Ventricular Septation of the Double-Inlet Ventricle: Over Three Decades of Follow-Up.

There is renewed interest in septation of the double-inlet ventricle as an alternative to Fontan palliation. We examined our septation experience with over 30 years of follow-up. We retrospectively reviewed patients with double-inlet ventricle from 1990 to 2011.

Staged Ventricular Septation of the Double-Inlet Ventricle: How-I-Do-It.

Ventricular septation of the double-inlet ventricle is a largely abandoned operation due to poor historical outcomes. However, there has been renewed interest in septation as an alternative to Fontan palliation given its long-term sequelae. As one of the few centers to revisit septation in the early 1990s, our institution has long-term data on a series of patients with a double-inlet ventricle who underwent biventricular repair.

Outcomes of Surgical Repair of Complex D-Transposition of the Great Arteries.

Background: D-transposition of the great arteries (TGA) or TGA-type double outlet right ventricle (DORV) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) may be treated with the arterial switch operation (ASO), Rastelli, Réparation à l'Etage Ventriculaire (REV), or Nikaidoh procedures. We evaluated midterm results of these techniques.

Methods: We retrospectively reviewed 42 cases of anatomic repair from 2005 to 2014 at our institution for TGA (n = 29) or TGA-type DORV (n = 13) with VSD and LVOTO.

Coding of coronary arterial origin and branching in congenital heart disease: The modified Leiden Convention.

Objectives: Variations in coronary anatomy are common and may relate to the position of the coronary ostium relative to the aortic sinus, the angle of coronary take-off, or the course of the coronary arterial branches. Several classification systems have been proposed. However, they all lack a simple rationale that is applicable irrespective of the relative position of the great arteries, as well as in bicuspid aortic valves.

Outcomes of the Arterial Switch Operation in ≤2.5-kg Neonates.

Although low birth weight is a known risk factor for mortality in congenital heart lesions and may consequently delay surgical repair, outcomes in low-weight neonates undergoing the arterial switch operation (ASO) have not been well described. Our objective was to assess the safety of this procedure in infants weighing ≤2.5 kg at the time of surgery.

Significance of Intraoperative Revision During Arterial Switch Operation in the Current Era.

Background: Arterial switch operation (ASO) is a relatively safe operation nowadays. We hypothesize that intraoperative revision for coronary malperfusion still plays an important role during ASO in the current era.

Methods: From January 2005 to May 2016, 244 patients underwent ASO.

Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair.

Background: Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) is a challenging operation increasingly being performed as a complete, primary repair in infancy. Previous studies have focused on perioperative outcomes; however, midterm valve function, ventricular function, and residual obstruction have received little attention.

Methods: We retrospectively reviewed 20 patients who underwent CAVC/TOF repair (January 2005 to December 2014).

Aiming to Preserve Pulmonary Valve Function in Tetralogy of Fallot Repair: Comparing a New Approach to Traditional Management.

Pulmonary valve (PV) incompetence following transannular patch (TAP) repair of tetralogy of Fallot (TOF) results in long-term morbidity and mortality. Valve-sparing repairs have recently gained recognition; however, they may be associated with residual pulmonary stenosis (PS) in patients with small PV z scores. We sought to determine whether a repair that increases the PV annulus and augments the valve leaflet with a biomaterial would result in annular growth and in longer duration of valve competence compared with TAP.

Postoperative tricuspid regurgitation after adult congenital heart surgery is associated with adverse clinical outcomes.

Objective: Many patients with adult congenital heart disease will require cardiac surgery during their lifetime, and some will have concomitant tricuspid regurgitation. However, the optimal management of significant tricuspid regurgitation at the time of cardiac surgery remains unclear. We assessed the determinants of adverse outcomes in patients with adult congenital heart disease and moderate or greater tricuspid regurgitation undergoing cardiac surgery for non-tricuspid regurgitation-related indications.

Repair of Anomalous Left Coronary Artery From the Right Pulmonary Artery: A Series of Nine Cases.

Background: Repair of anomalous left coronary artery from the right pulmonary artery presents a particular technical challenge to the congenital cardiac surgeon. There is disagreement in the literature over the optimal technique for this defect, with some authors advocating for unroofing of the periaortic segment of coronary artery, while others prefer direct aortic reimplantation of the artery.

Methods: We performed a retrospective study examining outcomes of patients who were repaired for this anomaly at our institution.

Norwood Stage I Palliation in Patients Less Than or Equal to 2.5 kg: Outcomes and Risk Analysis.

Background: Hospital mortality of stage I palliation in patients with hypoplastic left heart syndrome and weighing less than or equal to 2.5 kg remains high. We aimed to (1) assess outcomes in this specific population in a dedicated neonatal cardiac program and (2) determine factors associated with poor outcomes.

Early Complete Atrioventricular Canal Repair Yields Outcomes Equivalent to Late Repair.

Background: Repair of complete atrioventricular canal early in infancy has traditionally carried greater morbidity and mortality than repair performed later. However, an individualized anatomy-based repair may give young infants outcomes that are equivalent to older patients.

Methods: We retrospectively reviewed 139 patients who underwent complete atrioventricular canal repair from January 2005 to December 2012.

Repair of Tetralogy of Fallot in Children Less Than 4 kg Body Weight.

We reviewed our experience of surgical repair of Tetralogy of Fallot (TOF) in children weighing less than or equal to 4 kg to compare outcome of early palliation versus complete repair as the initial surgical approach. Seventy-six patients, weighing ≤ 4 kg, with TOF surgery between January 2005 and September 2013 were included in this single-center retrospective study. Twenty-five patients who underwent initial shunt procedure followed by later full repair were compared to 51 patients who had primary full repair for differences in baseline characteristics and outcomes.

Outcomes of cardiac surgery in patients weighing <2.5 kg: affect of patient-dependent and -independent variables.

Objective: A recent Society of Thoracic Surgeons database study showed that low weight (<2.5 kg) at surgery was associated with high operative mortality (16%). We sought to assess the outcomes after cardiac repair in patients weighing <2.

Earlier arterial switch operation improves outcomes and reduces costs for neonates with transposition of the great arteries.

Objectives: This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries (d-TGA).

Background: Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates.

Primary arterial switch operation as a strategy for total correction of Taussig-Bing anomaly: a 21-year experience.

Background: Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the long-term outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly.

Methods And Results: A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011.

Intact right ventricle-pulmonary artery shunt after stage 2 palliation in hypoplastic left heart syndrome improves pulmonary artery growth.

For patients with hypoplastic left heart syndrome who have undergone the Norwood procedure with a right ventricle-pulmonary artery (RV-PA) shunt, the shunt can either be removed or left intact at the time of the stage 2 procedure. This study aimed to determine the effects of an intact shunt on pulmonary artery growth and clinical outcomes after the stage 2 procedure. A retrospective review of patients who underwent Norwood with an RV-PA shunt from 2005 to 2010 was performed.

Hypoplastic right cervical aortic arch.

We describe a neonate with a rare congenital anomaly of the aorta. The anomaly included a hypoplastic aortic arch that was cervical and right sided. This complex combination was treated by a Norwood type procedure reconstructing a right-sided arch and, in a later stage, a Rastelli procedure.

Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence.

Objectives: The Ross procedure is used to treat aortic valve disease in children. The advantages include autograft growth, long-term durability, and avoidance of anticoagulation. Long-term follow-up of the Ross procedure in infancy is limited.

The effect of cardiopulmonary bypass prime volume on the need for blood transfusion after pediatric cardiac surgery.

Objective: There is increasing awareness that erythrocyte transfusions after pediatric cardiac surgery have deleterious effects. Despite reports of decreased transfusion requirements associated with smaller cardiopulmonary bypass circuits, the relationship between circuit prime volume and need for transfusion has not been systematically examined.

Methods: Pediatric patients at our institution who underwent cardiopulmonary bypass between January 2005 and December 2010 were reviewed.

Cardiac troponin I after cardiopulmonary bypass in infants in comparison with older children.

Objective: At the present time, there is a trend towards performing open heart surgery at a younger age. Myocardium of infants has been thought to be more vulnerable to cardiopulmonary bypass in comparison with adults. For this study, we evaluated the degree of myocardial injury by measurement of cardiac troponin levels in infants in comparison with older children for similar surgeries.

Outcomes after transplantation for "failed" Fontan: a single-institution experience.

Objective: Despite the excellent outcomes in the current era after the Fontan procedure, it continues to have an inherent risk of failure. Cardiac transplantation provides 1 option for treating these patients; however, the indications for, timing of, and outcomes after, transplantation remain undefined. We examined our own institutional experience with transplantation for failed Fontan.

Outcomes in pediatric cardiac transplantation with a positive HLA cross-match.

Previous studies have shown poor outcomes in pediatric heart transplant recipients with a high PRA or a positive direct donor-recipient cross-match. This study describes outcomes in patients with a positive cross-match at a large pediatric program. Pediatric heart transplant patients at a large single center between January 1993 and July 2009 were reviewed; those with cross-match data were analyzed.