The syndrome of cardiopulmonary schistosomiasis (cor pulmonale)

Abstract

Seven cases of bilharzic cor pulmonale were described. In one of the patients necropsy was performed and the detailed pathological findings recorded. In the myocardium of this patient, scattered foci of cellular infiltrations consisting mainly of lymphocytes, some histiocytes, fibroblasts, and eosinophile cells were demonstrated. No bilharzia ova were seen. The clinical, radiological, and electrocardiographic findings were fully described. Stress was laid on the possible diagnosis of concomitant pulmonary bilharzic infiltrations and their radiological appearances. Diagnosis of early cases can be made and confirmed radiologically. The x-ray appearance is that of the well-known “mitral configuration”. The responsible lesions are mainly vascular, consisting of obliterative arteriolitis, produced by the passage of bilharzia ova through the arteriolar wall in an attempt to form extravascular parenchymatous tubercles. Healing of the intimal and medial lesions results in obstruction, which in some arterioles is complete. This is eventually followed by dilatation and atheroma of the main pulmonary artery and by marked hypertrophy of the right ventricle. In advanced cases the occluded tissue becomes canalized and expanded by capillary or cavernous formations to form the “angiomatoid” which is a characteristic feature of bilharzic arteriolitis. The symptomatology was discussed. Hemoptysis was observed in two patients in the series. It was stressed that the term “Ayerza's disease” was unfortunately applied. A better terminology is “cardiopulmonary bilharziasis” or, still better, “bilharzic cor pulmonale”. Cyanosis is not a constant feature of the syndrome and is only present when heart failure sets in, or if there is concomitant pulmonary infection with septic organisms (such as chronic bronchitis). Clubbing of the digits is also not constant, but may be present in a good number of patients.