Persistence of the ductus arteriosus is not usually associated with a normal life expectancy.All but three of 62 patients were under 40 when they died (Shapiro, 1944). Three patients, all women, have lived to over 70 years. One who died at 79 is mentioned briefly as having a small opening and great dilatation of the pulmonary arteries with general cardiac hypertrophy (Holman et al., 1953). Another who lived to 75 had auricular fibrillation and congestive failure: the ductus measured 3 mm. across (Fishman and Silverthorne, 1951 (Fig. 1). Subsequently the paroxysms of tachycardia became more frequent and lasted longer. The ductus murmur became less obvious and in 1955 only the systolic component was present. Finally in 1956, she became anzmic from gastro-intestinal bleeding and died, aged 85. She never had cardiac failure.Necropsy (Dr. J. V. Wilson). The body was of normal development and nutrition. The heart weighed 270 g. The walls of the ventricles were of normal thickness and there was neither hypertrophy nor dilatation of any chamber. The heart valves were normal. The aorta showed extensive areas of degeneration and calcification and the ascending aorta and the arch were slightly dilated due to this degeneration. The coronary arteries were thickened and sclerosed but were nowhere occluded. The myocardium was healthy apart from some fibrosis.The ductus arteriosus was patent. The aortic opening (Fig. 3) was funnel-shaped due to extensive calcification of the wall of the aorta. This calcification extended for a radius of about 1 cm. around the opening so that the wall of the opening itself was firm and largely calcified: it was 4 mm. in diameter. The total length of the ductus was 6-5 mm. and it was calcified for about 3-5 mm. distal to the aortic opening forming a rigid channel. The pulmonary opening (Fig. 4) was surrounded by a thin fringe of endothelium and was freely mobile on its attachment to the pulmonary artery. The main pulmonary artery showed no evidence of calcification and the pulmonary branches were not thickened.The kidneys were contracted and granular. The bowel mucosa showed considerable congestion and the contents of the lumen were extensively blood-stained, especially in the upper part of the small intestine.There was no visible intestinal ulceration and the blood appeared to be oozing from the congested mucous membrane. Death was due to uremia. A remarkable feature of the necropsy was the absence of evidence or circulatory embarrassment from the ductus arteriosus. 574
A lad of eighteen was first seen on May 18, 1942. A congenital heart lesion had been diagnosed when he was a few weeks old, and though he was not then cyanosed at rest, he soon became blue and breathless on exertion, especially in cold weather. He had scarlet fever at the age of ten, but never rheumatism in any form. At the age of twelve, he was too breathless for any but short walks. He was unfit to play any games at school. A change had taken place six months before (October, 1941) when cyanosis became permanent and the dyspncea more distressing. There had been neither orthopncea nor cedema; he had never fainted, and he had not lost weight. Pain had started during these last months of increased dyspncea, and now constituted the greater complaint, though he was no complainer. On exertion it began in the left arm and if severe it affected the right. It was felt across the chest, especially on the left, but not in the back. Rest, especially if with warmth, relieved it, yet it might last 10 to 20 minutes once evoked by exertion, for it never began at rest. Palpitation was felt on emotion or exertion. There was a slight occasional cough.Examination.-He was a pleasant and fairly well-developed youth, with some cyanosis of the skin and moderate clubbing of the fingers and toes. The pulse was 70-80, and regular. The blood pressure was 125/100. A heaving apex beat was felt in the fifth space, two inches outside the nipple line. A long diastolic thrill was felt widely in the apical region, yet there was no thrill at the base. The first sound was snapping internal to the apex, and a thundering diastolic murmur was heard over the whole apical region, up to the fourth rib, though loudest internal to the apex. At the base the murmur was less loud; the basal second sound was just audible. There were no abnormal signs in the lungs or abdomen.The electrocardiogram (Fig. IA) showed a small R and a large S in all leads, and an isoelectric T in lead I. Two months later, three days before his death on July 18, 1942, T I was inverted (Fig. lB).Cardioscopy showed enlargement of the heart, mainly to the right, but with a single big rounded prominence on the left, in the region of the pulmonary artery and extending upwards to embrace the aortic area so that no aortic knob was visible. The right pulmonary artery was, however, not enlarged. In the right (I) oblique position, a barium swallow showed a combined aorto-pulmonary impression and no particular displacement of the left auricle. Radiographs by Major Peter Kerley are shown in Fig. 2 and 3.Course. A tentative diagnosis was made of patent ductus arteriosus with aneurysmal dilatation of the pulmonary artery, presumably combined with some other defect. This presumption seemed to preclude operation when this was considered because of the deterioration in his condition as already described. The physical signs by July 14, 1942 had altered as follows. Cyanosis was extreme, though he was not, even now, breathless at rest and could walk slowly in the street. The murmur had become more con...
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