Group 2 pulmonary hypertension: from diagnosis to treatment

Pulmonary hypertension associated with left heart disease (PHLHD) is common and associated with adverse prognosis. Proper diagnosis is critical to avoid inappropriate treatment. Practical approaches to noninvasive diagnosis are available, though invasive hemodynamics including volume loading or exercise are often necessary for definitive diagnosis. Treatment strategies and research in the field is rapidly evolving.

Combined pre and post capillary pulmonary hypertension is associated with remodeling of the pulmonary vasculature that is proportional to the severity of the pulmonary hypertension. Even quite mild elevation of pulmonary vascular resistance (PVR), (greater than 2.0 Wood units (WU)) is associated with adverse outcome. Guideline-directed medical therapy (GDMT) for left heart failure has rapidly evolved, including approval of SGLT2 inhibitors for treatment of heart failure with preserved ejection fraction (HFpEF). However, full implementation of GDMT is lagging. Utilization of implanted pulmonary artery pressure monitoring can facilitate patient management in selected patient phenotypes and has been utilized to demonstrate improvement in pulmonary pressures with emerging therapies in HFpEF including SGLT2 inhibitors and sacubitril/valsartan. A variety of shunt devices to create a left-to-right shunt in order to decompress the left heart are available or undergoing clinical trials. There is concern that there could be adverse response to such devices in patients who have even mild elevation of pulmonary vascular resistance. Sleep disordered breathing is common in PHLHD and should be aggressively sought out and treated.

Even mild elevation in pulmonary vascular resistance is associated with adverse outcome. A systematic approach to diagnosis of PHLHD is essential. Guideline-directed medical therapy for PHLHD prioritizes optimal management of the left heart disease. Pulmonary artery pressure sensors are useful in selected patients.