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      A ventricular septal defect restricted by the tricuspid septal leaflet and discrete subaortic membrane presenting with high-grade atrioventricular block and syncope

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      , M.D.
      Turkish Journal of Emergency Medicine
      Elsevier

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          Abstract

          A 32-year-old woman presented to emergency department with syncope. Her medical history was unremarkable, and cardiac auscultation revealed a localised, high-frequency, 3/6 pansystolic murmur at the 3rd and 4th left intercostal spaces. The 12-lead electrocardiogram showed high-grade atrioventricular block with a rate of 46 bpm (Fig. 1, Panel A). Transthoracic echocardiography revealed normal left ventricular systolic functions with an ejection fraction of 63%, mild-to-moderate mitral regurgitation, mild tricuspid regurgitation with a pulmonary systolic pressure of 30 mmHg and normal right ventricular chamber size and systolic functions. In the apical four-chamber view, a 20-mm sized membranous ventricular septal defect (VSD) was seen in the baseline portion of the interventricular septum and left-to- right shunt was observed in color and continuous wave Doppler echocardiography (Fig. 1, Panel B–D). The VSD and left-to-right shunt were restricted by the tricuspid septal leaflet and the patient's Qp/Qs ratio was <1.5 (Fig. 1, Panel E). Additionally, a discrete subaortic membrane between baseline portion of the interventricular septum and mitral anterior leaflet was observed in the apical four-chamber view without left ventricular outflow tract gradient (Fig. 1, Panel F). Ventricular septal defect and discrete subaortic membrane diagnoses were confirmed by cardiac magnetic resonance imaging (Fig. 2, Panel A–D). Following refusal of cardiac pace-maker implantation, the patient was discharged with a program of intensive follow-up. Fig. 1 The 12-lead electrocardiogram reveals high-grade atrioventricular block (Panel A), transthoracic echocardiography apical four-chamber view reveals a 20-mm sized membranous ventricular septal defect (Panel B, arrow), left-to- right shunt was observed in color (Panel C) and continuous wave Doppler echocardiography with a gradient of 103 mmHg (Panel D). Transthoracic echocardiography apical four-chamber view reveals a restriction of ventricular septal defect and left-to- right shunt by the tricuspid septal leaflet (Panel E, arrow) and a discrete subaortic membrane between baseline portion of the interventricular septum and mitral anterior leaflet (Panel F, arrow) (LA: left atrium, LV: left ventricle, RA: right atrium, RV: right ventricle, IVS: interventricular septum, MV: mitral valve, TV: tricuspid valve). Fig. 1 Fig. 2 Cardiac magnetic resonance imaging shows ventricular septal defect and discrete subaortic membrane (arrows) (LA: left atrium, LV: left ventricle, RA: right atrium, RV: right ventricle, IVS: interventricular septum, TV: tricuspid valve). Fig. 2 Ventricular septal defects may cause rhythm and conduction disturbances such as high-grade atrioventricular block. Cases with membranous ventricular septal defect and atrioventricular block become symptomatic in the 3rd or 4th decade of life.1, 2, 3 In this case report, we described a 32-year-old woman with ventricular septal defect and discrete subaortic membrane without left ventricular outflow tract obstruction associated with high-grade atrioventricular block and syncope. Funding statement None. Conflict of interest None.

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          Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum.

          An apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.
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            [Aneurysm of membranous ventricular septum associated with complete atrioventricular block].

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              Membranous interventricular septal aneurysm and associated atrioventricular block causing symptoms in a 65-year-old man.

              Membranous interventricular septal aneurysm has been reported to accompany atrioventricular (AV) conduction defects. If it is accompanied by AV block, patients generally become symptomatic before the age of 40.
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                Author and article information

                Contributors
                Journal
                Turk J Emerg Med
                Turk J Emerg Med
                Turkish Journal of Emergency Medicine
                Elsevier
                2452-2473
                27 October 2017
                December 2017
                27 October 2017
                : 17
                : 4
                : 146-147
                Affiliations
                [1]Department of Cardiology, Edremit State Hospital, Balikesir, 10300, Turkey
                Article
                S2452-2473(17)30149-8
                10.1016/j.tjem.2017.10.003
                5812915
                8ec9801a-ba1c-4ac7-844d-c943a7d9ab51
                Copyright © 2017 The Emergency Medicine Association of Turkey. Production and hosting by Elsevier B.V. on behalf of the Owner.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 1 July 2017
                : 28 September 2017
                : 18 October 2017
                Categories
                Visual Diagnosis

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