CASE HISTORY

Blue baby, cyanosed ever since. Very backward, unable to walk but sits about happily playing with toys. For the last month has suffered considerably from toothache, and an anesthetic was refused him at Bart's on account of the risk. Admitted here for tooth extraction. Examination: this revealed a cyanosed child with clubbing of fingers and obvious polycythaemia. No murmurs, but the apex beat suggested right ventricular enlargement. ECG showed striking biphasic QRS complexes in all leads with a deep S in lead 1 and a deep Q in leads 2 and 3. No X-ray. The clinical diagnosis was pulmonary atresia. An anesthetic was administered, ether and oxygen, and was taken very well by the child. Four teeth were extracted very quickly and cleanly and the patient returned to consciousness very soon afterwards. During the next two or three hours he sat up on bed happily playing with his toys and seemed no worse at all for his adventure. However, about four hours after the anaesthetic he fell back dead, presumably from ventricular fibrillation. Autopsy was performed. 

PATHOLOGY

Heart 175mmx205mmx70mm. The heart and lungs have been mounted in continuity and the two ventricles have been opened, the right in front and the left behind. The aorta is large and arises from both right and left ventricles, being situated in a mid-position. The ventricles communicate at their upper end via a patent intraventricular septum. The pulmonary artery is represented by a solid fibrous cord which lies to the right of and parallel to the aorta. The two pulmonary arteries are supplied via a large patent ductus arteriosus. The heart as a whole is large and globular in shape