Interrupted aortic arch

Reversed flow in the basilar artery can be acquired or congenital. Acquired reversed flow in the basilar artery can result from acute thrombosis of the basilar artery or retrograde vertebral artery flow. Congenital continuous retrograde basilar artery flow has not been described. We report a 2-day-old male presenting with hypocalcemic seizures which led us to obtain a Duplex echoencephalogram. An echocardiogram was subsequently ordered. In the coronal plane through the anterior fontanelle, retrograde flow was seen in the basilar artery and the right vertebral artery...

Heterotaxy syndrome (HS) occurs in developing embryos due to an inability to establish the normal anatomy, which manifests as abnormal symmetry and malposition of the thoracoabdominal viscera and vasculature, including cardiac and extracardiac anomalies. It is classified as right or left atrial isomerism. This classification depends on the atrial appendage morphology and the extracardiac defect associated with it. Right isomerism usually presents with right atrial appendages (RAA), asplenia, total anomalous pulmonary venous return, and severe pulmonary stenosis...

Aberrant subclavian artery (ASCA) is frequently observed in interrupted aortic arch (IAA) with aortic/subaortic obstruction. Developmental significance of ASCA in IAA in utero remains elusive. Newborns with prenatally diagnosed isolated IAA under continuous prostaglandin E1 infusion were studied. Cross-sectional areas of aortic valve opening (AVOCSA ) and patent ductus arteriosus (PDACSA ) were represented by echocardiographic measurement of (diameter)2 indexed by body surface area (m2 ). Types of IAA and presence of ASCA were examined in relation to sizes of AVOCSA and PDACSA ...

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions...

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other...

BACKGROUND: Prenatal diagnosis of Berry syndrome, a rare combination of cardiac anomalies including aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), interrupted aortic arch (IAA), hypoplastic aortic arch, or coarctation of the aorta (COA), poses a significant challenge. Due to the rarity of the disease, and the limited case reports available to features the complex malformation of Berry syndrome postpartum, this article introduces an innovative approach to visually showcase this unusual disease...

Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions...

INTRODUCTION AND OBJECTIVES: In contrast to developed countries, cardiac CT is not widely used in West Africa for the assessment of congenital heart disease, and has only recently been introduced in Côte d'Ivoire. The lack of data prompted this study, the aim of which was to describe our experience of the contribution of CT to the management of congenital heart disease in the Ivorian cardiology setting. PATIENTS AND METHOD: This was a prospective study which took place in the pediatric cardiology department over a period of 9 months (September 2022 to June 2023) which included all patients with congenital heart disease explored by echocardiography and cardiac scan...

RATIONALE: The resiliency of embryonic development to genetic and environmental perturbations has been long appreciated; however, little is known about the mechanisms underlying the robustness of developmental processes. Aberrations resulting in neonatal lethality are exemplified by congenital heart disease (CHD) arising from defective morphogenesis of pharyngeal arch arteries (PAA) and their derivatives. OBJECTIVE: To uncover mechanisms underlying the robustness of PAA morphogenesis...

BACKGROUND: Interrupted aortic arch (IAA) is associated with left ventricular outflow tract obstruction (LVOTO) and DiGeorge syndrome. High-risk infantile surgery is required to address IAA, with limited data available on long-term outcomes. We used the Pediatric Cardiac Care Consortium, a multicenter US-based registry for pediatric cardiac interventions, to assess long-term outcomes after IAA repair by patient characteristics and surgical approach. METHODS: This is a retrospective cohort study of patients undergoing IAA repair between 1982 and 2003...

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BACKGROUND: The prevalence and risk factors for failure to thrive (FTT) in pediatric patients with congenital heart disease (CHD) remain ambiguous. We aimed to investigate the prevalence, growth profiles, risk factors, and vulnerable subtypes of CHD associated with FTT in pediatric patients with CHD. METHODS: This was a cross-sectional study based on Chinese Database for Congenital Heart Surgery. FTT was defined as either stunting or underweight (height or weight standard deviation score <-2), and they were standardized by references of normal Chinese population...

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated...

Truncal valve regurgitation and interrupted aortic arch increase the need for reintervention and mortality rates in neonates undergoing truncus arteriosus repair. This featured case report presents a surgical video demonstrating the tricuspidization of a quadricuspid truncal valve during repair of truncus arteriosus and interrupted aortic arch in a 9-day-old girl. The patient had a quadricuspid and dysplastic truncal valve with moderate to severe regurgitation. Tricuspidization was successfully performed. The Institutional Review Board committee of Children's National approved the study protocol and publication of data (RB Number: Pro00015566, Initial Approval: 7/1/2021)...

BACKGROUND: Previously published studies suggest that genetic or environmental causes can be observed in 20-30% of congenital heart disease (CHD) patients, which include aneuploidy, single gene defects, pathological copy number variations, and de novo autosomal dominant and recessive inheritance. Moreover, the genetic background of childhood cardiomyopathies (CMs) has not been elucidated well. OBJECTIVE: The study highlights the value of genetic assessment in diagnosing and family counseling for CHD and pediatric CM patients referred to the genetic clinic in a pediatric cardiology department...

OBJECTIVE: Total aortic arch replacement (TAR) necessitates hypothermic circulatory arrest (CA). The frozen elephant trunk technique (FET) additionally requires commercial hybrid grafts. Herein we describe a novel modified FET technique without CA using standard grafts thanks to left axillary artery (LAxA) cannulation in patients with acute type A aortic dissection. METHODS: LAxA anastomosis is made first using a homemade debranching graft, and cardiopulmonary bypass is initiated, followed by anastomoses of left common carotid and innominate arteries...

Interrupted descending aorta (IDA) is an extremely rare congenital heart defect characterized by a complete loss of connection between the ascending and descending aorta. This condition is typically diagnosed in infancy or early childhood, but there have been very few cases reported in adulthood. Here, we present a unique case of an IDA in a 16-year-old patient with concomitant aortic stenosis (AS) and bicuspid aortic valve (BAV), making it an extremely rare scenario. This case highlights the importance of early diagnosis and appropriate management in patients with an IDA, particularly when in association with other cardiovascular abnormalities...

BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and interrupted aortic arch (IAA) is even rarer, with the hallmark characteristics of high peri-operative mortality and postoperative obstruction of the aortic arch, pulmonary artery, and left main bronchus. These complications often need re-interventions. CASE REPORT We present 2 cases with diagnoses of APW and IAA that were treated with single-stage repair...

BACKGROUND: Interrupted aortic arch (IAA) is a rare congenital heart disease characterized by loss of continuity between the ascending and the descending aorta. Prenatal diagnosis of IAA by echocardiography is challenging but nonetheless can be accomplished via a systematization of cardiac fetal evaluation. CASE PRESENTATION: We report a case of fetal IAA type A prenatally diagnosed through two-dimensional echocardiography using both a three vessel-trachea view and a sagittal view...

UNLABELLED: Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. We report a case series of adult forms of IAA. Even if the incidence is very rare, IAA should be could excluded in the setting of resistant asymmetrical hypertension. The first case is singular regarding its association with a bicuspid aortic valve and aortic aneurysm, while the second case is characterized by massive left ventricular hypertrophy...