Congenital coronary artery fistula is a rare abnormality in which a branch of either coronary artery communicates with a cardiac chamber or with the pulmonary trunk. Recently Papaioannou and his colleagues (1962) reviewed 70 adequately documented cases, including one of their own; in 18 of these a branch of the right coronary artery entered the right ventricle. A further patient with this anomaly is described here, with observations on the problem of diagnosis and a possible surgical approach to the correction of the lesion.
Case ReportA man, aged 31 when he died, was first seen at this hospital in 1957 six years previously, when he was admitted for a hernia operation. He had been attending a physician elsewhere and was known to have congenital heart disease, which was then symptomless. At no time did he have a continuous murmur.On examination, he had a collapsing pulse with capillary pulsation and a wide pulse pressure (blood pressure 150/45 mm. Hg). There was no cyanosis or clubbing and the venous pressure was normal. The heart was very large with hypertrophy of both ventricles. There was a very loud pan-systolic murmur, with thrill, maximal in the third intercostal space to the left of the sternum, an apical mid-diastolic murmur, and a soft blowing early-diastolic murmur down the left border of the sternum. The second sound was normally split. The electrocardiogram showed left ventricular hypertrophy, without ST or T wave changes but with notching of the R wave in leads I and V6 (Fig. la). On the chest radiograph there was enlargement of aorta and pulmonary trunk, and considerable pulmonary plethora. At cardiac catheterization, a large left-toright shunt at ventricular level was demonstrated (pulmonary/systemic flow ratio approximately 4:1), the pulmonary arterial pressure was slightly raised (35/7 mm. Hg above the sternal angle), and the pulmonary vascular resistance was normal. He was thought to have a ventricular septal defect with aortic regurgitation, but the discrepancy between the width of the pulse pressure and the inconspicuous early diastolic murmur was noted. Operation was not advised as he was then symptomless and as the presence of presumably free aortic reflux would have posed problems which at that time were insuperable.He was not seen again until five years later when he returned with increasing effort dyspncea and palpitation, and was found to have atrial fibrillation, with clinical and radiological evidence of pulmonary congestion. The signs were otherwise unchanged (phonocardiogram shown in Fig. lc), but the electrocardiogram now showed evidence of right ventricular hypertrophy (Fig. lb). The blood pressure was 170/30 mm. Hg. In spite of treatment his condition deteriorated, and he was readmitted to hospital with right and left ventricular failure, pulmonary infarction, and secondary chest infection. He never improved sufficiently for further investigations to be undertaken, and he died in May 1963.At necropsy (Dr. W. J. Hanbury) there was peripheral cedema, with pleural, pericardial, and p...