Increased Body Mass Index and Ventricular Cardiac Magnetic Resonance Characteristics in Adults With Fontan Circulation.

Background: In structurally normal hearts, increased body mass index (BMI) is associated with larger ventricular mass and volume, however, this association has yet to be described in adult Fontan patients.

Objectives: This study evaluates the relationship of increased BMI and ventricular characteristics by cardiac magnetic resonance imaging (CMR) in a multi-institutional adult Fontan population.

Methods: We conducted a multicenter, cross-sectional study using the Fontan Outcomes Registry using CMR Examinations.

Construct and Predictive Validity of the Kansas City Cardiomyopathy Questionnaire in Adult Congenital Heart Disease.

Background: The Kansas City Cardiomyopathy Questionnaire (KCCQ) is an Food and Drug Administration-approved health status measure for patients with heart failure (HF) but has not been directly assessed in adult congenital heart disease (ACHD).

Objectives: This study evaluates construct and predictive validity of the KCCQ in ambulatory ACHD patients.

Methods: We performed cross-sectional and prospective cohort studies of ACHD patients who completed the KCCQ to assess construct and predictive validity, respectively.

Ethical considerations in access to heart transplant for adults with congenital heart disease.

Purpose Of Review: Heart failure is a final common pathway for many patients with adult congenital heart disease (ACHD). As such, ACHD heart transplantation is increasingly being considered and performed. Ethical considerations exist at each step in the transplantation process.

Outcomes in infective endocarditis among adults with CHD: a comparative national study.

Background: Given increased survival for adults with CHD, we aim to determine outcome differences of infective endocarditis compared to patients with structurally normal hearts in the general population.

Methods: We conducted a retrospective cross-sectional study identifying infective endocarditis hospitalisations in patients 18 years and older from the National Inpatient Sample database between 2001 and 2016 using International Classification of Disease diagnosis and procedure codes. Weighting was used to create national annual estimates indexed to the United States population, and multivariable logistic regression analysis determined variable associations.

A Multisite Retrospective Review of Direct Oral Anticoagulants Compared to Warfarin in Adult Fontan Patients.

Purpose: Direct oral anticoagulants (DOACs) are not recommended in adult Fontan patients (Level of Evidence C). We hypothesized that DOACs are comparable to warfarin and do not increase thrombotic and embolic complications (TEs) or clinically significant bleeds.

Methods: We reviewed the medical records of adult Fontan patients on DOACs or warfarin at three major medical centers.

Endovascular Repair of a Descending Thoracic Aortic Aneurysm in a Pediatric Patient with Tuberous Sclerosis: A Case Report and Review of the Literature.

Aortic aneurysm in children is rare, but has been described in the tuberous sclerosis complex (TSC) population. While surgical repair has been utilized as the primary means of intervention, we present the first known case reporting exclusion of a descending thoracic aortic aneurysm with percutaneous covered stent implantation in a pediatric patient with TSC. A review of the literature is also included herein.

Sizing Up Fontan Failure: Association with Increasing Weight in Adulthood.

Obesity has become increasingly recognized in adults with Fontan palliation, yet the relationship between weight changes in adulthood and Fontan failure is not clearly defined. We hypothesize that increasing weight in adulthood among Fontan patients is associated with the development of Fontan failure. Single-center data from adults with Fontan palliation who were not in Fontan failure at their first clinic visit in adulthood and who received ongoing care were retrospectively collected.

HeartMate 3 in a ccTGA patient.

A 49-year-old female with congenitally corrected (or levo-) transposition of the great arteries complicated by nonischemic cardiomyopathy presented for worsening heart failure despite guideline-directed medical therapy and was found to be in cardiogenic shock. She successfully underwent ventricular assist device placement with a HeartMate III to her systemic right ventricle as a bridge to transplantation.

Sizing heart transplant donors in adults with congenital heart disease.

Objective: Optimal donor sizing for heart transplantation (HT) in adults with congenital heart disease (CHD) remains unclear, given the propensity for pulmonary hypertension related to shunting, staged repairs, and periods of pulmonary overcirculation. We studied HT outcomes related to donor size matching in the adult CHD population.

Methods: We conducted a retrospective cohort analysis of patients with CHD undergoing HT in the United States from January 1, 2000, to December 31, 2015.

More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients.

Setting: Fontan-associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra-cardiac dysfunction as measured by MELD and MELD-XI.

: An Unusual Case of Purulent Pericarditis.

Purulent pericarditis is a rare diagnosis with life-threatening implications due to the rapid accumulation of pericardial material, swiftly progressing to tamponade physiology. The nature of its quickly evolving and severe implications demands a low threshold for diagnostic consideration where appropriate. We present an unusual case of purulent pericarditis secondary to in a previously healthy male adolescent without traditional risk factors, which raises the question of whether emergent species may have acquired novel molecular mechanisms.