Clinical and pathologic findings of an unusual case of cardiac malformation are presented. The main features were those of atresia of the right atrioventricular valve associated with two distinct atrioventricular orifices connecting the morphologically left atrium to the underlying morphologically left ventricle. Other distinguishing characteristics were ostium secundum atrial septal defect, no...

The occurrence of aortic obstruction in patients with cervical arch is very rare. The clinical and angiocardiographic findings of two patients with this combination of defects are presented. One of these patients, in whom tricuspid atresia with reduced pulmonary blood flow was also present, had a successful resection of the obstruction together with construction of a Waterston shunt.

A patient with tricuspid atresia and characteristic electrocardiographic features of counterclockwise and superiorly oriented frontal plane QRS loop (left anterior hemiblock) is presented. Operative intervention resulted in a clockwise and inferior rotation of the frontal QRS loop (left posterior hemiblock) without the development of complete left bundle branch block. This observation suggests ...

The simultaneous existence of double orifice right and left atrioventricular valves in the absence of ostium primum defects is rare and scarcely reported. We present the case of a 20-month old boy diagnosed with tetralogy of Fallot with pulmonary atresia who was found to have associated double-orifice mitral and tricuspid valves.

OBJECTIVE Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia. METHODS A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul), from August 1980 through January 2000, of 25 patients with a long-term follow-up, out of a series of 36 patients who underwent the Fontan...

Shinebourne, E. A., Haworth, S. G., Anderson, R. H., and Ulgur, A. (1974). Archives of Disease in Childhood, 49, 729. Differential diagnosis of congenital heart disease in the first 3 months of life: significance of a superior (left) QRS axis. The ECGs of 473 infants under the age of 3 months who were referred to a paediatric cardiological unit were analysed; 47 (10%) of the ECGs showed a super...

Increased inspiratory right ventricular filling with a consequent shift of the ventricular septum to the left, thereby decreasing left ventricular filling, has been suggested as a mechanism of pulsus paradoxus. We recently saw a patient with tricuspid atresia and hypoplastic right ventricle who developed pulsus paradoxus. His course may help clarify the contribution of this mechanism.

Enlargement of right atrium is usually secondary to pulmonary hypertension due to valvular heart diseases or obstructive pulmonary disorders, atrial septal defect, tricuspid atresia or stenosis, pulmonary stenosis, primary pulmonary hypertension, Ebstein's anomaly. Congenital enlargement of right atrium is rare and it commonly presents in children. Our patient presented with congenital giant ri...

OBJECTIVES To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia. METHODS Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302)...

In tricuspid atresia, an obligatory right to left shunt occurs at the atrial level. We have observed several patients with left to right interatrial shunts. Data from cardiac catheterisation in 40 consecutive patients were reviewed to determine the frequency and mechanism of left to right shunting in tricuspid atresia. An increase of 6% or more in oxygen saturation between the superior vena cav...