Tips on recognizing HFpEF, a common pulmonary arterial hypertension mimic

Pulmonary arterial hypertension (PAH) is a familiar condition with familiar treatment approaches for pulmonologists. But what if the culprit isn’t PAH? Heart failure with preserved ejection fraction (HFpEF) is the most common mimic of PAH, and PAH-specific vasodilator therapies can adversely affect HFpEF.

Getting the diagnosis wrong means getting the treatment wrong and potentially harming your patient, said Veronica Franco, MD, MSPH, Head of Pulmonary Hypertension Research at The Ohio State University Wexner Medical Center. Half of all heart failure patients admitted to the hospital have preserved ejection fraction, and the prevalence of HFpEF is increasing.

The diagnostic problem is that PAH and HFpEF share multiple clinical signs and symptoms. There is no single test that can distinguish between the two.

“If someone has heart failure with low left ventricular ejection fraction, that’s easy to distinguish from pulmonary arterial hypertension because PAH has a normal ejection fraction,” Dr. Franco said. “But when your patient has a normal ejection fraction, you have to look more carefully. Sometimes, cardiac catheterizations done after several days of IV diuresis may show a normal wedge pressure. The real key for pulmonologists is just remembering to check for HFpEF. If you don’t think about it, you won’t see it.”

Dr. Franco will chair a case-based discussion on Recognizing and Treating HFpEF: the Biggest Pulmonary Arterial Hypertension Imitator on Tuesday at 2:45 pm CT. The panel will focus on clinical signs and diagnostic right-sided heart catheterization (RHC) maneuvers that can help distinguish between the two common conditions and appropriate diuretic management for HFpEF.

Heart failure scores can help distinguish between PAH and HFpEF, but the differential diagnosis relies on multiple factors, not any single score. Patients with HFpEF tend to be older, more often women, and have more cardiovascular comorbidities such as hypertension, diabetes, obesity, and coronary artery disease compared with patients with PAH.

These simple clinical characteristics can reliably distinguish between the two conditions. But again, only if you look for them, Dr. Franco emphasized.

There are also several maneuvers that can be done during RHC that can confirm the diagnosis. Saline loading or exercise during RHC typically shows an abnormal response in pulmonary capillary wedge pressure in patients with HFpEF vs PAH.

Treatment approaches are also different. Vasodilators are the typical first-line treatment for PAH, while high-dose diuretics are the preferred approach for HFpEF. Diuretic dosing in pulmonology and critical care is typically lower than in heart failure, which can leave pulmonologists feeling uncomfortable and uncertain with unfamiliar dosing regimens.

“We tend to be quite aggressive with diuretics in heart failure,” Dr. Franco said. “If you dose under our norms, you may find that they are not working. During the session, we will share some of the tips and tricks that we use on a daily basis to pull excess fluid out with our heart failure patients.”

Recognizing HFpEF and treating it appropriately makes an important difference in patient outcomes, she said. Inappropriate use of diuretics and failing to reduce the fluid load leaves HFpEF patients with a 20% increased risk of readmission within the next 30 days.

“HFpEF is increasing faster than PAH because our population is getting older and they are getting more obese,” Dr. Franco said. “Both of those are important risk factors for HFpEF. We can all expect to see more HFpEF in the coming years.”