Definitions and diagnosis of pulmonary hypertension

Abstract

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure ≥ 25 mm Hg at rest, measured during right heart catheterization. There is still insufficient evidence to add an exercise criterion to this definition. The term pulmonary arterial hypertension (PAH) describes a subpopulation of patients with PH characterized hemodynamically by the presence of pre-capillary PH including an end-expiratory pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg and a pulmonary vascular resistance >3 Wood units. Right heart catheterization remains essential for a diagnosis of PH or PAH. This procedure requires further standardization, including uniformity of the pressure transducer zero level at the midthoracic line, which is at the level of the left atrium. One of the most common problems in the diagnostic workup of patients with PH is the distinction between PAH and PH due to left heart failure with preserved ejection fraction (HFpEF). A normal PAWP does not rule out the presence of HFpEF. Volume or exercise challenge during right heart catheterization may be useful to unmask the presence of left heart disease, but both tools require further evaluation before their use in general practice can be recommended. Early diagnosis of PAH remains difficult, and screening programs in asymptomatic patients are feasible only in high-risk populations, particularly in patients with systemic sclerosis, for whom recent data suggest that a combination of clinical assessment and pulmonary function testing including diffusion capacity for carbon monoxide, biomarkers, and echocardiography has a higher predictive value than echocardiography alone.

Keywords: CO; CTD; DLCO; HFpEF; HPAH; IPAH; LVEDP; N-terminal pro–B-type natriuretic peptide; NT-proBNP; PAH; PAPm; PAWP; PH; PVR; RHC; SSc; WU; Wood units; cardiac output; connective tissue disease; diagnosis; diffusion capacity for carbon monoxide; heart failure with preserved ejection fraction; hemodynamics; heritable pulmonary arterial hypertension; idiopathic pulmonary arterial hypertension; left ventricular end-diastolic pressure; mean pulmonary artery pressure; pulmonary arterial hypertension; pulmonary artery wedge pressure; pulmonary hypertension; pulmonary vascular resistance; right heart catheterization; scleroderma; screening.