Kelly Chin, M.D. Answers Questions On: Pulmonary Hypertension

There are several categories of pulmonary hypertension. The first group has several subgroups within it, including connected tissue disease, congenital heart disease, and idiopathic (unknown cause). All three of those are treated with the same medications.

The other groups are hypertension related to left heart disease like heart failure; pulmonary hypertension related to lung disease like COPD and interstitial lung disease; and pulmonary hypertension from chronic pulmonary embolism.

After we determine what type of pulmonary hypertension it is, we know how best to treat it. That's important because if you start the wrong treatment, patients can waste money on expensive medications and they aren't going to get better.

It affects mainly women. And it hits women hardest between the ages of 30 and 50.

In the idiopathic form, we see a genetic predisposition in about a quarter of patients. We don't know why the remainder of patients get it. It's probably some combination of a predisposition and maybe something in the environment.

Many patients have connective tissue diseases, like scleroderma and lupus, while others have adult congenital heart disease. If congenital heart disease is repaired in infancy, that usually solves the problem. But if it's missed or repaired much later, patients can develop irreversible elevations in pulmonary pressure, and closing it won't fix it at that point.

IV therapy is our most potent therapy, but it also asks a lot from patients. They carry around a pump 24 hours a day, and they have an IV line in. Because of this, I frequently have to convince my patients that quality of life actually goes up when they use IV therapy.

Patients often get so focused on the negatives  – the pump and the line – that they sometimes don't think they're going to benefit from the upside, which is less shortness of breath, more energy, and being able to do the kinds of things they want to do.