Systemic sclerosis (SSc) is a rare immune-mediated rheumatic disease characterized by diffuse vasculopathy leading to multiorgan fibrosis.
We report the case of a 61-year-old man who was diagnosed with SSc one year before. At the time of diagnosis, the patient exhibited exertional dyspnea (NYHA II-III) due to mild signs of interstitial impairment at chest HRCT and stage 3b of chronic kidney disease. Echo was performed, showing severe impairment of the right ventricular systolic function in the absence of LV dysfunction. Right heart catheterization (RHC) was unremarkable (mPAP 19 mmHg, PCWP 16 mmHg).
Several months later, despite immunosuppressive treatment, the patient experienced clinical worsening, in the absence of remarkable worsening of Echo and RHC parameters. Therefore, he was started with mycophenolate mofetil and tocilizumab.
After 60 days of second line therapy, the patient presented to the ER with severe resting dyspnea (NYHA IV), in a clinical-laboratory data of acute heart failure due to new onset left ventricular dysfunction (EF 36%), and severe impairment of renal function (AKI on CKD).
ECG (figure 1) showed chaotic ectopic atrial rhythm with prolonged atrio-ventricular conduction and frequent polymorphic premature ventricular contractions.
This report presents a rare case of primary myocardial involvement in SSc, where right ventricular failure was the initial cardiac manifestation, rapidly progressing to severe biventricular dysfunction.
As a potential cause of biventricular systolic dysfunction, we ruled out both drug-induced cardiotoxicity and autoimmune or infectious myocarditis, based on consistently negative results for inflammation and viral markers.
We assumed fibrosis to play a significant role in this case of heart failure, as supported by electrocardiographic features of several conduction disturbances. Unfortunately, second level imaging with cardiac magnetic resonance was rejected by patient because of claustrophobia, and coronary angiography was excluded because of severe renal impairment. Patient was successfully empirically treated with diuretics, antiarrhythmic therapy, peritoneal dialysis and levosimendan. After 40 days of hospitalization, we reported complete regression of left ventricular systolic disfunction (last Echo showing EF 50%), and the patient was discharged in decent hemodynamic stability.
