Year: 1961
Type: article
Study design: Observational Study
Abstract: The relative importance of heredity and environment in causing malformation of the heart is still uncertain.We hoped that details about the families of our patients might provide some evidence to help in this problem.After a preliminary enquiry about the families of 384 patients, most of whom had Fallot's tetralogy (Polani and Campbell, 1955), we thought it would be more useful to make our enquiries in more homogeneous groups.So far, we have covered 261 patients with persistent ductus arteriosus (Polani and Campbell, 1960) and 151 patients with coarctation of the aorta (Campbell and Polani, 1961).In this paper we are applying the same method to the families of 170 patients with atrial septal defect.We are grateful to Dr. Evan Bedford for sending out our questionnaires to about 100 consecutive patients he had seen at the Middlesex Hospital: the 94 of these who replied (Cases 1-94) and 76 of our own patients (101-176) make up this series.We think that the diagnosis is certain in practically all these, and that the defect was of the ostium secundum type in all but three.In those seen at the Middlesex, it was confirmed by cardiac catheterization or operation in all, and generally by both.In the Guy's patients it was confirmed by catheterization, necropsy, or operation in all but five, in whom there seemed no doubt about the clinical diagnosis.Several had some pulmonary hypertension, but with A.S.D. this is generally a secondary result of the defect: in seven it was severe enough to have lessened the left-to-right shunt and produced a right-to-left shunt as well.I. OTHER MALFORMATIONS IN THE PROPOSITI (a) Cardiac Malformations.It is difficult to decide the frequency of these, because an atrial septal defect may often be a part of the picture in some more complex lesion, e.g. in tricuspid atresia or isolated levocardia, and may be hard to diagnose when combined with others, e.g. with Fallot's tetralogy.Such cases were excluded and this series was composed of examples of atrial septal defect (A.S.D.), relatively uncomplicated, though several were found to have some less important cardiac malformations as well.Most were of the common ostium secundum type but two were of the ostium primum type (Cases 115 and 132) and a third was thought to be (150), so that probably all these three had a ventricular septal defect and some deformity of the A-V valves as well as an A.S.D. (1 8%).One of these (132) and one other (171) had a persistent left superior vena cava.Patients with total anomalous pulmonary venous drainage were excluded, but some degree of this is common with atrial septal defects, especially with the sinus venosus type.Five of our patients (2.9%) (Cases 20, 34, 118, 120, and 165) were thought to have some anomalous pulmonary venous drainage.The combination of mitral stenosis with A.S.D. is well recognized: it occurred in eight of our patients (4-7%°).Six of the Guy's patients (171-176) and three of the Middlesex ones (27, 31, and 87) had pulmonary valve stenosis also, an incidence of 5-3 per cent.Six others had systolic gradients across the pulmonary valve between 16 and 34 mm.Hg but, with the increased flows, these gradients
Source: Heart
DOI: 10.1136/hrt.23.5.477
Authors: M. Campbell, P. E. Polani
Institution: Guy's Hospital
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2072002229