AB0638 Cardiac transplant in systemic sclerosis-associated cardiomyopathy: monocentric experience of 3 cases. (15th June 2017) - Lenaerts, J
Abstract : Background: Cardiac involvement in systemic sclerosis (SSc) is a frequent complication, but end-stage cardiac failure remains uncommon and represents a poor prognosis. Heart-lung and lung transplant is an established treatment option for SSc-related pulmonary disease. Due to the limited published data, no recommendations exist for cardiac transplant in the context of SSc. Objectives: We present our monocentric experience of 3 patients with SSc who underwent cardiac transplant for SSc-related end-stage heart disease (multiple hospitalisations due to failure of medical therapy and life-threatening complications). Results: Case 1 is a 59-year-old male with limited cutaneous SSc. Antinuclear antibody (ANA) was negative. He had vascular (digital ulcers) and cardiac (heart failure (left ventricular ejection fraction (LVEF) 20%, NYHA class IV)) involvement, without major gastrointestinal or pulmonary involvement (no interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH: assessed by right heart catheterization (RHC))). He underwent a cardiac transplant at the age of 51, after a disease duration of 6 years. Post-transplantation immunosuppressant therapy consists of tacrolimus and mycophenolic acid, initially associated with methylprednisolon, which is the standard immunosuppression protocol at our institution. Case 2 is a 55-year-old male with limited cutaneous SSc. ANA was positive, 1/320, speckled pattern, but no SSc-related autoantibody has been identified. He had gastrointestinal (upper gastrointestinal tract dysmotility), muscular (myositis) and cardiac (heart failure with secondary cardiac cirrhosis (LVEF 40%, NYHA class III)) involvement, without major pulmonary involvement (no ILD or PAH). He underwent a cardiac transplant at the age of 54, after a disease duration of 7 years. Standard immunosuppressants were initiated. Case 3 is a 50-year-old male with diffuse cutaneous SSc. ANA was negative. He had vascular (digital ulcers), gastrointestinal (upper gastrointestinal tract dysmotility) and cardiac (heart failure with secondary cardiac cirrhosis (LVEF 40%, NYHA class III)) involvement, without major pulmonary involvement (no ILD or PAH). He underwent a cardiac transplant at the age of 49, after a disease duration of 4 years. Standard immunosuppressants were initiated. At present, 1, 5 years (case 2 and 3) and 8 years (case 1) after transplant, the donor hearts are still functioning well. No other SSc-related organ manifestations have occurred. Conclusions: We present 3 patients with SSc who successfully underwent cardiac transplant for SSc-related end-stage heart disease. None had other major SSc-related organ involvement. This supports the limited published data that cardiac transplant is feasible and can be considered in end-stage SSc-related cardiomyopathy. Disclosure of Interest: None declared