Angiocardiography in Congenital Heart Disease of Cyanotic Type

In the first of this series of articles (6), we discussed the angiocardiographic changes in the tetralogy of Fallot and in “pseudo-truncus arteriosus.” In this communication we shall direct our attention to another congenital cardiac disorder of the cyanotic type, namely, tricuspid stenosis or atresia with hypoplasia of the right ventricle. Until a short time ago, this anomaly was thought rather rare. Gibson and Clifton (8) discovered it only twice in 105 autopsies of children with congenital heart disease. Manhoff and Howe (9), in their recent review of the literature, however, found 33 instances of the condition; they added a case of their own, which was accompanied by transposition of the great vessels, atresia of the pulmonary artery, and a patent ductus arteriosus. Since this review, seven other cases have been reported, by Alexander and White (1), Taussig (14), Robinson and Howard (11), and Dunsky (7). Tricuspid stenosis or atresia with hypoplasia of the right ventricle has recently stimulated considerable interest among cardiac surgeons. It has been found that in many patients with this anomaly the operative procedures used in the treatment of congenital pulmonic stenosis (2, 10) are beneficial. The functional improvement that is obtained by such surgical intervention appears to be greatest when there is a well established interauricular septal defect. Furthermore, surgery has been most effectively carried out when an exact knowledge of the anatomy of the cardiac structures and great vessels was established before the operation. It is therefore important that the diagnostic procedures used in the evaluation of these cases be directed toward a precise determination of the existing anatomical and physiological relationships if operative correction is planned. Abnormal Anatomy and Course of the Circulation Tricuspid stenosis or atresia is always associated with a poorly functioning or non-functioning right ventricle. The hypoplastic ventricle seems to be the fundamental abnormality in this disorder, since it may occur without tricuspid stenosis or atresia as we have recently seen in two instances at autopsy. There is always an associated patent foramen ovale or interauricular septal defect, generally combined with other defects which provide a circulation compatible with life. The narrowing of the tricuspid valve is variable. In the extreme, it may be represented by only a small nodule (Fig. 1) or depression in the floor of the right auricle. In such an instance the right ventricle may be so small that it is found only with difficulty at autopsy (Fig. 8). On the other hand, the tricuspid valve may have well developed leaflets even though the orifice is reduced in size (Fig. 2). In such instances, the right ventricle, although small, has a normal myocardium, and it may be assumed that it participates in the maintenance of the pulmonary circulation.