BACKGROUND:Sex-based differences may affect recipient profiles, donor selection, support, and outcomes after heart transplantation. Using the International Society for Heart and Lung Transplantation Registry, we evaluated trends by recipient sex. METHODS:Adults undergoing primary heart-alone transplantation from 2005 through 2023 were analyzed. Recipient and donor characteristics, support strategies, donor utilization, 1-year survival, and 5-year conditional survival among 1-year survivors were compared by recipient sex. Multivariable Cox models were adjusted for clinical covariates and predicted heart mass difference. RESULTS:Among 50,614 recipients, 13,248 (26.2%) were female. Compared with male recipients, female recipients were younger and more often had nonischemic cardiomyopathy, hypertrophic cardiomyopathy, or congenital heart disease. They also had higher rates of allosensitization, were less often bridged with durable left ventricular assist devices, and were more often transplanted without mechanical circulatory support. Expanded donor use increased in both sexes, while durable left ventricular assist device use declined and temporary support increased. One-year survival improved over time and was similar by recipient sex within eras. After adjustment, most donor-recipient sex-pairing associations were attenuated. Female donor-female recipient transplantation remained associated with higher 1-year mortality versus male donor-male recipient transplantation (hazard ratio, 1.12; 95% confidence interval, 1.02-1.22), and male donor-female recipient transplantation was associated with higher 5-year conditional mortality (hazard ratio, 1.17; 95% confidence interval, 1.04-1.31). CONCLUSIONS:Recipient sex is associated with distinct case mix, support strategies, donor utilization, and outcome patterns. These findings support matching strategies that incorporate size, immunologic, and clinical risk.

Background Barth syndrome is an X-linked mitochondrial disorder caused by pathogenic variants in the TAZ gene, resulting in impaired cardiolipin remodeling and mitochondrial dysfunction. Cardiomyopathy is the most prominent manifestation, often presenting in early infancy and potentially progressing to advanced heart failure requiring heart transplantation. Due to phenotypic overlapping with other cardiac diseases, diagnosis is frequently delayed. The aim of this study was to describe our experience with pediatric patients with Barth syndrome referred to heart transplantation and their clinical outcomes at the largest heart transplantation center in Latin America. Methods We retrospectively reviewed pediatric patients with Barth syndrome referred for heart transplantation evaluation at the Heart Institute (InCor), University of São Paulo Medical School, between 1992 and 2025. Clinical characteristics, heart failure severity, transplant evaluation, treatment response, listing status, and outcomes were described. Results We retrospectively reviewed three pediatric patients with genetically confirmed Barth syndrome referred to our center for heart transplantation evaluation. Despite severe early cardiac involvement, they exhibited markedly different heart failure trajectories. Barth syndrome cardiomyopathy follows a highly heterogeneous clinical course. Although some patients demonstrate significant recovery with optimized heart failure therapy, others progress to end-stage heart failure requiring transplantation. Our experience reinforces that early referral to pediatric heart transplant centers and individualized transplant listing based on longitudinal clinical evolution, rather than left ventricular ejection fraction alone, may optimize patient outcomes. Conclusions Early referral to pediatric heart transplant centers and individualized transplant listing based on longitudinal clinical evolution may optimize outcomes in Barth syndrome.

ABO-incompatible (ABOi) heart transplantation, first performed in infants by West and colleagues in 1996, transformed pediatric heart allocation by challenging longstanding immunologic constraints. Early success of this approach leveraged the developmental immaturity of the neonatal immune system and has since shown comparable short- and long-term outcomes to ABO-compatible (ABOc) transplantation. Over three decades, increasing clinical experience and policy evolution, including broader ABOi eligibility criteria and relaxed titer thresholds, have expanded access to donor hearts and reduced waitlist mortality, particularly among blood group ABO-O candidates. Despite these advances, marked variability persists among centers in the measurement, interpretation, and reporting of ABO antibody titers, directly influencing candidate selection, perioperative management, and post-transplant surveillance. Most assays rely on manual hemagglutination techniques that are subject to significant inter-laboratory and intra-laboratory variability. Emerging single-antigen bead-based methods utilizing the Luminex™ platform may provide an opportunity for standardized, semi-quantitative assessment of graft-relevant anti-A and -B antibody levels. Perioperative strategies are individualized according to ABO antibody titer, with intraoperative plasma exchange or immunoadsorption typically employed when titers exceed a center-specific level. Standard immunosuppression regimens are generally sufficient, with anti-B cell therapies (e.g., rituximab) reserved for elevated or rebound titers. Long-term outcomes remain excellent, with most patients not producing donor-specific ABO antibodies after ABOi transplant. Most centers perform surveillance biopsies only when rising titers, an uncommon occurrence, are accompanied by graft dysfunction or biopsy-proven antibody-mediated rejection, managed with combinations of plasmapheresis, IVIG, and anti-B cell therapies as indicated. Future multicenter collaborations incorporating standardized reporting and longitudinal follow-up will be critical to optimize candidate selection, refine management algorithms, and further improve utilization and outcomes of ABOi heart transplantation.

As cardiomiopatias são condições patológicas associadas a disfunção progressiva dos cardiomiócitos, sendo exclusas situações nas quais outras enfermidades concomitantes possam justificar tal progressão. Suas apresentações podem evoluir com diversos fenótipos, sendo os principais: hipertrófico, dilatado, restritivo, não compactado e arritmogênicos do ventrículo direito (Tabela 1). Relativo à importância acerca do tema, destaca-se que de 30 a 50% das cardiomiopatias dilatadas e hipertróficas possuem origem genética, de forma que a avaliação destas etiologias se tornou uma ferramenta essencial no manejo desses pacientes. No intuito de abordar o risco de transmissão genética e a probabilidade de manifestação fenotípica em diferentes gerações familiares, é fundamental reconhecer e classificar a forma de herança e tipo de mutação de cada gene associado à cardiomiopatia estrutural analisada. Além disso, determinar a proteína estrutural acometida, facilita a compreensão do fenômeno fisiopatológico e do fenótipo manifestado pelo portador da variante. Considerando o advento crescente da abordagem genética das cardiomiopatias, a Heart Failure Society of America em colaboração com o American College of Medical Genetics and Genomics elaborou um guia para investigação e seguimento destes casos. Apesar disso, neste estudo o momento de solicitar os testes genéticos não foi avaliado. Recomendando-se que os testes sejam solicitados em qualquer momento após a confirmação do diagnóstico, visto que o resultado desses pode contribuir na orientação do seguimento mais adequado. Apesar da infinidade de variantes existentes relacionadas a cardiomiopatias genéticas, algumas são de maior interesse clínico por seu impacto em estratificação de risco e interesse em rastreamento familiar, são elas: MYH7, PRKAG2, MYBPC3, GLA, LAMP2, TTN, DSP, MYH7, FLNC, LMNA, DSP, MYBPC3 e Complexo de Troponina (Tabela 2). Reconhecendo as principais mutações associadas às cardiomiopatias e os fenótipos a ela relacionados, é possível realizar uma medicina mais individualizada com intervenções precoces e estratificação de risco. Dessa maneira, a avaliação genética dos pacientes diagnosticados com cardiomiopatias estruturais é fundamental para o rastreamento de familiares e para o manejo específico mais vantajoso, o qual pode alterar de acordo com a mutação identificada.

Pediatric heart failure (HF) secondary to cardiomyopathies, acquired heart disease, and congenital heart disease is associated with significant morbidity and mortality. These guidelines represent an update from the International Society for Heart and Lung Transplantation guidelines for the management of pediatric HF that were published in 2014 and incorporate interval advancements in medical therapies and new approaches in the evaluation and management of children with HF.

Congenital heart diseases, including hypoplastic left heart syndrome (HLHS), frequently lead to malnutrition, which can result in worse outcomes in critical and perioperative situations. This study presents a case report based on clinical and nutritional data extracted from the electronic medical record of a 1-year and 10-month-old male infant diagnosed with HLHS, hospitalized in a tertiary cardiology hospital while awaiting a heart transplant. The length of hospitalization while waiting for the organ was approximately 13 months. Upon hospital admission, the child presented with severe malnutrition according to the World Health Organization Child Growth Standards. Thus, the decision was made to initiate enteral nutritional therapy in combination with an oral diet. Despite being complementary, the enteral nutritional intake ranged from 35 to 100 kcal/kg/d and 1.6 to 2.7 g of protein/kg/d. As a result, the child presented progressive weight gain, reaching normal values, as per World Health Organization growth standards, by the time of transplantation. Although limited, there was also an improvement in the child's length-for-age metric. Physical examination also revealed other improvements, such as in hair pigmentation and recovery of body reserves. Most notably, the child had a satisfactory recovery following heart transplant surgery. Therefore, it appears that the nutritional therapy applied was effective in improving the nutritional status of a child with severe malnutrition, which may have contributed to the surgical success and good postoperative recovery. Children with HLHS may benefit from alternative feeding methods to achieve higher nutritional intake.

ABSTRACT Background Short‐term outcomes following heart transplantation in children have improved, but comparable improvements in long‐term survival continues to have barriers. We sought to investigate long‐term outcomes following heart transplantation and to identify protective and risk factors associated with long‐term survival in children. Methods The Pediatric Heart Transplant Society (PHTS) database was queried for heart transplant recipients from 1993 to 2010 who were ≤ 10 years of age at time of transplant. Patients with conditional graft survival > 3 years and at ≥ 10 years were analyzed. Survival and time‐to‐event were compared using the Kaplan–Meier method with a log‐rank test for significance. Factors associated with graft loss were identified using Cox proportional hazard modeling. Results There were 1610 patients ≤ 10 year of age who were transplanted between 1993 and 2010 with conditional survival to 3 years post‐transplant. Of those patients, there were 1170 with conditional survival to 10 years post‐transplant. Patients < 1 year at transplant had improved survival compared to other age groups. Risk factors for graft loss after 3 years post‐transplant were malignancy, rejection, cardiac allograft vasculopathy (CAV), age, congenital heart disease, female sex, and Black race ( p value for all < 0.05). Conclusions Heart transplantation remains an effective therapy in children with a growing number of long‐term survivors. Risk factors for mortality in patients ≤ 10 years of age at transplant with conditional survival to 3 years post‐transplant include CAV, rejection, malignancy, female sex, and Black race. Further studies are needed to understand the social and biologic causes of racial and sex disparities in pediatric transplant patients.

The purpose of this study was to describe the first successful Heartmate 3 implantation in the pediatric population in Brazil.

Chylopericardium is a rare condition, typically associated with the postoperative period following cardiac surgery, lymphatic system obstruction, or congenital malformations. We report the case of a school-aged child with Noonan syndrome who developed treatment-resistant chylopericardium after undergoing heart transplantation. Further investigation revealed an anomalous lymphatic system.

Background:Heart transplantation (HT) has been the treatment of choice for children with end-stage heart disease. Since the first transplant in 1967, almost 6 decades on, vast improvements have occurred, the number of transplants has increased over time, and post-transplant survival curve has progressively improved. Methods:The purpose of this review is to analyze the current status of pediatric HT, including epidemiology of heart failure, number of ventricular assist devices, number of transplants, and outcomes. The pretransplant, contemporary issues of children with heart failure, the waiting list for transplant, the role of multidisciplinary team, perspectives to overcome the storage of donors with xenotransplantation, donation after circulatory death, and ABO-incompatible transplantation are discussed. Results:The outcome of transplantation in children and the main post-transplant issues related to immunosuppression, transition, and adherence were addressed, as well as the use of telemedicine as an additional tool for surveillance. Conclusion:HT in pediatric population is an established treatment for end-stage heart disease patients. The survival has increased over time, and there is a promising improvement in the care of these patients in relation to quality of life based on advances in the current researches and research and innovations in the future, improving life expectancy.

INTRODUCTION:Fear of cardiac arrest among parents of infants with heart disease can cause stress and anxiety. Literature is scarce on the effects of cardiopulmonary resuscitation training (CPRt) on anxiety and stress of parents. We analysed the impact of CPRt on anxiety, stress, and comfort levels on parents of infants with heart disease. METHODS:Cardiopulmonary resuscitation (CPR) and choking relief manoeuvre (CRM) comfort level, Parental State-Trait Anxiety Inventory (STAI), and Parenting Stress Index (PSI) scores were prospectively collected pre-, immediately post-, and 3 months post-CPRt. RESULTS:There were 97 participants: 80% (n = 78) mothers/grandmothers and 20% (n = 19) fathers. The mean (SD) age of participants was 28.7 (5.6) years old. There was a significant decrease in STAI across the three time points collected; STAI decreased by 12% from baseline to immediately post-CPRt and 19% from baseline to 3 months post-CPRt (p < .0001). There were no significant changes in PSI across the time points. Baseline to immediately post-teaching, we found that CPRt significantly increased comfort performing CPR, CRM, and comfort in knowing what to do (p=< .001, p=< .001, p=< .001, respectively). Comfort levels persisted elevated when comparing pre- to 3 months post-CPRt (p=< .001, p= .002, p= .001, respectively), maintaining at least a 177% average increase up to 3 months post-CPRt for all aspects. CONCLUSION:CPRt can aid in improving anxiety and comfort levels of parents of infants with heart disease around hospital discharge. Parental preparedness and reassurance to know what to do in emergency situations can be enhanced by a simple intervention such as CPRt.

Background Arrhythmias can lead to cardiac arrest and heart failure. When intractable, heart transplantation (HTX) can become the only viable treatment. This rare high-risk cohort has not been reported as a distinct group. Objective The purpose of this study was to characterize the outcomes of pediatric patients listed for HTX with the primary indication being malignant arrhythmia (MA). Methods Using the Pediatric Heart Transplant Society prospective registry, we identified all patients younger than 18 years listed between 2014 and 2022. MA as the listing indication was categorized into primary tachyarrhythmia (PT), inherited arrhythmia (IA), congenital heart disease, and cardiomyopathy (CM) with secondary arrhythmia. Demographic, listing, and transplant data were analyzed. Results Among 4630 patients listed and 3317 transplanted, MA was the indication in 63 (1.4%) and 49 (1.5%), respectively. Patients with MA were categorized as PT in 11 (17%), IA in 4 (6%), congenital heart disease in 6 (10%), and CM in 42 (67%). Compared with the non-MA cohort, patients listed for MA were older (mean age 10.6 ± 6.2 years vs 6.1 ± 6.2 years; P < .01), more likely to present with cardiac arrest (43% vs 11%; P < .01), and less likely to be in the intensive care unit (40% vs 58%; P < .01) or on inotropes (30% vs 60%; P < .01) at the time of listing. Outcomes including waitlist mortality, transplantation, posttransplant survival, and freedom from rejection were comparable to those of the non-MA cohort. Conclusion Patients with MA constitute a small proportion of those listed for HTX in childhood. CM was the most common category, while IA and PT were rare. Their waitlist mortality and posttransplant outcomes were comparable to those of the non-MA cohort.

Heart transplantation is the treatment of choice for patients with refractory heart failure treatment. Although there were advances in medical therapy and immunological knowledge regarding rejection, it is one of the major limitations for long-term survival for those who were submitted to heart transplantation. In this chapter, the main issues of rejection and prevention in heart transplantation will be discussed.