Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation.

Background: The Fontan operation is a surgical procedure to palliate single ventricle congenital heart disease. Hepatocellular carcinoma (HCC) is a rare complication of Fontan-associated liver disease (FALD).

Objectives: The authors aim to examine characteristics of individuals with Fontan circulation diagnosed with HCC and to describe tumor characteristics, treatment, and survival outcomes of these patients.

Cardiovascular Disease Among Women and Birthing Individuals After Delivering a Child With Congenital Heart Disease.

Background: Individuals have a higher risk of cardiovascular disease later in life if they give birth to a child with congenital heart disease (CHD). The mechanism of this association has not been well documented.

Objectives: The authors aimed to describe the prevalence of cardiovascular disease and risk factors in women and birthing individuals 18 to 23 years after delivery of a child with CHD compared to normative data.

Postpartum readmissions among patients with adult congenital heart disease.

Background: Given the risks associated with congenital heart disease in the postpartum period, epidemiologic data identifying risk factors and timing of complications may be useful in improving postpartum care.

Objective: This study aimed to determine the timing of, risk factors for, and complications associated with 60-day postpartum readmissions following deliveries with maternal congenital heart disease.

Study Design: The 2010-2020 Nationwide Readmissions Database was used for this retrospective cohort study.

Contribution of rare chromosome 22q11.2 copy number variants to non-syndromic bicuspid aortic valve.

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect in adults, often leading to complications such as thoracic aortic aneurysms and aortic stenosis. While BAV is frequently associated with 22q11.2 deletion syndrome (22q11.

Rare genomic copy number variants implicate new candidate genes for bicuspid aortic valve.

Bicuspid aortic valve (BAV), the most common congenital heart defect, is a major cause of aortic valve disease requiring valve interventions and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications (EBAV) to sporadic late onset disease. Rare genomic copy number variants (CNVs) have previously been implicated in the development of BAV and thoracic aortic aneurysms.

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.

Mortality and morbidity after combined heart and liver transplantation in the failing Fontan: An updated dual center retrospective study.

Introduction: As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation.

Methods: We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022.

Results: The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively.

Pregnancy-related cardiac outcomes among patients with congenital heart disease after formalization of a cardio-obstetrics program.

Background: The prevalence of pregnant patients with congenital heart disease (CHD) is increasing, and these patients are at high risk for cardiac morbidity.

Objective: This study aimed to examine the pregnancy outcomes in patients with congenital heart disease before and after the establishment of formal cardio-obstetrics collaboration between adult congenital heart disease and maternal-fetal medicine programs.

Study Design: This was a retrospective cohort study of pregnant patients with congenital heart disease from 2002 to 2020 at a single urban academic institution in the United States.

Whole Exome Sequencing Uncovers the Genetic Complexity of Bicuspid Aortic Valve in Families with Early Onset Complications.

Bicuspid Aortic Valve (BAV) is the most common adult congenital heart lesion with an estimated population prevalence of 1%. We hypothesize that early onset complications of BAV (EBAV) are driven by specific impactful genetic variants. We analyzed whole exome sequences (WES) to identify rare coding variants that contribute to BAV disease in 215 EBAV families.

Rare Genomic Copy Number Variants Implicate New Candidate Genes for Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV), the most common congenital heart defect, is a major cause of aortic valve disease requiring valve interventions and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications (EBAV) to sporadic late onset disease. Rare genomic copy number variants (CNVs) have previously been implicated in the development of BAV and thoracic aortic aneurysms.

Novel Approaches to the Failing Congenital Heart.

Purpose Of Review: Heart failure is the leading cause of morbidity and mortality in adults with congenital heart disease and is characterized by a variety of underlying mechanisms. Here, we aim to elaborate on the medical and technological advancements in the management of heart failure in adult patients with congenital heart disease and highlight the use of imaging modalities to guide therapy.

Recent Findings: There have been several advances over the past decade with angiotensin receptor neprilysin and sodium-glucose cotransporter-2 inhibitors, atrioventricular valve clips, transcatheter pulmonary valves, catheter ablation, and cardiac resynchronization therapy, as well as the introduction of lymphatic interventions.

Prolonged labor and adverse cardiac outcomes in pregnant patients with congenital heart disease.

Background: Patients with congenital heart disease are at high risk for peripartum cardiac morbidity, yet data on the impact of duration of labor on cardiac outcomes are limited. Prolonged labor is a known risk factor for maternal morbidity, but the impact of prolonged labor on cardiac outcomes in patients with congenital heart disease has not been evaluated.

Objective: This study aimed to evaluate the association between prolonged labor (≥24 hours) and adverse peripartum maternal cardiac outcomes in pregnant patients with congenital heart disease.

Psychological Outcomes and Interventions for Individuals With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Although resilience and high quality of life are demonstrated by many individuals with congenital heart disease, a range of significant psychological challenges exists across the life span for this growing patient population. Psychiatric disorders represent the most common comorbidity among people with congenital heart disease. Clinicians are becoming increasingly aware of the magnitude of this problem and its interplay with patients' physical health, and many seek guidance and resources to improve emotional, behavioral' and social outcomes.

Durable Mechanical Circulatory Support in Adult Congenital Heart Disease: Reviewing Clinical Considerations and Experience.

Adults with congenital heart disease (ACHD) patients are one of the fastest growing populations in cardiology, and heart failure (HF) is the most common cause of morbidity and mortality amongst them. The need for advanced HF therapies in ACHD patients stands to grow substantially. The anatomic considerations for placing durable mechanical circulatory support (MCS) devices in ACHD patients often require specialized approaches.

Delivery outcomes associated with maternal congenital heart disease, 2000-2018.

Purpose: To characterize temporal trends and outcomes of delivery hospitalization with maternal congenital heart disease (CHD).

Materials And Methods: For this repeated cross-sectional analysis, deliveries to women aged 15-54 years with maternal CHD were identified in the 2000-2018 National Inpatient Sample. Temporal trends in maternal CHD were analyzed using joinpoint regression to estimate the average annual percentage change (AAPC) with 95% CIs.

Diuretic use in the adult Fontan.

Objectives: To explore diuretic use in Fontan patients and its association with clinical features and adverse outcomes.

Background: In Fontan circulatory failure, ventricular dysfunction, chronically elevated systemic venous pressures, lymphatic abnormalities, and preload deprivation lead to volume expansion, prompting initiation of diuretics, yet use of diuretics has not been widely studied in the adult Fontan patient.

Methods: Single center retrospective review of Fontan patients ≥18 years between 2005 and 2018.

Capturing Adult Congenital Heart Disease: Framework for Development of an Adult Congenital Heart Disease Mortality Risk Model.

Background: As a formative step toward development of adult congenital heart disease (ACHD) risk-adjusted metrics, we describe The Society of Thoracic Surgeons (STS) ACHD population, procedural frequency, and early mortality.

Methods: Adults (≥18 years) with CHD (2014-2019) were identified in the STS Adult Cardiac Surgery (ASCD) and the Congenital Heart Surgery (CHSD) Databases. After deduplication, variable mapping, data concatenation, and harmonization of preprocedure factors, procedures were grouped, and unadjusted mortality was catalogued for the overall cohort and the cohort excluding patients with an isolated bicuspid aortic valve (BAV).

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry.

The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with "stable" Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening.

Laparoscopic Surgery Requiring Abdominal Insufflation in Patients With Congenital Heart Disease.

Objective: To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach.

Design, Setting, Participants: This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study.