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by de la Rocha K
(IPAH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension)

Definition

Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease. It is high blood pressure in the arteries (blood vessels) of the lungs.

The right side of the heart will have to work harder to push blood to the lungs. Over time this can lead to heart failure.

Heart and Lungs
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Causes

The cause of IPAH is unknown.

A person with IPAH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them.

Risk Factors

IPAH is more common in women aged 50 to 55 years old. Other factors that may increase your risk of IPAH include:

  • Portal hypertension
  • HIV infection
  • Family history of IPAH
  • Use of appetite suppressants (diet pills)
  • Cocaine use

Symptoms

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

  • Shortness of breath (when you are active or at rest)
  • Increased difficulty breathing
  • 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

Your doctor will ask about your symptoms and past health. A physical exam will be done. Diagnosis of IPAH may be delayed. Symptoms are similar to other more common conditions like asthma. It is hard to detect IPAH in early stages.

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 IPAH

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

There is no cure for IPAH. Treatment is used to help alleviate and control the symptoms and slow progress of the disease. 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:

  • Phosphodiesterase inhibitors
  • Endothelin receptor blockers
  • Prostanoids like epoprostenol or treprostinil
  • Stimulators of soluble guanylate cyclase (riociguat)
  • Prostacyclin receptor antagonist (selixipag)
  • Calcium channel blockers

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

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

Prevention

There are no current guidelines to prevent IPAH 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.ca 

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. Accessed January 29, 2019.

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:  https://www.dynamed.com/topics/dmp~AN~T115043/Pulmonary-arterial-hypertension-PAH  . Updated November 20, 2018. Accessed January 29, 2019.

Pulmonary hypertension. American Lung Association website. Available at: http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/pulmonary-hypertension. Accessed January 29, 2019.

Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension%5FUCM%5F301792%5FArticle.jsp#.Wbr2xrKGNxA. Updated May 23, 2017. Accessed January 29, 2019.

Revision Information

  • Reviewer: EBSCO Medical Review Board Michael J. Fucci, DO, FACC
  • Review Date: 09/2018
  • Update Date: 01/29/2019