Insights from 3D Echocardiography in Hypoplastic Left Heart Syndrome Patients Undergoing TV Repair.

Background: Tricuspid regurgitation (TR) in hypoplastic left heart syndrome (HLHS) is associated with morbidity and mortality. TR mechanisms and the impact of tricuspid valve repair (TVR) are unclear. We examined HLHS TR mechanisms, TVR's impact on tricuspid valve (TV), and features of poor TVR durability.

Echocardiographic Screening for Postoperative Pericardial Effusion in Children.

Pericardial effusion (PE) after cardiac surgery can be life threatening without timely detection, and the optimal screening method is unknown. We sought to evaluate the role of a surveillance echocardiogram on postoperative day 10 (± 2), determine the incidence of postoperative PE, and identify risk factors. We conducted a retrospective cohort study including all pediatric patients who underwent open heart surgery at a single institution over a 7-month period.

Impact of early surgical correction or palliation of congenital heart defects in infants with symptomatic viral respiratory tract infections in the current era.

Objective: This study investigates the influence of timing of surgery among infants with congenital heart disease and active respiratory tract infections in a contemporary Western Canadian cohort.

Methods: This was a retrospective matched cohort study of infants aged 1 week to 6 months undergoing surgical repair of congenital heart disease between 2014 and 2017. Case patients had active respiratory tract infections preoperatively and were matched to control patients based on primary heart lesion.

Tricuspid Regurgitation in Hypoplastic Left Heart Syndrome: Three-Dimensional Echocardiography Provides Additional Information in Describing Jet Location.

Background: Twenty-five percent of patients with hypoplastic left heart syndrome (HLHS) require tricuspid valve (TV) repair. The location of tricuspid regurgitation (TR) is important in determining the type of repair performed. Studies using three-dimensional echocardiography (3DE) have reported a high incidence of error on two-dimensional echocardiography (2DE) for the identification of TV leaflets.

Fetal Umbilical Arterial Pulsatility Correlates With 2-Year Growth and Neurodevelopmental Outcomes in Congenital Heart Disease.

Background: Children with congenital heart disease (CHD) are at risk of adverse long-term neurodevelopmental outcomes, believed to be, in part, secondary to prenatal insults. Placental pathology and altered fetal middle cerebral arterial (MCA) flow suggestive of brain sparing have been documented in fetal CHD. In the present study we investigated the relationship between MCA and umbilical arterial (UA) flow patterns in fetal transposition of the great arteries (d-TGA) and hypoplastic left heart syndrome (HLHS) and growth and 2-year neurodevelopmental outcomes.

Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome.

Background Management of patients with hypoplastic left heart syndrome has benefited from advancements in medical and surgical care. Outcomes have improved, although survival and long-term functional and cognitive deficits remain a concern. Methods and Results This is a cohort study of all consecutive patients with hypoplastic left heart syndrome undergoing surgical palliation at a single center.

Avoiding Furosemide Ototoxicity Associated With Single-Ventricle Repair in Young Infants.

Objective: To reduce bilateral delayed-onset progressive sensory permanent hearing loss using a systems-wide quality improvement project with adherence to best practice for the administration of furosemide.

Design: Prospective cohort study with regular audiologic follow-up assessment of survivors both before and after a 2007-2008 quality improvement practice change.

Setting: The referral center in Western Canada for complex cardiac surgery, with comprehensive multidisciplinary follow-up by the Complex Pediatric Therapies Follow-up Program.

Acetylation contributes to hypertrophy-caused maturational delay of cardiac energy metabolism.

A dramatic increase in cardiac fatty acid oxidation occurs following birth. However, cardiac hypertrophy secondary to congenital heart diseases (CHDs) delays this process, thereby decreasing cardiac energetic capacity and function. Cardiac lysine acetylation is involved in modulating fatty acid oxidation.

Neurocognitive outcomes after heart transplantation in early childhood.

Background: Children requiring heart transplantation (HTx) for congenital heart disease (CHD) or failing anatomically normal hearts (CMP) face different challenges pre-HTx. We compared the neurocognitive capabilities in pre-school-age children receiving HTx for CHD vs CMP and determined factors predicting outcomes.

Methods: Data were collected within a prospective multi-provincial project from children who underwent HTx ≤4 years of age between 1999 and 2011.

Hypoplastic Left Heart Syndrome Is Not a Predictor of Worse Intermediate Mortality Post Fontan.

Background: An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation.

Methods: All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included.

Post-operative Outcomes in Children Undergoing Fontan Palliation in a Regionalized Surgical System.

Evidence suggests that outcomes in pediatric cardiac surgery are improved by consolidating care into centers of excellence. Our objective was to determine if outcomes are equivalent in patients across a large regional referral base, or if patients from centers without on-site surgery are at a disadvantage. Since 1996, all pediatric cardiac surgery has been offered at one of two centers within the region assessed, with the majority being performed at Stollery Children's Hospital.

Glutaraldehyde Treatment of Allografts and Aortic Outcomes Post-Norwood: Challenging Surgical Decision.

Background: Glutaraldehyde (GA) treatment of allografts used for arch reconstruction prevents the immunologic sensitization that occurs with untreated allografts, but its use may cause tissue changes that predispose to recurrent obstruction. The objective was to determine whether GA treatment of allografts used in Norwood procedures increases the risk of recurrent aortic obstruction.

Methods: All infants who underwent a Norwood procedure between 2000 and 2015 were included.

A Unique Intracardiac Shunt: Identifying Complex Anatomy in Reoperative Adult Congenital Heart Disease.

Adult patients with repaired congenital heart disease are presenting with previously unseen types of residual lesions and consequences of prior repair. Patients with d-transposition of the great arteries repaired with atrial switch operations are returning with dysrhythmias and atrioventricular valve disease requiring intervention. We present the challenging case of a young adult with a residual shunt identified on preoperative three-dimensional transthoracic echocardiography, the precise anatomy of which was only characterized intraoperatively.

Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood-Sano Operation.

There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included.

Pilot randomized controlled trial on early and late remote ischemic preconditioning prior to complex cardiac surgery in young infants.

Background: Remote ischemic preconditioning involves providing a brief ischemia-reperfusion event to a tissue to create subsequent protection from a more severe ischemia-reperfusion event to a different tissue/organ. The few pediatric remote ischemic preconditioning studies in the literature show conflicting results.

Aim: We conducted a pilot randomized controlled trial to determine the feasibility of conducting a larger trial and to gather provisional data on the effect of early and late remote ischemic preconditioning on outcomes of infants after surgery for congenital heart disease.

Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort.

Background: Pulmonary vein stenosis is emerging as an important clinical problem in ex-premature infants.

Methods: We sought to describe the epidemiology of pulmonary vein stenosis affecting ex-premature infants by a multicenter retrospective cohort study of patients from seven children's hospitals diagnosed between 2000-2014.

Results: We identified 39 ex-premature patients (26 males, median gestational age 28 weeks range 22-36 weeks, birth weight 1.

An Unusual Cause of Hemopericardium 22 Years After a Fontan Procedure.

Late tamponade after cardiac operations is rare but reasonably well described. We report a case of exceedingly late tamponade secondary to a spontaneous coronary bleed 22 years after a Fontan operation, which was repaired with catheter intervention.

Neurologic, Neurocognitive, and Functional Outcomes in Children Under 6 Years Treated with the Berlin Heart Excor Ventricular Assist Device.

The objective of this study is to describe the neurologic, neurocognitive, and functional outcomes of children aged under 6 years supported on the Berlin Heart EXCOR ventricular assist device (VAD) followed in the Complex Pediatric Therapies Follow-up Program (CPTFP). Sixteen patients were prospectively followed through this longitudinal, developmental program. The patients were evaluated with neurologic physical examination.

Early childhood language outcomes after arterial switch operation: a prospective cohort study.

Background: Developmental and language outcomes at 2 years of age of children who had arterial switch operation (ASO) for transposition of the great arteries 2004-2010 are described.

Methods: In this prospective cohort study, 91/98 (93 %) children who underwent ASO were assessed at 2 years of age with the Bayley Scales of Infant & Toddler Development-3rd Edition. Outcomes were compared by patient and perioperative variables using bivariate and multivariate regression analyses to identify predictors of language delay.

Acetylation and succinylation contribute to maturational alterations in energy metabolism in the newborn heart.

Dramatic maturational changes in cardiac energy metabolism occur in the newborn period, with a shift from glycolysis to fatty acid oxidation. Acetylation and succinylation of lysyl residues are novel posttranslational modifications involved in the control of cardiac energy metabolism. We investigated the impact of changes in protein acetylation/succinylation on the maturational changes in energy metabolism of 1-, 7-, and 21-day-old rabbit hearts.

Suprasternal Approach Aortopexy Relieves Tracheal Compression After Nikaidoh Procedure.

Tracheal obstruction secondary to vascular and soft tissue compression, after Nikaidoh procedure, can effectively be managed with aortopexy from a suprasternal incision.

Supporting pediatric patients with short-term continuous-flow devices.

Background: Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being used in the pediatric population. However, little is known about the outcomes in patients supported with these devices.

Methods: All pediatric patients supported with a STCF-VAD, including the Thoratec PediMag or CentriMag, or the Maquet RotaFlow, between January 2005 and May 2014, were included in this retrospective single-center study.

Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study.

Background: Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair.

Methods: All patients who underwent a TA repair between 1996 and 2012 were included.