What is Pulmonary Hypertension?

Pulmonary hypertension is an increase in blood pressure within the arteries of the lungs and the right side of the heart.

When the blood vessels of the lungs become narrowed, pressure builds up in these arteries, therefore called pulmonary hypertension (high blood pressure).

When the heart begins to work harder, over time the right side of the heart becomes enlarged leading to right heart failure or cor pulmonale.

What are the causes?

Pulmonary hypertension may be caused by the following:
  • Autoimmune diseases i.e. scleroderma, rheumatoid arthritis, collagen vascular disease
  • Familial
  • Congenital heart defects
  • Pulmonary embolism
  • Congestive heart failure
  • Heart valve disease
  • Hepatic cirrhosis/ portal disease
  • Infection i.e. HIV
  • Chronic hypoxia i.e COPD, pulmonary fibrosis, interstitial lung disease, obstructive sleep apnea, chronic exposure to high altitudes
  • Medications i.e. diet pills
  • Idiopathic (unknown)

What are the risk factors?

The risk factors for pulmonary hypertension include:
  • Age: older patients are more likely to have secondary hypertension, such as pulmonary hypertension
  • Sex: more common in women than men
  • Family history: genetic inheritance
  • Weight: overweight persons are at higher risk of developing pulmonary hypertension
  • Other diseases: heart, lung, or liver disease
  • Other conditions: HIV infection, blood clots, sickle cell disease
  • Drugs i.e. cocaine or diet pills
  • Those who live at high altitudes

What are the symptoms?

Symptoms of pulmonary hypertension include:
  • Shortness of breath or light-headedness during activity
  • Fast heart rate (palpitations)
  • Swelling of the ankles or legs
  • Cyanosis (bluish discoloration of the skin)
  • Dizziness or syncope
  • Fatigue or weakness
  • Chest pain
  • Upper right quadrant abdominal pain

How is it classified?

Pulmonary hypertension can be classified into:
  • Idiopathic Pulmonary Hypertension (IPH)
Unknown cause of high blood pressure in the lungs; Uncommon
  • Secondary Pulmonary Hypertension
High pulmonary pressure due to other medical problems; Common
Causes include:
  • Pulmonary Embolism
  • COPD
  • Pulmonary fibrosis
  • Connective tissue disorders
  • Congenital heart defects
  • Sickle cell anemia
  • AIDS
  • Cirrhosis
  • High altitude
  • Drugs including cocaine, diet pills

What are the complications?

Pulmonary hypertension can lead to complications including:
  • Cor pulmonale (right heart failure)- The right ventricle has to work harder therefore it becomes thickened and enlarged. Eventually the right side of the heart gives out leading to right heart failure.
  • Pulmonary embolism- dislodgement of a blood clot to the lung
  • Arrhythmia
  • Hemoptysis- coughing up of blood

Call your doctor immediately if you have any of the following:

Call your doctor if you develop symptoms such as:
  • Shortness of breath during physical activity
  • Fatigue
  • Chest pain
  • Or other symptoms

How is it diagnosed?

Pulmonary hypertension is diagnosed in three different parameters including patient history, physical examination, and tests.


Patient history
Patients may complain of symptoms in severe pulmonary hypertension. Including symptoms of:
  • Shortness of breath
  • Angina (chest pain)
  • Fatigue/ tiredness
  • Upper right quadrant abdominal pain
  • Palpitations (racing heart beat)
Physical examination
During a routine physical examination, your health care provider will notice the following:
  • Cyanosis- bluish discoloration of the skin and lips
  • Weak pulse may be felt
  • Distended neck veins
  • During auscultation, a murmur may be heard
The following tests may be performed:
  • Blood tests – complete blood counts, liver function tests, PT/INR.
  • Electrocardiogram (EKG) – a noninvasive test performed in our clinic which determines the electrical activity of your heart. This test may be used to detect whether your heart’s rhythm is steady or irregular. Right ventricle enlargement can also be detected.
  • Chest X-ray– a simple test performed in our office showing the size and shape of the heart to determine whether the right ventricle is enlarged.
  • Echocardiogram (ECHO)– a noninvasive test using sound waves to determine the size and function of the heart’s chambers and the structure and function of the heart valves. This test can estimate the pressure in the pulmonary arteries.
  • Right heart catheterization– a hospital procedure used to measure the pressure in the pulmonary arteries. A thin tube (catheter) is inserted into an artery in your arm or groin to reach the right side of the heart to the pulmonary arteries.
  • Chest Computed Tomography (CT) – shows pictures of the heart, lungs, and blood vessels.
  •  Magnetic Resonance Imaging (MRI) – shows how the right ventricle of the heart is working as well as the blood flow to the lungs.
  • Pulmonary Function Tests (PTFs) – are used to measure the amount, rate, and quality of air you can breathe in and out. This helps to determine any lung disease that may be causing pulmonary hypertension.

How is it treated?

The treatment of pulmonary hypertension is aimed at early recognition, controlling symptoms, treatment of the underlying disease, and the prevention of more lung damage.
Treatment depends on what type of pulmonary hypertension is present and the severity.
Lifestyle modification:
  • Rest
  • No smoking
  • Exercise: Ask your physician which exercise routine is best for you. Exercise for at least 30 minutes a day, 5 days a week. This can improve symptoms of shortness of breath and fatigue
  • Avoid pregnancy and birth control pills
  • Avoid traveling at high altitudes
  • Control weight: maintain a healthy weight with a BMI less than 25. Extra weight makes the heart work harder. By reducing your weight you can reduce the strain on your heart.
  • Nutrition: Eat foods high in fiber such as whole grain cereals, oatmeal, and figs. Eat plenty of fruits such as apples, bananas, prunes, oranges, and pears. Include fish and legumes such as beans or chickpeas
  • Avoid fatty foods including bad saturated fat that is found in some meats, dairy products, chocolates, baked goods, and deep-fried food
  • Reduce stress
  • Prostacydinces
  • Vasodilators: Vasodilators dilate the blood vessels. Epoprostenol (Flolan) is the most commonly prescribed vasodilator for pulmonary hypertension. Another medication that can be prescribed is Iloprost (Ventavis), which can be inhaled and taken directly to the lungs.
  • Endothelin receptor antagonist: These medications reverse the effect of endothelin (vasoconstrictor) which makes vessels narrower. Bosentan (Tracleer) helps to improve energy and symptoms.
  • Sildenafil (Viagra) and Tadalafil (Cialis): These medications are used to treat pulmonary hypertension by opening up the blood vessels in the lungs to allow more blood flow.
  • Calcium channel blockers: Calcium channel blockers may be given in high dosage to help relax the muscles surrounding the arteries and cause the vessels to open. This increases blood flow to the lungs and lowers blood pressure. Calcium channel blockers include amlodipine (Norvasc), diltiazem (Cardizem), and nifedipine (Adalat, Procardia)
  • Ambrisenten (Lentairis): helps dilate the blood vessels
  • Anticoagulants: help prevent clot formation
  • Diuretics: these are sometimes referred to as ‘water pills’ that is used for patients with leg swelling or fluid in the lungs. This helps excrete excess water in the body
  • Oxygen: breathing in pure oxygen can help treat pulmonary hypertension
Surgical Procedures:
Surgery is required if medications are unable to control your pulmonary hypertension.
  • Atrial septostomy: an opening is created between the left and right atriums of the heart to relieve the pressure from the right side of the heart.
  • Lung transplant: is a surgery to replace a diseased lung with a healthy lung from a donor. This is reserved for patients with severe lung disease causing pulmonary hypertension.
  • Heart-lung transplant: both the heart and lung are replaced with healthy organs from a donor.

What is the prognosis?

The long term outlook for pulmonary hypertension is poor, yet new treatments may lead to better results.

Death can result in patients with associated heart failure.

Pregnancy is not encouraged for patients with this condition.