Angiographic and catheterization findings in patients with difficulties after total correction of tetralogy of Fallot.

Abstract

Tetralogy of fallot is an anatomically complex intracardiac defect resulting in right-to-left or bidirectional shunts at the ventricular level and decreased pulmonary blood flow. The peripheral arterial unsaturation varies with the size of the interventricular defect and its anatomic relationship to the enlarged aortic root, as well as with the severity of the right ventricular outflow obstruction. The muscular hypertrophy of the right ventricle may be pronounced. Total repair of the anomaly achieves physiologic correction by separation of the intracardiac streams and by relief of the pulmonic stenosis. The repair may or may not result in perfect anatomic or entirely normal intracardiac pressure relationships. Of 124 patients operated upon, there were 102 long-term survivors in this series. Twelve of these were restudied by cardiac catheterization and angiocardiography performed from two months to ten years after total correction of the defects. The most common indication for re-examination was the presence of a loud systolic murmur over the pulmonary area suggesting significant residual pulmonary stenosis. Other indications were a loud diastolic murmur or parasternal systolic murmur, cardiac enlargement and failure, a bulge along the left upper cardiac border on the chest roentgenogram suggestive of right ventricular outflow tract aneurysm, and electrocardiographic evidence of increasing right ventricular hypertrophy. Table I summarizes the catheterization and angiographic findings in these patients as well as their subsequent course. Three have had repeat operations with good results. Examples of the lesions found in seven patients are described in the following brief case reports and illustrated in the accompanying figures. Case Reports Case I (N. R): The diagnosis of tetralogy of Fallot was established in this boy at three years of age. Preoperatively a bidirectional shunt was found at the ventricular level. No atrial septal defect was diagnosed. The right ventricular systolic pressure was 88 mm Hg, and there was a 76 mm Hg gradient across the pulmonary valve. One of the views from the lateral angiocardiogram is shown in Figure 1, A. At operation, a moderate amount of infundibular stenosis was encountered and resection was done. The stenosed pulmonary valve was opened by incising two of the commissures, and the ventricular septal defect was closed with a Teflon patch. After surgery, digitalization was necessary because of congestive heart failure, and systolic and diastolic murmurs persisted over the pulmonic area. The electrocardiogram revealed complete right bundle branch block. The child was recatheterized at five years of age because of the persistent murmurs and cardiac failure. At this time the systolic right ventricular pressure was 110 mm Hg in the inflow tract.