Three-Dimensional Imaging Defines Straddling Mitral Valve Anatomy to Allow Successful Biventricular Repair.

Biventricular repair of a straddling mitral valve (MV) can involve relocating ectopic papillary muscles and chordae. However, this increases operative complexity and risks MV incompetence. We describe a nine-month-old with D-transposition of the great arteries and straddling MV.

Ventricular Pseudoaneurysms After Perventricular Closure of Ventricular Septal Defect.

Perventricular ventricular septal defect closure has become an accepted method for treatment of some muscular and perimembranous ventricular septal defects. This report describes the case of a child who had left ventricular pseudoaneurysms after perventricular closure of a ventricular septal defect performed at 3 months of age. At 18 months, the ventricular pseudoaneurysms were closed surgically.

Pediatric Heart Donor Assessment Tool (PH-DAT): A novel donor risk scoring system to predict 1-year mortality in pediatric heart transplantation.

Background: In this study we sought to quantify hazards associated with various donor factors into a cumulative risk scoring system (the Pediatric Heart Donor Assessment Tool, or PH-DAT) to predict 1-year mortality after pediatric heart transplantation (PHT).

Methods: PHT data with complete donor information (5,732) were randomly divided into a derivation cohort and a validation cohort (3:1). From the derivation cohort, donor-specific variables associated with 1-year mortality (exploratory p-value < 0.

Current Practices in the Timing of Stage 2 Palliation.

Background: Mortality through single-ventricle palliation remains high and the effect of the timing of stage 2 palliation (S2P) is not well understood. We investigated current practice patterns in the timing of S2P across two professional societies and compared them to actual practice patterns from two databases of patients who underwent S2P.

Methods: A ten-question survey was distributed to the members of the Congenital Heart Surgeons' Society (CHSS) and the European Congenital Heart Surgeons' Association (ECHSA).

Postapproval Outcomes: The Berlin Heart EXCOR Pediatric in North America.

The Berlin Heart EXCOR Pediatric Ventricular Assist Device (BH) was approved for use in the United States in December 2011, based on a prospective investigational device exemption (IDE) trial. Strict exclusion criteria for the trial selected a low-risk "ideal" cohort. We sought to determine whether postapproval usage of the BH in a "real world" cohort of recipients would result in similar outcomes.

Simplified mitral valve repair in pediatric patients with connective tissue disorders.

Background: In pediatric patients with connective tissue disorders (CTDs), early cardiac presentation often involves severe mitral regurgitation (MR) associated with severe bileaflet prolapse and, less frequently, aortic root enlargement. We adopted a simplified repair to address MR and prevent systolic anterior motion (SAM) in this unique group of patients.

Materials And Methods: Retrospective review of clinical and echocardiographic data of all pediatric patients (age < 18 years) with CTD and MR undergoing simplified repair at 3 institutions (2000-2014).

Natural history of nonimmune-mediated thrombocytopenia and acute kidney injury in pediatric open-heart surgery.

Objective: Thrombocytopenia and acute kidney injury (AKI) are common following pediatric cardiac surgery with cardiopulmonary bypass (CPB). However, the relationship between postoperative nadir platelet counts and AKI has not been investigated in the pediatric population. Our objective was to investigate this relationship and examine independent predictors of AKI.

Closing in on the PumpKIN Trial of the Jarvik 2015 Ventricular Assist Device.

The Infant Jarvik ventricular assist device (VAD; Jarvik Heart, Inc., New York, NY) has been developed to support the circulation of infants and children with advanced heart failure. The first version of the device was determined to have elevated hemolysis under certain conditions.

Pulmonary Valve Replacement With a Trifecta Valve Is Associated With Reduced Transvalvular Gradient.

Background: Outcomes after surgical pulmonary valve replacement (PVR) in patients with congenital cardiac disease are limited by long-term valve deterioration, which may be hastened by turbulent flow. The use of the Trifecta valve (St. Jude Medical, Little Canada, MN) at our institution (Duke University Medical Center, Durham, NC) appears to result in low postimplantation transvalvular gradients.

Cardiopulmonary Bypass Strategy for a Cyanotic Child With Hemoglobin SC Disease.

Hemoglobin SC (HbSC) disease is a hemoglobinopathy that may produce sickling under conditions of hypoxemia, dehydration, and acidosis. We present a case of HbSC disease and tricuspid atresia, type IB. We describe management by cardiopulmonary bypass CPB using exchange transfusion at initiation of bypass and fractionation of collected blood, allowing platelet and plasma apheresis, as an option for patients unable to undergo this procedure off pump.

Adverse events in children implanted with ventricular assist devices in the United States: Data from the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS).

Background: Ventricular assist devices (VADs) have been used in children on an increasing basis in recent years. One-year survival rates are now >80% in multiple reports. In this report we describe adverse events experienced by children with durable ventricular assist devices, using a national-level registry (PediMACS, a component of INTERMACS) METHODS: PediMACS is a national registry that contains clinical data on patients who are <19 years of age at the time of VAD implantation.

Is Four Stage Management the Future of Univentricular Hearts? Destination Therapy in the Young.

For the child born with a functionally univentricular heart, multi-stage surgical palliation culminating in the Fontan operation is now well established as the standard of care. Though this is an effective approach, there are several inherent disadvantages to the Fontan circulation: subnormal resting cardiac output, impaired exercise capacity, increased risk of thromboembolism, and increased risk of arrhythmia. Perhaps most importantly, the long-term deleterious effects of substantial systemic venous hypertension, which is necessary to "drive" blood through the pulmonary vascular bed, include progressive dysfunction of other organ systems, particularly renal, lymphatic, gastrointestinal, and hepatic systems.

Mechanical Circulatory Support in Children.

The field of pediatric mechanical circulatory support has undergone a significant evolution with the advent of devices designed for children and the implementation of new strategies for deployment. With the ongoing shortage of organs the demand for new devices specifically designed for children will only increase. This review discusses the evolution of mechanical circulatory support, available devices, and the implementation of new strategies for their deployment.

Reduction in blood transfusion in a cohort of infants having cardiac surgery with cardiopulmonary bypass after instituting a goal-directed transfusion policy.

Background: Current trends in pediatric cardiac surgery and anesthesiology include goal-directed allogeneic blood transfusion, but few studies address the transfusion of platelets and cryoprecipitate. We report a quality improvement initiative to reduce the transfusion of platelets and cryoprecipitate in infants having cardiac surgery with cardiopulmonary bypass (CPB).

Methods: Data from 50 consecutive patients weighing four to ten kilograms having cardiac surgery with CPB were prospectively collected after the institution of a policy to obtain each patient's platelet and fibrinogen levels during the rewarming phase of CPB.

Atrial Rhabdomyoma Resection With Extracellular Matrix Reconstruction of the Right Atrial Free Wall in an Infant.

A neonate was diagnosed with a mediastinal mass after presenting with bradycardia. At surgery, she was found to have a 4-cm mass replacing most of the right atrial free wall. After tumor resection, the right atrium was reconstructed with an extracellular matrix biomaterial that supports native tissue regeneration.

Mechanical Circulatory Support in Children.

The field of pediatric mechanical circulatory support has undergone a significant evolution with the advent of devices designed for children and the implementation of new strategies for deployment. With the ongoing shortage of organs the demand for new devices specifically designed for children will only increase. This review discusses the evolution of mechanical circulatory support, available devices, and the implementation of new strategies for their deployment.