Primary heart disease in systemic sclerosis (scleroderma): advances in clinical and pathologic features, pathogenesis, and new therapeutic approaches.

Heart disease in SSCL may be primary or secondary. Primary involvement includes pericarditis, myocardial fibrosis, and contraction band necrosis with congestive cardiomyopathy, conduction system fibrosis, intramural coronary artery lesions and, rarely, valvular disease. Symptoms include those of left and right ventricular failure, chest pain, palpitations, syncope, and sudden death. Pathogenesis may be related to functional Raynaud's phenomenon of the heart and/or structural small vessel disease. Therapy at present is symptomatic; however, new therapeutic approaches are warranted.