Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis

Groote, Pascal de; Gressin, Virginie; Hachulla, É.; Carpentier, Patrick; Guillevin, Loı̈c; Kahan, André; Cabané, J.; Françès, Camille; Lamblin, Nicolas; Diot, Élisabeth; Patat, F.; Sibilia, Jean; Petit, H.; Jl, Cracowski; Clerson, Pierre; Humbert, Marc; Investigators, ItinerAIR-Scleroderma

doi:10.1136/ard.2006.057760

Cited by 138 publications

(92 citation statements)

References 34 publications

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“…5 Additionally, SSc often causes left ventricular (LV) hypertrophy, nonsystolic LV dysfunction, and left atrial enlargement. 37 Overall, the cumulative infl uence of SSc on the heart may impair the heart's ability to compensate against increasing PVR in SSc-PAH. Our group has shown that despite similar hemodynamic indices, patients with SSc-PAH have higher mortality and are less responsive to therapy than patients with IPAH.…”

Section: Cardiac Involvement In Ssc-pahmentioning

confidence: 99%

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Pavec

Humbert

Mouthon

et al. 2010

Am J Respir Crit Care Med

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Systemic sclerosis (SSc) is a complex, multisystem disease characterized by fi brosis and excessive collagen deposition within the skin and internal organs, chronic infl ammation, autoimmune dysregulation, and microvascular endothelial dysfunction. There are two forms of SSc that are characterized by the extent of skin involvement. Limited cutaneous SSc predominantly involves the skin of the hands, arms, and face, and diffuse SSc involves large areas of skin and multiple organs. 1 Both forms are systemic diseases associated with signifi cant morbidity and mortality. Historically, mortality was largely caused by renal disease. With the advent of angiotensin-converting enzyme inhibitors to treat SSc renal crisis, SSc-associated pulmonary arterial hypertension (SSc-PAH) has emerged as a leading cause of morbidity and mortality, accounting for up to 30% of premature deaths. 2 Although SSc-PAH represents a signifi cant proportion of the pulmonary arterial hypertension (PAH) population (15%-30%), 3,4 much of what we know about PAH is derived from studies of idiopathic PAH (IPAH).Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc ) and affects up to 12% of all patients with SSc, with a 50% mortality rate within 3 years of PAH diagnosis. Compared with the idiopathic form of PAH (IPAH), patients with SSc-associated PAH (SSc-PAH) have a threefold increased risk of death and may receive a diagnosis late in the course of disease because of insidious onset and the high prevalence of cardiac, musculoskeletal, and pulmonary parenchymal comorbidities. Treatment with conventional forms of PAH therapy often yield poor results compared with IPAH cohorts; unfortunately, the exact reasons behind this remain poorly understood but likely include variations in the pathologic mechanisms, differences in cardiovascular response to increasing afterload, and inadequate strategies to detect and treat SSc-PAH early in its course. Current methods for screening and longitudinal evaluation of SSc-PAH, such as the 6-min walk test, transthoracic echocardiography, and MRI, each have notable advantages and disadvantages. We provide an up-to-date, focused review of SSc-PAH and how it differs from IPAH, including pathogenesis, appropriate screening for disease onset, and new approaches to treatment and longitudinal assessment of this disease. CHEST 2013; 144(4):1346-1356Abbreviations: 6MWD 5 6-min walk distance; CCB 5 calcium channel blocker; CTD 5 connective tissue disease; D lco 5 diffusing capacity of lung for carbon monoxide; EIPAH 5 exercise-induced pulmonary arterial hypertension; ERA 5 endothelin receptor antagonist; FC 5 functional class; ILD 5 interstitial lung disease; IPAH 5 idiopathic pulmonary arterial hypertension; LV 5 left ventricular; mPAP 5 mean pulmonary artery pressure; PAH 5 pulmonary arterial hypertension; PASP 5 pulmonary artery systolic pressure; PDE-5I 5 phosphodiesterase type 5 inhibitor; PFT 5 pulmonary function test; PH 5 pulmonary hypertension; pro-BNP 5 pro-brain natriuretic pe...

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Section: Cardiac Involvement In Ssc-pahmentioning

confidence: 99%

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Pavec

Humbert

Mouthon

et al. 2010

Am J Respir Crit Care Med

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“…The presence of diastolic dysfunction in patients with SSc, detected by the majority of previous studies [19][20][21], was commonly demonstrated by pulsed wave Dopplerderived transmitral inflow patterns. However, the use of TDI, less influenced by loading conditions, suggests that the Em determined by pulsed TDI is more reliable than conventional transmitral Doppler flow [22].…”

Section: Discussionmentioning

confidence: 93%

Contractile reserve in systemic sclerosis patients as a major predictor of global cardiac impairment and exercise tolerance

Cadeddu

Deidda

Giau

et al. 2014

Int J Cardiovasc Imaging

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Several studies have evidenced high prevalence of myocardial systolic and diastolic dysfunction among patients with systemic sclerosis (SSc). Exercise echocardiography has shown a diagnostic and prognostic role in identifying early left ventricular (LV) dysfunction in several myocardial pathological settings. The aim of our study was to evaluate early signs of LV impairment under exercise and their correlation to patient's exercise tolerance. Forty-five patients (age 60.4 ± 10.3 years) with SSc and 20 age and sex comparable controls were enrolled in the study. All patients underwent clinical evaluation, 2D echocardiography associated with Tissue Doppler and speckle tracking to evaluate LV deformation indexes, and an exercise echocardiography to evaluate left ventricle contractile reserve (LVCR) and exercise pulmonary pressures. Finally, a 6-minute walking test (6MWT) to evaluate exercise tolerance was also performed. Compared to controls, SSc patients showed an impaired diastolic function (E/E' 10.9 ± 3.7 vs 8.36 ± 2.01; p < 0.01) associated with larger left atrial dimensions (LAVI 28.4 ± 8.7 vs 19.3 ± 4.6 mL/m(2); p < 0.01). During exercise echocardiography, a reduced global longitudinal strain at peak exercise (S-GLS) was highlighted compared to controls (15.7 ± 3.6 vs 18.2 ± 2.2; p = 0.001). A S-GLS cutoff <18%, identified by ROC analysis, identified SSc patients with a reduced diastolic function, exercise tolerance at the 6MWT and higher pulmonary pressures. Our data show that in SSc patients a reduced LVCR characterizes the patients with a more extensive cardiovascular impairment in terms of LV diastolic function, pulmonary pressures and exercise tolerance. These data underline the importance of exercise echocardiography for the preclinical screening of the LV impairment in this population.

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“…The earliest, most frequent and characteristic echocardiographic feature of SHD is the left ventricular diastolic dysfunction, and multiple symptoms of SSc patients (including decreased exercise capacity, dyspnoea, and decompensation) are strongly related to LV filling pressure. Its prevalence ranges from 17.7% in a large cohort using traditional Doppler echocardiography11 to 63% in the latest studies performing tissue Doppler echocardiography 2. Left ventricular diastolic dysfunction is related to the degree of myocardial fibrosis and is inversely and independently associated to the survival of the patients 12…”

Section: Discussionmentioning

confidence: 99%

Autoimmune myocarditis in systemic sclerosis: an unusual form of scleroderma heart disease presentation

et al. 2017

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Primary cardiac involvement in systemic sclerosis is common, often subclinical, and is associated with significant mortality. We report the case of a patient who developed autoimmune myocarditis at an early stage of systemic sclerosis, who completely recovered from cardiac dysfunction under optimal medical therapy for heart failure and immunosuppression. This challenging case aims at increasing awareness around the fact that the heart is a target organ of scleroderma disease. It also highlights the importance of screening and early diagnosis of cardiac involvement, because a timely treatment may impact the quality of life of these patients and improve their prognosis.

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Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis

Cited by 138 publications

References 34 publications

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Contractile reserve in systemic sclerosis patients as a major predictor of global cardiac impairment and exercise tolerance

Autoimmune myocarditis in systemic sclerosis: an unusual form of scleroderma heart disease presentation

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