AimAssessment of the principles of qualification and the range of organ transplantation in a patient with diagnosis of system sclerosis with pulmonary manifestation and severe myocardial insufficiency. Methodswe present a 43-year-old patient with confirmed systemic sclerosis with pulmonary manifestation and biventricular heart insufficiency, after disease exacerbation and sudden cardiac arrest in the PEA mechanism with effective resuscitation, with the growing of shortness of breath and the need to use inotropes and levosimendan infusion without significantly improvement in general state. Due to the diagnosis of a systemic disease with no option of pharmacological or any other treatment of the heart failure he was re-evaluated and put on an urgent waiting list for isolated heart transplantation. After 7 days the patient was transplanted. Because of the risk of progression of the disease and possible lung transplantation in the future the pleural cavities remained untouched. The standard protocol of immunosuppression was induced with the induction of the therapy with rabbit anti-thymocyte globulin. ResultsPatient was extubated 24 hours after heart transplantation. The results of endomyocardial biopsy during the hospital stay and 6 months follow-up were negative. Patient was discharged home after 22 days of uneventful hospital stay. ConclusionsScleroderma as an autoimmunological disease still remains a challenge for transplant team as a possible progressive multiorgan insufficiency requiring organ transplantation qualification. The course of the disease varies depending on the form of systemic sclerosis. Careful assessment, qualification and determination of appropriate pre- and post-procedure immunosuppressive treatment determines the uncomplicated course of treatment.