A genetic analysis was made of rofamilies in which the propositi had a secundum atrial septal defect associated with abnowrmal atrioventricular conduction (first, second, or third degree heart block) or unexplained left axis deviation or a combination of these conduction disturbances. Diagnostic information was available on 51 (8I%) of the first degree relatives. Three of the families appeared to be examples of a new syndrome which, with variable expression, was inherited as a non-sex-linked autosomal dominant. The main features were a secundum atrial septal defect; disease of the conducting tissue, which in some cases was progressive; unexplained left axis deviation; and unexpected death. These families did not seem to be examples of the Holt-Oram syndrome,for the upper limbs were clinically and radiologically normal in the I9 members examined.The importance of recognizing this syndrome is the occurrence ofprogressive disease of the conducting tissue and the risk ofsudden death. When, therefore, unexplained left axis deviation or prolonged atrioventricular conduction is found in association with a secundum atrial septal defect all available relatives should be examined.In the remaining seven families there was only one affectedfirst degree relative out of 39 examined. He was the son of one of the propositi and had paroxysmal coronary sinus rhythm with an intact atrial septum.
The radiographic study of coronary arteries in man has been attempted only in recent years. The earliest workers in this sphere employed thoracic aortography, the opaque medium being introduced by needle puncture (Radner, 1945) (1960), the aim being to fill the aortic sinuses so that a high proportion of the medium enters the coronary arteries. In our animal experiments we found that cardiac arrest induced by acetylcholine (Sloman and Jefferson, 1960) and timed diastolic injections of contrast medium (Michell and Jefferson, 1962) gave encouraging results, but we considered that cardiac arrest was potentially too dangerous to use in man. Although Thal et al. (1958) have applied timed diastolic injections successfully in man, our experience convinced us that it was unreliable in filling diseased coronary arteries. Failure to fill a severely diseased right coronary artery is illustrated by Case 4 ( Fig. 1 and 2). This patient, a man aged 44, had had severe angina of effort for five years. Aortography with 75 ml. opaque medium showed occlusion of the anterior descending artery but failed to outline the right coronary artery (Fig. 1). Selective coronary arteriography was subsequently performed, and the right coronary artery was successfully cannulated and clearly outlined by an injection of only 2 ml. (Fig. 2).Thus, in 1960, faced with the problem of how to improve aortography without increasing the hazards of the procedure, we decided to investigate selective coronary arteriography, introduced by Sones in 1960 and practised by him with success and safety. Apart from Sones, who has had an extensive experience with selective coronary arteriography (Sones and Shirley, 1962), Lang and Sabiston (1961) and Tapia, Bolton, and Mazel (1961) have also reported favourably.
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