Preserving the pulmonary valve in Tetralogy of Fallot repair: Reconsidering the indication for valve-sparing.

Background: Tetralogy of Fallot (TOF) repair is a frequent procedure, and although valve-sparing (VS) repair is preferred, determining which patients can successfully undergo this operation remains controversial. We sought to identify parameters to determine a selective, accurate indication for VS repair.

Methods: We reviewed 71 patients (82%) undergoing VS repair.

Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery of an Infant.

A female presented 2 weeks after birth with an unbalanced atrioventricular canal, double outlet right ventricle, mild pulmonary stenosis, and patent ductus arteriosus that eventually caused pulmonary over circulation. After pulmonary artery banding, she experienced myocardial ischemia, suggesting interference with coronary blood flow by the band that had been placed on the main pulmonary trunk. The band was removed and revised to bilateral branch pulmonary artery banding, and cardiac function improved.

Predicting the Severity and Outcome of Persistent Pulmonary Hypertension of the Newborn Using New Echocardiography Parameters.

To examine echocardiographic parameters correlation with clinical severity indices, Alveolar- arterial gradient (A-a gradient), oxygenation index and clinical outcomes in newborns with persistent pulmonary hypertension of the newborn (PPHN). Retrospective cohort study of 67 subjects, >35 weeks' gestation with the diagnosis of PPHN admitted to the University of Kentucky neonatal intensive care unit (NICU) between September 2014 and December 2016. High left ventricular end-systolic eccentricity index (EIs) correlates with the overall clinical severity of PPHN as it is associated with higher A-a gradient and oxygenation index (P = 0.

Pediatric aortic valve repair: Any development in the material for cusp extension valvuloplasty?

Background: Aortic cusp extension is a technique for aortic valve (AV) repairs in pediatric patients. The choice of the material used in this procedure may influence the time before reoperation is required. We aimed to assess postoperative and long-term outcomes of patients receiving either pericardial or synthetic repairs.

Mortality and Reoperation Risk After Bioprosthetic Aortic Valve Replacement in Young Adults With Congenital Heart Disease.

Bioprosthetic aortic valve replacement (bAVR) in patients with congenital heart disease is challenging due to age, size and complexity. Our objective was to assess survival and identify predictors of re-operation. Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014.

Technical Modifications That Might Improve Long-term Outcomes of the Ross Procedure in Children.

Background: Failure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children.

Methods: Sixty-nine patients (median age, 12 years; range, 0.

Aortic root aneurysm repair in a neonate with Loeys-Dietz syndrome.

Loeys-Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow.

Background: Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure.

Methods: We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis.

Outcomes From Three Decades of Infant and Pediatric Heart Transplantation.

Infants are a unique transplant population due to a suspected immunologic advantage, in addition to differences in size and physiology. Consequently, we expect infants to have significantly different diagnoses, comorbidities, and outcomes than pediatric transplant recipients. In this study, we compare patterns and trends in pediatric and infant heart transplantation during three decades.

Congenitally Abnormal Aortic Valve Causing Coronary Obstruction and Cardiac Arrest in Infancy.

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge.

Reintervention rates after bioprosthetic pulmonary valve replacement in patients younger than 30 years of age: A multicenter analysis.

Objectives: To assess the difference in time to and predictors of reintervention according to valve type in surgical bioprosthetic pulmonary valve replacement (PVR) in patients younger than 30 years of age from multiple centers.

Methods: Data were retrospectively collected for 1278 patients <30 years of age undergoing PVR at 8 centers between 1996 and 2015.

Results: Mean age at PVR was 19.

Efficacy of Nitric Oxide Administration in Attenuating Ischemia/Reperfusion Injury During Neonatal Cardiopulmonary Bypass.

Objective: Nitric oxide (NO) plays several protective roles in ischemia/reperfusion (I/R) injury. Neonates undergoing the Norwood procedure are subject to develop I/R injury due to the immaturity of their organs and the potential need to interrupt or decrease systemic flow during surgery. We hypothesized that NO administration during cardiopulmonary bypass (CPB) ameliorates the I/R and could help the postoperative recovery after the Norwood procedure.

Repair of Superior Sinus Venosus Atrial Septal Defect Using a Modified Two-Patch Technique.

Background: Surgical repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection is usually performed with either a two-patch technique or the Warden procedure. However, both techniques can lead to superior vena cava or pulmonary vein stenosis, as well as sinus node dysfunction. To prevent these complications, we have utilized a modified two-patch technique.

Aortic Valvuloplasty or Rootplasty for Aortic Regurgitation.

At present, aortic valvuloplasty is considered an effective procedure for treatment of aortic regurgitation in pediatric patients. It has encouraging mid- and long-term results. The improved outcome is primarily related to better understanding of the functional anatomy of the normal valve and the different factors that alter it.

Surgical Management of Neonatal Ebstein's Anomaly Associated With Circular Shunt.

Ebstein's anomaly is a rare congenital cardiac malformation that may present in the neonatal period with life-threatening physiologic derangement, especially when it is associated with circular shunt. Urgent surgical intervention is critical for survival because of hemodynamic compromise. We review our experience with surgical management of neonates with Ebstein's anomaly associated with circular shunt and review the literature for similar reported cases.

Femoral vein homograft as Sano shunt results in improved pulmonary artery growth after Norwood operation.

Objective: To evaluate differences in interstage growth of pulmonary arteries between use of polytetrafluoroethylene and femoral vein homograft as Sano shunt during stage-I Norwood palliation.

Methods: A retrospective review of all patients who survived to the second stage following Norwood-Sano operation at two institutions was performed. Either polytetrafluoroethylene or the valved segment of femoral vein homograft was used for construction of the Sano shunt.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.

Objectives: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC).

Methods: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC.

Surgical Correction of Aberrant Right Coronary Anomalies Stranding an Aortic Commissure with and Without Unroofing.

The technique for successful surgical correction of an anomalous origin of the right coronary artery from the opposite aortic cusp with an aberrant course between the aorta and pulmonary artery is illustrated in a symptomatic 62-year-old woman. The intramural course of the right coronary artery traversed the tip of the commissure between the anterior and posterior leaflets, and its repair entailed unroofing of the intramural segment from inside the aortic intima. This technique required resuspension of the overlying commissure to maintain optimal aortic valve leaflet coaptation and prevent aortic insufficiency.

Relationship of Normal Aortic Valve Cusp Dimensions: A Tool to Optimize Cusp Reconstruction Valvuloplasty.

Outcomes of surgical aortic valvuloplasty hinge primarily on optimal leaflet design. Imprecise valve reconstruction can result in insufficiency or stenosis. Predicting postrepair leaflet parameters from pre- or intraoperative readily measurable valve dimensions could result in improved reconstruction.

Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Coronary artery anatomy represents a challenging and, often, determining predictor of outcome in an arterial switch operation (ASO). Impact of specific coronary artery variants, such as single, intramural and inverted, on time-related events following ASO, is, yet, to be determined. We sought to compare early and late outcomes within the group of nonstandard coronary artery variants.

Post-cardiotomy Rescue Extracorporeal Cardiopulmonary Resuscitation in Neonates with Single Ventricle After Intractable Cardiac Arrest: Attrition After Hospital Discharge and Predictors of Outcome.

Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified.

Hybrid pulmonary artery plication followed by transcatheter pulmonary valve replacement: Comparison with surgical PVR.

Objective/Background Historically, the sole option for patients with a dysfunctional native right ventricular outflow tract (RVOT) requiring re-establishment of pulmonary competence has been surgical PVR. We sought to compare early outcomes of hybrid pulmonary valve replacement (PVR) combining surgical plication of the main pulmonary artery followed by transcatheter PVR, with a contemporary cohort of surgical PVR patients. Methods Retrospective chart analysis of all patients with a dilated native RVOT eligible for surgical PVR over 36 months was performed.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

Background: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described.

Methods: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P.

Extended Application of the Hybrid Procedure in Neonates with Left-Sided Obstructive Lesions in an Evolving Cardiac Program.

The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed.