A Novel Case of Marfan Syndrome in an Infant With Hypoplastic Left Heart Syndrome.

This report describes a case of hypoplastic left heart syndrome (HLHS) and Marfan syndrome presenting in conjunction, and highlights how a connective tissue disorder may alter medical and surgical management of newborns with HLHS.

Successful Salvage of an Extracardiac Fontan in the Setting of Purulent Mediastinitis using Antibiotic-Impregnated Beads.

Post-cardiotomy mediastinitis is an especially serious complication after the implantation of prosthetic vascular grafts. Standard of care is irrigation, debridement, and removal of all prosthetic material present in the surgical field. The use of antibiotic impregnated beads at the site of infection has been reported in the salvage of vascular grafts in the adult population.

Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study.

Objectives: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch.

Methods: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141).

Quality of Life for Historic Cavopulmonary Shunt Survivors.

Background: Beginning with Dr William Glenn in 1958, 90 patients with congenital heart lesions underwent cavopulmonary (Glenn) shunts over a 30-year period. In 2015, the follow-up data on this original cohort were reported. The study focuses on the current quality of life of this cohort.

Early Extubation After Repair of Tetralogy of Fallot and the Fontan Procedure: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

Background: There is increasing interest in reducing the duration of mechanical ventilation after pediatric cardiac operations. However, the contemporary use of an early extubation strategy and its effect on clinical outcomes is poorly understood.

Methods: Data from The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2013) were analyzed to determine the association of early extubation with postoperative length of stay (PLOS).

Half a Century's Experience With the Superior Cavopulmonary (Classic Glenn) Shunt.

Background: Five decades after its introduction, the Glenn shunt remains an integral step for patients undergoing single-ventricle palliation. We performed a longitudinal follow-up of the original cohort of patients who underwent Glenn shunt.

Methods: We performed a retrospective study of the original cohort of patients who underwent Glenn shunt at Yale between 1958 and 1988.

Anomalous Origins of Coronary Arteries From the Pulmonary Artery: A Comprehensive Review of Literature and Surgical Options.

Anomalous origins of coronary arteries from the pulmonary artery are rare malformations in which the coronary arteries originate from pulmonary artery sinuses or branches. The consequences are variable although, in most cases, these anomalies lead to severe coronary hypoperfusion and ventricular dysfunction. Surgical correction is indicated once the diagnosis is established due to high early mortality associated with the disease.

Survival and right ventricular performance for matched children after stage-1 Norwood: Modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery conduit.

Objective: Early survival advantages after Norwood with right-ventricle-(RV)-to-pulmonary-artery conduit (NW-RVPA) over Norwood-operation with a Blalock-Taussig shunt (NW-BT) are offset by concerns regarding delayed RV dysfunction. We compared trends in survival, RV dysfunction, and tricuspid valve regurgitation (TR) between NW-RVPA and NW-BT for propensity-matched neonates with critical left ventricular outflow tract obstruction (LVOTO).

Methods: In an inception cohort (2005-2014; 21 institutions), 454 neonates with critical LVOTO underwent Norwood stage 1.

Persistent Pulmonary Hypertension in a Neonate With Transposition of Great Arteries and Intact Ventricular Septum: A Case Report and Review of the Literature.

Transposition of the great arteries (TGA) with intact ventricular septum (IVS) has very favorable short- and long-term surgical outcome. Although rare, when associated with persistent pulmonary hypertension (PPH), it exhibits significant mortality risk and management challenges. We report the case of a neonate with TGA with IVS and PPH who underwent successful early surgical repair with emphasis on clinical management and review of the literature.

Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I.

Objectives: To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia.

Methods: Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%).

Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.

Background: Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants.

Methods: Patients<30 years old (n=113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry.

Surgical repair of pulmonary venous stenosis: a word of caution.

Background: Pulmonary vein stenosis (PVS), both congenital and acquired, is challenging to treat surgically with uncertain long-term results. We reviewed an 11-year surgical experience in 52 children.

Methods: From 2002 to 2012, 52 children age 0 days to 13 years (mean 1.

Association of pulmonary conduit type and size with durability in infants and young children.

Background: Treatment of congenital heart disease may include placement of a right ventricle to pulmonary artery conduit that requires future surgical replacement. We sought to identify surgeon-modifiable factors associated with durability (defined as freedom from surgical replacement or explantation) of the initial conduit in children less than 2 years of age at initial insertion.

Methods: Since 2002, 429 infants were discharged from 24 Congenital Heart Surgeons' Society member institutions after initial conduit insertion.

Hemolytic anemia after placement of an intraluminal pulmonary artery band.

A newborn with double outlet right ventricle, malposed great vessels, hypoplastic aortic arch, and coarctation of the aorta with no restriction to pulmonary blood flow underwent palliation with arch augmentation and placement of an intraluminal Dacron patch pulmonary artery band (PAB). She subsequently presented in shock because of profound hemolytic anemia. Her intraluminal PAB was taken down and replaced with a traditional extraluminal band with resolution of her hemolysis.

Extracorporeal cardiopulmonary resuscitation for pediatric cardiac patients.

Background: Extracorporeal cardiopulmonary resuscitation (ECPR) has been shown to improve survival after in-hospital pediatric cardiac arrest. We describe our experience with ECPR for refractory cardiac arrest in pediatric cardiac patients.

Methods: We performed a retrospective analysis of the use of venoarterial extracorporeal membrane oxygenation (ECMO) for in-hospital cardiac arrest from 2002 to 2011.

Biventricular repair with the Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles.

Background: The Yasui procedure is employed in neonates with interrupted aortic arch and left ventricular outflow tract obstruction (IAA/LVOTO) or aortic atresia-severe stenosis with ventricular septal defect (AA/VSD) and 2 adequate-sized ventricles. This combines a Norwood arch reconstruction with a Rastelli operation establishing a biventricular repair.

Methods: From 2002 to 2011, 21 neonates aged 3 to 55 days (mean 12.

Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type.

Objective: The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt.

Methods: We used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors.

Impact of early fundoplication or gastrostomy tube on midterm outcomes for patients with single ventricle.

Objective: Patients with single-ventricle heart disease experience early and late failure during and after staged palliation. Little is known about the factors related to continued risk of transplantation and mortality after completion of staged palliation. The long-term outcome of patients with single-ventricle disease who require a gastric fundoplication early in life has not been assessed.

Association of feeding modality with interstage mortality after single-ventricle palliation.

Objective: Interstage mortality has been reported in 10% to 25% of hospital survivors after single-ventricle palliation. The purpose of this study was to examine the impact of feeding modality at discharge after single-ventricle palliation on interstage mortality.

Methods: We conducted a retrospective review of all neonates undergoing single-ventricle palliation from January 2003 to January 2010.

Supra-annular mitral valve replacement in children.

Background: Despite improved mitral repair techniques, some children need mitral valve replacement (MVR). Due to small annulus size, supra-annular MVR is useful.

Methods: From 2003 to 2010, 15 children had 23 supra-annular MVRs.