Fallot

BACKGROUND: Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction...

OBJECTIVES: Efficacy of dexmedetomidine (DEX) as a cardioprotective agent in Indonesian children undergoing classic tetralogy of Fallot (TOF) repair with cardiopulmonary bypass (CPB). DESIGN: A prospective, parallel trial using block randomization along with double-blinded preparation of treatment agents by other parties. SETTING: National Cardiovascular Center Harapan Kita, Indonesia. PARTICIPANTS: Sixty-six children with classic TOF scheduled for corrective surgery...

Computed tomography (CT) has emerged as a leading imaging modality in the evaluation of congenital heart disease (CHD). With ever-faster acquisition speed, decreasing radiation exposure, impeccable anatomic detail, optional functional data, and numerous post-processing tools, CT offers broad utility in CHD diagnosis, preoperative planning, and postoperative assessment. In this article, the far-reaching role of CT in CHD is reviewed, focusing on technical imaging considerations and key clinical applications.

We describe a rare and extremely challenging case of transcatheter pulmonary valve implantation in repaired tetralogy of Fallot and anomalous origin of the left main coronary artery from the right coronary sinus. Procedural planning based on advanced multimodality imaging and 3-dimensional technology proved to be the key to procedural success.

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs...

Despite significant improvements in techniques, the treatment of neonates and infants with congenital heart disease resulting in duct-dependent pulmonary circulation is still significantly challenging. Despite current trends toward early primary surgical repair, temporary palliation is still necessary in those patients who are at high surgical risk for complete correction due to unfavorable clinical or anatomic characteristics. Recent advances in interventional cardiology have led to the emergence of right ventricular outflow tract and arterial duct stenting as cost-effective alternatives to surgical palliation in high-risk surgical candidates or whenever short-term pulmonary blood flow support is anticipated...

INTRODUCTION: Brain abscesses are rare but potentially fatal condition and can be associated with cyanotic congenital heart disease of which 5-18.7% of these patients that develop cerebral abscess commonly have tetralogy of Fallot (TOF). CASE PRESENTATION: We report a case of 3-year-old Muganda male that presented with convulsions, cyanosis and difficulty in breathing. The patient had a combination intervention of medical treatment and surgical drainage of the abscess...

AIMS: Slow conduction (SC) anatomical isthmuses (AIs) are the dominant substrate for monomorphic ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTF). This study aimed to evaluate the utility of automated propagational analysis for the identification of SC-AI in patients with rTF. METHODS AND RESULTS: Consecutive rTF patients undergoing VT substrate characterization were included. Automated isochronal late activation maps (ILAM) were obtained with multielectrode HD Grid Catheter...

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• Residual MAPCAs result in post-tricuspid shunt and PH. • Distinction between PH and conduit stenosis requires attention to TTE findings. • Management of complex adult CHD should be reserved for centers with expertise. • A multimodal imaging approach is often required to complete this understanding.

• TOF with PA hypoplasia can be diagnosed in adult dogs. • MAPCAs were suspected on the basis of TTE detection of collateral vessels. • Acyanosis in this dog was attributed to multiple sources of pulmonary blood flow. • Augmented reality can provide unique imaging capabilities for complex heart defects.

A case of a newborn with tetralogy of Fallot, corpus callosum hypoplasia, and phenotypic features similar to DiGeorge syndrome. Chromosomal microarray analysis did not reveal any alterations. Whole exome sequencing and Sanger sequencing identified a de novo variant in the HIRA gene resulting in the loss of the start codon.

BACKGROUND: Congenital heart disease poses a therapeutic challenge, specifically pulmonary valve stenosis. This has been treated for many years with invasive procedures and bioprostheses, which over time, become dysfunctional due to the accumulation of fibrous tissue and calcification. OBJECTIVE: The aim of this study is to describe the use of endovascular management in the right ventricular outflow tract, as the beginning of an ongoing effot to improve pediatric outcomes in developing countries...

Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for cardiovascular morbidity and mortality. The aim of this study was to evaluate whether body mass index (BMI) is associated with health-related quality of life (HRQoL) in patients with variants of Tetralogy of Fallot (TOF). Patients and parents of children with variants of TOF-CHD were asked to fill out the PedsQL 4.0 questionnaire and provide weight and length. Patients were categorized into low, normal, and high BMI percentiles...

INTRODUCTION: Chest x-rays are widely used for diagnosing chest pathology worldwide. Pediatricians frequently interpret chest radiographs in the emergency department, guiding patient management. This study aims to assess the competency of non-radiologists in interpreting emergency chest x-rays and compare it with trainees of different levels to determine the necessity of radiologist input. METHODOLOGY: A cross-sectional online survey was conducted in Saudi Arabia from September to October 2023, involving 385 participants, including pediatricians and medical interns from various regions...

BACKGROUND: 4D flow magnetic resonance imaging (4D flow MRI) can assess and measure the complex flow patterns of the right ventricle (RV) in congenital heart diseases, but its limited availability makes the broad application of intracardiac flow assessment challenging. Color Doppler imaging velocity reconstruction from conventional echocardiography is an emerging alternative, but its validity against 4D flow MRI needs to be established. OBJECTIVE: To compare intracardiac flow parameters measured by color Doppler velocity reconstruction (DoVeR) against parameters measured from 4D flow MRI...

BACKGROUND: Junctional ectopic tachycardia (JET) complicates congenital heart surgery in 2-8.3% of cases. JET is associated with postoperative morbidity in single-center studies. We utilized the Pediatric Cardiac Critical Care Consortium data registry for the first multicenter epidemiologic description of treated JET. METHODS: This is a retrospective study (2/2019 - 8/2022) of patients with treated JET. INCLUSION: 1) <12 months old at index operation; 2) treated for JET <72 hours after surgery...

While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic variability of QAVs in children (median age at diagnosis 4.3 y IQR 0-12 y). Associated congenital heart diseases were present in 54% (most commonly tetralogy of Fallot (TOF) and valvar pulmonary stenosis). Among patients with isolated QAV, 11 had genetic syndrome and 5 had skeletal anomalies...

BACKGROUND: Transthoracic Echocardiography is the first-line, non-invasive, and accessible imaging modality to evaluate heart disease anatomy, physiology, and hemodynamics. We aim to describe the trans-thoracic echocardiography pattern of pediatric heart diseases and reasons for referral in children referred to Bahir Dar University Tibebe-Ghion Hospital and Adinas General Hospital. METHOD: A descriptive cross-sectional study of the archived Transthoracic, Two Dimensional, and Doppler Echocardiography assessments of children from birth to fifteen years of age performed between June 2019 to May 2023 was done...

INTRODUCTION AND IMPORTANCE: Raghib syndrome is a rare congenital complication consisting of the termination of the left superior vena cava (LSVC) in the left atrium, an unroofed coronary sinus, and an atrial septal defect most often found in the posterior-inferior angle of the atrial septum. Both a right-to-left and a left-to-right intracardiac shunt exist. In most circumstances, they do not show any symptoms. PRESENTATION OF CASE: The patient presented with a persistent left superior vena cava draining into the left atrium, an unroofed coronary sinus, and a secondary atrial septal defect (ASD)...