Sclerodermic Cardiomyopathy—A State-of-the-Art Review

Giucă, Adrian; Gegenava, T; Mihai, Carmen Marina; Jurcuţ, Ciprian; Săftoiu, Adrian; Girnita, Diana M.; Popescu, Bogdan A.; Marsan, Nina Ajmone; Jurcuţ, Ruxandra

doi:10.3390/diagnostics12030669

Cited by 4 publications

(4 citation statements)

References 102 publications

(261 reference statements)

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“…Patchy myocardial fibrosis is considered the hallmark of myocardial involvement in SSc. It is present in both ventricles and does not correlate with the distribution of coronary arteries [ 139 , 146 ]. Histopathological patterns of the myocardium involvement in SSc are described in detail in Table 6 , from the first reported autopsies to the most recent endomyocardial biopsies.…”

Section: Cardiac Muscle Involvementmentioning

confidence: 99%

“…For a proper diagnosis of myocardial involvement in SSc, various investigations are being used to assess cardiac injury, such as cardiac biomarkers, electrocardiography, transthoracic echocardiography, cardiac magnetic resonance (CMR), nuclear techniques, or endomyocardial biopsy [ 139 , 144 , 146 ]. CMR is one of the most accurate non-invasive and non-irradiating methods used to evaluate myocardial inflammation and fibrosis, being able to identify cardiac disease through specific indexes and measurements.…”

Section: Cardiac Muscle Involvementmentioning

confidence: 99%

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The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

Bratoiu

Burlui

Cardoneanu

et al. 2022

IJMS

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Systemic sclerosis (SSc) is a complex autoimmune disease characterized by heterogeneous changes involving numerous organs and systems. The currently available data indicate that muscle injury (both smooth and striated muscles) is widespread and leads to significant morbidity, either directly or indirectly. From the consequences of smooth muscle involvement in the tunica media of blood vessels or at the level of the digestive tract, to skeletal myopathy (which may be interpreted strictly in the context of SSc, or as an overlap with idiopathic inflammatory myopathies), muscular injury in scleroderma translates to a number of notable clinical manifestations. Heart involvement in SSc is heterogenous depending on the definition used in the various studies. The majority of SSc patients experience a silent form of cardiac disease. The present review summarizes certain important features of myocardial, as well as smooth and skeletal muscle involvement in SSc. Further research is needed to fully describe and understand the pathogenic pathways and the implications of muscle involvement in scleroderma.

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Section: Cardiac Muscle Involvementmentioning

confidence: 99%

Section: Cardiac Muscle Involvementmentioning

confidence: 99%

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

Bratoiu

Burlui

Cardoneanu

et al. 2022

IJMS

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“…In SSc patients, the presence of left ventricular (LV) remodeling and dysfunction are well-studied to date [ 5 ]. In particular, studies which used a two-dimensional speckle tracking echocardiography (2DSTE) were able to describe cardiac deformation and detect early contraction abnormalities [ 3 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Right Ventricular Morphology and Function after Exercise Training in People with Systemic Sclerosis: A Randomized Controlled Pilot Study

Anifanti

Teloudi

Mitropoulos

et al. 2023

Life

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Background: Vascular dysfunction and its concomitant multi-organ involvement, including cardiac involvement, affects prognosis in systemic sclerosis (SSc) patients. Regular exercise has demonstrated to be able to improve vascular function in SSc. However, the effects of an exercise program on the heart and specifically in right ventricular (RV) morphology and function in SSc have yet to be explored. The study aimed to examine whether a 3-month combined exercise program can affect RV morphology and function in SSc patients. Methods: Twenty-eight SSc patients were randomly allocated to either the exercise training (ET) or the control (CON) group. Baseline and follow-up assessments consisted of a cardiopulmonary exercise test along with both a conventional and a two-dimensional speckle tracking echocardiography (2DSTE) focused on RV morphology and function. Following the baseline assessments, Group ET participated in a supervised combined exercise program for 12 weeks, while group CON received their usual care. Results: The ET group demonstrated increases in peak oxygen consumption by 25.1% (p < 0.001), global RV free wall longitudinal systolic strain by 6.69% (p < 0.03), RV free wall longitudinal systolic strain of the basal segment by 13.5% (p < 0.001), and global RV four-chamber longitudinal systolic strain by 6.76% (p < 0.03) following the exercise program. No differences were observed in group CON. Conclusions: Combined exercise improved cardiorespiratory efficiency and indices of RV systolic function, as assessed by the 2DSTE, in SSc patients.

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“…Currently, cardiac complications are a prominent cause of mortality in SSc patients 1 – 4 . Although there has been no standard consensus regarding the definition of primary cardiac complication in SSc, it is assumed that it is a direct consequence of SSc which involves the pericardium, myocardium, conduction systems, valvular structure 5 – 7 and coronary arteries 8 . Whereas, secondary cardiac complications in SSc are usually a result of pulmonary hypertension (PH) and renal involvement 6 .…”

Section: Introductionmentioning

confidence: 99%

Predictors and survival of cardiomyopathy determined by echocardiography in Thai patients with early systemic sclerosis: an inception cohort study

Wangkaew

Prasertwitayakij

Intum

et al. 2023

Sci Rep

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Available data including the incidence, predictors and long-term outcome of early systemic sclerosis patients associated with suspected cardiomyopathy(SSc-CM) is limited. Therefore, we aimed to study the incidence, predictors and survival of SSc-CM. An inception cohort study was conducted for early SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, from January 2010 to December 2019. All patients were determined for clinical manifestations and underwent echocardiography and HRCT at enrollment and then annually. SSc-CM was determined and classified using echocardiography. 135 early SSc patients (82 female,108 DcSSc) were enrolled. With the mean follow-up period of 6.4 years, 32 patients developed SSc-CM. The incidence of SSc-CM was 5.3 per 100-person years. The multivariate Cox regression analysis showed that baseline anti-topoisomerase I-positive (Hazard ratio[HR] 4.86, p = 0.036), dysphagia (HR 3.35, p = 0.001), CK level ≥ 500 U/L(HR 2.27, p = 0.045) and low oxygen saturation (HR 0.82, p = 0.005) were predictors of SSc-CM. The survival rates after SSc-CM diagnosis at 1, 5 and 10 years were 90.3%, 73.1%, and 56.1%, respectively. In this study cohort, the incidence of SSc-CM was 5.3 per 100-person years, and tended to have low survival. The presence of anti-topoisomerase I antibody, dysphagia, CK level ≥ 500 U/L, and low oxygen saturation were independent baseline predictors for developing SSc-CM.

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Sclerodermic Cardiomyopathy—A State-of-the-Art Review

Cited by 4 publications

References 102 publications

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

Right Ventricular Morphology and Function after Exercise Training in People with Systemic Sclerosis: A Randomized Controlled Pilot Study

Predictors and survival of cardiomyopathy determined by echocardiography in Thai patients with early systemic sclerosis: an inception cohort study

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