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.

Calcium and phosphorus concentrations in native and decellularized semilunar valve tissues.

Background And Aim Of The Study: Native, allograft, xenograft and bioprosthetic semilunar valves are all susceptible to calcific degeneration. However, intrinsic differences in baseline calcium and phosphorus tissue concentrations within mammalian normal valve structural components (e.g.

Transesophageal Echocardiography in Healthy Young Adult Male Baboons (): Normal Cardiac Anatomy and Function in Subhuman Primates Compared to Humans.

Implantable, viable tissue engineered cardiovascular constructs are rapidly approaching clinical translation. Species typically utilized as preclinical large animal models are food stock ungulates for which cross species biological and genomic differences with humans are great. Multiple authorities have recommended developing subhuman primate models for testing regenerative surgical strategies to mitigate xenotransplant inflammation.

Pulmonary arterioplasty with decellularized allogeneic patches.

Background: Decellularized allogeneic nonvalved pulmonary artery patches for arterioplasty are a relatively new option compared with cryopreserved allogeneic, crosslinked xenogeneic bioprosthetic or synthetic materials. This study examines the midterm experience with a new decellularized allogeneic patch for congenital cardiac reconstructions.

Methods: For this prospective postmarket approval, nonrandomized, inclusive observational study, we collected data on a consecutive cohort of 108 patients with cardiovascular reconstructions using 120 decellularized allogeneic pulmonary artery patches (MatrACELL; LifeNet Health, Inc, Virginia Beach, VA) between September 2009 and December 2012.

Neonatal acute myocardial infarction of unknown etiology treated with surgical thrombectomy.

Coronary artery thrombosis and resultant myocardial infarction in neonates are exceedingly rare. Seldom does a neonate survive this myocardial insult. Often there is an underlying predisposition to coronary artery thrombosis, such as thrombophilia, central line placement, or myocarditis.

Bioengineered human and allogeneic pulmonary valve conduits chronically implanted orthotopically in baboons: hemodynamic performance and immunologic consequences.

Objective: This study assesses in a baboon model the hemodynamics and human leukocyte antigen immunogenicity of chronically implanted bioengineered (decellularized with collagen conditioning treatments) human and baboon heart valve scaffolds.

Methods: Fourteen baboons underwent pulmonary valve replacement, 8 with decellularized and conditioned (bioengineered) pulmonary valves derived from allogeneic (N = 3) or xenogeneic (human) (N = 5) hearts; for comparison, 6 baboons received clinically relevant reference cryopreserved or porcine valved conduits. Panel-reactive serum antibodies (human leukocyte antigen class I and II), complement fixing antibodies (C1q binding), and C-reactive protein titers were measured serially until elective sacrifice at 10 or 26 weeks.

Noncoding RNA expression in myocardium from infants with tetralogy of Fallot.

Background: The importance of noncoding RNAs (ncRNA), especially microRNAs (miRNAs), for maintaining stability in the developing vertebrate heart has recently become apparent; however, there is little known about the expression pattern of ncRNA in the human heart with developmental anomalies.

Methods And Results: We examined the expression of miRNAs and small nucleolar RNAs (snoRNAs) in right ventricular myocardium from 16 infants with nonsyndromic tetralogy of Fallot (TOF) without a 22q11.2 deletion, 3 fetal heart samples, and 8 normally developing infants.

Effects of cryopreservation, decellularization and novel extracellular matrix conditioning on the quasi-static and time-dependent properties of the pulmonary valve leaflet.

Decellularized allografts offer potential as heart valve substitutes and scaffolds for cell seeding. The effects of decellularization on the quasi-static and time-dependent mechanical behavior of the pulmonary valve leaflet under biaxial loading conditions have not previously been reported in the literature. In the current study, the stress-strain, relaxation and creep behaviors of the ovine pulmonary valve leaflet were investigated under planar-biaxial loading conditions to determine the effects of decellularization and a novel post-decellularization extracellular matrix (ECM) conditioning process.

Initial pediatric cardiac experience with decellularized allograft patches.

Purpose: This study examines use patterns, early outcomes, and technical surgical adaptability of a newly approved decellularized allograft pulmonary artery patch for right ventricular outflow tract reconstructions in neonates and infants, including primary operations and reoperations.

Description: The study includes the 44 consecutive initial patients in which 46 patches were used for cardiovascular reconstruction between September 2009 and September 2010. Recorded variables include sex, age at operation, adverse outcome end points (eg, death, stenosis, aneurysm), congenital diagnoses, postoperative complications, and number, type, and location of patches.

Performance and morphology of decellularized pulmonary valves implanted in juvenile sheep.

Background: Because of cryopreserved heart valve-mediated immune responses, decellularized allograft valves are an attractive option in children and young adults. The objective of this study was to investigate the performance and morphologic features of decellularized pulmonary valves implanted in the right ventricular outflow tract of juvenile sheep.

Methods: Right ventricular outflow tract reconstructions in juvenile sheep (160±9 days) using cryopreserved pulmonary allografts (n=6), porcine aortic root bioprostheses (n=4), or detergent/enzyme-decellularized pulmonary allografts (n=8) were performed.

Streptococcus pneumoniae causing mycotic aneurysm in a pediatric patient with coarctation of the aorta.

Mycotic aneurysms are rare in patients with congenital heart disease, but may occur in those with aortic coarctation and abnormal aortic valve. Rapid diagnosis of mycotic aneurysm is of extreme importance given the significant reported incidence of morbidity and mortality across all age groups. Aortic aneurysm is uncommon before the second decade of life, and here we report a 10-year-old male patient with new diagnosis of aortic coarctation and bicuspid aortic valve, who developed a rapidly enlarging mycotic aneurysm from Streptococcus pneumoniae.

Determining cell seeding dosages for tissue engineering human pulmonary valves.

Background: This study examines in vitro seeding of decellularized human pulmonary valves (hPVs) with human valve interstitial cells (hVICs) isolated from unrelated donor aortic valve leaflets. An assay was developed to assess seeding using precut uniform sized biopsies from whole hPVs for sequential evaluation of seeding efficiency, proliferation, and migration.

Materials And Methods: Scaffolds for seeding were created from decellularized hPVs using a reciprocating osmolality, double detergent, enzyme, multiple solvent protocol.

Gene expression in cardiac tissues from infants with idiopathic conotruncal defects.

Background: Tetralogy of Fallot (TOF) is the most commonly observed conotruncal congenital heart defect. Treatment of these patients has evolved dramatically in the last few decades, yet a genetic explanation is lacking for the failure of cardiac development for the majority of children with TOF. Our goal was to perform genome wide analyses and characterize expression patterns in cardiovascular tissue (right ventricle, pulmonary valve and pulmonary artery) obtained at the time of reconstructive surgery from 19 children with tetralogy of Fallot.

Pediatric cardiopulmonary bypass adaptations for long-term survival of baboons undergoing pulmonary artery replacement.

Cardiopulmonary bypass (CPB) protocols of the baboon (Papio cynocephalus anubis) are limited to obtaining experimental data without concern for long-term survival. In the evaluation of pulmonary artery tissue engineered heart valves (TEHVs), pediatric CPB methods are adapted to accommodate the animals' unique physiology enabling survival up to 6 months until elective sacrifice. Aortic access was by a 14F arterial cannula and atrial access by a single 24F venous cannula.

Persistent risk of subsequent procedures and mortality in patients after interrupted aortic arch repair: a Congenital Heart Surgeons' Society study.

Objective: Multiple subsequent procedures directed at the arch and/or the left ventricular outflow tract are frequently required after interrupted aortic arch repair. We the investigated patterns and factors associated with these subsequent procedures and mortality.

Methods: We reviewed the data from 447 patients with interrupted aortic arch at 33 institutions enrolled from 1987 to 1997.

The nonfenestrated extracardiac Fontan procedure: a cohort of 145 patients.

Background: There are limited data available on the outcomes of single-ventricle patients who have undergone a nonfenestrated extracardiac Fontan procedure. This study examined the clinical experience of a large cohort of patients from a single institution who received this procedure.

Methods: A retrospective data set of 145 patients who underwent a nonfenestrated extracardiac Fontan procedure from July 1997 to September 2008 was reviewed.

Intraoperative hyperglycemia and postoperative bacteremia in the pediatric cardiac surgery patient.

Background: Intraoperative hyperglycemia has been found to be associated with a higher incidence of postoperative infections in the adult cardiac surgery population. The goal of this study was to determine the association of intraoperative hyperglycemia and postoperative bacteremia in the pediatric population.

Methods: A retrospective chart review of all cardiac surgical cases for patients 18 years of age or younger requiring cardiopulmonary bypass support between June 2002 and July 2007 yielded 1,132 total cases representing 992 unique patients.

Decellularization reduces calcification while improving both durability and 1-year functional results of pulmonary homograft valves in juvenile sheep.

Objective: The juvenile sheep functional valve chronic implant calcification model was used to compare long-term calcification rates, functional performance, and durability for 3 types of right ventricular outflow tract implants: classically cryopreserved homografts and 2 decellularized pulmonary valved conduits.

Methods: Fifteen juvenile sheep were randomly assigned to one of 3 study arms and underwent pulmonary valve replacement. The arms included the following: (1) cryopreserved ovine pulmonary valves; (2) cryopreserved, decellularized, saline (1 degrees C-10 degrees C)-stored ovine pulmonary valves; and (3) cryopreserved, decellularized, glycerolized (-80 degrees C) stored ovine pulmonary valves.

Critical left ventricular outflow tract obstruction: The disproportionate impact of biventricular repair in borderline cases.

Objective: In critical left ventricular outflow tract obstruction, the common perception that biventricular physiology is superior to univentricular physiology has led to a bias favoring biventricular repair. We hypothesized that pursuit of biventricular repair in borderline candidates increases mortality.

Methods: Between 1994 and 2001, 362 neonates with critical left ventricular outflow tract obstruction were prospectively enrolled by 26 institutions.

Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study.

Background: Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group.

Methods: From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA.

Gene expression in pediatric heart disease with emphasis on conotruncal defects.

Developmental abnormalities of the heart are the underlying cause of many congenital heart malformations. The embryological development of the integrated cardiovascular tissue is the result of multiple tissue and cell-to-cell interactions involving temporal and spatial events under genetic control. Recent technological advances, like microarray analysis of gene expression, are providing new tools to aid in deciphering the complex networks of gene expression that regulate cardiac development.

Pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries.

Pulmonary atresia with ventricular septal defect and major aorta pulmonary collaterals arteries is a rare and complex congenital cardiac defect. There is considerable variability in the anatomy, morphology, and geometry of the native pulmonary arteries and the collateral vessels. While the ultimate goal of therapy is a biventricular correction with complete unifocalization, establishment of right ventricular to pulmonary arterial continuity, and closure of all intracardiac defects, achieving this endpoint can be frustrating and difficult.

Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum.

Objective: We sought to define the prevalence of definitive end states and their determinants in children given a diagnosis of pulmonary atresia and intact ventricular septum during the neonatal period.

Methods: Between 1987 and 1997, 408 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective study by 33 institutions. Competing risks analysis was used to demonstrate the prevalence of 6 end states.

Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia.

Objective: This study was undertaken to determine the demographic, anatomic, institutional, and surgical risk factors associated with outcomes after the Norwood operation.

Methods: A total of 710 of 985 neonates with critical aortic stenosis or atresia enrolled in a prospective 29-institution study between 1994 and 2000 underwent the Norwood operation. Admission echocardiograms were independently reviewed for 64% of neonates.

Endocarditis caused by Staphylococcus lugdunensis.

We describe a case of native valve endocarditis caused by Staphylococcus lugdunensis, a perineal skin commensal, and review 28 other cases from the medical literature. Correctly identifying this coagulase-negative organism is critical because endocarditis is usually associated with left sided valvular disruption and life-threatening embolic complications, reminiscent of disease caused by Staphylococcus aureus. Urgent surgical intervention is necessary in most cases.