Pulmonary Hypertension (cont.)
George Schiffman, MD, FCCP
Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.
William C. Shiel Jr., MD, FACP, FACR
Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
In this Article
- Pulmonary hypertension definition and facts
- What is pulmonary hypertension?
- Signs and symptoms of pulmonary hypertension
- Which diseases are common in people with the condition?
- Classification of primary and secondary pulmonary hypertension
- What causes pulmonary hypertension?
- How is pulmonary hypertension diagnosed?
- What is the treatment for pulmonary hypertension?
- Is there a cure?
- What is the life expectancy?
- Which types of doctors treat pulmonary hypertension?
- Find a local Cardiologist in your town
Signs and symptoms of pulmonary hypertension
Many people with pulmonary hypertension may have no symptoms at all, especially if the disease is mild or in the early stages.
Pulmonary hypertension symptoms may include:
- Shortness of breath that worsens with activity
- Other common complaints are cough, fatigue, dizziness, and lethargy.
- With the advancement of the condition and ensuing right heart failure, shortness of breath may become worse and retention of fluid in the body may increase (due to failure of the heart to pump blood forward) resulting in swelling the legs.
- People may also complain of chest pain and angina.
- Depending on the underlying associated disease, pulmonary artery hypertension can have other manifestations. For example, characteristic skin changes seen in scleroderma, or the signs of liver disease seen in portopulmonary hypertension.
Signs of pulmonary hypertension may include:
- Rapid breathing, hypoxia (low oxygen level in the blood), and swelling in the legs.
- In severe pulmonary hypertension, the health care professional may hear louder than normal components of heart sounds when he or she listens to the heart with a stethoscope (auscultation).
- The doctor may also feel elevation of the chest wall when the heart pumps and this may indicate enlargement of the right side of the heart suggestive of pulmonary hypertension (right ventricular heave).
Which diseases are common in people with the condition?
There are no clear statistical data on the prevalence of pulmonary hypertension. Since pulmonary hypertension is seen in multiple different conditions, it is perhaps better to look at the prevalence in each condition. It is fairly common in patients with heart failure, COPD, and sleep apnea. It occurs in 7% to 12% of patients with collagen vascular disease, and in less than 5% of HIV patients.
Classification of primary and secondary pulmonary hypertension
The conventional classification, primary (idiopathic) and secondary pulmonary hypertension, which also is called pulmonary arterial hypertension, has been reclassified and is now is based on the main underlying cause, characteristics, and treatments of pulmonary hypertension. These terms were updated in 2013.This system classifies the condition based on five classes.
Class 1 includes:
Pulmonary arterial hypertension including idiopathic previously referred to as primary pulmonary arterial hypertension), inherited pulmonary arterial hypertension, drug-induced pulmonary arterial hypertension
Pulmonary arterial hypertension associated with the following:
- Connective tissue diseases like systemic lupus erythematosus, or scleroderma
- Liver disease (elevated pressures in the liver, portal hypertension);
- Congenital heart disease
- Schistosomiasis (a parasite infection that can clog the pulmonary arteries)
- Pulmonary Veno-occlusive disease (very rare)
- Persistent pulmonary hypertension of the newborn
Class 2 includes:
Pulmonary hypertension due to left heart disease; an elevation of pulmonary arterial pressure because of increased pulmonary venous pressure, backing up into the lungs. This includes heart failure, valvular heart disease, and some congenital heart disease.
Pulmonary hypertension due to lung diseases and/or hypoxia (low oxygen levels). This includes:
- Chronic obstructive lung disease (COPD)
- Interstitial lung diseases
- Sleep apnea
- Chronic exposure to high altitudes
- Congenital lung disease
Class 4 includes:
Chronic thromboembolic pulmonary hypertension (chronic blood clots to the lungs that don't dissolve).
Class 5 includes:
Pulmonary hypertension with unclear multifactorial mechanisms, for example:
- Blood disorders (including hemolytic anemias)
- Systemic disorders(such as sarcoid)
- Metabolic disorders where cellular chemistry is abnormal (such as glycogen storage diseases)
- Tumors obstructing flow through the lungs
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