Atrioventricular Septal Defect With Tetralogy of Fallot and Exclusive Shunting at the Ventricular Level.

Atrioventricular septal defect (AVSD) with shunting restricted to the ventricular level is a rare form of AVSD. To our knowledge, this shunting pattern has not been reported in AVSD with tetralogy of Fallot. We report a child with this unusual combination who underwent a successful single-stage repair at two years of age.

A Rare Entity: Double Outlet of Both Ventricles.

Double outlet of both ventricles is an anomaly wherein both ventricles equally share the arterial trunks. A majority of the literature describes a variant of this disease with a muscular outlet septum which is perpendicular to the plane of the ventricular septum although a variant with a fibrous muscular septum can also be present. The condition may be associated with obstruction of either outflow tract, which may complicate repair. We report a child with double outlet of both ventricles and a fibrous outlet septum with unobstructed outflows. The child underwent a successful surgical repair using a two-patch technique.

Tricuspid Valve Dysplasia at Fetal Autopsy.

Introduction: Tricuspid valve abnormalities detected in fetal life include Ebstein anomaly and tricuspid valve dysplasia. The differentiation between these 2 entities can sometimes be challenging in the 2nd trimester fetus. We report a case of tricuspid valve dysplasia diagnosed on fetal autopsy. Case Report: A primigravida was diagnosed at 22 weeks' gestation to have Ebstein anomaly with severe tricuspid regurgitation. There was intra-uterine fetal demise. On fetal autopsy, the tricuspid valve leaflets were not apically displaced and the leaflets were nodular with rolled up edges. This supported a diagnosis of tricuspid valve dysplasia. Conclusion: The difficulties in differentiating Ebstein anomaly from tricuspid valve dysplasia due to inherent limitations in fetal imaging can be resolved by fetal autopsy. Valvular dysplasia will not have apical displacement of the valve leaflets.

Septal defect with polyvalvular involvement: A cardiac imaging hallmark of Trisomy 18.

Congenital Heart Diseases occur in close to 90% of children with Trisomy 18. A ventricular septal defect along with abnormalities of more than one cardiac valve is considered to be an imaging hallmark of Trisomy 18. We present echocardiographic images of an infant with Trisomy 18 who had a large ventricular septal defect and abnormalities of all cardiac valves.

Ivabradine in Post-operative Junctional Ectopic Tachycardia (JET): Breaking New Ground.

Junctional ectopic tachycardia (JET) is the commonest tachyarrhythmia in the early post-operative period in children undergoing open-heart surgery. It frequently leads to hemodynamic instability and needs to be managed aggressively. Amiodarone is the first-line agent along with non-pharmacological interventions. We report our initial experience with the use of Ivabradine in post-operative JET. A retrospective case records review of children with post-operative JET during the period from June 2018 to May 2019 was performed. Eight patients with post-operative JET were treated with Ivabradine during this period. The first patient was initially treated with Amiodarone. All eight patients responded to Ivabradine. The initial response was rate control permitting overdrive pacing. One patient had recurrence of JET 10 h after Ivabradine and after return to sinus rhythm. Amiodarone was administered along with the second dose of Ivabradine resulting in remission to sinus rhythm. Ivabradine appears to be an effective alternative to Amiodarone in children with post-operative JET based on our initial clinical experience.

Antenatal diagnosis of isolated infracardiac infradiaphragmatic total anomalous venous connection - Pictorial essay and discussion.

INTRODUCTION: Antenatal diagnosis of isolated infracardiac total anomalous pulmonary venous connection (TAPVC) is rare. Making the diagnosis antenatally is critical as delayed management could result in neonatal deterioration and poor outcome after surgery. METHOD: A multipara at 29 weeks of gestation was referred to our tertiary unit for ultrasound review. The fetal growth and biophysical profile were normal. A fetal echocardiogram revealed normal cardiac position with atrioventricular and ventriculoarterial concordance. There was a mild discrepancy in size of the right and left chambers of the heart. A connection between the pulmonary veins and the left atrium could not be established. A pulmonary venous confluence was noted posterior to the left atrium, from which a descending vertical vein emerged traversing the diaphragm and draining into the left portal vein into the liver. RESULTS: A diagnosis of infracardiac infradiaphragmatic total anomalous pulmonary venous connection was made. The pregnancy was delivered at 39 weeks by lower segment caesarean section. The antenatal findings were confirmed by postnatal echocardiogram. Successful sutureless repair of the pulmonary veins was performed. CONCLUSION: Isolated infracardiac total anomalous pulmonary venous connection can be diagnosed antenatally. This ensures early postnatal evaluation and successful repair.

Emergency Ross Procedure for Pediatric Aortic Valve Myxofibrosarcoma.

Primary cardiac tumors in children are uncommon and rarely demand surgical intervention. We report a malignant tumor arising from the aortic root in a 5-year-old boy presenting with left ventricular outflow tract obstruction and tumor embolism, its surgical management using the Ross procedure, and the unique histopathological aspects of the tumor.

A life-threatening infective pseudoaneurysm of the left ventricle in a toddler.

Pseudoaneurysms of the ventricle are an uncommon occurrence in children. They may be secondary to previous cardiac surgery or infection of the pericardial space. Infective pseudoaneurysms require urgent surgery because rupture of the pseudoaneurysm may have catastrophic consequences. The outcome with surgery is excellent. We report a toddler with ruptured pseudoaneurysm secondary to purulent pericarditis who recovered with an emergency surgical closure of the opening of the pseudo-aneurysm.