary hypertension. In general, the symptoms of early pulmonary hypertension include:

  • Shortness of breath
  • Fatigue
  • Easy tiring
These early symptoms happen because the pulmonary hypertension is slowly affecting the heart's ability to pump blood. As these effects worsen, the symptoms of pulmonary hypertension become very similar to the symptoms of heart failure, and include:
  • Fainting during exercise
  • Swelling of the lower body
  • Chest pain
  • Loss of appetite
  • Abdominal pain
These are considered to be later symptoms of pulmonary hypertension, and a heart murmur can usually be heard once these symptoms appear.

Pulmonary Hypertension Treatment

Pulmonary hypertension is a very serious disease, and should be treated as aggressively as possible. Specific treatment plans are very individualized, and often require extensive pre-treatment testing. Options that have been found to be helpful in almost all patients. These options include:

  • Diuretics to eliminate excess fluid and decrease stress on the heart
  • Oxygen therapy to compensate for the impaired ability of the heart to circulate blood
  • Anticoagulation therapy to prevent blood clots (Warfarin is the drug of choice)
  • Digoxin to help the heart pump blood more efficiently
  • Exercise to strengthen the cardiovascular system and prevent further decline
In addition, newer drugs such as Flolan (epoprostenol) and Tracleer (bosentan) represent advanced forms of treatment that are making a positive impact on the prognosis of pulmonary hypertension.

Different Types of Pulmonary Hypertension

The World Health Organization classification scheme describes five different types, or "groups," of pulmonary hypertension, which are organized based on the underlying cause of the disease. These groups are:

  • Group 1: Pulmonary artery hypertension because of congenital problems (heart defects) or HIV. The terms "idiopathic" or "primary" pulmonary hypertension also generally mean Group 1.
  • Group 2: Pulmonary vein hypertension because of problems with the left side of the heart
  • Group 3: Pulmonary artery or vein hypertension associated with underlying lung disease or a chronic state of oxygen deprivation
  • Group 4: Pulmonary artery or vein hypertension caused by blood clots blocking vessels in the lungs
  • Group 5: Pulmonary artery or vein hypertension as a result of chronic inflammation, obstruction or compression

Abuse of stimulants like cocaine and methamphetamine are an important and growing cause of Group 1 pulmonary hypertension.

Sources:
1. Rich, S (ed). Executive summary from the World Symposium on Primary Pulmonary Hypertension, Evian, France, September 6-10, 1998, co-sponsored by The World Health Organization.
2. Barst, RJ, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. Journal of the American College of Cardiology 2004; 43:40S.
3. D'Alonzo, et al. Survival in patients with primary pulmonary hypertension. Annals of Internal Medicine 1991; 115:343.
4. McLaughlin, VV, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:78S.
5. Simonneau, G, et al. Clinical Classification of Pulmonary Hypertension. Journal of the American College of Cardiology 2004 Jun 16;43(12 Suppl S):5S-12S.

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