Sinus rhythm QRS morphology reflects right ventricular activation and anatomical ventricular tachycardia isthmus conduction in repaired tetralogy of Fallot.

Background: Patients with repaired tetralogy of Fallot (TOF) are at risk for ventricular tachycardia (VT) related to well-described anatomical isthmuses.

Objective: The purpose of this study was to explore QRS morphology as an indicator of anatomical isthmus conduction.

Methods: Patients with repaired TOF and complete right bundle branch block referred for transcatheter pulmonary valve replacement (PVR) or presenting with sustained VT underwent comprehensive 3-dimensional mapping in sinus rhythm.

Rationale and Design of the Multicenter Catheter Ablation of Ventricular Tachycardia Before Transcatheter Pulmonary Valve Replacement in Repaired Tetralogy of Fallot Study.

Patients with repaired tetralogy of Fallot are at elevated risk for ventricular arrhythmia and sudden cardiac death. Over the past decade, the pathogenesis and natural history of ventricular tachycardia has become increasingly understood, and catheter ablation has emerged as an effective treatment modality. Concurrently, there has been great progress in the development of a versatile array of transcatheter valves that can be placed in the native right ventricular outflow tract for the treatment of long-standing pulmonary regurgitation.

Clinical and invasively-measured predictors of high exercise capacity in Fontan patients.

Introduction: Fontan patients have variable exercise capacity. Contemporary understanding as to which factors predict high tolerance is limited.

Methods: Records from the Ahmanson/University of California, Los Angeles Adult Congenital Heart Disease Center were reviewed for adult Fontan patients who underwent CPET.

Intrahepatic transcriptomics differentiate advanced fibrosis and clinical outcomes in adults with the Fontan circulation.

Background: The molecular mechanisms underlying Fontan associated liver disease (FALD) remain largely unknown. We aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes.

Methods: This retrospective cohort study included adults with the Fontan circulation at the Ahmanson/UCLA Adult Congenital Heart Disease Center.

Predictors of arrhythmia during pregnancy in adults with congenital heart disease.

Background: Risk prediction of arrhythmia during pregnancy in adult congenital heart disease (ACHD) patients is currently lacking, and the impact of preconception catheter ablation on future antepartum arrhythmia has not been studied.

Methods: We conducted a single-center, retrospective cohort study of pregnancies in ACHD patients. Clinically significant arrhythmia events during pregnancy were described, predictors of arrhythmia were analyzed, and a risk score devised.

Conduction system pacing in pediatric and congenital heart disease.

Conduction system pacing (CSP) has evolved rapidly to become the pacing method of choice for many adults with structurally normal hearts. Studies in this population have repeatedly demonstrated superior hemodynamics and outcomes compared to conventional pacing with the recruitment of the native conduction system. Children and patients with congenital heart disease (CHD) are also likely to benefit from CSP but were excluded from original trials.

Conduction System Pacing Versus Conventional Cardiac Resynchronization Therapy in Congenital Heart Disease.

Background: Dyssynchrony-associated left ventricular systolic dysfunction is a major contributor to heart failure in congenital heart disease (CHD). Although conventional cardiac resynchronization therapy (CRT) has shown benefit, the comparative efficacy of cardiac conduction system pacing (CSP) is unknown.

Objectives: The purpose of this study was compare the clinical outcomes of CSP vs conventional CRT in CHD with biventricular, systemic left ventricular anatomy.

Vascular malperfusion and abruption are prevalent in placentas from pregnancies with congenital heart disease and not associated with cardiovascular risk.

Congenital heart disease (CHD) in pregnancy is associated with an increased risk of adverse maternal, obstetric, and neonatal outcomes, plausibly through mechanisms involving abnormal placental development and function. This retrospective study aims to elucidate how maternal CHD influences placental health. Demographic and clinical information were collected via electronic medical record review, and placentas underwent histopathological evaluation.

Socioeconomic Factors and their Impact on Access and Use of Coronary and Structural Interventions.

In the past few decades, the accelerated improvement in technology has allowed the development of new and effective coronary and structural heart disease interventions. There has been inequitable patient access to these advanced therapies and significant disparities have affected patients from low socioeconomic positions. In the US, these disparities mostly affect women, black and hispanic communities who are overrepresented in low socioeconomic.

Postural orthostatic tachycardia syndrome and orthostatic hypotension in post-acute sequelae of COVID-19 during pregnancy: a case report.

Background: Patients with post-acute sequelae of COVID-19 (PASC) often experience the addition of new symptoms after recovery from COVID-19 illness. These may include orthostatic intolerance and autonomic dysfunction, and postural orthostatic tachycardia syndrome has been described to occur in a proportion of patients with PASC.

Case Summary: In this report, we present a 32-year-old pregnant woman (G3P2) who experiences severe orthostatic symptoms as part of her PASC syndrome, which is decoupled from normal physiologic changes of pregnancy.

Management of Heart Failure With Arrhythmia in Adults With Congenital Heart Disease: JACC State-of-the-Art Review.

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed.

Outcomes of malignancy in adults with congenital heart disease: a single center experience.

Background: Malignancy is known to be a major cause of death in adult congenital heart disease (ACHD). However, data regarding cardiovascular and cancer-related outcomes in ACHD are lacking.

Methods: We conducted a retrospective single-center cohort study comprising patients with ACHD and malignancy.

Cardiac Resynchronization Therapy for Adult Patients With a Failing Systemic Right Ventricle: A Multicenter Study.

Background The objective of this international multicenter study was to investigate both early and late outcomes of cardiac resynchronization therapy (CRT) in patients with a systemic right ventricle (SRV) and to identify predictors for congestive heart failure readmissions and mortality. Methods and Results This retrospective international multicenter study included 13 centers. The study population comprised 80 adult patients with SRV (48.

Catheter Ablation for Atrial Fibrillation in Adult Congenital Heart Disease: An International Multicenter Registry Study.

Background: Data on atrial fibrillation (AF) ablation and outcomes are limited in patients with congenital heart disease (CHD). We aimed to investigate the characteristics of patients with CHD presenting for AF ablation and their outcomes.

Methods: A multicenter, retrospective analysis was performed of patients with CHD undergoing AF ablation between 2004 and 2020 at 13 participating centers.

Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation.

Background: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPM) may be associated with additional adverse long-term outcomes.

Objectives: The goal of this study was to quantify the attributable risk of PPM in patients with SV, and to identify modifiable risk factors.

Transcatheter Interventions in Patients With Adult Congenital Heart Disease.

Patients with congenital heart disease now live well into adulthood because of advances in surgical techniques, improvements in medical management, and the development of novel therapeutic agents. As patients grow older into adults with congenital heart disease, many require catheter-based interventions for the treatment of residual defects, sequelae of their initial repair or palliation, or acquired heart disease. The past 3 decades have witnessed an exponential growth in both the type and number of transcatheter interventions in patients with congenital heart disease.

Critical congenital heart disease detection in the ANDES: Challenges and opportunities.

Critical congenital heart disease (CCHD) represents a challenging problem in global health equity due to the need for specialized surgical or transcatheter intervention within the first year of life. CCHD screening using pulse oximetry (POS) has led to significant improvements in mortality due to early referral and intervention. Andean America represents one of the few regions in the world with increasing CHD deaths and variable POS implementation.

Delivery Timing and Associated Outcomes in Pregnancies With Maternal Congenital Heart Disease at Term.

Background Current recommendations for delivery timing of pregnant persons with congenital heart disease (CHD) are based on expert opinion. Justification for early-term birth is based on the theoretical concern of increased cardiovascular stress. The objective was to evaluate whether early-term birth with maternal CHD is associated with lower adverse maternal or neonatal outcomes.

Right coronary artery originating from the left ventricular outflow tract diagnosed after a Ross procedure: a case report.

Background: Anomalous coronary origin from the left ventricular outflow tract (LVOT) is an exceedingly rare condition thought to be associated with the bicuspid aortic valve (BAV). While the malignant presentation of this entity has been described, its pathophysiology and diagnostic evaluation are poorly understood.

Case Summary: A 33-year-old woman status post Ross procedure in childhood for congenital aortic stenosis due to BAV with presumed common origin of right and left coronary arteries based on single coronary ostium seen on aortic valve inspection, presented with symptomatic pulmonary regurgitation and stenosis.

Suture connection of overlapping covered CP stents for transcatheter treatment of sinus venosus atrial septal defect with anomalous pulmonary venous connection.

Transcatheter correction of a superior sinus venosus defect and partial anomalous pulmonary venous connection with covered stents is a feasible alternative to surgical repair in select patients. Commercially available balloon-expandable covered stents may be of inadequate length to treat some patients. This may require multiple stents to be placed, which increases the risk of stent migration or embolization.