Patient-Reported Outcomes Among Adults With Congenital Heart Disease in the Congenital Heart Initiative Registry.

Importance: In the US, there are more than 1.5 million adults living with congenital heart disease (CHD). The Congenital Heart Initiative (CHI) is a digital, online, patient-empowered registry that was created to advance multicenter research and improve clinical care by gathering patient-reported outcomes (PROs) in adults with CHD.

Experiences and Attitudes Toward Telemedicine in an Adult Congenital Heart Disease Clinic: Lessons Learned from the COVID-19 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has driven a broader adoption of telemedicine (TM). We aim to describe adult congenital heart disease (ACHD) patient experiences with TM and explore factors associated with positive attitude toward future TM visits. This is a cross-sectional, single-center study in an outpatient ACHD clinic from February to June, 2022.

Introduction of transcatheter edge-to-edge repair in patients with congenital heart disease at a children's hospital.

Background: Atrioventricular valve regurgitation (AVVR) is a devastating complication in children and young adults with congenital heart disease (CHD), particularly in patients with single ventricle physiology. Transcatheter edge-to-edge repair (TEER) is a rapidly expanding, minimally invasive option for the treatment of AVVR in adults that avoids the morbidity and mortality associated with open heart surgery. However, application of TEER in in CHD and in children is quite novel.

Clinical complications of liver disease in adults after the Fontan operation.

Background: The natural history and complications of Fontan-associated liver disease (FALD) are not well-characterized. We aim to describe the incidence and prevalence of and risk factors for adverse liver-related complications in FALD.

Methods: This is a single-center, retrospective cohort study of adult Fontan patients followed in an adult congenital heart disease center from 2006 to 2019.

Successful in vitro fertilization in women with Fontan physiology.

Purpose: Patients with single ventricle anatomy palliated with Fontan operation are at risk for thromboembolism, arrhythmia, and heart failure rendering pregnancy high risk or even contraindicated. Infertility and high rates of first trimester miscarriage are not uncommon. In vitro fertilization (IVF) with or without gestational surrogacy can be an option, but poses risks during ovarian stimulation, oocyte retrieval, and the post-procedural period.

Improvement in ventricular function with rhythm control of atrial arrhythmias may delay the need for atrioventricular valve surgery in adults with congenital heart disease.

Objective: Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy.

Long term follow-up after ventricular tachycardia ablation in patients with congenital heart disease.

Background: Ventricular tachycardia (VT) is frequently encountered in patients with repaired and unrepaired congenital heart disease (CHD), causing significant morbidity and sudden cardiac death. Data regarding underlying VT mechanisms and optimal ablation strategies in these patients remain limited.

Objective: To describe the electrophysiologic mechanisms, ablation strategies, and long-term outcomes in patients with CHD undergoing VT ablation.

Pulmonary arterial hypertension in adults with systemic right ventricles referred for cardiac transplantation.

Background: Systemic right ventricular (RV) failure may progress necessitating referral for orthotropic heart transplantation (OHT). Pulmonary hypertension (PH) frequently coexists in adult congenital heart disease and can complicate the assessment for OHT.

Methods: Single-center case series of six patients (median age 34.

Accuracy of risk prediction scores in pregnant women with congenital heart disease.

Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.

Design: Single-center retrospective study.

Setting: Tertiary care academic hospital.

Perioperative electrophysiology study in patients with tetralogy of Fallot undergoing pulmonary valve replacement will identify those at high risk of subsequent ventricular tachycardia.

Background: Ventricular tachyarrhythmias are the most common cause of death in patients with repaired tetralogy of Fallot (TOF), but predicting those at risk remains a challenge. An electrophysiology study (EPS) has been proposed to risk stratify patients with TOF.

Objective: We sought to evaluate a perioperative EPS-guided approach to risk stratify patients with TOF undergoing pulmonary valve replacement (PVR) and guide concomitant cryoablation.

Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

Background: Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias.

Hypothesis: A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort.

Methods: We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015.

Noninvasive Predictors of Ventricular Arrhythmias in Patients With Tetralogy of Fallot Undergoing Pulmonary Valve Replacement.

Objectives: This study sought to test the hypothesis that a vectorcardiographic parameter, the QRS vector magnitude (QRSVm), can risk stratify those patients at risk for sustained spontaneous ventricular arrhythmias (VAs) or ventricular arrhythmia inducibility (VAI) in a large cohort of patients with tetralogy of Fallot (TOF).

Background: Patients with TOF have an increased risk of VAs, but predicting those at risk can often be challenging.

Methods: Blinded retrospective analyses of 177 TOF patients undergoing pulmonary valve replacement (PVR) between 1997 and 2015 were performed.

Factors Associated with the Accurate Diagnosis of Obesity.

Obesity is a growing epidemic, yet few patients with obesity receive a clinical diagnosis of obesity or appropriate counseling. We examined the socioeconomic and demographic factors associated with the accurate diagnosis of obesity during ambulatory care visits. We used data from the National Hospital Ambulatory and National Ambulatory Medical Care Surveys (NHAMCS and NAMCS) to determine if a patient with obesity had been clinically diagnosed with obesity during the visit by either of the following: (1) a diagnosis listed in the patient's record; or (2) the provider's answer to the question "despite the diagnoses listed, does this patient have obesity?" We used multivariate models to examine the association between the accurate diagnosis of obesity and socioeconomic and demographic factors.

Approach to residual pulmonary valve dysfunction in adults with repaired tetralogy of Fallot.

Residual right ventricular outflow tract and pulmonary valve disease is common in adults with repaired tetralogy of Fallot. Chronic severe pulmonary regurgitation as a result of surgical repair can lead to myriad complications including right ventricular dysfunction, decreased exercise tolerance, right heart failure and symptomatic arrhythmias. The aim of restoring pulmonary valve integrity is to preserve right ventricular size and function with the intent of mitigating the development of symptoms and poor long-term outcomes.

Slow Pathway Modification in a Patient with D-Transposition of the Great Arteries and Atrial Switch Procedure.

Patients with systemic right ventricles are often not able to tolerate frequent, rapid, or incessant atrial arrhythmias without developing significant symptoms and ventricular dysfunction. Atrial arrhythmias are associated with an increased risk of ventricular arrhythmias and sudden cardiac death. Rhythm disturbances must be aggressively addressed in this population with frequent screening, follow-up, and treatment.

A Case of Cough-induced Ventricular Tachycardia in a Patient with a Left Ventricular Assist Device.

In this case, the patient's ventricular tachycardia (VT) was specifically induced by coughing, which has not previously been described. Decreasing the rotational speed of the left ventricular assist device (LVAD) and increasing preload by stopping the patient's nitrates and reducing diuretic dose allowed improved filling of the left ventricle (LV) and increased LV volumes. When coughing recurred, the effects on the LV cavity were less pronounced and thus VT was reduced.

Vectorcardiographic predictors of ventricular arrhythmia inducibility in patients with tetralogy of Fallot.

Introduction: Vectorcardiography (VCG) may have predictive value in Tetralogy of Fallot (TOF) patients undergoing ventricular arrhythmia inducibility (VAI) electrophysiology studies (EPs).

Materials And Methods: Blinded, retrospective analyses of 37 adult TOF patients undergoing EPs prior to pulmonary valve replacements were performed (21 female, median age 37years). VAI was evaluated from EPs and resting 12-lead electrocardiograms, respectively using QRS and heart rate adjusted Q-T intervals, spatial QRS-T angles (peaks), T-wave and QRS-wave (QRSwave vc) component vector root mean squares.

Pulmonic regurgitation and management challenges in the adult with tetralogy of fallot.

Patients with tetralogy of Fallot (TOF) are living longer than ever because of advances in surgery in childhood since the 1950s. However, surgery in childhood is not a cure and remains only a palliative procedure because almost all patients will require further intervention throughout life. The most common intervention required in adulthood is pulmonary valve replacement (PVR) because of residual pulmonary regurgitation leading to right ventricular dilation and eventual dysfunction.

Preprocedural imaging in patients with transposition of the great arteries facilitates placement of cardiac resynchronization therapy leads.

Objective: The purpose of this study is to review a series of patients with complex congenital heart disease in whom preprocedural imaging was used to assist placement of cardiac resynchronization therapy (CRT) leads.

Background: CRT may be beneficial in patients with a failing systemic ventricle and transposition of the great arteries (TGA). However, complex coronary venous anatomy can be challenging for placement of CRT leads.

Differential effects of mechanical ventilatory strategy on lung injury and systemic organ inflammation in mice.

Patients with acute respiratory distress syndrome are at increased risk for developing multiorgan system dysfunction. The goal of this study was to establish an in vivo murine model to assess the differential effects of ventilation-protective strategies on the development of acute lung injury and systemic organ inflammation. C57B/6 mice were randomized to mechanical ventilation (MV) with conventional, high (17 ml/kg) or protective, low (6 ml/kg) tidal volume (VT) after intratracheal hydrochloric acid or no intervention.