CASE HISTORY

The baby was a full-term child of healthy parents and was noticed to be cyanosed from birth. At the age of 6 weeks congenital heart disease was diagnosed. He became deeply blue on exertion but never squatted. He was admitted to this hospital at the age of 3 years, when he weighed 23 lbs. He was deeply cyanosed and this became worse on crying. Fingers and toes clubbed. Not dyspnoeic at rest and jugular venous pressure not raised. Blood pressure was 110/90, systolic thrill in pulmonary area and systolic murmur maximal in pulmonary area and conducted to back. Pulmonary 2nd sound split. Screening showed a large globular heart with a left sided aorta and underfilled lung fields. Pulmonary arteries seen. Arterial oxygen saturation 66% ECG suggested pulmonary stenosis. Angiocardiogram showed a large single auricle; the aorta filled before the pulmonary artery and appeared to arise from the right ventricle. The right pulmonary artery filled but no left pulmonary artery was seen. Right ventricle very large. It was concluded that the child had a single auricle, a pulmonary stenosis and transposition of the aorta and pulmonary artery or an extreme degree of over-riding. On 22.8.51 the child was operated on. At operation the position of the great vessels indicated complete transposition. The pulmonary arteries were very small and the pulmonary artery pressure was 26cm. of saline. It was decided to undertake a Pott's operation as the only feasible treatment, although the risk of left ventricle failure was appreciated. A Blalock operation was impossible owing to a very small subclavian artery. Soon after anastomosis was complete, there was a sudden alteration in cardiac rhythm, with arterial venous dissociation and the child died half an hour later in spite of treatment. 

PATHOLOGY

Heart 160mmx125mmx65mm. The heart and the main pulmonary vessels have been mounted together. At the back of the specimen there is a large single atrium. The pulmonary veins can be seen opening into this near its top. The inferior vena cava opened into the lower part of it but has been removed in exposing the auricle. The superior vena cava can be seen from the front of the specimen lying across the root of the right lung, between it and the aorta. This common atrium connects via a large three-cusped valve with a single ventricle which has been opened in front. This dilated and hypertrophied ventricle gives rise to the aorta. Below and to the left of the aortic valve is a circular orifice which leads to a small pulmonary artery. A blue rod has been passed through this. There is, in addition, a stenosed pulmonary valve which only just admits the glass rod. The main pulmonary artery divides into two equal sized branches, both of which were patent though collapsed. A surgical communication has been made between the aorta and the left pulmonary artery (Pott's operation).