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In progressive systemic sclerosis, cardiac involvement, evidenced by histology, is rather unusual and even more rarely it gives clinical manifestations. In our patient cardiac symptoms, characterized by recurrent pulmonary edema as well as by serious conduction defects and arrhythmias, are prevailing and have a rapid and fatal course, whereas the involvement of the skin and of other internal organs is not important. The post-mortem examination gives an evident explanation of the clinical course of the disease, which we can consider as a typical manifestation of sclerodermic heart.
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