Browsing by Author "Hunter, J. C."

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    Progressive familial heart block type I : clinical and pathological observations
    (Health & Medical Publishing Group, 1991) Van der Merwe, P.-L.; Rose, A. G.; Van der Walt, J. J.; Weymar, H. W.; Hunter, J. C.; Weich, H. F. H.
    Progressive familial heart block type I (PFHB-I) is an autosomal inherited disease. It was previously postulated that the disease is limited to the cardiac conduction tissue. The presentation of a patient with dilated cardiomyopathy focused on the possibility that this might be part of PFHB-I. This observation led to routine echocardiographic examination of patients with complete heart block, who belonged to PFHB-I families, and another 5 cases with signs of dilated cardiomyopathy were identified. This is the first time, to our knowledge, that the histological picture of PFHB-I has been described. From these case reports it is clear that in the presence of a dilated cardiomyopathy the prognosis in PFHB-I tends to be poor.