Systemic sclerosis (SSc) is a chronic autoimmune disorder with unknown triggering factors, and complex pathophysiologic links which lead to fibrosis of skin and internal organs, including the heart, lungs, and gut. However, more than 100 years after the first description of cardiac disease in SSc, sclerodermic cardiomyopathy (SScCmp) is an underrecognized, occult disease with important adverse long-term prognosis. Laboratory tests, electrocardiography (ECG) and cardiovascular multimodality imaging techniques (transthoracic 2D and 3D echocardiography, cardiac magnetic resonance (CMR), and novel imaging techniques, including myocardial deformation analysis) provide new insights into the cardiac abnormalities in patients with SSc. This state-of-the-art review aims to stratify all the cardiac investigations needed to diagnose and follow-up the SScCmp, and discusses the epidemiology, risk factors and pathophysiology of this important cause of morbidity of the SSc patient.

Systemic sclerosis (SSc) is a rare disease, with unfavorable clinical course and prognosis, characterized by progressive multisystemic involvement. SSc associated pulmonary hypertension (SSc-PAH) and interstitial lung disease (ILD) are the most important factors for morbi-mortality in these patients, being responsible for more than 60% of total deaths. Though pulmonary arterial hypertension (PAH) is the dominant subtype seen in SSc, PH secondary to ILD, left-heart pathology, and pulmonary veno-occlusive disease (PVOD) are also possible occurrences. Initial evaluation of a SSc case is complex and should be performed with a multidisciplinary approach. Early detection of SSc-PAH is imperative, given the fact that new and effective medications are available and early treatment was shown to improve outcomes. Therefore, screening algorithms must be used adequately and in a cost-effective manner. Sensitivity and negative predictive value (NPV) are the most important performance measures in a screening test. Several algorithms were developed in the last decade (e.g., DETECT and ASIG) and demonstrated higher efficiency when compared to older algorithms. The present manuscript details the risk factors for SSc-PAH and includes a critical description of current detection algorithms, as a primer for clinicians working in the field of cardio-rheumatology.

Systemic sclerosis (SSc) is a multisystemic autoimmune disorder in which cardiac involvement is frequent and portends negative prognosis. Left ventricular (LV) diastolic dysfunction is one of the most common cardiac alterations in these patients, and left atrial (LA) reservoir strain (Ɛ_R) measurement using speckle tracking echocardiography has been proposed as a novel parameter for a better assessment of LV diastolic function. Therefore, the aim of this study was to test the prognostic value of Ɛ_R in a large multicenter cohort of SSc patients. In total, 311 SSc patients (54 ± 14 years, 85% female) were included from two different centers. Echocardiography was performed at the time of first visit, including Ɛ_R measurement. Over a median follow-up of 132 (interquartile range: 110 to 157) months, 67 (21.5%) patients experienced the outcome of all-cause mortality. Spline curve analysis identified an optimal cut-off value of 30% for Ɛ_R, and patients with Ɛ_R ≤ 30% showed a 10-year cumulative survival rate of 71% as compared to 88% for patients with Ɛ_R > 30% (log-rank p < 0.001). At the multivariable Cox regression analysis, Ɛ_R was independently associated with the endpoint (HR 1.830; 95% confidence interval (CI) 1.031–3.246; p = 0.039) together with age (HR 1.071, 95% CI 1.043 to 1.099; p < 0.001), sex (female) (HR 0.444, 95% CI 0.229 to 0.861; p = 0.016), and diffusing lung capacity for carbon monoxide (HR 0.969 95% CI 0.956 to 0.982; p < 0.001). Ɛ_R is of independent prognostic value in SSc and might help optimizing risk stratification in these patients.

Background This review is based on the case of a 71-year-old female patient, with cardiovascular risk factors, who was referred for emergency cardiological evaluation after experiencing severe anterior chest pain that had lasted for more than 5 hours at the time of admission. A diagnosis of inferior-posterior ST-segment elevation myocardial infarction was established, and emergency coronary angiography was performed. It revealed severe, diffuse dilation of the entire arterial coronary tree with slow flow and the presence of a large quantity of thrombotic material in the right coronary artery. Thromboaspiration was deemed unfit since the operator believed that there was a high risk of distal embolization with subsequent no-reflow, when passing with the catheter. As such, the patient started receiving anticoagulant therapy, and after one month she was scheduled for a control angiogram. The follow-up coronary angiography identified complete dissolution of the thrombus from the right coronary artery and a moderate stenosis in the distal segment, so no stent implantation was required. Rationale for review After performing a complete and comprehensive differential diagnosis, presence of isolated coronary artery ectasia was established. Coronary artery ectasia is a rare disease, usually found incidentally during coronary angiograms performed for other indications (chronic and acute coronary syndromes) with a vast