Introduction to Primary Pulmonary Hypertension
First recognized by Dr. Ernst Von Romberg in 1891, primary pulmonary hypertension (PPH) is a condition consisting of increased blood pressure in the major blood vessel that takes blood from the right ventricle of the heart to lungs (pulmonary artery). Once in the lungs, blood can be oxygenized and carbon dioxide can be eliminated during exhalation.
Primary Pulmonary Hypertension
Primary pulmonary hypertension has been linked to the use of certain diet drugs, some of which include:
- Fen Phen
- Redux (dexfenfluramine)
- Pondimin (fenfluramine)
Primary pulmonary hypertension is typically considered to be a genetic (familial) condition as it has no underlying cause; often referred to as idiopathic pulmonary hypertension or unexplained pulmonary hypertension for this reason.
A relatively rare disorder, PPH has approximately 500 to 1,000 new cases being diagnosed yearly in the United States. Most PPH sufferers are women aged 20 to 40; however, men, women and children of all ages can develop the condition. Patients suffering from PPH are often asymptomatic until they reach their late-twenties or early-thirties. PPH symptoms tend to come on gradually, beginning with shortness of breath and excessive fatigue, eventually culminating in fainting, peripheral edema (swelling) and angina pectoris.
Secondary Pulmonary Hypertension
Secondary pulmonary hypertension is caused as a result of other disease processes. Some of the common causes of secondary pulmonary hypertension include:
- Chronic obstructive pulmonary disease (COPD)
- Pulmonary embolism
- Pulmonary fibrosis
- Portal hypertension
- Mitral regurgitation
- Systemic sclerosis
- Congenital heart disease
- Asthma / chronic bronchitis
Pulmonary Hypertension Diagnosis & Treatments
Blood pressure in the pulmonary artery is typically between 12 to 16 mmHg. The diagnosis of primary pulmonary hypertension is viewed to be present when blood pressure in the pulmonary artery is about 30% to 50% higher, exceeding 25 mmHg. In order to confirm a suspected case of pulmonary hypertension, a series of diagnostic tests are required, including:
- Blood tests
- Electrocardiography (recording of heart activity)
- Arterial blood gas measurements
- CT scan of the chest
- Lung biopsy (when secondary pulmonary hypertension is suspected)
A complete family medical history will also help to confirm a suspected case of primary pulmonary hypertension as the disease is most often contracted as a result of a genetic predisposition.
There is no cure for primary pulmonary hypertension. Increased arterial blood pressure forces the heart muscles to work harder in order to pump blood into the lungs. Overexertion takes its toll, eventually culminating in right ventricle heart failure and heart attack. A number of PPH treatments are used to help sufferers live with the debilitating condition. Drugs such as vasodilators and anticoagulants can be used to help ease the trauma caused by increased arterial blood pressure.
Vasodilators are drugs that work to open (dilate) the arteries, lowering the blood pressure and relieving the heart muscles of a certain amount of stress. Vasodilators are often effective in the treatment of severe PPH cases and have side effects that can include increased heart rate, headache, and fluid buildup.
Anticoagulants (also called "blood thinners") are drugs that are used to limit the clotting (coagulation) of blood in the lungs. Anticoagulants are often used to help safeguard against heart attack or stroke in pulmonary hypertension sufferers. Side effects of anticoagulant use can include unusual bleeding or bruising, abdominal / stomach pain or swelling, cough, fever, skin rash, difficult urination, chest pain and constipation.