SSc is considered a rare chronic multisystemic disease. It is characterized by the dysregulation of adaptive and innate immunity, microvascular damage, and skin and multiple internal organs progressive fibrosis [
6]. Symptoms of conduction disorders and arrhythmias such as fatigue, palpitations, or syncope are frequently reported in patients suffering from SSc. According to the large European League against Rheumatism Scleroderma Trials and Research database (EUSTAR), arrhythmias and pericardial effusion represent the most frequent cardiac complications [
7]. ECG abnormalities in SCC are common. Up to 75% of patients with SCC have abnormal ECG, and their presence is considered an independent predictor of mortality [
8]. Among 128 SSc-related deaths reported in the EUSTAR database, 6% were attributable to arrhythmias [
7]. A large study examining the noninvasive assessment of cardiac arrhythmias in 35 patients with scleroderma demonstrated that conduction defects were detected in only 19% of cases by ECG, essentially first- or second-degree atrioventricular block, and, unlike our case, not a complete atrioventricular block, whereas this percentage reached 33% with 24-hour Holter monitoring [
9]. This further supports the use of 24-Holter ECG in this patient population when the context is suggestive of arrhythmia. In our case, the diagnosis of complete atrioventricular block was made using 12-lead ECG, whereas the diagnosis of tachycardia–bradycardia syndrome was made through Holter ECG. Nowadays, new noninvasive tools are used for myocardial subclinical dysfunction assessment. One of the most powerful is cardiac MRI [
10], allowing the screening of late gadolinium enhancement (LGE) which is considered the most reliable method for myocardial fibrosis detection. In fact, an arrhythmia was diagnosed in almost 75% of patients with SSc with an LGE pattern [
10]. Unfortunately, cardiac MRI remains a rather expensive tool, not easily available in our context. This can represent a real challenge, as early diagnosis of cardiac complications in SSc is crucial and allows early management of arrhythmia treatments. Anti-arrhythmic drugs should be used with extreme caution, because of their potentially harmful effects on other SSc organ disorders. For instance, beta-blockers are very effective arrhythmia treatments but, with the exception of certain new products, may aggravate Raynaud phenomenon in the other hand [
8]. For this very reason, treatment with metoprolol was considered more appropriate in our case after pacemaker implantation.