Idiopathic Pulmonary Arterial Hypertension

(PPH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Pulmonary Arterial Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension; Primary Pulmonary Hypertension)

Pronounced: PRY-mair-ee PUL-mo-nair-ee hi-per-TEN-shun

Definition

Primary pulmonary hypertension (PPH) is a rare disease. It is high blood pressure in the blood vessels of the lungs.

A person with PPH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them. As a result, the right side of the heart has to work harder to push blood to the lungs. This additional strain can eventually lead to heart failure.

PPH is a serious condition. It requires care from your doctor.

Heart and Lungs

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Causes    TOP

The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:

  • Autoimmune diseases
  • Exposure to certain drugs or chemicals
  • Genetic defects

Risk Factors    TOP

PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:

  • Cirrhosis of the liver
  • Portal hypertension
  • HIV infection
  • Family history of PPH
  • Use of appetite suppressants (diet pills)
  • Cocaine use

Symptoms    TOP

Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause:

  • Shortness of breath (when you are active or at rest)
  • Abnormally rapid, deep breathing—hyperventilation
  • Fatigue
  • Progressive weakness
  • Fainting spells
  • Lightheadedness
  • Coughing up blood
  • Bluish tint to the lips and skin—cyanosis
  • Swelling of the legs and hands
  • Chest pain
  • Lack of appetite
  • Cold hands and feet
  • Low blood pressure

Diagnosis    TOP

Your doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis of PPH may be delayed. It is hard to detect until symptoms worsen.

A physical exam by your doctor may show:

  • Swelling of the veins in your neck
  • Enlarged liver and swollen abdomen
  • An abnormal sound in the heart— heart murmur

Tests may include:

  • Blood tests
  • Pulse oximetry to evaluate how much oxygen is in your blood
  • ECG —to test your heart’s electrical activity
  • Pulmonary function tests —noninvasive tests, like blowing into a tube, that measure how well your lungs are working
  • Cardiac catheterization —to detect problems with the heart and its blood supply
  • Six-minute walk to determine the amount of shortness of breath, an indirect measure of the severity of PHH

Imaging tests evaluate the lungs and surrounding structures. These may include:

  • Chest x-ray
  • CT scan of the chest
  • Echocardiogram
  • Pulmonary arteriogram
  • Nuclear lung scan

Treatment    TOP

There is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best plan for you. Treatment includes the following:

Medication

Medication can improve blood flow, decrease the risk of blood clots, and improve the ability of the heart to pump blood. These may include:

  • Calcium channel blockers
  • Prostacylins
  • Digoxin
  • Anticoagulants
  • Diuretics
  • Vasodilators
  • Endothelin receptor antagonists
  • Phosphodiesterase-5 inhibitors

Supplemental Oxygen

If breathing becomes difficult, oxygen therapy may be given. It may be given through a mask or tubes inserted into the nostrils.

Transplantation    TOP

If PPH is severe or other treatment methods fail, a lung transplant or heart-lung transplant may be needed.

Prevention    TOP

There are no current guidelines to prevent PPH because the cause is unknown.

RESOURCES:

National Heart, Lung, and Blood Institute
https://www.nhlbi.nih.gov
Pulmonary Hypertension Association
https://phassociation.org

CANADIAN RESOURCES:

Heart and Stroke Foundation of Canada
http://www.heartandstroke.com/
The Lung Association
https://www.lung.ca

References:

Explore pulmonary hypertension. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health/health-topics/topics/pah. Updated August 2, 2011. Accessed September 14, 2017.
Nuclear lung scan. Cedars-Sinai Medical Center website. Available at:
...(Click grey area to select URL)
Accessed September 14, 2017.
Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at: https://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 14, 2017.
Pulmonary arterial hypertension (PAH). EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated June 14, 2017. Accessed September 14, 2017.
Pulmonary hypertension. American Lung Association website. Available at:
...(Click grey area to select URL)
Accessed September 14, 2017.
Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at:
...(Click grey area to select URL)
Updated May 23, 2017. Accessed September 14, 2017.
Last reviewed September 2018 by EBSCO Medical Review Board Michael J. Fucci, DO, FACC
Last Updated: 10/9/2015

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