Type 2 persistent fifth aortic arch: An elusive entity diagnosed on computed tomography angiography

A 2-month-old male child presented with central cyanosis, poor feeding and failure to thrive. Chest radiograph revealed mild cardiomegaly with right ventricular configuration and right sided aortic arch. Echocardiography demonstrated presence of non-committed VSD with pulmonary atresia. Aorta was seen arising from right ventricle. CT angiography confirmed the presence of VSD with pulmonary atresia. The aortic arch was right sided with mirror image branching of the arch vessels. The pulmonary arteries were non-confluent with the right pulmonary artery reformed via a patent arterial duct.
Source: Journal of Cardiovascular Computed Tomography - Category: Radiology Authors: Source Type: research

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Conclusion: Most infants with BA have liver fibrosis at diagnosis and it progresses rapidly. The presence of cirrhosis is correlated with the age at surgery, which suggests the importance of this clinical variable in the evolution of fibrosis.RESUMEN Objetivo: Analizar la evoluci ón de los hallazgos histológicos de pacientes con atresia de vías biliares (AVB), resaltando la progresión de la fibrosis y comparando la biopsia hepática diagnóstica (BHD) con la biopsia hepática quirúrgica (BHQ) mediante la hepatoportoenterostomía de Kasai. Método: Estudio ret...
Source: Jornal Brasileiro de Patologia e Medicina Laboratorial - Category: Pathology Source Type: research
Authors: Brązert M, Kranc W, Celichowski P, Ożegowska K, Budna-Tukan J, Jeseta M, Pawelczyk L, Bruska M, Zabel M, Nowicki M, Kempisty B Abstract Under physiological conditions, human ovarian granulosa cells (GCs), are responsible for a number of processes associated with folliculogenesis and oogenesis. The primary functions of GCs in the individual phases of follicle growth are: Hormone production in response to follicle stimulating hormone (FSH), induction of ovarian follicle atresia through specific molecular markers and production of nexus cellular connections for communication with the oocyte. In recent ...
Source: Molecular Medicine Reports - Category: Molecular Biology Tags: Mol Med Rep Source Type: research
Publication date: October 2019Source: Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms, Volume 1862, Issue 10Author(s): Tianya Guo, Jinbi Zhang, Wang Yao, Xing Du, QiQi Li, Long Huang, Menglan Ma, Qifa Li, Honglin Liu, Zengxiang PanAbstractMammalian ovarian follicular atresia is a complex and fine-regulated biological process with active involvement of connective tissue growth factor (CTGF). The emergence of studies of endogenous non-coding RNAs has raised a new aspect for exploration of the regulatory mechanisms involved in follicular atresia. Here, we aimed to illustrate a circRNA involved in the CTGF reg...
Source: Biochimica et Biophysica Acta (BBA) Gene Regulatory Mechanisms - Category: Genetics & Stem Cells Source Type: research
Conclusion Patients undergoing laparoscopic CDO repair at our institution benefited from shorter time to full enteral feeds, and reduced the need for PN as well as postoperative pain medication. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
AbstractObjectiveTracheo-oesophageal fistula (TOF) is a rare pathology. It can be congenital and concurrent with other congenital anomalies such as oesophageal atresia, laryngeal and tracheal agenesis, or it can be acquired. The purpose of this study was to analyse various management strategies and their outcomes in infants and children with TOF and identify potential areas for standardisation of the fistula repair procedures.MethodsAt a single institution, a retrospective analysis of infants and children with congenital or acquired TOF between 2013 and 2019 was performed. Thirteen patients were identified. Data collection...
Source: European Archives of Oto-Rhino-Laryngology - Category: ENT & OMF Source Type: research
We describe a technical modification to approach for dissection of proximal pouch in patients undergoing the primary repair of congenital Esophageal atresia with tracheoesophageal fistula (EA-TEF).
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Operative Techniques Source Type: research
Mutations in the tetratricopeptide repeat domain 7A (TTC7A) gene cause very early onset inflammatory bowel diseases (VOIBD) or multiple intestinal atresia associated with immune deficiency of various severities, ranging from combined immune deficiency to mild lymphopenia. In this manuscript, we report the clinical, biological and molecular features of a patient born from consanguineous parents, presenting with recurrent lymphoproliferative syndrome and pan-hypergammaglobulinemia associated with chronic intestinal pseudo obstruction (CIPO). Genetic screening revealed the novel c.974G>A (p.R325Q) mutation in homozygosity ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Publication date: Available online 11 September 2019Source: JACC: Heart FailureAuthor(s): Karen Sliwa, Lucia Baris, Christoph Sinning, Elvin Zengin-Sahm, Lina Gumbiene, Israa F. Yaseen, Ghada Youssef, Mark Johnson, Hasan Al-Farhan, Malgorzata Lelonek, Roger Hall, Jolien Roos-HesselinkAbstractObjectivesThe purpose of this work was to study maternal and fetal outcomes of women with uncorrected congenital heart disease (CHD).BackgroundGlobally, CHD is an important cause of maternal morbidity and mortality in women reaching reproductive stage. Data are lacking from larger cohorts of women with uncorrected CHD.MethodsThe 10-yea...
Source: JACC: Heart Failure - Category: Cardiology Source Type: research
Cystic Fibrosis (CF) is the most frequent life-limiting autosomal disease in the Caucasian population. It is a channelopathy caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, which acts as a cAMP chloride (Cl −) channel after activation by protein kinase A (PKA). The typical phenotype includes chronic sinopulmonary disease, pancreatic exocrine insufficiency, salt loss syndrome and congenital bilateral atresia of the vas deferens [1]. The diagnosis is based on a positive newborn screen test, characteris tic phenotype, and/or a family history of CF in conjunction with a...
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
ConclusionsReoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
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