Complete atrioventricular septal defect with absent or diminutive primum component: Incidence, anatomic characteristics, and outcomes.

Background: Repair of complete atrioventricular septal defect with absent or diminutive primum defect is challenging because of atrial septal malposition and abnormal anatomy of the left atrioventricular valve. We sought to define the incidence, anatomy, and surgical outcomes of this entity.

Methods: We identified all patients in our institutional database presenting for complete atrioventricular septal defect repair from 2006 to 2018.

Neonatal venoarterial extracorporeal membrane oxygenation cannulation technique.

Peripheral cannulation for initiation of neonatal venoarterial extracorporeal membrane oxygenation is an essential technical skill in the armamentarium of every congenital cardiac surgeon and is necessary for the care of newborns with congenital heart disease and other conditions presenting with cardiopulmonary failure. With certain exceptions, it is usually performed via a right neck cut-down to expose the right internal jugular vein and common carotid artery.   A strong understanding of the fundamental techniques, as well as strategies for avoiding common pitfalls, is critical to the success of cardiopulmonary rescue afforded by cannulation, especially considering the emergent conditions under which cannulation must usually be performed.

Serum Protein Changes in Pediatric Sepsis Patients Identified With an Aptamer-Based Multiplexed Proteomic Approach.

Objectives: Sepsis, a life-threatening organ dysfunction caused by a dysregulated host response to infection, is a leading cause of death and disability among children worldwide. Identifying sepsis in pediatric patients is difficult and can lead to treatment delay. Our objective was to assess the host proteomic response to infection utilizing an aptamer-based multiplexed proteomics approach to identify novel serum protein changes that might help distinguish between pediatric sepsis and infection-negative systemic inflammation and hence can potentially improve sensitivity and specificity of the diagnosis of sepsis over current clinical criteria approaches.

Multimodality and multiterritory vascular imaging in a toddler with acquired, complex, and unusual aortic arch anatomy.

A 20-month-old otherwise well child with no cardiac history presented for evaluation of a recent onset cardiac murmur. His initial examination revealed significant right carotid bruit along with a transthoracic echocardiogram (ECHO) concerning for severe transverse arch hypoplasia with unusual appearance and left ventricular hypertrophy with preserved function. This report demonstrates the utility of various modalities including echo, CT, and MRI to assess vascular structures in different vascular territories in a challenging patient who underwent a satisfactory arch repair with unexpected pathology findings.

Hybrid stage 1 palliation as a bridge to cardiac transplantation in patients with high-risk single ventricle physiology.

Background: The hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) was first described in 1993 as a bridge to heart transplant for HLHS. There are limited data on this strategy as primary heart transplantation for HLHS has become less common.

Methods: This is an observational, single-center study comparing pre- and post-transplant outcomes of patients listed for transplant following hybrid palliation with those following surgical stage 1 palliation.

A tale of two cases of pulmonary arteriovenous malformation: How they fared after cardiac transplantation.

Background: In single ventricle patients, aortopulmonary collaterals (APCs) and pulmonary arteriovenous malformations (PAVMs) following superior cavopulmonary shunt (CPS) can complicate orthotopic heart transplant (OHT) by cyanosis and hemoptysis. Although PAVMs can regress with the restoration of hepatic venous flow to the pulmonary circulation, the effects of hypoxemia on the "unconditioned" allograft are not known.

Cases: Two patients with significant PAVMs after CPS were cyanotic following OHT.

Diagnostic Accuracy of a Host Gene Expression Signature That Discriminates Clinical Severe Sepsis Syndrome and Infection-Negative Systemic Inflammation Among Critically Ill Children.

Objectives: SeptiCyte Lab (Immunexpress, Seattle, WA), a molecular signature measuring the relative expression levels of four host messenger RNAs, was developed to discriminate critically ill adults with infection-positive versus infection-negative systemic inflammation. The objective was to assess the performance of Septicyte Lab in critically ill pediatric patients.

Design: Prospective observational study.

Impact of donor-recipient sex match on long-term survival after heart transplantation in children: An analysis of 5797 pediatric heart transplants.

The effect of donor-recipient sex matching on long-term survival in pediatric heart transplantation is not well known. Adult data have shown worse survival when male recipients receive a sex-mismatched heart, with conflicting results in female recipients. We analyzed 5795 heart transplant recipients ≤ 18 yr in the Scientific Registry of Transplant Recipients (1990-2012).

Tricuspid valve replacement with a melody stented bovine jugular vein conduit.

Atrioventricular valve replacement options are limited in infants and small children. The Melody stented bovine jugular vein conduit is being used with increasing frequency for percutaneous pulmonary valve replacement. The Melody valve can be serially dilated over time to accommodate the somatic growth of pediatric patients.

One size does not fit all: the influence of age at surgery on outcomes following Norwood operation.

Background: Given our large catchment area that often results in later presentation age, we sought to understand our institutional outcomes for the Norwood operation in the context of published data. Specifically, we studied whether operative and late death post-Norwood are dependent on age at operation.

Methods: Retrospective review of 105 consecutive infants undergoing Norwood (2004-2011) at our institution.

Significance of positive mediastinal cultures in pediatric cardiovascular surgical procedure patients undergoing delayed sternal closure.

Background: Many pediatric cardiac surgery centers obtain mediastinal cultures at the time of delayed sternal closure (DSC). There are no recommendations regarding how to treat patients with positive cultures. We explored the clinical significance of positive mediastinal cultures with regard to surgical site infections (SSI).

Modified Blalock-Taussig shunt versus ductal stenting for palliation of cardiac lesions with inadequate pulmonary blood flow.

Objective: The modified Blalock-Taussig shunt is the most commonly used palliative procedure for infants with ductal-dependent pulmonary circulation. Recently, catheter-based stenting of the ductus arteriosus has been used by some centers to avoid surgical shunt placement. We evaluated the durability and safety of ductal stenting as an alternative to the modified Blalock-Taussig shunt.

Downsized Contegra graft as a right ventricle-to-pulmonary artery conduit in the setting of mediastinitis.

Polytetrafluoroethylene (PTFE) grafts are commonly used for construction of the right ventricle-to-pulmonary artery conduit in the modified Norwood procedure. Dehiscence of a PTFE conduit in the setting of purulent mediastinitis presents a challenging clinical problem because of limited availability of appropriately sized replacement vascular homografts. The Contegra bovine jugular vein graft is an alternative to placing another PTFE graft in an infected space when a homograft of appropriate size is not available.

Increased extracorporeal membrane oxygenation center case volume is associated with improved extracorporeal membrane oxygenation survival among pediatric patients.

Objective: We sought to examine the relationship between extracorporeal membrane oxygenation center case volume and survival in pediatric patients requiring extracorporeal membrane oxygenation support.

Methods: Pediatric patients (≤ 20 years) undergoing extracorporeal membrane oxygenation cannulation were identified using the Healthcare Cost and Utilization Project Kids' Inpatient Database for 2000 to 2009. Annual hospital extracorporeal membrane oxygenation volume tertiles were <15 patients/year (low volume), 15 to 30 patients/year (medium volume), and >30 patients/year (high volume).

Benign mediastinal lymphadenopathy presenting as cor triatriatum.

This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy referred for oncologic care. During her evaluation, an abnormal cardiovascular examination resulted in an echocardiographic evaluation and an unanticipated diagnosis of a highly obstructive left atrial cor triatriatum was uncovered. The patient underwent repair of cor triatriatum and lymph node biopsy shortly after the diagnosis was made.

Association of thymectomy with infection following congenital heart surgery.

Background: Congenital absence of the thymus can lead to profound immunodeficiency, suggesting that thymic function during fetal development is essential to normal lymphocyte development. How vital the thymus after birth is to human immune competence and regulation is not known. Routine thymectomy, especially at an early age, may influence immunity, and therefore the risk of infection, autoimmunity, or malignancy.

Direct optical measurement of intraoperative myocardial oxygenation during congenital heart surgery.

This study demonstrates use of novel technology to measure cellular oxygenation during corrective congenital heart surgery. Cellular oxygenation was measured using a custom-designed optical probe placed on the free wall of the right ventricle. Cellular oxygenation, determined from myoglobin saturation, was calculated using multiwavelength analysis.

Predictors of ECMO support in infants with tachycardia-induced cardiomyopathy.

The development of tachycardia-induced cardiomyopathy (TIC) is related to the rate and duration of supraventricular tachycardia (SVT). Infants may be more susceptible to TIC because early symptoms might be unrecognized. Extracorporeal membrane oxygenation (ECMO) may improve outcome in patients with SVT and TIC; however, clinical predictors of infants who require ECMO support have not been determined.

Minimizing bleeding associated with mechanical circulatory support following pediatric heart surgery.

Objective: The use of extracorporeal membrane oxygenation (ECMO) to support patients with early postcardiotomy heart failure may be associated with catastrophic bleeding, making its use undesirable. However, postcardiotomy mechanical circulatory assistance is necessary in some patients to allow for myocardial recovery. We have assembled a centrifugal pump system (CPS) that does not require early systemic anticoagulation.

Direct microvascular repair of an infant's transected coronary artery: case report.

The authors report the direct, microvascular repair of a right coronary artery transected during reoperation fOr complications of arterial switch operation (ASO) in a 3 month-old child. This is the first documented use of direct microsurgical anastamosis in the repair of an infant's transected coronary artery. Deviation from standard coronary bypass graft repair was permitted by vessel characteristics, as well as close collaboration between plastic surgery and cardiac surgery services.

Decision making for mechanical cardiac assist in pediatric cardiac surgery.

The practice of pediatric cardiac surgery has evolved to the point where the majority of patients operated on represent the most complex end of the spectrum of congenital heart disease. Given this, the potential role for mechanical cardiac assist will continue to expand. Although extracorporeal membrane oxygenation remains the mainstay of mechanical circulatory assist, the increased use of centrifugal ventricular assist devices is changing the approach to the treatment of acute cardiac failure.