Major aortopulmonary collateral arteries in association with atrioventricular septal defect with balanced ventricles and trisomy 21.

A major aortopulmonary collateral artery is a rare and easily missed diagnosis that is usually associated with Tetralogy of Fallot or pulmonary atresia. We present two cases of major aortopulmonary collaterals associated with trisomy 21 and atrioventricular septal defect with balanced ventricles in which the diagnosis went undetected until after initial cardiac repair.

Electrophysiological characteristics of atrial tachycardia recurrence: Relevance to catheter ablation strategies in adults with congenital heart disease.

Background: Catheter ablation outcomes for adults with congenital heart disease (ACHD) are described, but recurrence mechanisms remain largely unknown.

Objective: The purpose of this study was to identify the electrophysiological characteristics of atrial tachycardia (AT) recurrence in ACHD.

Methods: ACHD atrial tachycardia procedures over a 10-year period were explored for AT or atrial fibrillation (AF) recurrence.

Multicenter Outcomes of Catheter Ablation for Atrioventricular Reciprocating Tachycardia Mediated by Twin Atrioventricular Nodes.

Objectives: This study sought to describe the electrophysiologic properties and catheter ablation outcomes for atrioventricular reciprocating tacchycardia via twin atrioventricular nodes (T-AVRT).

Background: Although catheter ablation for T-AVRT is an established entity, there are few data on the electrophysiological properties and outcomes of this procedure.

Methods: An international, multicenter study was conducted to collect retrospective procedural and outcomes data for catheter ablation of T-AVRT.

Mechanisms and outcomes of catheter ablation for biatrial tachycardia in adults with congenital heart disease.

Background: Biatrial tachycardia (BiAT) is a rare form of macroreentry not previously characterized in adults with congenital heart disease (ACHD).

Objective: The purpose of this study was to determine the prevalence, mechanisms, and outcomes of catheter ablation for BiAT in ACHD.

Methods: All ACHD undergoing catheter ablation for macroreentrant atrial tachycardia over a 10-year period were evaluated for evidence of BiAT.

Electrophysiological Characteristics of Intra-Atrial Reentrant Tachycardia in Adult Congenital Heart Disease: Implications for Catheter Ablation.

Background Ultra-high-density mapping enables detailed mechanistic analysis of atrial reentrant tachycardia but has yet to be used to assess circuit conduction velocity (CV) patterns in adults with congenital heart disease. Methods and Results Circuit pathways and central isthmus CVs were calculated from consecutive ultra-high-density isochronal maps at 2 tertiary centers over a 3-year period. Circuits using anatomic versus surgical obstacles were considered separately and pathway length <50th percentile identified small circuits.

Pulmonary vein and left atrial posterior wall isolation for the treatment of atrial fibrillation: Comparable outcomes for adults with congenital heart disease.

Introduction: Optimal treatment strategies for ACHD with AF are unknown. This study sought to assess outcomes of pulmonary vein isolation (PVI) ± left atrial (LA), posterior wall isolation (PWI) for adults with congenital heart disease (ACHD), and atrial fibrillation (AF).

Methods: A retrospective review of all cryoballoon (CB) PVI ± PWI procedures at a single center over a 3-year period were performed.

Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.

Objectives: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs).

Background: LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown.

Methods: A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes.

Peripheral venous pressure accurately predicts central venous pressure in the adult Fontan circulation.

Background: Patients with the Fontan circulation lack a subpulmonary ventricular pump and thus the main driver for pulmonary blood flow is a high central venous pressure. Peripheral venous pressure (PVP) measurement has been shown to be a reproducible and fairly accurate surrogate for central venous pressure (CVP), but not specifically for the adult Fontan circulation. This study aims to determine the relationship of PVP to CVP in adult Fontan patients.

Ten-year outcomes of transcaval cardiac puncture for catheter ablation after extracardiac Fontan surgery.

Background: Although they are at lower risk, patients with previous extracardiac conduit (EC) Fontan still may require catheter ablation for supraventricular arrhythmia.

Objective: The purpose of this study was to determine the optimal approach to pulmonary venous atrium (PVA) access after EC Fontan operation.

Methods: All electrophysiological procedures requiring PVA over a 10-year period at the UCLA Medical Center were reviewed.

Permanent conduction system pacing for congenitally corrected transposition of the great arteries: A Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) Collaborative Study.

Background: Congenitally corrected transposition of the great arteries (CCTGA) is associated with spontaneous atrioventricular block and pacing-induced cardiomyopathy. Conduction system pacing is a potential alternative to conventional cardiac resynchronization therapy (CRT).

Objective: The purpose of this study was to determine the outcomes of conduction system pacing for CCTGA.

Ultrahigh-density mapping supplemented with global chamber activation identifies noncavotricuspid-dependent intra-atrial re-entry conduction isthmuses in adult congenital heart disease.

Objective: To evaluate the role of ultrahigh-density mapping for conduction isthmus (CI) characterization in adult congenital heart disease (ACHD).

Background: Catheter ablation remains suboptimal for ACHD with atypical intra-atrial reentrant tachycardias (IART) that can be challenging to define using existing mapping technology.

Methods: An ultrahigh-density mapping system was selectively employed over a 1-year period for procedures involving noncavotricuspid isthmus-dependent-IART.

Catheter ablation of supraventricular tachycardia after tricuspid valve surgery in patients with congenital heart disease: A multicenter comparative study.

Background: Tricuspid valve (TV) surgery is often required for adult congenital heart disease (ACHD), but may hinder catheter ablation when an artificial material or imbricated tissue covers the tricuspid annulus.

Objective: The purpose of this study was to determine the outcomes of catheter ablation after TV surgery in a large ACHD cohort.

Methods: An international retrospective study involving 7 centers was conducted.

A minimally invasive hybrid approach for cardiac resynchronization of the systemic right ventricle.

Background: Patients with systemic right ventricle (RV) often develop progressive heart failure and may benefit from cardiac resynchronization therapy (CRT); however, the optimal strategy for CRT has not been defined.

Methods: A retrospective review of all the patients with systemic RV failure undergoing a hybrid transcatheter-surgical approach was performed. Procedural technique and outcomes are reported.

Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients.

Objectives: The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA).

Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population.

Clinical and electrophysiological properties of atrial tachycardia after pediatric heart transplantation.

Background: Pediatric heart transplant recipients are at an elevated risk for development of atrial tachycardia (AT); however, the underlying mechanisms and long-term outcomes are unclear.

Objective: We hypothesized that occurrence of AT in pediatric heart transplant recipients would be associated with a higher frequency of adverse events during follow-up.

Methods: A single-center retrospective review of all pediatric heart transplant recipients with suspected AT between 1997 and 2017 was performed.

Single-Center Experience Using Selexipag in a Pediatric Population.

Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi), was FDA approved in the US.

Mechanisms and predictors of recurrent tachycardia after catheter ablation for d-transposition of the great arteries after the Mustard or Senning operation.

Background: The Senning and Mustard operations for dextro-transposition of the great arteries are associated with an increased risk for supraventricular tachycardia. Catheter ablation has been shown to be acutely successful for achieving rhythm control in this population, but the mechanisms of recurrence are ill-defined.

Objective: We hypothesized that the type and degree of recurrence would vary by the surgical technique used.

Very long-term results of surgical and transcatheter ablation of long-standing persistent atrial fibrillation.

Background: New hybrid approaches for atrial fibrillation (AF) ablation, combining surgical and percutaneous procedures, are emerging to enhance the long-term success rate of these 2 procedures severally considered. Recent guidelines underline the need for long-term follow-up to really assess the efficacy of AF ablation.

Methods: From 2000 to 2002, 33 patients with long-standing persistent AF and valvular heart disease underwent valve surgery and cryoablation (pulmonary veins isolation and mitral isthmus and roof line lesions).

Neonatal ascending aortic thrombus: successful medical treatment.

Neonatal aortic thrombosis is a potentially life-threatening condition with significant morbidity and mortality if undiagnosed and untreated. The most common location of arterial thrombosis in neonates is in the abdominal aorta and is associated with umbilical artery catheterisation. There are only a few previous reports of thrombosis in the ascending aorta.

Use of the Valsalva graft and long-term follow-up.

Objective: The Valsalva graft is a specifically designed Dacron graft that, on implantation and pressurization, generates pseudosinuses of Valsalva. We reviewed a multicenter experience of the reimplantation procedure with the Valsalva graft in patients with aneurysms involving the aortic root.

Methods: A total of 278 patients underwent valve-sparing aortic root replacement using the Valsalva graft at 4 different Italian cardiac surgery centers and were studied by clinical assessment and echocardiography.

Reimplantation valve-sparing aortic root replacement with the Valsalva graft: what have we learnt after 100 cases?

Objectives: Reimplantation valve-sparing aortic root replacement has been increasingly performed with improving perioperative and mid-term results. The success of this operation primarily depends on preserving the highly sophisticated dynamic function of the aortic valve by recreating an anatomical three-dimensional configuration similar to the normal aortic root, thus minimizing the mechanical stress and strain on the cusps. Over the years several techniques have been proposed to reproduce the sinuses of Valsalva.

Reimplantation valve-sparing aortic root replacement for aortic root aneurysm in the elderly: are we pushing the limits?

Objectives: Reimplantation valve-sparing aortic root replacement has been increasingly performed with improving perioperative and midterm results. However, extending the age criterion in patient selection remains a debate. This study reviews the results of reimplantation valve-sparing aortic replacement in patients greater than 60 years of age.

Reoperation for aortic false aneurysms: our experience and strategy for safe resternotomy.

Background And Aim Of The Study: To review our experience with reoperation for aortic false aneurysms (FA) and to present an analysis of the relevant surgical approaches and risks.

Methods: From May 1999 to June 2006, 11 patients underwent a total of 13 reoperations due to aortic false aneurysms, with an incidence of 3% of all thoracic aortic cases. Cardiopulmonary bypass (CPB) and cooling were started before sternotomy in all cases.