Truncus arteriosus

INTRODUCTION: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. METHODS: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention...

BACKGROUND: Truncus arteriosus repair is associated with higher morbidity and mortality compared with many other congenital heart operations. We sought to determine factors associated with mortality and adverse outcomes in infants undergoing truncus arteriosus repair. METHODS: We used the Pediatric Health Information System Database to identify infants aged < 90 days who underwent truncus arteriosus repair from 2004 to 2019. The primary outcome was hospital mortality...

Data regarding the effect of significant TVI on outcomes after truncus arteriosus (TA) repair are limited. The aim of this meta-analysis was to summarize outcomes among patients aged ≤ 24 months undergoing TA repair with at least moderate TVI. A systematic literature search was conducted in PubMed, Scopus, and CINAHL Complete from database inception through June 1, 2022. Studies reporting outcomes of TA repair in patients with moderate or greater TVI were included. Studies reporting outcomes only for patients aged > 24 months were excluded...

Type A3 truncus arteriosus describes pulmonary atresia with non-confluent mediastinal pulmonary arteries in which one pulmonary artery arises from a patent ductus arteriosus and the contralateral pulmonary artery from the aorta resulting in ductal dependent pulmonary blood flow. We describe a premature neonate with caudal regression syndrome and type A3 truncus arteriosus who was palliated with a ductal stent allowing completion of a prolonged neonatal ICU hospitalisation for multiple comorbidities.

OBJECTIVE: To identify the late surgical outcomes of truncus arteriosus. METHODS: Fifty consecutive patients with truncus arteriosus who underwent surgery between 1978 and 2020 at our institute were enrolled in this retrospective, single institutional cohort study. The primary outcome was death and reoperation. The secondary outcome was late clinical status, including exercise capacity. The peak oxygen uptake was measured by a ramp-like progressive exercise test on a treadmill...

BACKGROUND: The goal of this study is to describe in-hospital and long-term mortality following single-stage repair of truncus arteriosus communis (TAC) and explore factors associated with these outcomes. METHODS: This is a cohort study of consecutive patients undergoing single-stage TAC repair between 1982 and 2011 reported to the Pediatric Cardiac Care Consortium registry. In-hospital mortality was obtained for the entire cohort from registry records. Long-term mortality was obtained for patients with available identifiers via matching with the National Death Index through 2020...

BACKGROUND: Bioprosthetic valves are often used for pulmonary valve replacement (PVR) and tricuspid valve replacement (TVR) because of concerns about mechanical valve thrombosis in the right heart. The purpose of this study was to assess prosthetic valve function and outcomes (prosthetic valve dysfunction, reoperations and major bleeding events) after mechanical PVR and TVR and to compare these to bioprostheses implanted in the same positions. METHOD: Case-control study of adults with congenital heart disease that underwent mechanical TVR or PVR (2003-2021) at Mayo Clinic Rochester, Minnesota...

No abstract text is available yet for this article.

The ventricular papillary muscles (VPMs) can be a source of premature ventricular contractions (PVCs). Catheter ablation of VPM PVCs is challenging because of the anatomical complexity, such as the apical structures in proximity to the ventricular walls. The QDOT MICRO catheter (Biosense Webster, Diamond Bar, CA, USA) has microelectrodes embedded along the circumference of its distal tip and can provide information on which side of its tip myocardial activation is earlier. This repaired truncus arteriosus case demonstrates the usefulness of the microelectrode recording in identifying a PVC origin in a right VPM apex close to the right ventricular anterior wall...

OBJECTIVES: We sought to determine the prevalence of and factors associated with gastrostomy tube placement and tracheostomy in infants undergoing truncus arteriosus repair, and associations between these procedures and outcome. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System database. PATIENTS: Infants less than 90 days old who underwent truncus arteriosus repair from 2004 to 2019. INTERVENTIONS: None...

Complex congenital heart defects may necessitate repeated surgical interventions throughout a patient's lifetime. Each subsequent procedure exposes patients to a greater cumulative risk, thus adding to the potential morbidity and mortality of the surgery. Transcatheter interventions can help mitigate the surgical risk for many defects and can delay or mitigate the need for surgery. This case report describes the rare use of a transapically delivered transcatheter aortic valve replacement (TAVR) therapy in a high-risk pediatric patient to postpone the need for surgery and potentially reduce the number of lifelong surgical interventions...

No abstract text is available yet for this article.

BACKGROUND: Congenital heart disease (CHD) represents the most widespread congenital birth defect among neonates worldwide, leading to substantial expenses and contributing significantly to premature death caused by birth defects. Despite the significance of CHD, research on its etiology remains limited and has failed to provide substantial evidence for the molecular basis of the disease. With the advancement of next-generation sequencing (NGS), genetic screening has become increasingly accessible, offering a greater capability for identifying potential genetic variants associated with CHD...

PHACES syndrome is a multiple congenital disorder with unknown etiology that is characterized by Posterior fossa anomalies, Hemangioma, Arterial lesions, Cardiac abnormalities/coarctation of the aorta, Eye anomalies, and Sternal cleft. Compound heterozygous or homozygous TMEM260 variants cause structural heart defects and renal anomalies syndrome (SHDRA). We describe a 10-year-old male patient with a PHACES-like syndrome and TMEM260 compound heterozygous variants who demonstrated overlapping phenotypes between the two syndromes...

We report a fetus with hydrops, congenital heart disease and bilateral radioulnar synostosis caused by a novel pathogenic MECOM variant. The female fetus was referred for post-mortem examination after fetal hydrops and intrauterine death was diagnosed at 20 weeks gestation. Post-mortem examination confirmed fetal hydrops, pallor, truncus arteriosus and bilateral radioulnar synostosis. Trio whole genome sequencing analysis detected a novel de novo heterozygous pathogenic loss-of-function variant in MECOM (NM_004991), associated with a diagnosis of Radioulnar Synostosis with Amegakaryocytic Thrombocytopenia 2 (RUSAT-2)...

We describe a neonate with a unique variant of truncus arteriosus with interrupted aortic arch, an absent ascending aorta, persistent right dorsal aorta, and an unusual brachiocephalic artery pattern in which all head and neck vessels were supplied from the ductal arch-descending aorta continuum. ( Level of Difficulty: Beginner. ).

OBJECTIVES: We aimed to investigate the utility of repeat fetal echocardiograms (FE) after a diagnosis of structural congenital heart disease (CHD) was made on the initial FE. We evaluated how often changes in management and counseling occurred based on subsequent FE findings and sought to determine which types of CHD were more likely to have changes in management and/or counseling based on repeat FE. METHODS: We performed a retrospective review of all patients who presented to our center between January 2012 to January 2019 and had more than one FE performed for structural CHD...

Congenital anomalies of the coronary arteries are rare. In this article, we reported a case of 6-month-old female who was brought with breathing difficulty and poor weight gain. Echocardiography was done which showed dextrocardia and truncus arteriosus. A cardiac computed tomography confirmed these findings and additionally revealed that the coronary arteries originated from a common vascular channel from the brachiocephalic artery. This is a very rare occurrence. To our knowledge, only a few such cases have been reported in the literature...

No abstract text is available yet for this article.

BACKGROUND: Truncus arteriosus (TA) or persistent arterial trunk describes the presence of a solitary arterial trunk arising from the base of the heart, supported by a common ventriculoarterial junction. The trunk gives rise to the coronary arteries, systemic arteries, and at least one pulmonary artery. Truncus arteriosus is a rare congenital cardiac disease, and even rarer is the absence of ventricular septal defect. CASE SUMMARY: We report a case of a 2-day-old infant who presented with cyanosis and a cardiac murmur...