Ebstein anomaly of the tricuspid valve

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EBSTEINANOMALYOF THE TRIcusPID VALVE IN AN ADULTCOHORT

Mohammed Ali Warsi

A thesis submitted in conforrnity with the requirements for the degree of Masters of Science in the field of Cardiovascular Science,

Graduate Department of Institute of Medical Science, University of Toronto

O Copyright by Mohammed AL Warsi (August, 1999)

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Ebstein Anomaly of the Tricuspid Valve in an Adult Cohort

Mohammed Ali Warsi Masters of Science, 1999 Institute of Medical Science

University of Toronto

Abstract

Objective: We examined the spectrum of aduits with Ebstein anomaly of the tricwpid valve.

Methods: Clinical data, EKG,chest x-rays, echocardiograms and surgical details were reviewed in patients with

Ebstein anomaly at the University of Toronto Congenital Cardiac Center for Adults.

Resuk: Seventy-four patients (27 M. 47 F) were seen at our clinic at a mean age of 3 3 2 1 3 . 6 years and were

followed for a mean of 7.5 t 5.8 years. Seven patients died. Thirty-eight patients (51%) presented with or developed sustained arrhythmia (atriai fluttedfibrillation n = 37,supraventricular tachycardia due to WPW n = 16): 7 of these patients with arrhythmia experienced transient ischemic attacks. Tricuspid valve surgery was undertaken in 24 patients; NYHA class improved in al1 23 survivors. Of the 12 patients that experienced atrial flutter/fibnllation before surgery, arrhythmia persisted in 6. Only 2 of these 6 patients had intraoperative arrhythmia ablation. The other 6 patients undergoing ablative procedures convened to sinus rhythm. Right atrial size (pdl.02) and the presence of atrid septal defect ( p d . 0 2 ) are independent predictors of atnd fiutter/fibriIlation (using Cox regression anaiysis), whereas cardiothoracic ratio (p4.03) is predictive of death. Conclusions: Atrial flutterlfibrillation is common in adults with Ebstein anomiily, and relates to RA size. Surgery for Ebstein anomaly results in symptomatic improvement. Atrial fluttedfibriilation may persist without concomitant intnopentive arrhythmia abIation.

1am moa thankf?l to my supewisor, Dr. Peter Liu, and my advisors, Dr. Michael Gatzoulis, Dr. Samuel Siu and Dr. Peter McLaughlin for their invaluable guidance. 1am also indebted to Dr. Gary Webb, Dr. William Williams, Dr. Louise Harris and Dr. Judith Themen for

their help. X am particularly grateful to Dr. Michael Gatmuiis for beiieving in me and investing so

much time and effort, and turning an idea into a dream, and into reality.

1would also like to thank Dr. Sara Thom, Dr. Vera Rose and Dr. J. Smaiihom for their invaluable comments and suggestions to polish up this thesis.

1wish to express my sincere gratitude and appreciationto:

- The Congenital Cardiac Centre for Adults for al1 their help in booking, retrieving patient

information, meeting patients in clinic, and for their companionship.

- Dr. Naeem Merchant for teaching me everything I know about cardiac MRI and to Nora for the

MRI bookings.

- Cathy Koost and Exercise Lab at the Toronto General Hospital for the exercising patients in the

study, even at very short notice.

- Graham Jencho for his help with the computer server and the congenital database. - Donna and Eleanor for onenting my into the department and their organizationai help.

- Clark Lang for his endless supply of bagels and coffee, and his advice.

This research was supported in part by the Heart and Stroke Foundation of Ontario.

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This thesis is dedicated to Dr. Gatzoulis who gave my academic career a clear direction. This thesis is aiso dedicated to the undergraduate students who are unsure of how much more can

be learned before entering medical school.

'7havert?failed. I've fmnd lO.000 ways that don't work " Sir ~ o m aEsdison

"Thisis not the end It is not even the beginning of the end It is the end of the beg?nning." Wimton Churchill

TABLE OF CONTENTS

ABSTRACT

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ACKNOWLEDGMENTS

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TABLE OF CONTENTS

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LIST OF FIGURES

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LIST O F TABLES

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LIST OF ABBREVIATIONS

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Chapter 1 Introduction: Pathologieal Anatomy o f Ebstein Anomaly

Introduction and Purpose of Study

2

History

5

The Tricuspid Valve in Ebstein Anomaly

7

The Right Ventricle

10

The Right Atrium

11

Interatrial Communication in Association with Ebstein Anomaly

The Right Ventr?cular Outflow Tract and Pulmonary

Art

The Left Ventricle

Other Congenital Malformations

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Chapter 2 Pathophysiology In Ebstein Anomdy

Tncuspid Valve with Regurgitation and Stenosis in Ebstein

Anomal y

16

Right Ventricular Dysfunction

18

Right Atrium Dilatation

20

Left Ventricular Dysf?nction

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