Genetic Testing Resources and Practice Patterns Among Pediatric Cardiomyopathy Programs.

The use of genetic testing has enhanced the diagnostic accuracy of heritable genetic cardiomyopathies. However, it remains unclear how genetic information is interpreted and incorporated into clinical practice for children with cardiomyopathy. The primary aim of this study was to understand how clinical practice differs regarding sequence variant classifications amongst pediatric cardiologists who treat children with cardiomyopathy.

Cardiac imaging and biomarkers for assessing myocardial fibrosis in children with hypertrophic cardiomyopathy.

Background: Myocardial fibrosis, as diagnosed on cardiac magnetic resonance imaging (cMRI) by late gadolinium enhancement (LGE), is associated with adverse outcomes in adults with hypertrophic cardiomyopathy (HCM), but its prevalence and magnitude in children with HCM have not been established. We investigated: (1) the prevalence and extent of myocardial fibrosis as detected by LGE cMRI; (2) the agreement between echocardiographic and cMRI measurements of cardiac structure; and (3) whether serum concentrations of N-terminal pro hormone B-type natriuretic peptide (NT-proBNP) and cardiac troponin-T are associated with cMRI measurements.

Methods: A cross-section of children with HCM from 9 tertiary-care pediatric heart centers in the U.

Single-drug immunosuppression is associated with noninferior medium-term survival in pediatric heart transplant recipients.

Background: Patients are usually maintained on at least 2 immunosuppressive drugs (ISDs) after the first year post heart transplant. Anecdotally, some children are switched to single-drug monotherapy (a single ISD) for various reasons and varying durations. Outcomes associated with differences in immunosuppression after heart transplantation are unknown for children.

COVID-19 Positive Versus Negative Complete Kawasaki Disease: A Study from the International Kawasaki Disease Registry.

To determine clinical differences for children with complete Kawasaki disease (KD) with and without evidence of preceding SARS-CoV-2 infection. From January 2020, contemporaneous patients with complete KD criteria were classified as either SARS-CoV-2 positive (KDCOVID+; confirmed household exposure, positive PCR and/or serology) or SARS-CoV-2 negative (KDCOVID-; negative testing and no exposure) and compared. Of 744 patients in the International Kawasaki Disease Registry, 52 were KDCOVID- and 61 were KDCOVID+.

Intraventricular Septation in the Context of Dilated Cardiomyopathy Associated With Mutation.

A 6-month-old infant boy presented with symptomatic heart failure. Dilated cardiomyopathy was found in association with a mutation in . Structural heart disease included novel septation of the left ventricle with a fenestrated membrane resulting from aberrant congenital mitral valve apparatus formation.

Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure.

Background: Fontan-associated liver disease (FALD) uniformly affects patients with long-term Fontan physiology. The effect of isolated heart transplant (HT) on the course of FALD post-HT is not well understood.

Methods: We evaluated serial liver imaging pre- and post-HT to assess liver changes over time in a single-center retrospective analysis of Fontan HT recipients who had pre- and ≥1-year post-HT liver imaging.

Clinical outcomes of children receiving ABO-incompatible versus ABO-compatible heart transplantation: a multicentre cohort study.

Background: ABO-incompatible heart transplantation increases donor availability in young children and is evolving into standard of care in children younger than 2 years. Previous smaller studies suggest similar outcomes to ABO-compatible heart transplantation, but persisting alterations of the immune system in ABO-incompatible recipients might increase the risk of some infections or benefit the graft owing to reduced HLA reactivity. We aimed to assess long-term outcomes in young children after they received ABO-incompatible or ABO-compatible heart transplantation.

Altered Biatrial Phasic Function after Heart Transplantation in Children.

Purpose: We used two-dimensional echocardiographic speckle-tracking to investigate whether left and right atrial (LA and RA) phasic function in pediatric heart transplantation (HT) patients is altered and explored the relationship to HT-related clinical variables.

Methods: Eighty-six subjects (36 HT and 50 normal children) were prospectively enrolled in two centers. Clinical data included age at HT, bypass time, ischemia time, donor age, and incidence of rejection.

A current era analysis of ABO incompatible listing practice and impact on outcomes in young children requiring heart transplantation.

Background: Heart transplantation from ABO incompatible (ABOi) donors has evolved into a progressively accepted therapy in young children. We assessed the recent practice of ABOi listing impact on waitlist and post-transplant outcomes.

Methods: Using the Pediatric Heart Transplant Society registry, we compared clinical presentation, waitlist parameters, and post-transplant survival of children < 2 years of age listed for ABOi vs ABO compatible (ABOc) heart transplant between January 2010 and June 2018 with sub-analysis of blood group O recipients.

Review of the impact of donor characteristics on pediatric heart transplant outcomes.

Heart transplantation (HTx) is a treatment option for end-stage heart failure in children. HTx is limited by the availability and acceptability of donor hearts. Refusal of donor hearts has been reported to be common with reasons for refusal including preexisting donor characteristics.

Vasopressin After the Fontan Operation.

Background: Prolonged pleural effusion following Fontan operation is common and increases morbidity and hospital length of stay. Vasopressin (VP), a neurohypophysial hormone, has numerous effects on the cardiovascular system. The most notable is increased peripheral vascular resistance, but it may also reduce capillary leakage by tightening endothelial intercellular junctions and reducing capillary hydrostatic pressure We reviewed our experience with the perioperative administration of VP following Fontan operation.

Apical Left Ventriculotomy is Safe in Infants and Young Children Requiring Cardiac Surgery.

Objectives: Incisions in the left ventricle have previously been associated with increased mortality and morbidity, particularly in infants. In order to determine whether this assumption is still true in the current era, we reviewed our recent experience with apical left ventriculotomy in neonates and infants.

Methods: The records of five consecutive patients requiring a left ventriculotomy between 2007 and 2010 were reviewed.

Iatrogenic pseudoaneurysm of the innominate artery in a neonate.

A 2.8-kg infant underwent urgent repair of a large iatrogenic pseudoaneurysm of the innominate artery, which was compressing the airway and superior vena cava, creating critical respiratory instability. The pseudoaneurysm was repaired with complete resolution of all respiratory symptoms.

Surgical Decision Making in Neonatal Ebstein's Anomaly: An Algorithmic Approach Based on 48 Consecutive Neonates.

Objective: There is currently no consensus of opinion regarding the optimal surgical management of Ebstein's anomaly (EA) in neonates and young infants. Reported early mortality rates range from 25% to 100%. In this study, we present an algorithm for choosing the best management option for neonates with EA based on analysis of our experience.

Early systemic-to-pulmonary artery shunt intervention in neonates with congenital heart disease.

Objective: To determine the incidence, risk factors, and outcomes after early, unplanned intervention on systemic-to-pulmonary artery shunts in neonates.

Methods: We retrospectively studied all neonates undergoing systemic-to-pulmonary artery shunt placement at The Children's Hospital of Philadelphia between September 1, 2002, and May 1, 2005. Patients requiring transcatheter or surgical systemic-to-pulmonary artery shunt intervention before discharge were compared with those not undergoing shunt intervention.

Reintervention for arch obstruction after stage 1 reconstruction does not adversely affect survival or outcome at Fontan completion.

Objective: To determine the effect of reintervention for coarctation after stage 1 reconstruction for hypoplastic left heart syndrome and variants on survival, suitability for Fontan, and morbidity at Fontan.

Methods: A retrospective review of echocardiograms, catheterizations, hospital records of patients who underwent stage 1 reconstruction from January 2002 to May 2005, with a cross-sectional analysis of hospital survivors, was performed. Kaplan-Meier curves were derived for patients alive more than 30 days after stage 1 reconstruction.

A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at Fontan completion.

Objective: We previously reported no difference in morbidity or mortality in a cohort of infants undergoing stage 1 and 2 reconstructions for hypoplastic left heart syndrome with either a modified Blalock-Taussig shunt or a right ventricular to pulmonary artery conduit. This article compares the hemodynamics and perioperative course at the time of the Fontan completion and reports longer-term survival for this cohort.

Methods: We retrospectively reviewed the hospital records of all patients who underwent stage 1 reconstruction between January 2002 and May 2005 and subsequent surgical procedures, as well as cross-sectional analysis of hospital survivors.

Hyperglycaemia after Stage I palliation does not adversely affect neurodevelopmental outcome at 1 year of age in patients with single-ventricle physiology.

Objective: Hyperglycaemia has been associated with worse outcome following traumatic brain injury and cardiac surgery in adults. We have previously reported no relationship between early postoperative hyperglycaemia and worse neurodevelopmental outcome at 1 year following biventricular repair of congenital heart disease. It is not known if postoperative hyperglycaemia results in worse neurodevelopmental outcome after infant cardiac surgery for single-ventricle lesions.

Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants.

Objective: The risk of death during the interstage period remains high after stage 1 reconstruction for single ventricle lesions, despite improved surgical results. The purpose of this study is to identify risk factors for interstage death and to describe the events leading to interstage death.

Methods: A nested case-control study was conducted of 368 patients who underwent stage 1 reconstruction at a single center between January 1998 and April 2005.

Hyperglycemia after infant cardiac surgery does not adversely impact neurodevelopmental outcome.

Background: Hyperglycemia has been associated with worse outcome after traumatic brain injury and cardiac surgery in adults. It is not known whether postoperative hyperglycemia results in worse neurodevelopmental outcome after infant cardiac surgery.

Methods: Secondary analysis of postoperative glucose levels was performed in infants younger than 6 months of age enrolled in a prospective study of genetic polymorphisms and neurodevelopmental outcomes who were undergoing repair of two-ventricle cardiac defects.

A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction.

Objective: We compare the hemodynamics and perioperative course of shunt type in hypoplastic left heart syndrome at the time of stage 2 reconstruction and longer-term survival.

Methods: We retrospectively reviewed the echocardiograms, catheterizations, and hospital records of all patients who had a stage 1 reconstruction between January 2002 and May 2005 and performed a cross-sectional analysis of hospital survivors.

Results: One hundred seventy-six patients with hypoplastic left heart syndrome and variants underwent a stage 1 reconstruction with either a right ventricle-pulmonary artery conduit (n = 62) or a modified Blalock-Taussig shunt (n = 114).

Neurodevelopmental outcomes following congenital heart surgery.

Advances in both surgical techniques and perioperative care have led to improved survival outcomes in infants and children undergoing surgery for complex congenital heart disease. An awareness is emerging that early and late neurological morbidities complicate the outcome of these operations. Adverse neurological outcomes after neonatal and infant cardiac surgery are related to both fixed and modifiable mechanisms.