Angiocardiographic findings in tricuspid atresia: correlation with hemodynamic and morphologic features.

Abstract

Although the general morphologic and hemodynamic features of tricuspid atresia are well established, exact angiocardiographic diagnosis of such associated conditions as pulmonary stenosis and transposition of the great arteries may be a problem. Therefore, we reviewed the angiocardiographic and hemodynamic findings in tricuspid atresia from 20 patients and from autopsies upon the hearts of 14 patients to determine the relationship of the angiocardiographic features to the morphologic and hemodynamic findings. Special attention was given to the relationship between the size of the right ventricle and the size of the ventricular septal defect (VSD), since recent morphologic studies of tricuspid atresia (1) indicate that the former measurement depends in part upon the latter. Material and Methods The records of 31 patients with tricuspid atresia seen between 1946 and 1966 were studied, and thoracic roentgenograms were available for review in 27 of them. Large-film, biplane angiocardiography was performed with injection of contrast material into the right atrium and left ventricle in 16 cases, while injections were made in only one of these sites in 4 other cases. Cardiac catheterization was carried out simultaneously in these 20 patients. Both atria and the left ventricle were entered to measure the pressures and saturations. To calculate pulmonary flow, it was assumed that pulmonary arterial saturation was equal to systemic arterial saturation in 16 cases. In 3 cases the pulmonary artery had been entered by suprasternal puncture to measure flow and pressure, and in one the catheter was directed from the left ventricle through a VSD into the pulmonary artery (Fig. 1). Morphologic Findings These comments pertain to the 14 cases in which autopsy was performed (Table I). No remnant of the tricuspid valve was present in the floor of the enlarged right atrium. A “dimple,” i.e., a tiny, endocardium-lined, blind pocket, was identified anterior and to the left of the ostium of the coronary sinus in 9 of the 14 cases. Ten of the 14 specimens were examples of Type I tricuspid atresia (2,3), in which the great arteries were normally related. In all 10, the right ventricle was a small chamber whose size varied. It appeared as a tiny, endocardium-lined muscular chamber in the one example in which the pulmonary valve was atretic (Type IA); no VSD was present in this heart. In the 8 cases of Type IB (with subpulmonary stenosis), the right ventricle was a small, elongated chamber that lay obliquely along the base of the heart, with a diameter the same as that of the pulmonary artery. The site of obstruction to pulmonary blood flow was the narrow, tubular-shaped VSD (1). The one specimen of Type IC (no obstruction to pulmonary flow; 4) had the largest right ventricle of any of the Type I hearts, and the VSD was large. Thus, the size of the right ventricle in these hearts was directly related to the size of the VSD.