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.

• 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)

• 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

• 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

• Idiopathic Pulmonary Hypertension (IPH)

• Secondary Pulmonary Hypertension

• Pulmonary Embolism
• COPD
• Pulmonary fibrosis
• Connective tissue disorders
• Congenital heart defects
• Sickle cell anemia
• AIDS
• Cirrhosis
• High altitude
• Drugs including cocaine, diet pills

• 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

• Shortness of breath during physical activity
• Fatigue
• Chest pain
• Or other symptoms

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

• Shortness of breath
• Angina (chest pain)
• Fatigue/ tiredness
• Upper right quadrant abdominal pain
• Palpitations (racing heart beat)

• Cyanosis- bluish discoloration of the skin and lips
• Weak pulse may be felt
• Distended neck veins
• During auscultation, a murmur may be heard

• 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.

• 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

• 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.

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.