Atrioventricular defects--a study of 92 families.
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Biomedical subjects
Publications and source records attributed to J Somerville.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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The evolution of diagnosis and treatment of congenital heart malformations can be traced through patients surviving into adulthood. We reviewed the heart specimens from 180 patients aged 16 to 86 years and considered the morphological features, the nature of any interventional procedures, and the events leading to death. Based on the mode of clinical presentation, 33 cases were considered covert, and the remaining 147 cases were known or suspected to have a cardiac abnormality during life. Of the symptomatic cases, 60 had no surgical intervention, whereas 167 surgical procedures had been performed in the remaining 87 cases. Acquired heart disease was noted in 7 of the covert cases and in 16 of the symptomatic cases. Overall, there were only 3 instances of errors in clinical identification of significant morphological abnormalities, and 2 cases related to surgical procedures. This review emphasizes the value of autopsy examination for clinicopathologic correlations and the case for retention of cardiac specimens for teaching purposes.
There is a stage in one's career when one is mature enough to be invited to give named lectures. Usually, one has never heard of the 'named' person. This occasion is different because I know Dr Keith. (Dr John Keith died February 8, 1989, aged 80 years. Obituary in Can J Cardiol 1989;5[3]:XI). His book was my reference when I became excited by congenital heart disease and two days ago I had the pleasure of speaking to him, explaining the title which he said was "quaint and interesting." He reminded me that when he started the Canadian Cardiovascular Society with Dr Harold Segall there were only a handful of people who came together to discuss mutual problems in cardiology. Looking at the remarkable list of distinguished physicians and surgeons who have come before me to give this important lecture, I hope that I can do justice to the honour that you have given in asking me to be the John Keith Lecturer in 1988. The title of this lecture was born when I received a blue anniversary card covered with pink hearts from a once deeply cyanosed, chairbound girl with Ebstein's anomaly, 20 years after she had reparative surgery and lived a normal life, for which she thanked me. I passed the message to Donald Ross, with whom I have been privileged to work with for 25 years in the National Heart Hospital in London, United Kingdom and whose courageous innovations have created so much good life for many with complex congenital heart disease.
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Ten patients with duplication of the atrioventricular valves were studied, nine of them with the double orifice located in the mitral valve and the other one in the tricuspid. In two cases the diameter of the secondary valve was the same as that of the normal valve, and in the other 8 cases, it was smaller; each had its own valvular apparatus with chordae tendinae attached to papillary muscles located in different sites. The principal malformation in nine was ostium primum. One of the cases with mitral duplication had pulmonary atresia, an association which has never before been published. In five patients regurgitation of the secondary orifice was present; the diagnosis was made during surgery in nine cases and at necropsy in one. The orifice was sutured in three cases because of regurgitation. Four patients died in the immediate post-operative period, including the one with pulmonary atresia. The other three had pulmonary hypertension. These findings agree with the literature. The etiological hypotheses are discussed and emphasis is given to the importance of the finding of a narrow jet of contrast material directed upward and to the right in the left angiocardiogram in frontal plane. The authors believe that this may be an important sign in diagnosing cases with ostium primum septal defect; taking into account the fact that double mitral orifice is the cause of valvular insufficiency, this can be the site of bacterial endocarditis and usually needs to be surgically closed.