An 8-month-old female infant was diagnosed with tricuspid atresia in utero by an ultrasound and the diagnosis was confirmed at birth. She underwent a modified Blalock-Tausig shunt at the age of 2 months to treat increasing cyanosis. She did well and is waiting to complete single ventricle palliation. On her recent follow-up, she was asymptomatic, with a systemic arterial saturation of 80% at ro...

OBJECTIVE To describe the spectrum of cardiac defects in monochorionic (MC) twins discordant for congenital heart disease (CHD) in a referral center population. METHOD Retrospective study of all twin gestations undergoing echocardiography between 2000 and 2009 at our institution. RESULTS A total of 356 twin pairs were evaluated during the study period, 202 for suspected twin-twin transfusio...

Objective: To compare the prevalence at live birth and the spectrum of cardiovascular malformations in infants born to diabetic mothers with pre-existing diabetes with that in infants of non-diabetic mothers. Design: Prospective study of all live births in the resident population of one health region, with recording of details of the outcome of all pregnancies of women with pre-existing diabete...

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patient's ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 4...

Raghib Syndrome is a rare developmental complex, which consists of persistence of the left superior vena cava (PLSVC) along with coronary sinus ostial atresia and atrial septal defect. This Raghib complex anomaly has also been associated with other congenital malformations including ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. Our case demonstrates a...

BACKGROUND The Fontan operation is the usual goal of therapy for children with tricuspid atresia. The influences of morphology and different management strategies on survival and subsequent suitability for this procedure are crucial but unstudied in an unselected population during the Fontan era. METHODS AND RESULTS The fates of 237 consecutive infants with tricuspid atresia were reviewed (19...

OBJECTIVE To evaluate the additional information provided by colour Doppler in the ultrasonic assessment of congenital heart disease. PATIENTS AND METHODS A prospective study of 215 children (age range 1 day-16 years) presenting with clinical signs of congenital heart disease. RESULTS Colour Doppler was essential for the diagnosis of an anomalous left coronary artery and altered the managem...

Hypoplasia of the right ventricle, unassociated with severe pulmonary or tricuspid valvar malformations, is a primary congenital abnormality in which the trabeculated sinus portion of the ventricle fails to develop. An atrial septal defect or a stretched foramen ovale serves as an escape valve. The clinical, hemodynamic, and angiocardiographic features of this rare type of cyanotic heart diseas...

We present a case of one-day-old newborn, without prenatal diagnosis, referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation 80%. Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation, functional pulmonary atresia intact ventricular septum reversal flow the large patent ductus arteriosus (PDA). ...

or atresia. Seventy-one per cent of the infant group and 90% of patients over 1 year of age were long-term survivors (up to 6 years). Tetralogy of Fallot was the most commonly encountered anomaly in all 141 patients (66%); transposition of the great vessels and pulmonary stenosis occurred in 18%; and tricuspid atresia with pulmonary stenosis in 10%. The presence of an excessively large shunt an...