Pulmonary Hypertension in the Context of Heart Failure With Preserved Ejection Fraction

Abstract

Heart failure with preserved ejection fraction (HFpEF) is the most common form of heart failure and frequently is associated with pulmonary hypertension (PH). HFpEF associated with PH may be difficult to distinguish from precapillary forms of PH, although this distinction is crucial because therapeutic pathways are divergent for the two conditions. A comprehensive and systematic approach using history, clinical examination, and noninvasive and invasive evaluation with and without provocative testing may be necessary for accurate diagnosis and phenotyping. After diagnosis, HFpEF associated with PH can be subdivided into isolated postcapillary pulmonary hypertension (IpcPH) and combined postcapillary and precapillary pulmonary hypertension (CpcPH) based on the presence or absence of elevated pulmonary vascular resistance. 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 PH for HFpEF associated with PH remain limited. In this review, we aim to provide an updated overview on clinical definitions and hemodynamically characterized phenotypes of PH, pathophysiologic features, therapeutic strategies, and ongoing challenges in this patient population.

Keywords: diastolic heart failure; heart failure with preserved ejection fraction; left heart disease; pulmonary hypertension; right ventricle.

Figure 1

Diagram showing the pathophysiologic mechanisms in HFpEF associated with PH. CpcPH = combined postcapillary and precapillary pulmonary hypertension; HFpEF = heart failure with preserved ejection fraction; IpcPH = isolated postcapillary pulmonary hypertension; LA = left atrial; LAP = left atrial pressure; LV = left ventricle; mPAP = mean pulmonary artery pressure; PAWP = pulmonary artery wedge pressure; PH = pulmonary hypertension; PVR = pulmonary vascular resistance; RAP = right atrial pressure; RV = right ventricle; WU = Wood unit.

Figure 2

A-B, Flow charts showing the hemodynamic assessment of PH resulting from HFpEF. A, Pretest probability of PH resulting from LHD is based on the features presented in Table 1. RHC is recommended in intermediate probability when risk factors of pulmonary arterial hypertension or CTEPH are present, if evidence exists of RV abnormality, or both. If the probability is high, patients should be managed according to recommendations for LHD. B, For the assessment of PH, RHC should be performed at expert centers. In patients with intermediate or high probability (Table 1) and PAWP between 13 and 15 mm Hg, HFpEF associated with PH is not excluded; provocative testing should be considered. ^#For patients with systemic sclerosis, risk factors for CTEPH, unexplained dyspnea, or both. ⁺If PAWP of > 15 mm Hg, LVEDP validation should be considered. CTEPH = chronic thromboembolic PH; HFpEF = heart failure with preserved ejection fraction; LHD = left heart disease; LVEDP = left ventricular end-diastolic pressure; PAWP = pulmonary arterial wedge pressure; PH = pulmonary hypertension; RHC = right heart catheterization; RV = right ventricle. Reproduced with permission of the European Respiratory Society from Eur Respir J. 2019;53(1):1801897. DOI: 10.1183/13993003.01897-2018. Published January 24, 2019. © 2021 European Respiratory Society.