Biomedical subjects

J Somerville

Publications and source records attributed to J Somerville.

At least 181 records · Page 10Linked to original sources

PubMed1975

Association of secundum atrial septal defect with abnormalities of atrioventricular conduction or left axis deviation. Genetic study of 10 families.

A genetic analysis was made of 10 families in which the propositi had a secundum atrial septal defect associated with abnormal 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 (81%) 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 19 members examined. The importance of recognizing this syndrome is the occurrence of progressive disease of the conducting tissue and the risk of sudden 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 affected first 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.

Adolescent↗

PubMed1975

Problems with radical corrective surgery after ascending aorta to right pulmonary artery shunt (Waterston's anastomosis) for cyanotic congenital heart disease.

Forty-five patients with Fallot's tetralogy or pulmonary atresia had total correction 2 to 6 years after palliative ascending aorta to right pulmonary artery anastomosis (Waterston's shunt). The operative mortality for total correction is increased in this group. Patients shown subsequently to have had a good anatomical correction died unexpectedly with high central pulmonary artery pressure, falling arterial PO2, low cardiac output, and progressive acidosis. The cause of this syndrome was difficult to determine. Histological examination of the lungs showed that severe pulmonary arteriolar thickening was not present. The factors apparently predisposing to these postoperative difficulties were kinking of the right pulmonary artery and/or lone perfusion of the right lung, remaining uncorrected for several years, in patients with pulmonary atresia or with Fallot's tetralogy with a shunt performed under the age of 3 years. In survivors, stenosis of the right pulmonary artery frequently occurred but was rarely severe. In view of these difficulties, early haemodynamic and angiocardiographic assessment is recommended in all patients with aorta to right pulmonary artery anastomosis, irrespective of the clinical result, in order to delineate the central pulmonary artery anatomy and assess perfusion of each lung; if kinking of the right pulmonary artery and inadequate perfusion of the central pulmonary arteries and left lung are demonstrated, early radical corrective surgery is recommended.

Adolescent↗

PubMed1975

Aortic regurgitation from infective endocarditis in Fallot's tetralogy and pulmonary atresia.

Three cases of aortic regurgitation acquired as the result of bacterial endocarditis complicating Fallot's tetralogy and pulmonary atresia have been described. One also had mitral regurgitation from a 'jet lesion' of the anterior cusp of the mitral valve. Surgical treatment of all abnormalities with aortic valve repair or replacement was undertaken in each patient and was successful in two. Difficulties in diagnosis and surgical treatment are discussed.

Adult↗

PubMed1975

Primitive ventricle with acquired subpulmonary stenosis.

The course and natural history of two children aged 1 yr and 5 yr with primitive ventricle with outlet chamber and normally related great arteries are described. They initially presented as ventricular septal defect with increased pulmonary blood flow and subsequently developed 'cyanotic attacks', chronic cyanosis and diminished pulmonary flow by acquiring subpulmonary obstruction of the bulboventricular foramen. Both had successful surgical treatment utilizing their own normal pulmonary valves by connecting the right atrium to the right ventricular outflow, with a Dacron conduit in one, and in the other by detaching the pulmonary artery and valve from the rudimentary outflow chamber and anastomosing it directly to the right atrial appendage. Changing morphology and its effect on function are discussed.

Cardiomyopathy, Hypertrophic↗

PubMed1975