Ventricular Septal Defect in Early Childhood

A particular group of children in whom a defect of the ventricular septum leads to cardiac dis- ability in early life is the subject of this paper.The clinical picture that serves to distinguish the graver disability from the better known maladie de Roger is outlined. THE MALADIE DE ROGER-CLINICAL FEATURESThe maladie de Roger has been a well-recognized clinical entity since the characteristic para- sternal murmur was first described (Roger, 1879).The absence of symptoms and the presence of striking physical signs have been taken as the essential points in the diagnosis (Muir and Brown, 1934).In the routine examination of school children, follow-up studies have confirmed the clinical impression that the isolated ventricular septal defect may be regarded as a benign lesion.As with Roger's original cases, repeated examination has revealed no clinical deterioration and no change in the physical signs.Commonly there is no significant radiological or electrocardiographic alteration during the school period (Perry, 1937).Such patients may lead useful, energetic lives and, provided they escape subacute bacterial endocarditis and other incidental diseases, die of congestive heart failure in middle or later life.Roger's oldest case was a woman of 50 years who had borne four children and was leading an unrestricted life; cases recognized as having congenital heart disease in infancy have been followed to over 70 years of age (Gilchrist, 1948).THE VENTRICULAR SEPTAL DEFECT-AUTOPSY FEATURES Pathological confirmation in the maladie de Roger is rare.Roger's contribution to medicine is all the more remarkable in that he did not have the opportunity to confirm the diagnosis in any one of his cases.None of Muir and Brown's (1934) cases had autopsy confirmation, and only two of Perry's cases had died at the time he gave his paper (1937).The first case diagnosed clinically to be proved post-mortem was a boy of 4 years who died from diphtheria (Dupre, 1891).The defect usually occurs through the muscular septum just anterior to the membranous septum.It is circular or oval in shape and its margins are surrounded by fibrous tissue.It opens into the right ventricle behind the septal cusp of the tricuspid valve.The size of the defect varies from a minute opening to 1 cm. in its longest diameter (Abbott, 1936;Brown, 1939).In sharp contrast to the benign clinical picture of the symptomless lesion, post-mortem statistics show that the ventricular septal defect alone, or in combination with some other minor flaw, has in fact a high mortality in infancy and early childhood.Of Abbott's 50 cases, no less than 21 died at or under 5 years of age (Bauer and Astbury, 1944).Of 110 consecutive cases of congenital heart disease coming to autopsy during the first three years of life in the Royal Hospital for Sick Children, Edinburgh, no less than 28 (25%) had such defects of the ventricular septum.This malformation thus constituted the commonest single lesion in this post-mortem series.