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Introduction/Aims: Duchenne muscular dystrophy (DMD) is characterized by fibrofatty replacement of muscle. This has been documented in the ventricular myocardium of DMD patients, but there is limited description of atrial involvement. The purpose of this study is to examine the arrhythmia and ectopy burden in patients with DMD and non‐DMD dilated cardiomyopathy (DCM) and to characterize the cardiac histopathologic changes in DMD patients across the disease spectrum. Methods: This was a retrospective analysis of age‐matched patients with DMD and non‐DMD DCM who received a Holter monitor and cardiac imaging within 100 days of each other between 2010 and 2020. Twenty‐four‐hour Holter monitors were classified based on the most recent left ventricular ejection fraction at the time of monitoring. Cardiac histopathologic specimens from whole‐heart examinations at the time of autopsy from three DMD patients and one DCM patient were reviewed. Results: A total of 367 patients with 1299 Holter monitor recordings were included over the study period, with 94% representing DMD patients and 6% non‐DMD DCM. Patients with DMD had more atrial ectopy across the cardiac function spectrum (p < 0.05). There was no difference in ventricular ectopy. Four DMD patients developed symptomatic atrial arrhythmias. Autopsy specimens from DMD patients demonstrated fibrofatty infiltration of both atrial and ventricular myocardium. Discussion: The atrial myocardium in patients with DMD is unique. Autopsy specimens reveal fibofatty replacement of the atrial myocardium, which may be a nidus for both ectopy and arrhythmias in DMD patients. [ABSTRACT FROM AUTHOR]

The number of Fontan patients with circulatory failure and systolic dysfunction is growing rapidly. The last decade has demonstrated that ventricular assist device (VAD) is an effective therapy in properly selected patients. Herein, we discuss the current approach to patient selection, implantation, and patient management. [ABSTRACT FROM AUTHOR]

The last 10 years have seen an increase in the number of Fontan patients with heart failure. There has been a coincident rapid evolution in the field of pediatric and congenital heart disease ventricular assist device therapy. Herein, we describe the existing body of literature regarding the use of ventricular assist device therapy in the Fontan circulation as well as the current approach to clinical decision-making and device implantation within the field. [ABSTRACT FROM AUTHOR]

Background Ventricular assist device (VAD) use as a bridge to transplant (BTT) for children with end-stage heart failure and congenital heart disease (CHD), although challenging, has increased, but its effect on posttransplant outcome is unknown. This study describes posttransplant outcomes of CHD patients BTT with a VAD. Methods All heart transplant recipients identified in United Network of Organ Sharing database from 2006 to 2015 (n = 21,865) were divided into four groups by those with (+) and without (–) a diagnosis of CHD and with (+) and without (–) VAD support at transplant: +CHD/+VAD, +CHD/–VAD, +VAD/–CHD, and –VAD/–CHD. Posttransplant survival of +CHD/+VAD was compared with +CHD/–VAD, –CHD/+VAD, and –CHD/–VAD in addition to pretransplant characteristics comparison between +CHD/+VAD and +CHD/–VAD. Results Of 1,871 patients (8.6%) with CHD, 1,348 (72%) were younger than 18 years old, and 143 (7.6%) were BTT with a VAD (+CHD/+VAD). At transplant, +CHD/+VAD compared with +CHD/–VAD were more likely to have worse functional status (<50%: 60% vs 46%, p = 0.004), infections (29% vs 14%, p < 0.001), to be sensitized (47% vs 30%, p < 0.001) and on ventilator support (20% vs 13%, p = 0.029) and dialysis (13% vs 2.5%, p < 0.001). Overall, 1-year (84% vs 87%) and 5-year (72% vs 75%) survival was similar for +CHD/+VAD and +CHD/–VAD ( p = 0.694). Survival was also similar when +CHD/+VAD were compared with –CHD/+VAD (n = 7,363; p = 0.529) and –CHD/–VAD (n = 12,613; p = 0.097). Conclusions Although more ill pretransplant, CHD patients BTT with a VAD have similar posttransplant survival compared with CHD patients without a VAD and with other non–CHD heart transplant patients. VAD support may mitigate certain risk factors for poor posttransplant outcomes in the challenging CHD cohort. [ABSTRACT FROM AUTHOR]

Abstract: Despite a limited supply of donors, potential donor hearts are often declined for subjective concerns regarding organ quality. This analysis will investigate the relationship between donor heart AR and patient outcome at pediatric transplant centers. The UNOS database was used to identify all match runs for pediatric candidates (age < 18 years) from 2008 through March 2015 in which a heart offer was ultimately placed. Centers which received ≥10 offers/y were included (10 634 offers, 38 centers). Transplant centers were stratified based on their AR: low (<20%, n = 13), medium (20%‐40%, n = 16), or high (>40%, n = 9). Low AR centers experienced worse negative WL outcome compared with medium (P = .022) and high (P = .004) AR centers. Low AR centers had similar post‐transplant graft survival to medium (P = .311) or high (P = .393) AR centers; however, medium AR centers had better post‐transplant graft survival than high AR centers (P = .037). E‐F survival from listing regardless of transplant was worse for low AR centers compared with medium (P < .001) or high (P = .001) AR centers. Low AR centers experience worse WL outcomes without improvement in post‐transplant outcomes. High AR centers experience higher post‐transplant graft failure than medium AR centers. AR of 20%‐40% appears to have optimal WL and post‐transplant outcomes. [ABSTRACT FROM AUTHOR]

Abstract: The effect of obesity stratification on pediatric heart transplant outcomes is unknown. The UNOS database was queried for patients ≥2‐<18 years listed for heart transplant and stratified by BMI: normal (BMI>5%‐≤85 percentile), overweight (BMI=86%‐95 percentile), class 1 (BMI=100%‐120% of 95 percentile), class 2 (BMI=121%‐140% of 95 percentile), and class 3 obesity (BMI>140% of 95 percentile). A total of 5056 individuals were listed for transplant, with 71% normal, 13% overweight, 10% class 1, 4% class 2, and 2% class 3 obesity. Waitlist survival was not different between groups. Post‐transplant survival was decreased in overweight and combined obese groups vs normal, with no further difference between overweight and obese classes. Overweight and obese patients had higher listing status and were more likely to have ventilator, inotrope, and mechanical circulatory support at listing. After transplant, there was an association of overweight‐obese patients with diabetes and rejection requiring hospitalization. Stricter definition of normal weight reveals overweight‐obese status was an independent risk factor for poorer post‐transplant survival, without further effect by stratification of weight class. However, because there is no difference in waitlist survival, this study does not allow the selection of absolute weight‐based criteria regarding transplant listing and suggests the need to look further for modifiable risk factors post‐transplant. [ABSTRACT FROM AUTHOR]

The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc.) and physiologic constraints (diastolic dysfunction). The total artificial heart (TAH), which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD. [ABSTRACT FROM AUTHOR]

While use of the total artificial heart (TAH) is growing, the use of the device is not uniform across the gender and age spectrum because the vast majority of implants are in adult males. SynCardia has recently developed a smaller 50 cc TAH that was designed to accommodate patients with a body surface area as low as 1.2 m 2 (potentially even lower using virtual implantation). Herein, we describe the early use of the 50 cc TAH (10 implants in the US and 18 outside the US). Twenty-eight devices have been implanted worldwide. Nineteen (68%) patients were female, 4 (14%) were 21 years of age or younger, and 2 (7%) had a diagnosis of congenital heart disease (1 Fontan). The smallest patient, by body surface area, was 1.35 m 2 . Six patients (21%) have been placed on the Freedom Driver, all of whom have survived. Fourteen patients (50%) have had a positive outcome to date. The development of the 50 cc TAH has expanded the population of patients who may benefit from TAH support and thus may help improve outcomes for patients who have had limited biventricular support options to date. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is available in the text. [ABSTRACT FROM AUTHOR]

An introduction is presented in which the editor discusses the implications of a report in the issue which examines the feasibility of cardiac destination therapy in pediatrics on basis of the current utilization of ventricular assist devices (VAD) and artificial implants in children.