Adults With Congenital Heart Disease Have an Increased Prevalence of Autoimmunity.

Background: Adult congenital heart disease (ACHD) individuals have increased risk of noncardiac comorbidities including cancer and infections. Whether they are at increased risk of autoimmunity is unknown.

Objectives: The purpose of this study was to understand the association of ACHD and risk for autoimmunity.

Training pediatric cardiology fellows in the current era of healthcare.

Purpose Of Review: This review explores the modern training of pediatric cardiology fellows, starting from prefellowship residency preparation, highlighting new tools and strategies to support trainees during fellowship, and setting up successful transition to independent practice.

Recent Findings: Pediatric cardiology fellows benefit from a variety of newer educational strategies. Most experience a boot camp at the start of fellowship to help exposure to the complex and technical elements of the field.

Arrhythmias in congenitally corrected transposition of the great arteries: an international study.

Background And Aims: There is a paucity of data on arrhythmia burden in patients with congenitally corrected transposition of the great arteries (ccTGAs). The present study sought to quantify the incidence of atrial arrhythmia (AA), ventricular arrhythmia (VA), and complete atrioventricular block (CAVB) in patients with ccTGA and identify associated factors.

Methods: An international, multi-centre, retrospective cohort study was conducted in 29 tertiary hospitals (six countries) between 1990 and 2018.

Advanced tissue-engineered pulsatile conduit using human induced pluripotent stem cell-derived cardiomyocytes.

Single ventricle congenital heart defects (SVCHDs) are life-threatening defects that can lead to severe circulation issues and increased stress on the heart. Without prompt treatment, these defects can prove fatal in infancy. Fontan surgery is a conventional treatment for SVCHDs, which reroutes oxygen-poor blood directly to the lungs, bypassing the non-functioning ventricle.

Fully biologic endothelialized-tissue-engineered vascular conduits provide antithrombotic function and graft patency.

Tissue-engineered vascular conduits (TEVCs), often made by seeding autologous bone marrow cells onto biodegradable polymeric scaffolds, hold promise toward treating single-ventricle congenital heart defects (SVCHDs). However, the clinical adoption of TEVCs has been hindered by a high incidence of graft stenosis in prior TEVC clinical trials. Herein, we developed endothelialized TEVCs by coating the luminal surface of decellularized human umbilical arteries with human induced pluripotent stem cell (hiPSC)-derived endothelial cells (ECs), followed by shear stress training, in flow bioreactors.

Single ventricle palliation in congenitally corrected transposition of the great arteries: An international multicenter study.

Background: Single ventricle palliation may be performed in patients with congenitally corrected transposition of the great arteries due to hypoplasia of 1 ventricle or anatomic complexity rendering biventricular repair unfeasible. There have been only a few small studies of the outcomes of single ventricle palliation in the setting of congenitally corrected transposition of the great arteries.

Methods: A multicenter, international, retrospective cohort study of patients with congenitally corrected transposition of the great arteries undergoing single ventricle palliation was conducted in 29 tertiary hospitals in 6 countries from 1990 to 2018.

Life and Death: A Multicenter Study Evaluating Cardiologists' Approach to Difficult Conversations with Fontan Patients and Families.

Outpatient cardiologists provide longitudinal care for Fontan patients. As these patients age, they face mounting morbidities, necessitating challenging conversations about prognosis and goals of care. We created a novel survey to evaluate cardiologists' attitudes surrounding risk counseling for patients/caregivers.

How Good Are Cardiologists at Predicting Major Adverse Events in Fontan Patients?

Background: It is unknown how well cardiologists predict which Fontan patients are at risk for major adverse events (MAEs).

Objectives: The purpose of this study was to examine the accuracy of cardiologists' ability to identify the "good Fontan" patient, free from MAE within the following year, and compare that predicted risk cohort to patients who experienced MAE.

Methods: This prospective, multicenter study included patients ≥10 years with lateral tunnel or extracardiac Fontan.

Identifying Gender and Racial Bias in Pediatric Fellowship Letters of Recommendation: Do Word Choices Influence Interview Decisions?

Objectives: To describe linguistic differences in letters of recommendation (LORs) for pediatric fellowship candidates based on applicant and letter writer demographics and to examine if these differences influenced the decision to interview a candidate for a fellowship position.

Study Design: LORs for applicants to 8 pediatric subspecialty fellowships at a single academic center from the 2020 Match were analyzed in this cross-sectional study. Frequency of validated agentic and communal terms in each letter were determined by a language processing web application.

Unique clinical challenges for the teenager with congenital heart disease.

Purpose Of Review: On top of existing rites of passage, adolescents with congenital heart disease (CHD) face additional challenges as related to their underlying medical condition. These include compliance with medications and appointments, establishing heart-healthy behaviors, navigating potential at-risk situations, and transition to adult-oriented care.

Recent Findings: Teenagers with CHD should focus on the importance of exercise as this seems to have important long-term benefits and may help improve weight management.

Words Used in Letters of Recommendation for Pediatric Residency Applicants: Demographic Differences and Impact on Interviews.

Objective: To describe differences in agentic (achievement) and communal (relationship) terms in letters of recommendation (LORs) for pediatric residency candidates by applicant and letter writer demographics and to examine if LOR language is associated with interview status.

Methods: A random sample of applicant profiles and LORs submitted to one institution were analyzed from the 2020-21 Match. Letters of recommendation text was inputted into a customized natural language processing application which determined the frequency of agentic and communal words in each LOR.

Unrepaired Maternal Vein of Galen Malformation in Pregnancy: A Case Report.

We present a case of a pregnant patient with an unrepaired vein of Galen malformation (VGAM) and left ventricular (LV) dilation. Patients with VGAM lesions typically present during childhood with cardiac failure or developmental delay prompting embolization. Therefore, it is highly unusual for an adult to present with an unrepaired lesion.

T Cell Response to Influenza Vaccination Remains Intact in Adults with Congenital Heart Disease Who Underwent Early Thymectomy.

Introduction: T cells developed in the thymus play a key role in vaccine immunity. Thymectomy occurs during infant congenital heart surgery and results in an altered T cell distribution. We investigated if adults with congenital heart disease (ACHD) who underwent early thymectomy have a diminished response to influenza vaccination.

Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns.

Methods: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands.

Frequency of QTc Interval Prolongation in Children and Adults with Williams Syndrome.

QTc prolongation (≥ 460 ms), according to Bazett formula (QTcB), has been identified to be increased in Williams syndrome (WS) and suggested as a potential cause of increased risk of sudden cardiac death. The Bazett formula tends to overestimate QTc in higher heart rates. We performed a retrospective chart review of WS patients with ≥ 1 electrocardiogram (EKG) with sinus rhythm, no evidence of bundle branch blocks, and measurable intervals.

Noonan syndrome and pregnancy outcomes.

Background: Noonan syndrome is a genetic disorder with high prevalence of congenital heart defects, such as pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy. Scarce data exists regarding the safety of pregnancy in patients with Noonan syndrome, particularly in the context of maternal cardiac disease.

Study Design: We performed a retrospective chart review of patients at Yale-New Haven Hospital from 2012 to 2020 with diagnoses of Noonan syndrome and pregnancy.

Impact of the new heart allocation policy on patients with restrictive, hypertrophic, or congenital cardiomyopathies.

Background: Patients with restrictive or hypertrophic cardiomyopathy (RCM/HCM) and congenital heart disease (CHD) do not derive clinical benefit from inotropes and mechanical circulatory support. Concerns were expressed that the new heart allocation system implemented in October 2018 would disadvantage these patients. This paper aimed to examine the impact of the new adult heart allocation system on transplantation and outcomes among patients with RCM/HCM/CHD.

Hair and skin predict cardiomyopathies: Carvajal and erythrokeratodermia cardiomyopathy syndromes.

Carvajal and erythrokeratodermia cardiomyopathy syndromes (EKC) are rare, inherited cardiocutaneous disorders with potentially fatal consequences in young children. Some patients display features of congestive heart failure and rapidly deteriorate; others exhibit no evident warning signs until sudden death reveals underlying heart disease. We present two patients to illustrate the characteristic hair, skin, teeth, and nail abnormalities, which-especially when distinct from that of other family members-should prompt cardiac evaluation and genetic analysis.

A window into portal hemodynamics in adult fontan patients?

Background: Fontan circulation alters portal venous hemodynamics, causing chronic passive hepatic congestion and fibrosis. This congestion increases liver stiffness (LS) leading to overestimates of liver fibrosis as measured by ultrasound shear wave elastography (SWE) of the liver. We evaluated whether Fontan circulation has a similar effect on spleen stiffness (SS) and SS/LS ratio as measured by SWE.

Cardio-obstetrics: Recognizing and managing cardiovascular complications of pregnancy.

Pregnancy can exacerbate known cardiovascular disorders and unmask previously unrecognized problems. Patients with congenital heart disorders, valvular disease, primary pulmonary hypertension, hypertensive disorders of pregnancy, and acquired peripartum cardiomyopathy need a collaborative interdisciplinary team that includes a cardiologist with specialty training in obstetrics.

Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation.

Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.

Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs.

Results: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians.

T-Cell Receptor Excision Circles in Newborns with Congenital Heart Disease.

Objectives: To determine if children with congenital heart disease (CHD) have lower newborn T-cell receptor excision circles (TREC) levels than the general population and to evaluate if low TREC levels in newborns with CHD are associated with clinical complications such as hospitalization for infection.

Study Design: The Connecticut Newborn Screening Program reported TREC levels for newborns with CHD delivered between October 2011 and September 2016 at 2 major Connecticut children's hospitals. TREC levels for children with CHD were compared with the general population.

Postpartum anticoagulation in women with mechanical heart valves.

Background: Women with mechanical heart valves (MHV) requiring anticoagulation (AC) are at high risk for hemorrhagic complications. Despite guidelines to manage antenatal and peripartum AC, there are few evidence-based recommendations to guide the initiation of postpartum AC. We reviewed our institutional experience of pregnant women with MHV to lay the groundwork for recommendations of immediate postpartum AC therapy.