Pulmonary hypertension is a rare blood vessel disorder of the lung. James Carroll, MD, pulmonologist at University of Iowa Hospitals and Clinics, talks more about pulmonary hypertension:
How does pulmonary hypertension develop in the lungs?
Blood enters the right side of the heart with very little oxygen. The right heart pumps the blood to the lungs through pulmonary arteries of the “pulmonary circulation.” In the lungs, oxygen moves into the blood, and carbon dioxide leaves the blood. The oxygen-rich blood returns to the left heart through the pulmonary veins. From the left heart, oxygenated blood is pumped out to the body through “systemic circulation.” In the organs and muscles of the body, oxygen is extracted. Oxygen poor blood returns to the right heart by veins to complete the circuit.
Pulmonary hypertension is high blood pressure inside the lungs. Normally, the blood pressure within the lungs is very low (15/5 mmHg). In comparison, the “systemic” blood pressure (measured by a blood pressure cuff on your arm) is normally ten times higher (120/80 mmHg).
Are there different types of pulmonary hypertension?
Yes. Several types of pulmonary hypertension are known. The current classification system was developed by the World Health Organization in 2003. This system distinguishes types of pulmonary hypertension based on the broad underlying cause.
The five categories include:
- Pulmonary arterial hypertension
- Pulmonary venous hypertension
- Pulmonary hypertension associated with low oxygen levels
- Pulmonary hypertension due to repeated blood clots
- Pulmonary hypertension due to compression of the blood vessels
Who is most likely to develop pulmonary hypertension?
This is a difficult question to answer, due to differences in the five categories of pulmonary hypertension. Pulmonary hypertension can be triggered by certain medical conditions or medications, but the exact cause is unknown.
Is pulmonary hypertension a genetic disorder?
There is an inherited version of group 1 pulmonary hypertension, known as “Familial pulmonary arterial hypertension.” This is caused by a mutation in the “bone morphogenetic protein receptor 2” gene. This mutation is found in up to 10 percent of people with Group 1 pulmonary arterial hypertension.
What are the symptoms?
Symptoms of pulmonary hypertension are non-specific. They include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells, and fainting.
How is pulmonary hypertension diagnosed?
Several tests, including cardiac catheterization of the right heart and pulmonary blood vessels, is required to diagnose pulmonary hypertension. Clues leading can be found by a careful medical history and detailed physical examination.
Why is it easy to misdiagnose pulmonary hypertension?
The symptoms of fatigue and shortness of breath are common and non-specific. Health care providers are trained to consider the most likely causes of symptoms such as asthma or heart disease, before thinking about rare diseases such as pulmonary hypertension.
What treatment options are available?
Several medications have been developed in the last decade to treat pulmonary hypertension. These include oral, inhaled, and intravenous therapies. Background therapies are also important, including the use of oxygen, diuretics, and blood thinners.
Can pulmonary hypertension be cured?
Pulmonary hypertension can be a challenging condition to live with. Currently there is no known cure for pulmonary hypertension. Because of this, involvement in a support group is essential. There are currently over 100 support groups sponsored through the Pulmonary Hypertension Association.
Is University of Iowa Hospitals and Clinics currently involved in any research with regard to pulmonary hypertension?
Yes. We are involved in several multi-centered clinical trials for new treatments of pulmonary hypertension. We also participate in national registries designed to increase our knowledge of the causes of pulmonary hypertension. |
