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Right-sided heart failure is the most common cause of death in pulmonary hypertension (PH). Echocardiographic measurements of right atrial (RA) size are associated with worse outcome in PH, however the association between RA function and death in PH has not been well-described. 160 PH patients (World Health Organization groups 1-5) underwent cardiac magnetic resonance imaging (cMRI) and right heart catheterization (RHC) within 6 weeks of each other at a tertiary care academic medical center in the United States. We measured cMRI RA maximum and minimum volumes indexed to body surface area and calculated RA emptying fraction (RAEF). We evaluated the relationship between RAEF and clinical variables with death using Cox proportional hazard models. 57 deaths occurred during a median follow-up of 3.5 years (36 % died overall, 10 % per year). RAEF was directly correlated in univariate analyses with right ventricular (RV) ejection fraction, left ventricular (LV) ejection fraction, LV size, cardiac index, absence of tricuspid and pulmonic regurgitation, absence of pericardial effusion, estimated glomerular filtration rate, 6-minute walk distance, and pulmonary arterial oxygen saturation, whereas it was inversely correlated with death, BNP, heart rate, mean RA pressure, mean PA pressure, pulmonary and systemic vascular resistance, RV size, and RA size. Using multivariate analyses, RAEF had a robust inverse association with death after adjusting for measured risk factors (HR per 5 % change in RAEF: 0.83 [95 % CI 0.73-0.94], p = 0.003). In PH patients, decreased RAEF by cMRI is independently associated with worse survival after adjustment for other risk factors.

In the last decade, the role of the right ventricle (RV) has been increasingly recognized in a variety of conditions, contributing to the pathophysiology of disease and the prediction of outcomes. Recent echocardiography guidelines focused on the RV have been published by the American Society of Echocardiography to encourage a standardized approach in assessing RV size and function. In this article, we review the recently published echocardiog- raphy guidelines for assessing RV size and function, and their importance in clinical practice. We discuss advantages and disadvantages of currently available imaging techni- ques for evaluating the RV morphology, size, and systolic function. Basic methods such as TAPSE, tissue Doppler, RIMP, and fractional area change are discussed, as are more emerging techniques such as strain and strain rate. Additional insights are provided into upcoming uses of echocardiography in the areas or RV dyssynchrony and three-dimensional echocardiography.

Chronic Thromboembolic Pulmonary Hypertension(CTEPH) is the only pulmonary hypertension group with a cure. 50-year-old male presented with disabling dyspnea. Right heart catheterization(RHC) showed pulmonary artery pressure(PAP) 90/30 mm Hg (mean 51 mm Hg), pulmonary capillary wedge pressure(PCWP)