Background—The underlying electrophysiological mechanism that causes an abnormal ECG pattern and ventricular tachycardia/ventricular fibrillation (VT/VF) in patients with the Brugada syndrome (BrS) remains unelucidated. However, several studies have indicated that the right ventricular outflow tract (RVOT) is likely to be the site of electrophysiological substrate. We hypothesized that in patie...

OBJECTIVE Patients with Williams syndrome (WS) undergoing cardiac surgery are at risk for major adverse cardiac events (MACE). Prevalence and risk factors for such events have not been well described. We sought to define frequency and risk of MACE in patients with WS using a multicenter clinical registry. METHODS We identified cardiac operations performed in patients with WS using the Society...

OBJECTIVES Surgical reconstruction of the right ventricular outflow tract (RVOT) often requires implantation of a valved conduit. A single-centre 10-year experience with the Hancock porcine-valved Dacron conduit was retrospectively assessed. METHODS The records of 63 patients who underwent RVOT reconstruction with Hancock conduit implantation between August 2000 and July 2010 were retrospecti...

OBJECTIVE To assess the indication, technical aspects, and outcome of stenting of the right ventricular outflow tract (RVOT) in the management of symptomatic patients with severely limited pulmonary blood flow. METHODS Retrospective case note and procedure review of patients undergoing stenting of the RVOT over an 8 year period. PATIENTS Between 2005 and 2012, 52 selected patients underwent...

OBJECTIVE Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation. METHODS We performed a retr...

BACKGROUND Ventricular arrhythmia is an important cause of late death in patients with repaired tetralogy of Fallot (rTOF). By using an rTOF canine model, we investigated the role of repolarization alternans and its electrophysiological mechanisms. METHODS AND RESULTS Six dogs received right ventricular outflow tract (RVOT) transannular patch, pulmonary valve destruction, and right bundle bra...

BACKGROUND Among patients undergoing transcatheter pulmonary valve (TPV) replacement with the Melody valve, risk factors for Melody stent fracture (MSF) and right ventricular outflow tract (RVOT) reintervention have not been well defined. METHODS AND RESULTS From January 2007 to January 2010, 150 patients (median age, 19 years) underwent TPV implantation in the Melody valve Investigational De...

We thank Yurekli et al. [1] for their questions and comments relating to our publication, where we examined how the postoperative pulmonary annular gradient related to pulmonary valve annular size after total correction of tetralogy of Fallot [2]. We examined the patients who did not have significant postoperative right ventricular outflow tract obstruction (RVOTO). We discussed the implication...

OBJECTIVE Follow-up after tetralogy of Fallot (ToF) repair is directed to detect timely right ventricular (RV) dysfunction by following pulmonary regurgitation and global RV size, with little attention for the effective contribution of regional RV dysfunction. This study investigates the contribution of regional RV dysfunction on exercise capacity after ToF repair. METHODS Forty-two patients ...

OBJECTIVE Pulmonary regurgitation may cause progressive right ventricular dilatation and dysfunction in adult patients previously repaired for right ventricular outflow tract obstruction (RVOTO), and who require subsequent valve implantation for relief of these symptoms. Right ventricular recovery after pulmonary valve implantation (PVI) may be closely linked to the functional importance of the...