What is it?
High blood pressure in the arteries that supply the lungs is called pulmonary hypertension (PHT). The blood pressure measured by cuff on your arm isn't directly related to the pressure in your lungs. The blood vessels that supply the lungs constrict and their walls thicken, so they can't carry as much blood. As in a kinked garden hose, pressure builds up and backs up. The heart works harder, trying to force the blood through. If the pressure is high enough, eventually the heart can't keep up, and less blood can circulate through the lungs to pick up oxygen. Patients then become tired, dizzy and short of breath.
If a pre-existing disease triggered the PHT, doctors call it secondary PHT. That's because it's secondary to another problem, such as a heart or lung disorder. Congenital heart disease can cause PHT.
The most common symptoms of primary pulmonary hypertension or pulmonary arterial hypertension are related to difficulty with breathing. They include:
These symptoms may occur at presentation, or progress over a period of time. A patient with primary pulmonary hypertension must be monitored for these symptoms by routine visits to their cardiologist or pulmonologist. If any new symptoms develop, patients should notify their doctor immediately.
A physical examination of patients with primary pulmonary hypertension may show:
All of these signs are related to the right ventricle working harder to pump blood into the high resistance of the lungs' blood vessels.
PPH remains a diagnosis of exclusion. This means that it is diagnosed only after the doctor finds pulmonary hypertension and excludes or cannot find other reasons for the hypertension, such as a chronic obstructive pulmonary disease (chronic bronchitis and emphysema), blood clots in the lung (pulmonary thromboemboli), or some forms of congenital heart disease
Cardiac Catheterization. The gold standard for making the diagnosis of primary pulmonary hypertension is a cardiac catheterization. This will actually measure the pressures on the right side of the heart and calculations can be made to measure the resistance of the blood vessels in the lungs. Various drugs may be used during a cardiac catheterization to determine if the blood vessels in the lungs will relax in response to them. This may lower the pressures in the lungs and can help guide therapy.