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      Aortopathy associated with congenital heart disease: A current literature review

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          Abstract

          In patients born with congenital heart disease, dilatation of the aorta is a frequent feature at presentation and during follow-up after surgical intervention. This review provides an overview of the pathologies associated with aortopathy, and discusses the current knowledge on pathophysiology, evolution, and treatment guidelines of the aortic disease associated with congenital heart defects.

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          Most cited references94

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          2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.

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            ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).

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              Cardiac chamber formation: development, genes, and evolution.

              Concepts of cardiac development have greatly influenced the description of the formation of the four-chambered vertebrate heart. Traditionally, the embryonic tubular heart is considered to be a composite of serially arranged segments representing adult cardiac compartments. Conversion of such a serial arrangement into the parallel arrangement of the mammalian heart is difficult to understand. Logical integration of the development of the cardiac conduction system into the serial concept has remained puzzling as well. Therefore, the current description needed reconsideration, and we decided to evaluate the essentialities of cardiac design, its evolutionary and embryonic development, and the molecular pathways recruited to make the four-chambered mammalian heart. The three principal notions taken into consideration are as follows. 1) Both the ancestor chordate heart and the embryonic tubular heart of higher vertebrates consist of poorly developed and poorly coupled "pacemaker-like" cardiac muscle cells with the highest pacemaker activity at the venous pole, causing unidirectional peristaltic contraction waves. 2) From this heart tube, ventricular chambers differentiate ventrally and atrial chambers dorsally. The developing chambers display high proliferative activity and consist of structurally well-developed and well-coupled muscle cells with low pacemaker activity, which permits fast conduction of the impulse and efficacious contraction. The forming chambers remain flanked by slowly proliferating pacemaker-like myocardium that is temporally prevented from differentiating into chamber myocardium. 3) The trabecular myocardium proliferates slowly, consists of structurally poorly developed, but well-coupled, cells and contributes to the ventricular conduction system. The atrial and ventricular chambers of the formed heart are activated and interconnected by derivatives of embryonic myocardium. The topographical arrangement of the distinct cardiac muscle cells in the forming heart explains the embryonic electrocardiogram (ECG), does not require the invention of nodes, and allows a logical transition from a peristaltic tubular heart to a synchronously contracting four-chambered heart. This view on the development of cardiac design unfolds fascinating possibilities for future research.
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                Author and article information

                Journal
                Ann Pediatr Cardiol
                Ann Pediatr Cardiol
                APC
                Annals of Pediatric Cardiology
                Medknow Publications & Media Pvt Ltd (India )
                0974-2069
                0974-5149
                Jan-Apr 2015
                : 8
                : 1
                : 25-36
                Affiliations
                [1]Department of Cardiac Surgery, University Hospital Gent, De Pintelaan, Gent, Belgium
                Author notes
                Address for correspondence: Dr. Katrien Francois, Department of Cardiac Surgery, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium. E-mail: katrien.francois@ 123456ugent.be
                Article
                APC-8-25
                10.4103/0974-2069.149515
                4322397
                25684884
                e3bc94c7-aae3-4d4a-9d22-2a4214446c55
                Copyright: © Annals of Pediatric Cardiology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Review Article

                Cardiovascular Medicine
                aortic root,aortopathy,congenital heart disease
                Cardiovascular Medicine
                aortic root, aortopathy, congenital heart disease

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