CONGENITAL HEART DISEASE WITH THE IVEMARK SYNDROME AND ABSENCE OF THE INFERIOR VENA CAVA.

Abstract

THIS COMMUNICATION reports a case of complicated congenital heart disease with the Ivemark syndrome in which an unusual malformation was observed. During catheterization performed to establish the above diagnosis, an anomalous inferior vena cava with azygos continuation was visualized. Case Report The patient was a four-year-old girl known to suffer a congenital heart disease because of severe cyanosis and a systolic murmur. She measured 96 em and weighed 12.500 g. Blood pressure was 95/ 60 mm Hg with palpable femoral pulsations. There was clubbing of the fingers. By auscultation, the first sound was slightly accentuated, the second sound pure, and a holosystolic murmur II/IV was heard over the third and fourth interspaces along the right sternal border. The phonocardiographic tracing confirmed the auscultation. Roentgenographic studies of the thorax (Figs. 1 and 2) showed an abnormal shift to the right in the cardiac shadow. Electrocardiography performed with the wires in the usual position disclosed an extreme right axis deviation and a biventricular hypertrophy. The presence of a wandering pacemaker prohibited the drawing of any conclusions concerning eventual atrial inversion. Heinz bodies were revealed in the examination of the blood, indicating agenesis of the spleen. A roentgen examination of the gastrointestinal tract with oral administration of contrast medium demonstrated a common mesentery. The clinical diagnosis of I vemark syndrome with multiple congenital malformations was stated. Right heart catheterization and, later, left heart catheterization, both with angiography, were undertaken. Right Heart Catheterization: After denudation of the vena saphena, a catheter reached the atrium through an azygos continuation of the inferior vena cava. A large atrial septal defect, perhaps a single atrium, was noted. The superior vena cava was placed normally on the right. A partial anomalous pulmonary venous return of the right superior lobe was proved by oximetry in the superior vena cava. Two ventricular cavities were penetrated, and the similar pressure in them indicated a large ventricular septal defect. The great vessels could not be catheterized. The angiography was performed in the azygos continuation which traced an arc before entering the right atrium (Figs. 3 and 4). The hepatic veins entered the right atrium directly. Once the medium penetrated this cavity, a simultaneous opacification of both atria and both ventricles occurred, which proved the atrial septal defect. No further diagnostic information, however, could be obtained from this procedure. Left Heart Catheterization: After denudation of the femoral artery, a catheter was introduced and advanced into the aorta and finally into the left ventricle. The aortic valve was easily passed. No aortic gradient was found.