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      Accessory mitral valve tissue causing severe left ventricular outflow tract obstruction in a post-Senning patient with transposition of the great arteries

      case-report

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          Abstract

          Accessory mitral valve tissue is a rare congenital anomaly associated with congenital cardiac defects and is usually detected in the first decade of life. We describe the case of an 18-year old post-Senning asymptomatic patient who was found to have accessory mitral valve tissue on transthoracic echocardiography producing severe left ventricular outflow tract obstruction.

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          Accessory mitral valve in an adult population: the role of echocardiography in diagnosis and management.

          S Thanigaraj, Aleksandr Rovner, E. Perez Codina (2005)
          Accessory mitral valve is a rare congenital abnormality and is an unusual cause for subvalvular left ventricular outflow tract (LVOT) obstruction. It is detected first in children and is very rarely noticed in adults. The most common clinical presentation is symptomatic LVOT obstruction. We present a case series of 5 adult patients with varying clinical presentations in which the accessory mitral valve was diagnosed using echocardiography. Three patients presented with varying degrees of symptomatic LVOT obstruction, one presented with recurrent transient ischemic attack and stroke, and one patient was incidentally diagnosed during echocardiography to exclude endocarditis. Accessory mitral valve should be suggested in patients with LVOT obstruction.
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            Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.

            Jean Rubay, Jose Gomez, W Daenen … (2010)
            Optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow obstruction (LVOTO) remains controversial. Although the Rastelli operation has been the most widely performed surgical procedure during the past decades, several studies have shown its suboptimal long-term prognosis. Other operations have been developed to improve results. This study was performed to compare the outcomes of the different surgical approaches for patients with TGA, VSD and LVOTO, as well as to determine risk factors for mortality and re-intervention. Records from 146 patients undergoing surgery from 1980 to 2008 from eight European hospitals were reviewed. Median age at operation was 21.5 months (range 0.2-165.1 months), and median weight was 10.0 kg (range 2.0-41.0 kg). Surgical procedures involved were the Rastelli procedure (82), arterial (24) and atrial (5) switch operation with relief of LVOTO, Réparation à l'Etage ventriculaire (REV) procedure (7) and the Metras modification (24), as well as the Nikaidoh procedure (4). The overall survival was 88%, 88% and 58% at 1, 10 and 20 years, respectively. The overall event-free survival was 80%, 45% and 26% at 1, 10 and 20 years, respectively. The REV procedure and the Metras modification were found to have the best long-term results in both survival and event-free survival rates. Multivariate analysis revealed year of operation, non-commitment of the VSD and prolonged cardiopulmonary bypass (CPB) time as risk factors for mortality while age at surgery, year of operation and type of corrective surgery were risk factors for re-intervention. Different surgical approaches have been developed for patients with TGA, VSD and LVOTO. The REV procedure and the Metras modification were observed to have favourable long-term results in survival and event-free survival rates. Aortic translocation techniques such as the Nikaidoh procedure seem promising, but further studies will be needed to confirm this in the long term. Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
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              The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study.

              Margot M. Bartelings, Mark G. Hazekamp, Francisco Portela (2007)
              To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Heart Int
                Journal ID (pmc): HI
                Journal ID (publisher-id): HI
                Title: Heart International
                Publisher: PAGEPress Publications (Pavia, Italy )
                ISSN (Print): 1826-1868
                ISSN (Electronic): 2036-2579
                Publication date (Electronic): 21 July 2011
                Publication date Collection: 02 June 2011
                Volume: 6
                Issue: 1
                Electronic Location Identifier: e6
                Affiliations
                [>1 ]Department of Adult Congenital Heart Disease, Royal Hospital, Muscat;
                [2 ]Pediatric Cardiology, Royal Hospital, Muscat, Oman
                Author notes
                Correspondence: Prashanth Panduranga, Department of Cardiology, Royal Hospital, Post Box 1331, Muscat-111, Sultanate of Oman. Tel. +968.92603746 - Fax: +968.24599841. E-mail: prashanthp_69@ 123456yahoo.co.in

                Conflict of interest: the authors report no conflicts of interest.

                Article
                Publisher ID: hi.2011.e6
                DOI: 10.4081/hi.2011.e6
                PMC ID: 3184715
                PubMed ID: 21977306
                SO-VID: 4c9a16cd-73ad-47bf-906b-8fdda31f3700
                Copyright © ©Copyright P. Panduranga et al., 2011
                License:

                This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0).

                Licensee PAGEPress, Italy

                History
                Date received : 07 May 2011
                Date revision received : 09 July 2011
                Date accepted : 15 July 2011
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: left ventricular outflow tract obstruction.,accessory mitral valve tissue
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: left ventricular outflow tract obstruction., accessory mitral valve tissue

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