Pediatric cardiology patient reported outcomes

Despite significant advancements in the care of patients with hypoplastic left heart syndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid regurgitation (TR) are associated with worse outcomes in HLHS. We aim to determine if right ventricle functional parameters and TR on fetal echocardiogram are associated with postnatal outcomes in HLHS patients. Retrospective review was performed on all fetuses with HLHS from 2014 to 2022 at our institution. Initial and follow up fetal echocardiogram measurements of right ventricular myocardial performance index (MPI), fractional area change (FAC) and global longitudinal strain (GLS) were retrospectively measured...

BACKGROUND: Carnitine palmitoyltransferase II (CPT II) deficiency is a rare inborn error of mitochondrial fatty acid metabolism with autosomal recessive pattern of inheritance. Its phenotype is highly variable (neonatal, infantile, and adult onset) on the base of mutations of the CPT II gene. In affected subjects, long-chain acylcarnitines cannot be subdivided into carnitine and acyl-CoA, leading to their toxic accumulation in different organs. Neonatal form is the most severe, and all the reported patients died within a few days to 6 months after birth...

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test...

BACKGROUND: Pediatric cardiomyopathies (CMP) can be familial or idiopathic with increasing detection of genetic mutations. The study is a retrospective single-center review of cardiomyopathy patients from January 2011 to May 2020. Results of the genetic study, as well as the outcome, were reported. Patients were divided according to the type of CMP, age of presentation, and EF at presentation. Univariate and multivariate analysis and ROC and survival curves were done. RESULTS: We reported 229 patients under 14 years of age with a diagnosis of cardiomyopathy, most commonly DCM (160 patients (70%)) followed by HCM (26...

Fetal arrhythmias are rare and carry significant morbidity and mortality without appropriate treatment. Initial reports exist of fetal arrhythmia in the setting of maternal COVID-19 infection. Our study sought to evaluate incidence of fetal arrhythmia before and during the COVID-19 pandemic at our institution. This retrospective cohort study from a tertiary care fetal cardiac center utilized the institutional REDCap database to search fetal arrhythmia diagnostic codes. Medical records of mother-fetus dyads were reviewed and data were collected on diagnoses, gestational age, treatment regimen, and postnatal outcomes...

BACKGROUND: The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. METHODS AND RESULTS: The American College of Cardiology Quality Network assembled a panel from 12 centers to refine a previously published PEC score developed in a single institution. The panel refined complexity categories and included study modifiers to account for complexity related to performance of the echocardiogram...

The number of device implantation procedures has increased in adult patients with congenital heart disease (ACHD). Despite significant improvements in materials and implantation techniques, these patients are exposed to higher risk of device related complications than general population. Herein, we describe our single tertiary referral center experience on transvenous pacemaker (PM) implantation and follow-up in adult patients with moderate and complex congenital heart disease (CHD) as limited data are available on long-term outcome...

BACKGROUND: Congenital heart disease (CHD) is common in children and associated with greater risk of thrombotic complications. Management of these complications with standard-of-care treatment is suboptimal for these children. METHODS AND RESULTS: The effectiveness and safety of dabigatran were demonstrated in pivotal pediatric studies for the treatment of acute venous thromboembolism (VTE; NCT01895777) and secondary VTE prevention (NCT02197416). We report safety and efficacy outcomes from subgroup analyses of these studies for children with CHD (diagnosed according to local practice) and those without...

The arterial switch operation for d-transposition of the great arteries achieves anatomic repair but creates the potential for right ventricular outflow tract obstruction as a result of the LeCompte maneuver. The resultant right ventricular hypertension is generally well tolerated but a select group are referred for cardiac catheterization. The outcomes of these catheterizations have not been well described. The objective of this study was to describe the degree and nature of right ventricular outflow tract obstruction found during cardiac catheterization among patients following the arterial switch operation as well as determine the rate of intervention and assess the acute impact of any catheter intervention undertaken...

Detecting dyslipidemia early is important because atherosclerosis originates in childhood and early treatment can improve outcomes. In 2022, CCS/CPCA published a Clinical Practice Update to detect, evaluate, and manage pediatric dyslipidemia. However, guidance on its translation into clinical laboratories is lacking. The Canadian Society of Clinical Chemists (CSCC) Working Group on Reference Interval Harmonization (hRI-WG) Lipid Team aims to aid guideline implementation and promote harmonized pediatric lipid reporting across Canada...

We previously reported short-term outcomes for stenting of aortic coarctation (CoA) (native or re-coarctation) with newer generation low-profile stents (Valeo, Formula, and Begraft stents) in children under 30 kg. We present here the medium-term outcomes of this procedure. Retrospective review of patients weighing under 30 kg who had percutaneous stent treatments for coarctation between 2012 and 2021 was performed. Clinical and procedural data were collected; 19 patients were included. The median age at the time of procedure was 5...

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis)...

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated...

Adults with complex congenital heart disease (CHD) are at risk for cognitive dysfunction. However, associations between cognitive dysfunction and psychosocial outcomes are poorly defined. Between June and November 2022, we prospectively recruited 39 adults with complex CHD who completed a computerized cognitive assessment (Cogstate) and validated psychosocial scales measuring psychological distress, health-related quality of life (HRQOL), and resilience. Participants had a mean age of 36.4 ± 11...

BACKGROUND: Congenital Heart Disease stands as a prominent cause of infant mortality, with notable disparities in surgical outcomes evident between high-income and low- to middle-income countries. OBJECTIVE: This study presents a collaborative partnership between a local governmental entity and an international private organization to establish a high-quality Pediatric Cardiac Surgery Program in a post-conflict limited resource country, Iraq. METHODS: A descriptive retrospective study analyzed pediatric cardiac surgery procedures performed by a visiting pediatric heart surgery team from October 2021 to October 2022, funded by the Ministry of Health (MOH)...

The SARS-CoV-2 pandemic has led to widespread research on associated clinical syndromes. While pediatric patients were initially deemed as a low-risk population for severe COVID-related disease, an increasing number of case reports have revealed a rare but potentially life-threatening syndrome, multisystem inflammatory syndrome in children (MIS-C). MIS-C is hypothesized to be due to hyperactivation of the immune system via a cytokine storm which leads to end-organ damage via endothelial dysfunction and changes in vascular permeability...

BACKGROUND: Turner syndrome (TS) is associated with left-sided cardiac lesions, including hypoplastic left heart syndrome (HLHS). Mortality as high as 80-90% has been reported following stage I single-ventricle palliation (S1P) in patients with TS and HLHS (TS + HLHS). The specific factors that relate to poor outcomes are not well understood. METHODS: This is a single-center, retrospective cohort study that includes 197 patients with HLHS who underwent S1P between 2008 and 2022...

BACKGROUND: Previous findings from the Swissped RECOVERY trial showed that patients with Pediatric Inflammatory Multisystem Syndrome-Temporally Associated with SARS-CoV-2 (PIMS-TS) who were randomly assigned to intravenous immunoglobulins or methylprednisolone have a comparable length of hospital stay. Here, we report the 6-month follow-up outcomes of cardiac pathologies and normalisation of clinical or laboratory signs of inflammation from this study population. METHODS: This pre-planned follow-up of patients with PIMS-TS included the Swissped RECOVERY Trial reports on the 6-month outcomes of the cohort after randomisation, with a focus on cardiac, haematological, and biochemical findings...

Vertical vein (VV) ligation during total anomalous pulmonary venous return (TAPVR) repair is controversial. While some surgeons prefer ligation of the VV to prevent adverse sequelae of shunting across it and to promote flow through the newly created anastomosis, others leave it to serve as a "pop off valve" to the left heart structures, which are believed to be hypoplastic and noncompliant, presumably contributing to a more favorable post-operative outcome. We report two patients post-Fontan procedure, who underwent cardiac catheterization to explore the etiology of hypoxia and were found to have a persistent VV responsible for right to left shunting...

PURPOSE OF REVIEW: Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis. Surgical and transcatheter closure techniques have been previously described...