Postoperative pathology of congenital heart disease.

Complete and unselected data concerning the postoperative pathology of congenital heart disease are presented for the first time. This study was based on 2,365 autopsies performed at the Children's Hospital Medical Center, Boston, in the 9 years from 1966 through 1974. Of these, 586 autopsies (25 percent) revealed congenital heart disease--238 performed in medically treated patients (41 percent) and 348 in surgically treated patients (59 percent). Tetralogy of Fallot, including cases with pulmonary outflow tract atresia and other associated malformations, was the congenital heart disease most often encountered in the postoperative autopsy series (88 cases, 25 percent of that series). D-transposition of the great arteries, including cases with other associated anomalies, was second (54 cases, 15.5 percent). Early death (hospital mortality) accounted for 320 (92 percent) of the 348 surgical cases; late death occurred in 28 patients (8 percent). Causes of late postoperative death included arrhythmias, excessively small ventricular septal defect with tricuspid atresia, massive hemoptysis, rupture of the pulmonary artery, cyanotic spell, congestive heart failure and infection. Prophylactic penicillin is recommended for patients with the asplenia syndrome because of their probably enhanced vulnerability to fulmfulminating septicemia by encapsulated bacteria such as the pneumococcus. Completeness and lack of selection in reporting data are essential in the interests of perspective and comparability of findings.