Distinct approach necessary for pulmonary hypertension from left heart disease

Roxana Sulica, MD
Roxana Sulica, MD

All pulmonary hypertension (PH) is not created equal. Group I PH is defined as pulmonary arterial hypertension (PAH) due to pulmonary vascular and right ventricular remodeling that can be readily diagnosed and treated. Group II PH stems from left heart disease and can be more difficult to diagnose and to treat.

“Group II is the most common type of PH and is not a uniform disease,” said Roxana Sulica, MD, associate professor and director of the pulmonary hypertension program at the New York University Grossman School of Medicine. “Various pathological processes of the left side of the heart may lead to the development of pulmonary hypertension, which has a much more cumbersome management. If you don’t do a very thorough workup, you may misdiagnose the patient as having PAH, give them PH-specific therapy, and run into complications like pulmonary edema.”

Dr. Sulica will chair the CHEST 2022 session, Pulmonary Hypertension in Patients With Left Heart Disease: A Tale of Two Hearts, Sunday, October 16, from 1:00 PM – 2:00 PM CT, in Room 104BC. She will discuss a proposed management algorithm for PH due to left heart disease with three experts.

Namita Sood, MBBCh, FCCP, director of the advanced lung disease program and pulmonary hypertension clinical trials at the University of California, Davis, will focus on the clinical presentation of PH due to left heart disease.

Anjali Vaidya, MD, professor of medicine at Temple University and co-director of the pulmonary hypertension, right heart failure, and chronic thromboembolic pulmonary hypertension program at Temple University Hospital, will explore the diagnostic challenges and novel strategies to distinguish Group II from Group I PH.

Ronald Oudiz, MD, director of the Liu Center for Pulmonary Hypertension at The Lundquist Institute and Professor of Medicine at the University of California, Los Angeles, will discuss current and future directions in the management of PH due to left heart disease.

“The standard assumption in Type II PH is that the left ventricle is not contracting properly, which you can easily see on echocardiograms, or there are valve malfunctions,” Dr. Sulica said. “The real problem is when you have heart failure with preserved ejection fraction, which is more difficult to diagnose by echocardiogram and more difficult to manage and can lead to pulmonary hypertension. At least 70% of new referrals I’m getting are patients with this type of pulmonary hypertension.”

It is not difficult to distinguish between the different types of PH, she said, but only if clinicians know to look and have access to the appropriate equipment. Exercise testing that includes specific, provocative maneuvers at the time of right heart catheterization can help. So can magnetic resonance imaging or a thorough and properly done echocardiogram.

Unfortunately, there are not many good management alternatives for PH with preserved ejection fraction beyond diuresis. There have been few clinical trials in this population, and results have not been promising.

There may be some potential for sodium-glucose cotransporter-2 inhibitors, originally developed to manage diabetes. Type 2 diabetes trials have shown survival benefits for patients who have heart failure with preserved ejection fraction, but there have been no randomized trials in patients with Group II PH.

“There are a lot of controversial ideas circulating out there because the management is so challenging, and these patients are so very complex,” Dr. Sulica said. “I work very closely with interventional cardiologists and surgeons because while you may have pulmonary hypertension, the root of the disease is on the other side of the interventricular septum. Ideally, you should first fix everything you can on that side, and even then, you can run into trouble when you try to apply medications specifically designed for use in PAH.”