Hemodynamic Effects of Phenylephrine, Vasopressin, and Epinephrine in Children With Pulmonary Hypertension: A Pilot Study.

Objectives: During a pulmonary hypertensive crisis, the marked increase in pulmonary vascular resistance can result in acute right ventricular failure and death. Currently, there are no therapeutic guidelines for managing an acute crisis. This pilot study examined the hemodynamic effects of phenylephrine, arginine vasopressin, and epinephrine in pediatric patients with pulmonary hypertension.

Exploring the Role of Polycythemia in Patients With Cyanosis After Palliative Congenital Heart Surgery.

Objectives: To understand the relationship between polycythemia and clinical outcome in patients with hypoplastic left heart syndrome following the Norwood operation.

Design: A retrospective, single-center cohort study.

Setting: Pediatric cardiovascular ICU, university-affiliated children's hospital.

Training Pathways in Pediatric Cardiac Intensive Care: Proceedings From the 10th International Conference of the Pediatric Cardiac Intensive Care Society.

The increase in pediatric cardiac surgical procedures and establishment of the practice of pediatric cardiac intensive care has created the need for physicians with advanced and specialized knowledge and training. Current training pathways to become a pediatric cardiac intensivist have a great deal of variability and have unique strengths and weaknesses with influences from critical care, cardiology, neonatology, anesthesiology, and cardiac surgery. Such variability has created much confusion among trainees looking to pursue a career in our specialized field.

Mitral Stenosis and Aortic Atresia--A Risk Factor for Mortality After the Modified Norwood Operation in Hypoplastic Left Heart Syndrome.

Background: There are conflicting reports regarding the importance of mitral stenosis and aortic atresia as a risk factor for Norwood mortality. This study reviews outcomes of this anatomic subgroup at our institution and examines the utility of preoperative cardiac catheterization and its correlation with clinical outcomes and pathology findings.

Methods: This is a single-center, retrospective review of hypoplastic left heart syndrome patients who underwent modified Norwood operation between October 2005 and May 2013.

COCATS 4: Securing the Future of Cardiovascular Medicine.

The latest iteration of the Core Cardiology Training Statement (COCATS 4) [Corrected] provides a potentially transformative advancement in cardiovascular fellowship training intended, ultimately, to improve patient care. This review addressed 3 primary themes of COCATS 4 from the perspective of fellows-in-training: 1) the evolution of training requirements culminating in a competency-based curriculum; 2) the development of novel learning paradigms; and 3) the establishment of task forces in emerging areas of multimodality imaging and critical care cardiology. This document also examined several important challenges presented by COCATS 4.

Reported sildenafil side effects in pediatric pulmonary hypertension patients.

Background: Sildenafil, a phosphodiestase type 5 inhibitor, was approved in 2005 for the treatment of pulmonary arterial hypertension (PAH) in adults and is commonly used off-label for pediatric patients. Little is known, however, about sildenafil's side effects in this population.

Methods: Single institution, longitudinal survey-based study performed in an outpatient pediatric cardiology clinic.

Ebstein anomaly and Trisomy 21: A rare association.

This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

Outcomes following cardiac catheterization after congenital heart surgery.

Objectives: Describe outcomes following unplanned cardiac catheterization after congenital heart surgery.

Background: Utility of cardiac catheterization following congenital heart surgery is relatively understudied.

Methods: Retrospective study examining demographics, indications, and outcomes of unplanned cardiac catheterization after congenital heart surgery at a single institution.

Orthotopic heart transplantation in two infants with histiocytoid cardiomyopathy and left ventricular non-compaction.

HC is a rare cause of congestive heart failure that typically presents with malignant ventricular arrhythmias in infants, often requiring urgent intervention. Successful heart transplantation in a patient with HC has only been reported once (J Heart Lung Transplant 2004: 23: 902). The combination of HC with concurrent LVNC has only been described three times (Int J Legal Med 2009: 123: 47; Hum Pathol 2005: 36: 403; Pediatr Dev Pathol 2012: 15: 397).

Incidence and risk factors of complete atrioventricular block after operative ventricular septal defect repair.

Background: Complete atrioventricular block (AVB) is a recognized complication of ventricular septal defect (VSD) repair. The objective of this study was to examine incidence and risk factors.

Methods: This is a single-center, retrospective study.

Home monitoring program reduces interstage mortality after the modified Norwood procedure.

Background: From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality.

Methods: Results of the first 5 years of the Norwood HMP were reviewed retrospectively.

Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics.

Background: Pediatric patients with severe pulmonary arterial hypertension (PAH) are treated with intravenous epoprostenol or intravenous or subcutaneous treprostinil. Little is known about longitudinal hemodynamics and outcomes of epoprostenol, treprostinil, and transitions from epoprostenol to treprostinil.

Methods: This was retrospective study of 77 pediatric patients (47 idiopathic PAH, 24 congenital heart disease-PAH) receiving epoprostenol or treprostinil from 1992 to 2010 at 2 centers.

Electrical and mechanical dyssynchrony in pediatric pulmonary hypertension.

Background: Electrical and mechanical dyssynchrony are often seen in patients with left ventricular failure. In pediatric pulmonary hypertension (PH), right ventricular failure predominates; however, the prevalence of electrical and/or mechanical dyssynchrony in these patients is unknown. We examined the prevalence of electrical and mechanical dyssynchrony in pediatric PH patients.

Clinical usefulness of a novel C1q assay to detect immunoglobulin G antibodies capable of fixing complement in sensitized pediatric heart transplant patients.

Background: Donor-specific antibodies (DSA) against human leukocyte antigens complicate transplantation with the potential for acute antibody-mediated rejection (AMR). Complement-fixing antibodies are required to initiate the complement cascade. Not all DSAs, however, can fix complement.

Hypersensitivity reactions to aprotinin re-exposure in paediatric surgery.

Objective: Hypersensitivity to aprotinin is low (1-3%) but more likely with re-exposure. The manufacturer issued a black box warning which lists aprotinin re-exposure within 1 year of prior exposure as a contraindication. We investigated the temporal relationship between re-exposure interval and hypersensitivity in children.

Early cavopulmonary anastomosis after Norwood procedure results in excellent Fontan outcome.

Background: Children with univentricular hearts and aortic arch obstruction are treated sequentially with Norwood procedure, superior cavopulmonary anastomosis (SCPA), and Fontan operation. Early SCPA results in lower initial O2 saturation and longer hospitalization, but not increased mortality. We sought to determine the impact of early SCPA on Fontan candidacy and outcomes.