Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by multisystem involvement. Heart involvement occurs in 80-100% of cases and represents one of the more common risk factors of death. Myocardial fibrosis and vascular microangiopathy lead to arrhythmias and impaired contractility with secondary left and right heart failure. Pulmonary arterial hypertension in some patients with systemic sclerosis results in right heart failure and low output syndrome during the end-stage of the disease. The aim of the present study was to analyze four cases of systemic sclerosis with severe cardiovascular complications and a fatal outcome.
Case descriptions: Case 1: A 68-year-old female who probably suffered from limited SSc (lcSSc) and pulmonary arterial hypertension for more than 10 years was unsuccessfully treated with vasodilatators (sildenafil, iloprost). Case 2: A 52-year-old female with a 5-year history of diffuse cutaneous SSc (dcSSc), interstitial lung disease, and pulmonary arterial hypertension underwent aggressive therapy with immunosuppressive and antiproliferative (treprostinil) agents but without effect on the progression of the disease. Case 3: A 50-year-old male with dcSSc and coexistent cardiomyopathy with dominating symptoms of right heart failure was placed on high doses of immunosuppressants (i.v. cyclophosphamide) and had a cardioverter-defibrillator implanted as part of primary prevention. The patient died with symptoms of severe, therapy-resistant, two-sided heart failure. Case 4: A 58-year-old female with a 6-year history of dcSSc, interstitial lung disease, and heart involvement with severe ventricular arrhythmias and significant mitral and tricuspid insufficiency received intensive immunosuppressive therapy (i.v. cyclophosphamide) and underwent arrhythmia ablation. Sudden cardiac arrest in this patient was attributed to arrhythmia due to cardiomyopathy associated with systemic sclerosis.
Conclusions: Lung and heart involvement is the most common reason for poor prognosis in systemic sclerosis. Arrhythmias, usually latent clinically, and right heart failure associated with cardiomyopathy or pulmonary hypertension are the main reasons of cardiac death in SSc patients. Severe and fatal cardiovascular complications occur more often in dcSSc, particularly during the first few years after diagnosis. Early detection of cardiovascular manifestations should be a priority in systemic sclerosis.