CONTINUOUS MURMURS IN CYANOTIC CONGENITAL HEART DISEASE

Abstract

The diagnosis, clinical picture, and prognosis in 45 patients with cyanotic congenital heart disease and a continuous murmur is discussed. The murmur was loud in 40 and soft and variable in 5 of them: it was due to a persistent ductus in 1/4 and to a bronchial artery blood supply to the lungs in 3/4. In 35 of the 45 patients, there was a single arterial trunk that carried the blood from both ventricles. When there is such a trunk and a pulmonary blood supply through bronchial arteries, it is often impossible to make the clinical distinction between persistent truncus arteriosus and pulmonary atresia: such patients are classified as having a solitary trunk. In all these there is a continuous range from patients with a reduced pulmonary blood flow, a heart that is not much enlarged, moderate cyanosis, and considerable disability (the classical and commoner picture of pulmonary atresia) to those with an increased pulmonary blood flow, a large heart, slight cyanosis, and relatively little disability until heart failure supervenes (the classical and commoner picture of presistent truncus in infancy). Of the 45 patients, 1 had a persistent truncus, 16 had a solitary trunk, and 18 had pulmonary atresia: 5 of the remaining 10 had Fallot''s tetralogy or a related condition and the last 5 could not be classified. Of the 16 patients with a solitary trunk, 9 had an increased blood flow to the lungs, although this was through bronchial arteries[long dash]a condition that has not been widely recognized. Corresponding to this, they were less disabled and cyanosed and had less increase in their hemoglobin than most patients with Fallot''s tetralogy or pulmonary atresia, they did not squat, and had little or no clubbing of the fingers. Most of them have got on well for 12 years and are now near the end of their second decade. No surgical treatment is indicated for such patients at present. In general, squatting was much less common than with Fallot''s tetralogy. It occurred in 3 of the 5 patients who were placed in the Fallot group but in only 7 of the other 40[long dash]in 3 of the 17 with a solitary trunk and in 4 of the 18 with pulmonary atresia. The aortic arch was right-sided in rather more than half of those with a solitary trunk, and in about a quarter of the patients with pulmonary atresia, this being about the same proportion in pulmonary atresia as in Fallot''s tetralogy. The pulmonary blood flow was through bronchial arteries in all the patients with a solitary trunk and in about 2/3 of those with pulmonary atresia: in the remaining 3rd it was through a persistent ductus. The continuous murmur was generally loudest on the same side as the aortic arch, even when the flow was through bronchial arteries. Several of the patients had other malformations of the heart, the commonest being some form of transposition of the viscera[long dash]situs inversus in 1, isolated dextrocardia in 3, and isolated levocardia in 1. There were 3 patients among these 5 who had no inferior vena cava and a blood flow from the lower half of the body through the azygos vein[long dash]an interesting syndrome that is not widely recognized.