Sclerodermic Cardiomyopathy—A State-of-the-Art Review
Abstract
:1. Introduction
2. Pathophysiology
3. Traditional Cardiovascular Risk Factors in SSc
4. Risk Factors for Primary Myocardial Disease in SSc
5. Myocardial Involvement in SSc
6. Cardiac Investigations in SSc-Related Myocardial Involvement
6.1. Cardiac Biomarkers
6.2. ECG Changes
6.3. Echocardiographic Assessment of Primary Myocardial Disease in SSc
6.3.1. LV Systolic Function
6.3.2. LV Diastolic Function
6.3.3. Ventricular-Arterial Coupling (VAC)
6.3.4. Right Ventricle (RV) Evaluation
6.3.5. Atrial Evaluation
6.4. CMR Assessment of Primary Myocardial Disease in SSc
6.5. Nuclear Imaging in Primary Myocardial Disease in SSc
7. Outcomes of Primary Myocardial Involvement in SSc
8. Treatment of SScCmp
9. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Investigation | Main Findings |
---|---|
Cardiac biomarkers | NTproBNP—first-line diagnostic tool; follow-up; does not vary across skin subtypes [23]. Cut-off value of 130 pg/mL-sensitivity 74%, specificity 70%, NPV 85% for detecting cardiac impairment in SSc [23] cTn—positive correlation with cardiac disease in SSc, but with low specificity and sensitivity [46] |
ECG | 25–75% SSc patients have abnormal tracings [39]. Independent predictor of mortality [39]. Anteroseptal myocardial infarction pattern—10% of cases [30]. High frequency of arrhythmias—especially ventricular events [47]. High frequency of PVB—correlates with mortality and SCD risk [48]; >1190 PVB/24 h—100% sensitivity and 83% specificity in predicting SCD or later necessity of ICD implantation [49]. Presence/severity of arrhythmias does not correlate with skin subtype/symptoms [39]. Arrhythmogeneity index—correlates with mRSS [50]. |
.2DTTE | SSc patients need annual 2DTTE for assessing LV systolic and diastolic function and sPAP [51]. LV systolic function: 5.4% prevalence of LV systolic dysfunction [52]; reduced strain rate values in the IVS and antero-lateral wall [12]; myocardial electromechanical proprieties—diffuse deterioration of LV segments [3]. LV diastolic function: 4–5 times more frequent than systolic dysfunction (17–30% of cases) [22,53]; Risk factors for low e’velocities: duration of the disease, age, CAD, HTN [53]; e’-baseline value predicts mortality risk and every decrease in standard deviation elevates mortality risk 3.2 times [53]. VAC: Higher in dcSSc compared to lcSSc [51]; VAC might predict MACE in SSc population [51]. RV: Frequently involved in systemic diseases, either by direct injury, or because of associated PH [54]. Early dysfunction in SSc—associated with the degree of skin and pulmonary involvement and with anti-Scl70+ [12,54]. Early diastolic dysfunction [54]; prolongation of pre-ejection times [54]; RVFWS—significantly lower in SSc cases compared to healthy controls (irrespective of skin subtype or sPAP) [55]; apical and middle segments of RVFW—more damaged compared to the basal segment [55]. |
CMR | Detects cardiac disease in up to 75% of SSc cases, superior sensitivity than echocardiography for detecting cardiac abnormalities in SSc [56]. Large areas of fibrosis with specific disposition: diffuse, in small intercellular quantities [28]; higher T1 values and larger ECV compared to controls [57]; patchy, mid-wall, linear pattern in the basal and mid-LV segments [57]; fibrosis more prevalent in dcSSc [42]. Adenosine stress CMR–79% SSc cases with cardiac disease have subendocardial perfusion anomalies [58]. |
Nuclear Techniques | Thallium-201 perfusion scintigraphy +SPECT—detect reversible induced myocardial perfusion defects in almost all patients [10]. Perfusion defects more prevalent in severe cutaneous disease and anti-Scl70+ [10]. Myocardial perfusion defects—one of the most important predictors for future myocardial damage and mortality, irrespective of skin subtype [10,18,29]. |
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Giucă, A.; Gegenava, T.; Mihai, C.M.; Jurcuţ, C.; Săftoiu, A.; Gȋrniţă, D.M.; Popescu, B.A.; Ajmone Marsan, N.; Jurcuț, R. Sclerodermic Cardiomyopathy—A State-of-the-Art Review. Diagnostics 2022, 12, 669. https://doi.org/10.3390/diagnostics12030669
Giucă A, Gegenava T, Mihai CM, Jurcuţ C, Săftoiu A, Gȋrniţă DM, Popescu BA, Ajmone Marsan N, Jurcuț R. Sclerodermic Cardiomyopathy—A State-of-the-Art Review. Diagnostics. 2022; 12(3):669. https://doi.org/10.3390/diagnostics12030669
Chicago/Turabian StyleGiucă, Adrian, Tea Gegenava, Carmen Marina Mihai, Ciprian Jurcuţ, Adrian Săftoiu, Diana Monica Gȋrniţă, Bogdan Alexandru Popescu, Nina Ajmone Marsan, and Ruxandra Jurcuț. 2022. "Sclerodermic Cardiomyopathy—A State-of-the-Art Review" Diagnostics 12, no. 3: 669. https://doi.org/10.3390/diagnostics12030669