Chest. 2021 Aug 12:S0012-3692(21)03666-7. doi: 10.1016/j.chest.2021.08.039. Online ahead of print.
Heart failure with preserved ejection fraction (HFpEF) is the most common form of heart failure and is frequently associated with pulmonary hypertension (PH). PH-HFpEF may be difficult to distinguish from pre-capillary forms of PH, though this distinction is crucial as therapeutic pathways are divergent for the two conditions. A comprehensive and systematic approach utilizing history, clinical exam, non-invasive and invasive evaluation with and without provocative testing may be necessary for accurate diagnosis and phenotyping. Once diagnosed, PH-HFpEF can be subdivided into isolated post-capillary pulmonary hypertension (IpcPH) and combined post- and pre-capillary pulmonary hypertension (CpcPH) based on the presence or absence of elevated pulmonary vascular resistance (PVR). CpcPH portends a worse prognosis than IpcPH. Despite its association with reduced functional capacity and quality of life, heart failure hospitalizations, and higher mortality, therapeutic options focused on pulmonary hypertension for PH-HFpEF remain limited. In this review, we aim to provide an updated overview on clinical definitions and hemodynamically characterized phenotypes of PH, pathophysiology, therapeutic strategies, and ongoing challenges in this patient population.