The Failing Right Heart

Transcription

1 The Failing Right Heart

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3 Kyriakos Anastasiadis Stephen Westaby Polychronis Antonitsis Editors The Failing Right Heart

4 Editors Kyriakos Anastasiadis Department of Cardiothoracic Surgery Aristotle University of Thessaloniki AHEPA University Hospital Thessaloniki Greece Polychronis Antonitsis Department of Cardiothoracic Surgery Aristotle University of Thessaloniki AHEPA University Hospital Thessaloniki Greece Stephen Westaby Department of Cardiac Surgery Oxford University Hospitals NHS Trust Oxford UK Institute of Life Sciences University of Swansea Wales UK ISBN DOI / ISBN (ebook) Library of Congress Control Number: Springer Cham Heidelberg New York Dordrecht London Springer International Publishing Switzerland 2015 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper Springer International Publishing AG Switzerland is part of Springer Science+Business Media (

5 In all things of nature there is something of the marvellous. Aristotle

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7 Foreword I Left heart and right heart failure are terms referring to conditions in which the left or right side of the heart, respectively, is primarily impaired. The stroke volume or the output of each side of the heart, however, cannot be different in the absence of an abnormal shunt, since both sides of the heart are in circuit. Thus, the output of one abnormal ventricle should be similar to the output of the other ventricle even if it is totally normal. In addition, one side of the heart affects the other side by the left heart right heart interrelationship and interactions mostly via the intra-atrial septum or intraventricular septum. Despite these close anatomical and functional associations between the left and right heart, the right heart until recently has received much less attention compared to the left heart [1]. The English physician William Harvey was the first known to describe the systemic circulation in the classic book De Motu Cordis, known as On the Motion of the Heart and Blood, in 1628; he thought that the purpose of the right ventricle was to transmit blood to the lungs. Thus, the right ventricle, may be said to be made for the sake of transmitting blood through the lungs, not to nourishing them [2]. Three hundred years later in 1931, Paul Dudley White suggested that pressure load might affect the right ventricle. Comparable to the effect of systemic hypertension on the left ventricle is that of pulmonary hypertension on the right ventricle [3]. At that time, the most common cause of pulmonary hypertension and subsequent right heart failure was mitral stenosis of rheumatic etiology. Other causes of right heart failure (besides mitral stenosis, which is extremely rare today in developed counties) include chronic left heart failure regardless of the etiology, chronic obstructive pulmonary disease, congenital heart disease, pulmonary emboli, pulmonary hypertension (primary or secondary), and tricuspid and pulmonic valvular disease, especially tricuspid regurgitation. While symptoms and clinical findings due to right heart failure have been extensively described over the last 100 years [4], assessment of right ventricular performance in the daily clinical practice has lagged well behind to that of left ventricular performance. Among others, reasons for this discrepancy have been related to the fact that until recently, available technology did not allow accurate measurements of right ventricular and right atrial structure and function and that effective therapy for the failing right heart was not available. It is for these reasons that while many books and monographs related to left heart failure have been published, there is a vacancy in publications related to the failing right heart, though clinicians have been aware of the poor prognosis associated vii

8 viii Foreword I with right heart failure. However, over the last several decades, new advances in diagnostic approaches related to the evaluation of right ventricular structure, function, and diseases have been developed. Further, recent developments in therapeutic modalities provide novel approaches (pharmacologic therapy and mechanical support) for the failing right heart in daily clinical practice. All these new developments related to the failing right heart are presented in this book. The editors, cardiac surgeons, and physician scientists contributing to this book have been actively involved with clinical work and have conducted research related to the failing right heart. In this book, right ventricular structure, function, and diseases producing right heart failure are presented. Traditional and newer diagnostic and therapeutic modalities are discussed at length. Clinicians and clinical investigators from Greece, United Kingdom, Australia, and China present their experience. Current concepts and beliefs related to the failing right heart are explored. Diagnostic imaging techniques including state-of-the-art two- and three-dimensional echocardiography, Doppler and tissue Doppler echocardiography, magnetic resonance imaging, and cardiac catheterization are presented. Pharmacologic and other therapeutic modalities including balloon pump and right ventricular assist devices are discussed. Attempts have also been made to bridge mechanisms of pathogenesis as they are related to clinical presentation and management, thus providing a basis for translational research. Diagnostic information obtained with current technology as presented in this book would have been obtained only at autopsy several years ago. A note of caution, however, should be given to clinicians and especially to young physician scientists. We should not lose sight of reality and be aware that despite all the impressive technology of time present, right heart failure and most of heart disease in general cannot be currently cured. Several years ago, Lewis Thomas [5] proposed a concept that he called halfway technology. Diseases cannot be cured with today s mostly halfway technology ; however, this technology does provide new insights into basic pathophysiology, gives us a better understanding of basic mechanisms of diseases, and eventually does result in the prevention and cure of diseases of time present as was the case with the diseases of time past. For example, rheumatic fever, rheumatic valve disease, syphilis with all of its cardiovascular involvements, poliomyelitis, and peptic ulcer disease are rarely seen today in developed countries. This is the ultimate goal of medicine. Until that time, the reader of this book will benefit from the information that is gathered in one place related to the failing right heart. This book will assist physician scientists who conduct clinical and translational research related to the failing right heart and to the failing heart in general in order to come up with new concepts that will help prevent and cure most of diseases we see today. Importantly, this book will help clinicians who take care of patients with heart failure and assist them to provide better care to sick individuals with the failing right heart. The editors should be commended for undertaking the task to edit this book on the failing right heart, a topic that until recently was essentially ignored. The time for publication of a book

9 Foreword I ix related to right heart failure has already passed. The present book will fill the vacancy on this important topic. Columbus, OH, USA Harisios Boudoulas References 1. Ziesenitz V, Kohler D, Gorenflo M. The failing right heart: the neglected ventricle? Clin Pharmacol Ther. 2014;96: Harvey W. Exercitatio de Motu Cordis et Sanguinis in Animalibus. Francofurti Guilielem Fitzeri, 1628, translated by C. D. Leake. Springfield: Charles C Thomas; White PD. Heart disease. Boston: Macmillan; 1931, p Mackenzie J. Diseases of the heart. New York: Oxford Medical Publications; Thomas L. The lives of a cell. New York: Viking Press; 1974.

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11 Foreword II The editors and the authors are to be congratulated for shedding light on some often neglected parts of the heart, i.e., the right ventricle and the right atrium. The advent of better treatment modalities has not only raised life expectancy and paved the way for unparalleled success in congenital cardiac repair but also, ironically, led to an increase in the incidence of cardiac diseases worldwide, many of which would previously have led to early mortality. The right heart plays a key role in many of these cardiac diseases, such as left heart failure, congenital heart diseases, right ventricular cardiomyopathy, right ventricular dysplasia, right ventricular infarction, pulmonary arterial hypertension, and pulmonary and/or tricuspid valvular heart disease and is of utmost importance in heart transplantation and implantation of mechanical circulatory support devices. Despite this, the implications of right heart failure have been overlooked for many years, owing to the fact that the systemic circulation can be maintained even without adequate right ventricular function in certain clinical situations (i.e., after total cavopulmonary connection in congenital heart diseases). Furthermore, the anatomy and pathophysiology of the right heart are difficult to understand and not well elucidated to date, and little is known about treatment strategies for right ventricular failure. Fortunately, our understanding of the right heart has increased in recent years, and more and more scientific publications dealing with right ventricular failure are emerging. With the aid of modern diagnostic tools (two- and three-dimensional echocardiography, velocity vector imaging, magnetic resonance imaging, and diffusion tensor MRI), the structural and functional relationships between the right and left ventricle are being studied more closely. The complex nature of the right heart is, in part, related to the functional interaction between the right and left ventricle (ventricular interdependence), and the septum is the central player in this interdependence. The integral role of the septum has been described over 100 years ago by Bernheim and more recently by Buckberg, and details of this important observation are examined and presented again in the chapter entitled Physiology of the Failing Right Heart. The Failing Right Heart covers all aspects of the right heart: functional anatomy, pathophysiology, echocardiographic assessment, magnetic resonance imaging, pharmacological treatment, aesthetic management, and mechanical support. Furthermore, it details the role of the failing right xi

12 xii Foreword II ventricle in adult congenital heart disease and pulmonary embolism and surgical treatment of the tricuspid valve and its role in heart transplantation. The authors are to be congratulated for drawing much needed attention to the right ventricle, for their comprehensive analysis on why the right ventricle fails, and for pointing the way ahead for treatment strategies in important clinical situations involving the right heart. This book is a must-read for anyone who is involved in all aspects of treating patients with cardiac diseases. Freiburg, Germany Friedhelm Beyersdorf

13 Preface Right ventricular function, though neglected for many years, attracted scientific attention lately. Numerous experimental and clinical data is currently accumulated, however dispersed, in the literature. This prompted us to summarize in this book the fundamental principles of right heart physiology and pharmacology, diagnostic tools, and strategies to define right heart failure. Moreover, emphasis is given on certain pathological conditions affecting the right heart, like congenital diseases, pulmonary embolism, and tricuspid valve disease as well as advanced treatment with mechanical support and transplantation. Algorithms and flow diagrams are included to provide the reader with illustrated snapshots of the decisions involved in the management of these patients. The Failing Right Heart book provides an up-to-date and comprehensive overview on the current evidence regarding right ventricular failure and aims to become an essential reference for cardiac surgeons, cardiologists, cardiac anesthesiologists, cardiac intensivists, and respiratory physicians on the topic. Thessaloniki, Greece Oxford, UK Thessaloniki, Greece Kyriakos Anastasiadis Stephen Westaby Polychronis Antonitsis xiii

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15 Contents 1 Introduction Kyriakos Anastasiadis, Stephen Westaby, and Polychronis Antonitsis 2 Functional Anatomy of the Right Heart Kyriakos Anastasiadis, Polychronis Antonitsis, and Stephen Westaby 3 Physiology of the Failing Right Heart Kyriakos Anastasiadis, Polychronis Antonitsis, and Stephen Westaby 4 Echocardiographic Assessment of the Right Ventricle Xu Yu Jin and Yu-Man Li 5 Magnetic Resonance Imaging of the Failing Right Heart Theodoros Karamitsos and Haralambos Karvounis 6 Right Heart Catheterization Stavros Hadjimiltiades 7 Pharmacologic Treatment of the Failing Right Heart Kyriakos Anastasiadis, Polychronis Antonitsis, and Stephen Westaby 8 The Failing Right Ventricle in Adult Congenital Heart Disease Stephen Westaby 9 Pulmonary Embolism and Right Heart Failure Aravinda Page, Ramesh De Silva, and David Jenkins 10 Surgery of the Tricuspid Valve Stephen Westaby and Kamran Baig 11 Anesthetic Management of the Failing Right Heart Helena Argiriadou xv

16 xvi Contents 12 Mechanical Support of the Right Heart Kyriakos Anastasiadis, Polychronis Antonitsis, and Stephen Westaby 13 Right Ventricular Failure and Heart Transplantation Yasir Abu-Omar and Ayyaz Ali Index

17 Contributors Editors Kyriakos Anastasiadis, MD, DSc, FETCS, FCCP, FESC Cardiothoracic Department AHEPA University Hospital Aristotle University of Thessaloniki Thessaloniki Greece Stephen Westaby, BSc, MS, PhD, FRCS, FETCS, FESC Consultant Cardiac Surgeon Department of Cardiac Surgery Oxford University Hospitals NHS Trust Oxford UK Professor of Bioengineering Institute of Life Sciences University of Swansea Wales UK Polychronis Antonitsis, MD, DSc Cardiothoracic Department Aristotle University of Thessaloniki AHEPA University Hospital Thessaloniki Greece xvii

18 xviii Contributors Authors Yasir Abu-Omar, MB ChB, DPhil, FRCS(CTh) Department of Cardiothoracic Surgery Papworth Hospital Cambridge UK Ayyaz Ali, MB BS, PhD, FRCS(CTh) Department of Cardiothoracic Surgery Papworth Hospital Cambridge UK Helena Argiriadou, MD, DSc Cardiothoracic Department AHEPA University Hospital Aristotle University of Thessaloniki Thessaloniki Greece

19 Contributors xix Kamran Baig, MD, FRCS(CTh) Department of Cardiac Surgery Oxford University Hospitals Trust Oxford UK Ramesh De Silva The University of New South Wales Medical School Sydney Australia Stavros Hadjimiltiades, MD Department of Cardiology AHEPA University Hospital Aristotle University of Thessaloniki Thessaloniki Greece

20 xx Contributors David Jenkins, BSc, MS, FRCS(CTh) Department of Cardiothoracic Surgery Papworth Hospital Cambridge UK Xu Yu Jin, MD, PhD, FRCS, FRCP Radcliffe Department of Medicine Oxford University Hospital University of Oxford Oxford UK Theodoros Karamitsos, MD Department of Cardiology AHEPA University Hospital Aristotle University of Thessaloniki Thessaloniki Greece Haralambos Karvounis, MD Department of Cardiology AHEPA University Hospital Aristotle University of Thessaloniki Thessaloniki Greece

21 Contributors xxi Yu-Man Li, MD Department of Echocardiography Wuhan Union Hospital Wuhan People s Republic of China Aravinda Page, MD Department of Cardiothoracic Surgery Papworth Hospital Cambridge UK Contributors Harisios Boudoulas, MD, Dr, DR Hon. Mult. Division of Cardiovascular Medicine The Ohio State University Columbus OH USA

22 xxii Contributors Friedhelm Beyersdorf, MD Department of Cardiovascular Surgery Heart Centre, University of Freiburg Freiburg Germany

23 Abbreviations 6MWD ACE AP ARDS ARVC ARVF ASD ASE AV BIS BiVAD BNP BPA BSA CABG cgmp CHD CMR CO CPB CRT CS CT CTEPH CTPA CVP DHCA DPG DT DTRS DVT EACVI ECG ECLS ECM ECMO EF 6-minute walk distance Angiotensin-converting enzyme Anteroposterior Acute respiratory distress syndrome Arrhythmogenic right ventricular cardiomyopathy Acute right ventricular failure Atrial septal defect American Society of Echocardiography Atrioventricular Bispectral index Biventricular assist device Brain natriuretic peptide Balloon pulmonary angioplasty Body surface area Coronary artery bypass grafting Cyclic guanosine monophosphate Congenital heart disease Cardiac magnetic resonance Cardiac output Cardiopulmonary bypass Cardiac resynchronization therapy Coronary sinus Computed tomography Chronic thromboembolic pulmonary hypertension Computed tomography pulmonary angiography Central venous pressure Deep hypothermic circulatory arrest Diastolic pressure gradient Destination therapy Destination therapy risk score Deep venous thrombosis European Association of Cardiovascular Imaging Electrocardiogram Extracorporeal life support Extracellular matrix Extracorporeal membrane oxygenation Ejection fraction xxiii

24 xxiv Abbreviations EI ESC ESP ET ET FAC FAO HIF HMRS HR IABP IAP ICD ICU IL IVA IVC IVCT LAO LAP LV LVAD LVEDP LVEF MCS MHC MiECC mpap MPI MRI NO NTG NYHA PA PAC PADP PAH PAP PCI PCWP PDE PE PEA PEEP PFO PG POC PV Eccentricity index European Society of Cardiology End-systolic pressure Endothelin Ejection time Fractional area change Fatty acid oxidation Hypoxia-inducible factor HeartMate II risk score Heart rate Intra-aortic balloon pump Intra-abdominal pressure Implantable cardioverter defibrillator Intensive care unit Interleukin Myocardial acceleration during isovolumic contraction Inferior vena cava Isovolumic contraction time Left anterior oblique Left atrial pressure Left ventricle Left ventricular assist device Left ventricular end-diastolic pressure Left ventricular ejection fraction Mechanical circulatory support Myosin heavy chain Minimal-invasive extracorporeal circulation Mean pulmonary artery pressure Myocardial performance index Magnetic resonance imaging Nitric oxide Nitroglycerin New York Heart Association Pulmonary artery Pulmonary artery catheter Pulmonary artery diastolic pressure Pulmonary arterial hypertension Pulmonary artery pressure Percutaneous coronary intervention Pulmonary capillary wedge pressure Phosphodiesterase Pulmonary embolism Pulmonary endarterectomy Positive end-expiratory pressure Patent foramen ovale Prostaglandin Point-of-care Pulmonary valve

25 Abbreviations xxv PVR RA RAO RAP RCA RHC RIMP ROS RT3DE RV RVAD RVEDV RVEF RVESV RVF RVFRS RVOT RVSP RVSWI SIRS SNP SPAP SR SSFP STI SVC SVR TAH TAPSE TCI TCO TDI TEE TGA TGF TOF TPG TR TTE TV TVF VEGF VQ VTE Pulmonary vascular resistance Right atrium Right anterior oblique Right atrial pressure Right coronary artery Right heart catheterization Right ventricular index of myocardial performance Reactive oxygen species Real-time three-dimensional echocardiography Right ventricle Right ventricular assist device Right ventricular end-diastolic volume Right ventricular ejection fraction Right ventricular end-systolic volume Right ventricular failure Right ventricular failure risk score Right ventricular outflow tract Right ventricular systolic pressure Right ventricular stroke work index Systemic inflammatory response syndrome Sodium nitroprusside Systolic pulmonary artery pressure Strain rate Steady-state free precession Speckle tracking imaging Superior vena cava Systemic vascular resistance Total artificial heart Tricuspid annular plane systolic excursion Target-controlled infusion Tricuspid (valve) closure opening Tissue Doppler imaging Transesophageal echocardiography Transposition of the great arteries Transforming growth factor Tetralogy of Fallot Transpulmonary gradient Tricuspid regurgitation Transthoracic echocardiography Tricuspid valve Tumor necrosis factor Vascular endothelial growth factor Ventilation/perfusion Venous thromboembolism