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Congenital Aortic Stenosis in Adults

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Congenital Aortic Stenosis in Adults Congenital Aortic Stenosis in Adults Update on clinical outcome, diagnostic methods and pregnancy 1 Colofon Yap, SC Congenital aortic stenosis in adults - Update on clinical outcome, diagnostic methods and pregnancy. ISBN: 978-90-8559-330-0 © Copyright 2007 S.C.Yap All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronical, mechanical, photocopying, recording, or otherwise, without prior written permission of the holder of the copyright. Several chapters of this thesis are based on published papers, which are reproduced with permission of the co-authors and the publishers. Copyright of these papers remains with the publishers. Cover: Véro Crickx, Sirene ontwerpers, Rotterdam, The Netherlands Lay-out: S.C.Yap Printed by Optima Grafische Communicatie, Rotterdam, The Netherlands Financial support by the Netherlands Heart Foundation for the publication of this thesis is gratefully acknowledged. 2 Congenital Aortic Stenosis in Adults Update on clinical outcome, diagnostic methods and pregnancy Congenitale aortastenose in volwassenen Nieuwe inzichten in klinische uitkomsten, diagnostische methoden en zwangerschap Proefschrift ter verkrijging van de graad van doctor aan de Erasmus Universiteit Rotterdam op gezag van de rector magnificus Prof.dr. S.W.J. Lamberts en volgens besluit van het College voor Promoties. De openbare verdediging zal plaatsvinden op woensdag 19 december 2007 om 9.45 uur door Sing-Chien Yap geboren te ’s-Hertogenbosch 3 Promotiecommissie Promotor: Prof.dr. M.L. Simoons Copromotor: Dr. J.W. Roos-Hesselink Overige leden: Prof.dr. A.J.J.C. Bogers Prof.dr. B.M.J. Mulder Prof.dr. W.A. Helbing 4 Contents Chapter 1. General introduction 9 Part 1. Clinical outcome of congenital aortic stenosis Chapter 2. Aortic stenosis at young adult age 18 Expert Rev Cardiovasc Ther 2005;3:1087-98 Chapter 3 Congenital aortic stenosis in adults: rate of progression and 42 predictors of clinical outcome Int J Cardiol 2007 Feb 7; [Epub ahead of print] Chapter 4 When to intervene in aortic ectasia 56 Focused review. Braunwald’s Heart Disease: A textbook of cardiovascular medicine Chapter 5 Outcome of implantable cardioverter defibrillators in adults with 66 congenital heart disease: a multi-centre study Eur Heart J 2007;28:1854-61 Part 2. Diagnostic methods for congenital aortic stenosis Chapter 6 Steepened aortoseptal angle may be a risk factor for discrete 86 subaortic stenosis in adult patients Int J Cardiol 2007 April 13; [Epub ahead of print] Chapter 7 Abnormal aortic elastic properties in adults with congenital 92 valvular aortic stenosis Int J Cardiol 2007 Aug 7; [Epub ahead of print] Chapter 8 Rapid and accurate measurement of LV mass by RT3DE 104 in patients with concentric LV hypertrophy: comparison to CMR Eur J Echocard 2007 Jun 29; [Epub ahead of print] Chapter 9 A simplified continuity equation approach to the quantification of 118 stenotic bicuspid aortic valves using velocity-encoded CMR J Cardiovasc Magn Reson 2007 (in press) 5 Part 3. Pregnancy and congenital aortic stenosis Chapter 10 Complications during pregnancy in women with congenital heart 136 disease: a literature review JACC 2007;49:2303-11 Chapter 11 Risk of complications during pregnancy in women with congenital 154 aortic stenosis Int J Cardiol 2007 May 3; [Epub ahead of print] Chapter 12 Outcome of pregnancy in women after pulmonary autograft valve 168 replacement for congenital aortic valve disease J Heart Valve Dis 2007;16:398-403 Chapter 13 Percutaneous triple-valve balloon valvuloplasty in a pregnant 180 woman using intracardiac echocardiography: case report J Heart Valve Dis 2006;15:459-64 Chapter 14 General discussion, conclusions and future directions 193 Chapter 15 Samenvatting 207 Chapter 16 Dankwoord 215 List of publications 218 Curriculum vitae 221 6 C H A P T E 1 R Congenital aortic stenosis in adults General introduction 7 Chapter 1 8 General introduction INTRODUCTION Congenital aortic stenosis (AS) encompass a series of stenotic lesions starting in the anatomic left ventricular outflow tract (LVOT) and stretches toward the ascending aorta. Obstruction may be subvalvar, valvar, or supravalvar. All of these lesions impose an increased afterload on the left ventricle (LV), which can result in LV hypertrophy, dilatation and eventually heart failure, when left untreated. Congenital valvar AS is usually the result of a stenotic bicuspid aortic valve (BAV). 1,2 Variants range from a nearly trileaflet bicommissural valve with mild cusp inequality to a unicuspid unicommissural valve. Recently, BAV has been the focus of much research as it identifies patients at risk of development of stenosis and regurgitation, and in addition predisposes patients to ascending aorta dilatation and dissection. BAV is the most common congenital malformations and has a prevalence of 1-2% in the general population. 3,4 It results from fusion of one of the commissures (usually present as a raphe), 5 resulting in two rather than three valve leaflets. No clear etiology has been defined for BAV, and no specific gene has been identified. Recent findings support the suggestion that all anomalies of the left ventricular outflow tract obstruction spectrum are developmentally related and multiple genes have been implicated. Experimental evidence suggests that the expression of endothelial nitric oxide synthase (eNOS) may have an influence on aortic valve anatomy and aneurysmal dilatation of the aorta. 6,7 Mutations in the signaling and transcriptional regulator NOTCH1 gene result in developmental aortic valve abnormalities and severe valve calcification in affected families.8 Ubiquitin fusion degradation 1-like gene is another potential candidate, which is highly expressed in the cardiac outflow tract during embryogenesis and is downregulated in patients with BAV. 9 Furthermore, familial clustering of BAV and other left ventricular outflow tract obstructions has been described. 10,11 PROGNOSIS AND TREATMENT Patients with congenital AS with severe symptoms in infancy and childhood have a poor prognosis without intervention. 12 Sudden cardiac death may occur, especially in the setting of physical acitivity and exertion. 13 Even after surgical valvotomy the incidence of sudden death is still 0.4% per year. 14 A bicuspid valve may function normally throughout a lifetime, but usually is associated with the development of either progressive stenosis or regurgitation. 15,16 BAV accounts for approximately 50% of all aortic valve replacements of isolated AS in adults. 1 The main indication for aortic valve replacement is the presence of symptoms (i.e., angina, syncope, congestive heart failure) as this is associated with a worse outcome (i.e., overall cardiac mortality and sudden cardiac death). 17-19 Ballloon valvotomy may be an 9 Chapter 1 attractive option in children, adolescents and young adults who have pliable, noncalcified valves with fusion of the commissures, at the cost of restenosis or regurgitation. 20,21 The Ross procedure (pulmonary autograft) is the treatment of choice in the pediatric population due to its growth potential, but the high reoperation rate and progressive autograft dilatation renders it unsuitable in adults. 22 AORTIC DILATATION There is a high incidence of aortic disease in patients with congenital aortic valve malformations suggesting a causal relationship between these two conditions. Controversy exists regarding the etiology of aortic dilatation in BAV patients. Once thought to be the consequence of post-stenotic dilatation, evidence is accumulating that intrinsic aortic medial weakness should be regarded as the underlying cause of aortic dilatation. Several findings support this hypothesis. Approximately 50% of patients with normally functioning BAV have an aortic dilatation, thus independent of the presence of stenosis or regurgitation.23-26 Also after aortic valve replacement aortic dilatation may occur. Furthermore, the histology of the ascending aortic wall is similar to that of the Marfan syndrome. 27 Medial disease is present, as are varying degrees of abnormalities of the smooth muscle, extracellular matrix, elastin, and collagen. 27-29 Dilatation of the aorta may be the result of the disruption of the extracellular matrix by upregulation of matrix metalloproteinase-2 that is triggered by an inherent deficiency of fibrillin-1. 28,30-32 Furthermore, premature smooth muscle cell apoptosis leads to upregulation of matrix metalloproteinase-2. 33,34 PREGNANCY Cardiovascular physiology changes profoundly during pregnancy. Cardiac output increases 30-50% due to increases in both stroke volume and heart rate. 35,36 During labour, cardiac output increases further due to pain and uterine contractions. 37,38 The haemodynamic impact of AS is aggravated by the physiological changes during pregnancy. Pregnancy in AS patients has been the focus of some reports because of concern for development of heart failure and mortality during pregnancy. 39,40 The review of Lao et al . published in 1993 demonstrated seven deaths among 65 women, resulting in a maternal mortality rate of 11% 41 . Earlier diagnosis and treatment of patients with severe stenosis (e.g. balloon aortic valvotomy) has resulted in more women with congenital AS reaching childbearing age in relatively good condition. Recent pregnancy reports in AS patients are encouraging, showing a favorable pregnancy outcome with low maternal mortality. 42-44 10 General introduction AIMS AND OUTLINE OF THE THESIS The
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