THE COEXISTENCE OF SICKLE CELL DISEASE AND CONGENITAL HEART DISEASE: A REPORT OF THREE CASES, WITH REPAIR UNDER CARDIO-PULMONARY BY-PASS IN TWO

Publisher Website
Google Scholar
Abstract
Sickle cell disease, characterized by protean manifestations, has been confused frequently with rheumatic fever and congenital heart disease. Though it has been described in combination with rheumatic mitral stenosis, little on no consideration has been given to the association of sickle cell disease with congenital heart disease. This communication describes the occurrence of hemoglobin S disease in combination with congenital heart disease in three patients, the lesions being an atrial septal defect of the secundum type in one and pulmonary stenosis in two other patients. The congenital cardiac abnormalities were repaired under cardiopulmonary by-pass in the case of atrio-septal defect and the more severe case of pulmonary stenosis. Preparation for surgery consisted in the suppression of hemoglobin S formation by blood transfusions. During cardiopulmonary by-pass, further dilution of the hemoglobin S cells occurred so that their concentration in the patients' blood was negligible. Following surgery, it was necessary to administer greater amounts of intravenous fluid than usual to allow for the reduced ability to concentrate urine. Convalescence was unremarkable in each case.

This publication has 0 references indexed in Scilit: