A 70-year-old woman with two very high-risk health conditions – heart valve disease and pulmonary hypertension – recently came to see us.

Both problems were triggered by a chronic autoimmune disease called scleroderma, a skin condition that can cause the skin to tighten or become thick and rigid, losing elasticity. Scleroderma comes with numerous symptoms and complications, and can affect any part of the body – in our patient's case, her heart and lungs.

She had been managing her heart valve disease for years. Specifically, she had aortic stenosis, meaning that her aortic valve was narrowed, causing congestive heart failure on the left side of her heart. In congestive heart failure, fluid builds up, and there is poor blood flow throughout the body because the heart can't pump efficiently.

But, the subsequent pulmonary hypertension diagnosis came as a surprise to her after she was brought into the emergency room with shortness of breath, fatigue, and weakness. Pulmonary hypertension is high blood pressure in the arteries in the lung, which, if untreated, can result in right heart failure – and our patient already was having problems with the left side of her heart.

Unfortunately, up to 15 percent of scleroderma patients can develop pulmonary hypertension, not good news for a patient already coping with congestive heart failure from aortic stenosis, as well.

Which condition should be treated first to best protect her heart and her overall health?


After the patient's cardiologist referred her to us, we came up with a treatment plan to address this double dose of high-risk medical conditions.

First, we knew we needed to improve her aortic stenosis with a balloon valvuloplasty in order to stretch the narrowed aortic valve. That procedure relieved some of her congestive heart failure, so we could safely administer medication to treat the pulmonary hypertension. This was critical, as when there is already left heart failure, medical therapy to treat pulmonary hypertension can make the congestive heart failure even worse.

Four weeks later, the patient came back to Temple so we could more permanently and safely treat her aortic stenosis. She underwent a minimally invasive surgical procedure called a transcatheter aortic valve replacement or TAVR to replace the faulty aortic valve.

We can now aggressively treat her pulmonary hypertension and her prognosis looks positive, a true testament to the value of a multidisciplinary approach.

Anjali Vaidya, M.D., is associate director of the Pulmonary Hypertension, Right Heart Failure, and Pulmonary Thromboendarterectomy Program at Temple University Hospital, and assistant professor of Medicine at the Lewis Katz School of Medicine at Temple University. Brian O'Neill, M.D., is assistant professor of Medicine at the Lewis Katz School of Medicine.