Clinical and Laboratory Correlates of QTc Duration in Adult and Pediatric Sickle Cell Disease.

Background: Sickle cell disease, a common genetic disorder in African Americans, manifests an increased risk of sudden death, the basis of which is incompletely understood. Prolongation of heart rate-corrected QT (QTc) interval on the electrocardiogram, a standard clinical measure of cardiac repolarization, may contribute to sudden death by predisposing to ventricular tachycardia.

Methods: We established a cohort study of 293 adult and 121 pediatric sickle cell disease patients drawn from the same geographic region as the Jackson Heart Study (JHS) cohort, in which significant correlates of QT duration have been characterized and quantitatively modeled.

Genetic variants and effect modifiers of QT interval prolongation in patients with sickle cell disease.

Background: Sickle cell disease (SCD) is a common inherited blood disorder among African Americans (AA), with premature mortality which has been associated with prolongation of the heart rate-corrected QT interval (QTc), a known risk factor for sudden cardiac death. Although numerous genetic variants have been identified as contributors to QT interval prolongation in the general population, their impact on SCD patients remains unclear. This study used an unweighted polygenic risk score (PRS) to validate the previously identified associations between SNPs and QTc interval in SCD patients, and to explore possible interactions with other factors that prolong QTc interval in AA individuals with SCD.

Haemodynamic effects of prophylactic post-operative hydrocortisone following cardiopulmonary bypass in neonates undergoing cardiac surgery.

Multiple studies have endeavoured to define the role of steroids in paediatric congenital heart surgery; however, steroid utilisation remains haphazard. In September, 2017, our institution implemented a protocol requiring that all neonates undergoing cardiac surgery with the use of cardiopulmonary bypass receive a five-day post-operative hydrocortisone taper. This single-centre retrospective study was designed to test the hypothesis that routine post-operative hydrocortisone administration reduces the incidence of capillary leak syndrome, leads to favourable postoperative fluid balance, and less inotropic support in the early post-operative period.

Pediatric Extracorporeal Membrane Oxygenation Anticoagulation Protocol Associated with a Decrease in Complications.

Extracorporeal membrane oxygenation (ECMO) in pediatrics has rapidly progressed in recent years; however, there continues to be considerable variation in anticoagulation practices. In 2016, we implemented a standardized anticoagulation protocol in effort to reduce clotting and bleeding complications. A single-center retrospective analysis of pediatric patients requiring ECMO between 2014 and 2018 was performed.

Two rare cases of paraganglioma in patients with Fontan physiology.

Pheochromocytoma/paraganglioma is an exceedingly rare tumour, thought to share an association with cyanotic CHD. This association is thought to be a result of chronic hypoxaemia (Antonio et al, Revista Española de Cardiología (English Edition) 2017; 70: 673-675; Folger et al, Circulation 1964; 29: 750-757; Opotowsky et al, J Clin Endocrinol Metab 2015; 100: 1325-1334) We report two cases of paraganglioma over a 4-year period in patients with hypoplastic left heart syndrome who had undergone Fontan completion by ages 2 and 4. Based on a very small number of reported cases of CHD, the mechanism of tumourigenesis is unclear.

Obesity and Diabetes Mellitus Adversely Affect Outcomes after Cardiac Surgery in Children's Hospitals.

Objective: To assess how obesity or diabetes mellitus impacts outcomes in patients undergoing cardiac surgery in pediatric hospitals.

Design: A multi-institutional, matched case-control study of the Pediatric Health Information System database was performed.

Setting: Tertiary children's hospitals in the United States.

Isolated Left Subclavian Artery From the Pulmonary Artery Masked by Pulmonary Hypertension.

Isolated subclavian artery arising from the pulmonary artery is exceedingly rare. Most cases are associated with other forms of congenital heart disease and typically present with subclavian or pulmonary steal syndromes. We report the case of a patient with pulmonary hypertension which masked the isolated subclavian artery, allowing it to masquerade as another common congenital heart lesion.

The Pathophysiology of Heart Failure in Children: The Basics.

Few data exist on the pathophysiologic changes in pediatric heart failure. Most of the knowledge has evolved from animal models of ischemic or idiopathic dilated cardiomyopathy. This review addresses the pathophysiologic changes that occur in the failing heart from animal models and the adult experience to unique aspects of heart failure in children.

Alternative strategies in newborns and infants with major co-morbidities to improve congenital heart surgery outcomes at an emerging programme.

Introduction: Debilitating patient-related non-cardiac co-morbidity cumulatively increases risk for congenital heart surgery. At our emerging programme, flexible surgical strategies were used in high-risk neonates and infants generally considered in-operable, in an attempt to make them surgical candidates and achieve excellent outcomes.

Materials And Methods: Between April, 2010 and November, 2013, all referred neonates (142) and infants (300) (average scores: RACHS 2.

Unusual cause of aborted sudden cardiac death in a teen athlete: homozygosity for the 4G allele of the plasminogen activase inhibitor type 1 gene.

Common aetiologies of sudden cardiac death in children include coronary anomalies, channelopathies, and cardiomyopathies. Less frequently, hypercoagulable states cause sudden arrest. We report an unusual case of aborted sudden cardiac death in a teenager, ultimately found to have homozygosity for the 4G allele of the plasminogen activase inhibitor type 1 gene.

Porcine xenograft aortic root replacement in a three month old with severe truncal insufficiency.

Outcomes for truncus arteriosus repair are impacted significantly by the severity of truncal valve dysfunction. When satisfactory repair of the regurgitant truncal valve is unattainable, replacement is required. Given our experience in children with stentless porcine xenografts in the aortic position and the incidence of early valve failure for aortic homografts in infants, we replaced a severely regurgitant truncal valve with a full-root porcine xenograft in a 3-month-old infant.

Peripheral pulmonary artery stenosis: an unusual case and discussion of genetic associations.

Peripheral pulmonary artery stenosis is a common congenital heart lesion associated with several genetic syndromes. We have reviewed the genetics of the lesion and present an unusual case of peripheral pulmonary stenosis involving a newly reported genetic deletion on chromosome 16. Further studies will be needed to confirm association of this genotype and phenotype.

Chondrosarcoma presenting with pulmonary embolism in a 9-year-old girl: a case report.

Chondrosarcoma is a malignant bone tumour common in adults, third to myeloma and osteosarcoma, but is exceptionally rare in children. Here we discuss a 9-year-old girl presenting with occlusive right pulmonary artery neoplastic embolus, resulting from a primary right proximal humerus chondrosarcoma. To the best of our knowledge, this the first pediatric and only second overall case reported in the United States of a neoplastic pulmonary embolus resulting from a primary chondrosarcoma.

Angiotensin-converting enzyme inhibitor nephrotoxicity in neonates with cardiac disease.

Angiotensin-converting enzyme inhibitors (ACEi) are commonly used for pediatric cardiology patients. However, studies examining their safety for neonates with cardiac disease are scarce. The current study aimed to test the hypothesis that ACEi-mediated nephrotoxicity occurs in neonates and may be underappreciated in this population.

Characteristics and outcomes of heart failure-related intensive care unit admissions in children with cardiomyopathy.

Objective: The purpose of this study was to describe patient characteristics and outcomes of heart failure (HF)-related intensive care unit (ICU) hospitalizations in children with cardiomyopathy (CM).

Methods And Results: A query of the Pediatric Health Information System database, a large administrative and billing database of 43 tertiary children's hospitals, was performed. A total of 17,309 HF-related ICU hospitalizations from 2005 to 2010 of 14,985 children ≤18 years old were analyzed.

Off-pump snare technique for congenital left atrial appendage aneurysm.

Left atrial appendage aneurysm is an extremely rare anomaly and as such has been rarely imaged or seen intraoperatively with very little accumulated management experience. The available scant published literature stresses resection on cardiopulmonary bypass as the safest and by far the most commonly applied technique. We suggest a novel alternative imaging-guided management utilising an off-pump tourniquet snare technique under live transoesophageal echocardiography.

Osteosarcoma with cardiac metastasis in a 22-year-old man: a case report and review of cardiac tumors.

Primary osteosarcoma accounts for 3% of all childhood cancer. It commonly occurs during the adolescent growth spurt and is more common in boys than girls and in African Americans than white people. The 5-year survival is approximately 79%.

Leptin-induced endothelial dysfunction: a target for therapeutic interventions.

Leptin has received much attention since its cloning in 1994. Initially, leptin research centered on satiety, energy balance and sympathetic activation. However, hyperleptinemia has since been identified as an independent risk factor for various cardiovascular pathologies including atherosclerotic coronary artery disease.

Coronary artery anatomy in congenital heart disease.

Expanded surgical options and improved outcomes for children born with structural heart defects have ushered a greater clinical interest in the normal and abnormal development of the coronary circulation. Anatomic variations of the coronary system may impact surgical candidacy or operative technique during neonatal life, while others may impact long-term clinical management and planning for subsequent interventions. This review aims to characterize coronary artery anatomy in symptomatic congenital heart disease, emphasizing the clinical consequence of these variations and anomalies.

Characterization of extracorporeal membrane oxygenation for pediatric cardiac arrest in the United States: analysis of the kids' inpatient database.

To characterize the overall use, cost, and outcomes of extracorporeal membrane oxygenation (ECMO) as an adjunct to cardiopulmonary resuscitation (CPR) among hospitalized infants and children in the United States, retrospective analysis of the 2000, 2003, and 2006 Kids' Inpatient Database (KID) was performed. All CPR episodes were identified; E-CPR was defined as ECMO used on the same day as CPR. Channeling bias was decreased by developing propensity scores representing the likelihood of requiring E-CPR.

Cardiopulmonary resuscitation in hospitalized children with cardiovascular disease: estimated prevalence and outcomes from the kids' inpatient database.

Objective: Hospitalized children with cardiovascular disease may be at increased risk of cardiac arrest; however, little data exist regarding prevalence, risk factors, or outcomes of cardiopulmonary resuscitation in these patients. We sought to characterize national estimates of cardiopulmonary resuscitation and death after cardiopulmonary resuscitation for hospitalized children with cardiovascular disease.

Setting: A total of 3,739 hospitals in 38 states participating in Kids' Inpatient Database.

Response to intravenous potassium chloride supplementation in pediatric cardiac intensive care patients.

Potassium chloride (KCl) supplementation is common among critically ill children. Intravenous (IV) KCl supplementation for pediatric patients is poorly characterized. This study aimed to examine the efficacy and safety of IV KCL and to determine factors affecting patient responses to IV KCL in the pediatric cardiac intensive care unit (CICU).

Prevalence and outcomes of pediatric in-hospital cardiopulmonary resuscitation in the United States: an analysis of the Kids' Inpatient Database*.

Objective: Population-based data on pediatric in-hospital cardiopulmonary resuscitation in the United States are scarce. Single-center studies and voluntary registries may skew the estimated prevalence and outcomes. This study aimed to determine the prevalence and outcomes of pediatric cardiopulmonary resuscitation on a national scale.