Pulmonary Hypertension

High blood pressure in the arteries that supply the lungs is called pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH). The blood pressure measured by a 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.

Once pulmonary hypertension has been diagnosed, often more medical therapy is needed. You'll require regular follow-up with a cardiologist or pulmonologist trained in caring for patients with this condition.

As long as the underlying disease exists, it will keep causing PAH. Once you have PAH (especially if you've had it for some time), curing the disease that caused it may not make the high blood pressure go away. In that case, the PAH will usually need special treatment. While PH is a serious illness, treatments are available. You may be treated with oxygen, agents to help your heart pump better, diuretics, anticoagulants (blood thinners) and medications to lower your PAH and reverse the scarring in the blood vessels in the lung. Sometimes lung transplants also are done for PAH, but in congenital heart disease, usually heart and lung transplantation is required.

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