Case Report: Filamin C gene mutation associated with restrictive cardiomyopathy leading to heart transplantation.

Background: Cardiomyopathy is a disease that affects the myocardium and can be classified as dilated, restrictive, or hypertrophic cardiomyopathy. Among the subtypes, restrictive cardiomyopathy is characterized by restriction of ventricular filling and its uncommon cause is a disease due to mutation on Filamin C (FLNC) gene. Filamin C is an actin-binding protein encoded by FLNC gene and participates in sarcomere stability maintenance, which is expressed on the striated muscle.

They Deserve to Live: Impact of the Berlin Heart EXCOR on Small Children's Heart Transplant Waitlist in Low-Resource Settings.

In newly emerging economy countries, the shortage of pediatric donor hearts and poor healthcare infrastructure poses a significant challenge. Although mechanical circulatory support (MCS) has been proven effective in enhancing heart transplant waiting list outcomes, economic barriers hinder its widespread adoption. A single-center retrospective study reviewed children under 10 kg on the heart transplant (HTx) list from 2012 to 2023.

Comparative Analysis of Respiratory and Functional Outcomes in Children Post-Fontan Procedure Versus Healthy Peers.

Studies have shown that respiratory muscle training enhances functional capacity and pulmonary function in Fontan patients. However, diaphragm muscle characteristics in Fontan children have not been fully elucidated. The aim of this study was to compare respiratory function, maximal and submaximal functional capacities, and quality of life, as well as to assess diaphragm mobility and thickness, between Fontan patients aged 8 to 12 years and healthy individuals.

HeartMate 3 Ventricular Assist Device in a Single-Ventricle and Single-Lung Patient Palliated With a Bidirectional Glenn.

We describe a 15 year old patient with failing second-stage single-ventricle palliation and left pulmonary artery thrombosis successfully supported with HeartMate 3 ventricular assist device and a Fontan completion as destination therapy.

Long-Term Effects of Pulmonary Valve Implantation and Prosthesis Evolution in Patients with Repaired Tetralogy of Fallot.

Background: Pulmonary valve regurgitation is a significant long-term complication in patients with tetralogy of Fallot (TOF).

Objective: This study aims to investigate the effects of pulmonary valve implantation (PVI) on the anatomy and function of the right ventricle (RV) and the long-term evolution of the implanted prosthesis in the pulmonary position.

Methods: A single-center retrospective cohort analysis was performed in 56 consecutive patients with TOF who underwent PVI.

Familial Profile of Pediatric and Adult Patients With Congenital Heart Disease in Heart Transplantation.

Congenital heart disease is the most common birth defect, and heart transplantation is the main treatment of choice. As genetic causes can be identified in a considerable proportion of cases, investigation of possible family cardiac history is essential. We analyzed the profiles of pediatric heart transplant recipients in terms of family history of heart disease.

Challenges of Congenital Heart Surgery in Brazil: It is Time to Designate Pediatric Congenital Heart Surgery Subspecialty.

Congenital heart disease (CHD) affects eight to ten out of every 1,000 births, resulting in approximately 23,057 new cases in Brazil in 2022. About one in four children with CHD requires surgery or other procedures in the first year of life, and it is expected that approximately 81% of these children with CHD will survive until at least 35 years of age. Professionals choosing to specialize in CHD surgery face numerous challenges, not only related to mastering surgical techniques and the complexity of the diseases but also to the lack of recognition by medical societies as a separate subspecialty.

Serologic Evaluation for Severe Acute Respiratory Syndrome Coronavirus 2 in Pediatric Heart Transplantation Recipients and Patients on a Pediatric Heart Transplantation Waiting List in a Quaternary Hospital.

Background: The Coronavirus disease 2019 (COVID-19) pandemic has been a global reality for longer than 3 years. Serologic studies have great importance for understanding the virus's behavior in populations, as it can suggest the status of the epidemic in a community. This cross-sectional study aimed to analyze the serologic profile for COVID-19 in patients before and after pediatric heart transplantation.

Myocardial tissue expression of mRNA and preoperative neutrophil-lymphocyte ratio in children undergoing congenital heart surgery.

Background: The neutrophil-lymphocyte ratio (NLR) is an easily accessible and inexpensive biomarker that has been shown to predict morbidity and mortality in congenital cardiac surgery. However, its regulatory mechanism remains unclear. This study aims to compare and correlate the tumor necrosis factor alpha (TNF-α), interleukin (IL)-1β, IL-6, and IL-10 messenger RNAs (mRNAs) with the NLR in patients with tetralogy of Fallot (ToF) and ventricular septal defect (VSD).

Fifth "Jatene Lecture on Surgical Innovation": Innovation in Congenital Heart Surgery: Contributions From South America.

We present the fifth "Jatene Lecture on Surgical Innovation" on Innovation in Congenital Heart Surgery, given at the Eighth Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery and Eighth World Congress of Pediatric Cardiology and Cardiac Surgery in Washington DC in 2023. We highlight what surgical innovation is and how innovation was accomplished in cardiac surgery and particularly in congenital heart surgery. A brief history of the development of congenital heart surgery across the world is summarized and we finally illustrate the South American contributions to congenital heart surgery, acknowledging the great innovations of Adib Jatene and Guillermo Kreutzer to our field.

Predictors of in-ICU length of stay among congenital heart defect patients using artificial intelligence model: A pilot study.

Objective: This study aims to develop a predictive model using artificial intelligence to estimate the ICU length of stay (LOS) for Congenital Heart Defects (CHD) patients after surgery, improving care planning and resource management.

Design: We analyze clinical data from 2240 CHD surgery patients to create and validate the predictive model. Twenty AI models are developed and evaluated for accuracy and reliability.

Performance Evaluation of Geometrically Different Pediatric Arterial Cannulae in a Pediatric Cardiopulmonary Bypass Model.

Objective: To define a reference chart comparing pressure drop vs. flow generated by a set of arterial cannulae currently utilized in cardiopulmonary bypass conditions in pediatric surgery.

Methods: Cannulae from two manufacturers were selected considering their design and outer and inner diameters.

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries.

A model for preservation of thymocyte-depleted thymus.

DiGeorge syndrome is a disorder caused by a microdeletion on the long arm of chromosome 22. Approximately 1% of patients diagnosed with DiGeorge syndrome may have an absence of a functional thymus, which characterizes the complete form of the syndrome. These patients require urgent treatment to reconstitute T cell immunity.

Heart Transplantation in Children and Adults With Congenital Heart Disease: 3 Decades of Evolution.

Heart transplantation is the treatment of choice for children and adults with congenital heart disease. We report the heart transplant single-center experience. The number of transplantations has increased over the last 3 decades.

Mechanical Circulatory Assistance in Children: Clinical Outcome.

Background: In countries where organ donation is scarce, mortality in the pediatric heart transplant waiting list is high, and ventricular assist devices (VADs) are therapeutic alternatives in these situations. Berlin Heart EXCOR is currently 1 of the few VADs specific for children.

Methods: This retrospective study includes pediatric patients who underwent Berlin Heart EXCOR placement in a Brazilian hospital between 2012 and 2021.

Brain tuberculoma in pediatric heart transplant recipient.

Introduction: Heart transplantation is the standard treatment for end-stage heart disease. Despite advances in the field, patients remain under risk of developing complications, including opportunistic infections, such as tuberculosis. We present the unprecedented case of cerebral tuberculoma in a 9-year-old heart transplant recipient.

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant.

We will report a case of a desmoid tumour (DT), which developed at the surgical site of the pacemaker after a late childhood heart transplant. Patients with idiopathic dilated cardiomyopathy followed up in the paediatric cardiology service. It evolved with the dissociation of ventricular rhythm caused by severe heart failure, which led to the implantation of a cardiac resynchronization device prior to heart transplantation.

Ischemic preconditioning does not prevent placental dysfunction induced by fetal cardiac bypass.

Background: Remote ischemic preconditioning (rIPC) has been applied to attenuate tissue injury. We tested the hypothesis that rIPC applied to fetal lambs undergoing cardiac bypass (CB) reduces fetal systemic inflammation and placental dysfunction.

Methods: Eighteen fetal lambs were divided into three groups: sham, CB control, and CB rIPC.

Neutrophil-Lymphocyte Ratio in Congenital Heart Surgery: What Is Known and What Is New?

Operative mortality after repair of congenital heart disease has improved dramatically over the past few decades. Nevertheless, there is always room for the additional mitigation of complications and mortality. Being able to anticipate adverse outcomes is clearly important, especially when using low-cost and easily accessible resources.