Background Right heart dysfunction and pulmonary artery hypertension (PAH) have been reported in almost every connective tissue disorder; 1 of 6 patients with systemic erythematous lupus (SLE) will develop it during their lifetime and sometimes it precedes the diagnostic of the disease. The presence of PAH is a consequence of endothelial dysfunction and smooth muscle proliferation, and damage of the pulmonary vasculature, mechanisms involving inflammation and autoimmunity, and is associated with an increased rate of morbidity and mortality1,2.
Objectives The aim of this pictorial review is to illustrate the most common echocardiographic changes in patients with different activity indexes of the disease (SLEDAI) focusing on right ventricle changes evaluated either directly through tricuspid regurgitation or using indirect markers such as inferior vena cava inspiratory collapse and tricuspid annulus systolic excursion.
Methods We present a pictorial review of the right heart echocardiographic findings of SLE patients treated in our Internal Medicine Department, which benefit from a thorough cardiac evaluation for each clinical visit, each of them undergoing echocardiography, including M-mode, 2-D conventional Doppler and TDI assessment.
Results We selected patients with different SLEDAI for which we estimated the pulmonary artery pressure (PAP) using the transtricuspid gradient, RA area (RAA), inferior vena cava inspiratory collapse (IVC), and tricuspid annular plane systolic excursion (TAPSE); The left ventricle ejection fraction (LVEF) was assessed using the modified biplane Simpson's equation in the apical 4- and 2-chamber views. The most common findings are mild-moderate PAH, right ventricular wall hypertrophy, diastolic impairment and valvular lesions with a high incidence of regurgitation – observations consistent with the published data3.
Conclusions It has been stated that SLE disease activity is related to the characteristics of PAH4 and sometimes PAH may be diagnosed first, thus leading to a clinical dilemma: should we take into consideration for a cardiologist to further referral of a patient to rheumatologic consultation in an unexplained case of PAH?.
Xia YK, Tu SH, Hu YH et al. Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population. Rheumatol Int 2013; 33:1211–1217
Yang X, Mardekian J, Sanders KN Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature. Clin Rheumatol 2013; 32:1519–1531
Zuily S, Regnault V, Selton-Suty C Increased Risk for Heart Valve Disease Associated With Antiphospholipid Antibodies in Patients With Systemic Lupus Erythematosus Meta-Analysis of Echocardiographic Studies. Circulation. 2011; 124:215-224
Tian Z, Liu Y, Xu D et al. Disease Activity Is Related to Acute Response to Vasodilator in Pulmonary Artery Hypertension Associated With Systemic Lupus Erythematosus. Circulation Journal 2014;78.
Disclosure of Interest None declared
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