Guide to Pulmonary Hypertension

Pulmonary hypertension is a condition caused by increased blood pressure in the lungs that can lead to serious complications including heart failure.

Key Info about Pulmonary Hypertension

  • Pulmonary hypertension is classified into 5 main groups
  • It has multiple causes and may be difficult to diagnose
  • Pulmonary Hypertension can often be managed with medications, but surgery is an option when medical therapy fails

Pulmonary hypertension is a rare and serious disease in which the pressure in the pulmonary (lung) circulation is above normal levels. Left untreated, pulmonary hypertension can cause permanent damage to the lungs, resulting in life-threatening right heart failure and death.

Types of Pulmonary Hypertension

Pulmonary hypertension is currently classified into five main groups:

  1. Pulmonary Arterial Hypertension
  2. Pulmonary Hypertension due to left heart disease
  3. Pulmonary Hypertension due to lung disease
  4. Pulmonary Hypertension due to blood clots in the lungs
  5. Blood and other rare disorders that lead to Pulmonary Hypertension

Most treatments are directed toward group 1, pulmonary arterial hypertension. When there is no known cause of pulmonary hypertension, it is called idiopathic (primary) pulmonary arterial hypertension. Pulmonary hypertension that occurs as a result of other disorders is called secondary pulmonary hypertension. One example of a common type of secondary pulmonary arterial hypertension, caused by abnormal development and defects in the heart and great vessels, is called Eisenmenger's syndrome.

Other causes of pulmonary hypertension include:

  • Congenital heart disease including Eisenmenger’s syndrome
  • Lupus, scleroderma and other connective tissue diseases
  • Chronic thromboembolic pulmonary hypertension — CTEPH
  • Chronic lung disease and advanced lung failure
  • Liver disease
  • HIV disease
  • Genetic (familial/inherited) pulmonary hypertension
  • HHT (hereditary hemorrhagic telangiectasia)
  • Pulmonary hypertension of the newborn
  • Sickle cell disease
  • Toxin (methamphetamines) exposure

While there have been many advances in the treatment of pulmonary arterial hypertension over the past two decades, there is still no cure aside from lung transplantation if medical therapy fails.


Pulmonary hypertension can be challenging to pinpoint as a diagnosis. Routine physical exams may not uncover the disease and symptoms may be similar to other lung and heart conditions. So a careful work up is important to diagnose pulmonary hypertension.

Some combination of the following tests will be done:

  • Echocardiogram, which uses sound waves to view the heart. A small, plastic instrument called a transducer is placed on the chest. It reflects sound waves (echoes) from the heart and sends them to a machine that uses the sound wave patterns to compose images of the beating heart on a monitor.
  • Blood tests to find signs of infection, inflammation, or other indicators of conditions that could lead to pulmonary hypertension.
  • Chest X-ray to see if the right ventricle of the heart or pulmonary arteries are enlarged.
  • Right heart catheterization, which is one of the most reliable ways of diagnosing pulmonary hypertension. Usually local anesthesia and sedation are given before a thin, flexible tube (catheter) is inserted into a vein in the neck or groin. The catheter is then guided into the right ventricle and pulmonary artery to measure the pressure there.

Additional tests to evaluate the pulmonary arteries and lungs include:

  • Pulmonary function tests to measure how much air your lungs can hold, and the airflow in and out of your lungs using a simple instrument called a spirometer
  • Perfusion lung scan, which uses tiny amounts of radioactive substances (radioisotopes) to look at blood flow (perfusion) in the lungs
  • Computerized tomography (CT) scans to allow the doctor to see lungs in 2-D cross-sections images
  • Magnetic resonance imaging (MRI), which uses no X-rays, to obtain images of the lung's blood vessels

Medical Therapies

The current treatment for pulmonary arterial hypertension includes the use of targeted pulmonary hypertension medications and other conventional treatments to support heart and lung function. Medical treatment of pulmonary arterial hypertension has advanced significantly during the past decade with multiple approved drugs including a number of oral and inhaled medications as well as alternate intravenous and subcutaneous infusions medications.

These medications commonly include:

  • Vasodilators, which open narrowed blood vessels
  • Endothelin receptor antagonists, which reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow
  • High-dose calcium channel blockers, which help relax the muscles in the walls of your blood vessels.
  • Anticoagulants, which help prevent the formation of blood clots within the small pulmonary arteries
  • Oxygen therapy, which breathing pure oxygen to allow the lungs to work more efficiently

Surgical Options

If medical treatment does not alleviate the symptoms of pulmonary hypertension, there are a few surgical options. These include:

  • Atrial septostomy: A surgeon creates an opening between the upper left and right chambers of the heart (atria) to relieve the pressure on the right side of the heart.
  • Transplantation: In some cases, a lung or heart-lung transplant might be an option, especially for younger people who have idiopathic pulmonary arterial hypertension.

Next Steps

If you need surgical help pulmonary hypertension, we’re here for you. Call (212) 305-3408 for existing patients, (212) 304-7535 for new patients, or request an appointment online to get started today. Our surgeons also work closely with pediatric and adult pulmonologists and cardiologists as part of one of the largest Pulmonary Hypertension Comprehensive Care Centers in the country. Columbia’s Pulmonary Hypertension Comprehensive Care Center can be reached at (212) 305-4436.

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