Remote magnetic navigation ablation of incisional atrial flutters in adolescent with a giant right atrium after surgical correction of tetralogy of Fallot and Ebstein's anomaly

We describe a clinical case of a 17-years-old adolescent with congenital heart disease after three open-heart surgery procedures for correction of tetralogy of Fallot and Ebstein's anomaly who presented with drug-resistant, persistent atrial flutter and giant right atrium (8.2 cm by transthoracic echocardiography). The successful ablation procedure of the two types of incisional atrial flutter was performed using remote magnetic navigation without any complications with 2.2 minutes of fluoroscopy. The patient remained free of any arrhythmias without antiarrhythmic drugs during 12 months of follow-up with a reduction of right atrium size (5.8 cm by transthoracic echocardiography).

Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein’s anomaly: A paradigm shift

Severe tricuspid valve regurgitation secondary to Ebstein’s anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. The overall results of neonatal tricuspid valve repair are unsatisfactory especially in sick neonates and those with preoperative hemodynamic instability. Single ventricle palliation utilizing the Starnes procedure with right ventricular exclusion provides a quicker way to improve hemodynamics and allows rapid decompression of the right ventricle but carries the long-term disadvantages of the single ventricle pathway. We were recently faced with a challenging case of neonatal Ebstein’s anomaly resulting in severe tricuspid valve regurgitation (TR) and significant hemodynamic and respiratory instability. We performed an initial stage I palliation with a modified Starnes’ procedure, which allowed stabilization and rapid recovery of the patient to be followed 5 months later with conversion to 2-ventricle repair using the cone technique. We believe combining these 2 strategies for suitable neonatal candidates may be a useful technique that should be considered in the algorithm for neonatal Ebstein’s anomaly.

Ebstein’s Anomaly, Left Ventricular Noncompaction and Gerbode-Like Defect Triad (Fetal Diagnosis and Neonatal Course)

Ebstein’s anomaly is characterized by the apical displacement of the septal and posterior leaflets of the tricuspid valve with atrialization of the right ventricle (RV). It is commonly associated with other heart defects including left ventricular noncompaction. We describe a case of prenatally diagnosed Ebstein’s anomaly in association with left ventricular noncompaction and a septal defect between the left ventricle and the atrialized portion of the RV (Gerbode-like defect). The patient underwent a modified Blalock−Taussig shunt followed by Glenn procedure because of severe RV hypoplasia and RV outflow tract obstruction. The patient tolerated both procedures and is doing clinically well in anticipation of Fontan procedure for single ventricle palliation.

Antenatal Diagnosis of a Partial Atrioventricular Canal with Ebstein’s Anomaly

The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein’s anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient’s antenatal diagnosis of both Ebstein’s anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.