Systemic Sclerosis as a Challenge for Heart Transplantation: A Case Report.

ABSTRACT

OBJECTIVE: To assess 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. METHODS: We present the case of a 43-year-old patient with confirmed systemic sclerosis with pulmonary manifestations and biventricular heart insufficiency after disease exacerbation and sudden cardiac arrest in the pulseless electrical activity (PEA) mechanism with effective resuscitation, with increasing shortness of breath and the need for inotropes and levosimendan infusion without a significant improvement in his general status. Owing to the diagnosis of a systemic disease with no option for pharmacologic or any other treatment for heart failure, he was reevaluated and put on an urgent waiting list for isolated heart transplantation. After 7 days, heart transplantation was performed. Given the risk of disease progression and the possibility of future lung transplantation, the pleural cavities were untouched. The standard immunosuppression protocol was followed with the use of rabbit antithymocyte globulin. RESULTS: The patient was extubated at 24 hours after heart transplantation. The results of endomyocardial biopsies performed during the hospital stay and at a 6-month follow-up were negative. The patient was discharged to home after 22 days of an uneventful hospital stay. CONCLUSIONS: Scleroderma as an autoimmunologic disease remains a challenge for the transplantation team as a possible progressive multiorgan insufficiency requiring qualification for organ transplantation. The course of the disease varies depending on the form of systemic sclerosis. Careful assessment, qualification, and determination of appropriate preprocedure and postprocedure immunosuppressive treatment are essential to an uncomplicated course of treatment.