PULMONARY HEART DISEASE PULMONARY HEART DISEASE edited by LEWIS J. RUBIN MARTINUS NI]HOFF PUBLISHING A MEMBER OF THE KLUWER ACADEMIC PUBLISHERS GROUP Boston/The Hague /Dordrecht/Lancaster Copyright 1984 @ by Martinus Nijhoff Publishing, Boston Softcover reprint of the hardcover 1s t Edition 1984 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, mechanical, photocopying, recording, or otherwise, without written permission of the publisher, Martinus Nijhoff Publishing, 190 Old Derby Street, Hingham, Massachusetts 02043 Distributors: for North America for all other countries Kluwer Academic Publishers Kluwer Academic Publishers Group 190 Old Derby Street Distribution Centre Hingham, MA 02043 P.O. Box 322 3300 AH Dordrecht The Netherlands Library of Congress Cataloging in publication Data Main entry under title: Pulmonary heart disease. Bibliography: p. Includes index. 1. Cor pulmonale. I. Rubin, Lewis J. [DNLM: 1. Pulmonary heart disease. WG 420 P975] RC685.C55P85 1984 616.1'2 84-1523 ISBN-13: 978-1-4612-9797-0 e-ISBN: 978-1-4613-2847-6 DOl: 10.1007/978-1-4613-2847-6 To my mother, Erna Rubin, and in memory of my father, Theodore Rubin. CONTENTS Preface xiii 1. Introduction 1 LEWIS J. RUBIN, M.D. 2. Pathophysiology of the pulmonary circulation 11 BRYDON J.B. GRANT, M.D. DAVID R. DANTZKER, M.D. 3. Pathology of pulmonary heart disease 65 WAYNE E. TAYLOR, M.D. 4. Clinical evaluation 107 LEWIS J. RUBIN, M.D. 5. The electrocardiogram in chronic lung disease 117 FRANCIS X. MCGOWAN, M.D. GALEN S. WAGNER, M.D. 6. Roentgenographic evaluation of pulmonary heart disease 135 CARL E. RAVIN, M.D. 7. Pulmonary function and exercise testing 177 TIMOTHY R. CHAPPELL, M.D. 8. Radionuclide angiocardiographic assessment of right and left ventricular performance 223 vii viii Contents RICHARD A. MATTHAY, M.D. HARVEY J. BERGER, M.D. 9. The left ventricle in chronic lung disease 247 EDGAR J. CALDWELL, M.D. 10. Arrhythmias in chronic lung disease 273 ERIC N. PRYSTOWSXY, M.D. GEORGE KLEIN, M.D. 11. Acute cor pulmonale 285 WARREN R. SUMMER, M.D. 12. Therapy of pulmonary heart disease 325 LEWIS J. RUBIN, M.D. ROBERT H. PETER, M.D. 13. Prognosis of pulmonary heart disease 355 RUSSELL DODGE, M.D. BENJAMIN BURROWS,. M.D. DOUGLASS MORRISON, M.D. CONTRIBUTING AUTHORS Harvey J. Berger, M.D. Assistant Professor of Diagnostic Radiology and Medicine Director of Cardiovascular Imaging Yale University School of Medicine New Haven, CT Benjamin Burrows, M.D. Professor of Medicine Chief, Pulmonary Division University of Arizona Health Sciences Center Tucson, AZ Edgar J. Caldwell, M.D. Associate Professor of Medicine University of Vermont School of Medicine Pulmonary Division, Maine Medical Center Portland, ME Timothy R. Chappell, M.D. Assistant Professor of Internal Medicine University of Texas Health Science Center at Dallas Assistant Chief, Pulmonary Section ix x Contributing Authors Veterans Administration Medical Center Dallas, TX David R. Dantzker, M.D. Professor of Medicine Chief, Pulmonary Division University of Texas Health Science Center at Houston Houston, TX Russell Dodge, M.D. Assistant Professor of Medicine Pulmonary Division University of Arizona Health Sciences Center Tucson, AZ Brydon J.B. Grant, M.D. Assistant Professor of Medicine Pulmonary Division State University of New York at Buffalo School of Medicine Buffalo, NY George J. Klein, M.D. Assistant Professor of Medicine Director, Clinical Electrophysiology Laboratory Cardiac Investigation Unit University Hospital London, Ontario Canada Richard A. Matthay, M.D. Associate Professor of Medicine Associate Director, Pulmonary Division Yale University School of Medicine New Haven, CT Francis X. McGowan, M.D. Research Fellow in Cardiology Department of Medicine Duke University School of Medicine Durham, NC Douglass Morrison, M.D. Assistant Professor of Medicine and Radiology University of Arizona Health Sciences Center xi Staff Cardiologist, Veterans Administration Medical Center Tucson, AZ Robert H. Peter, M.D. Professor of Medicine Associate Director, Cardiovascular Laboratory Duke University School of Medicine Durham, NC Eric N. Prystowsky, M.D. Associate Professor of Medicine Director, Clinical Electrophysiology Laboratory Krannert Institute of Cardiology Indiana University School of Medicine Indianapolis, IN Carl E. Ravin, M.D. Professor of Radiology Director, Imaging Division Duke University School of Medicine Durham, NC Lewis J. Rubin, M.D. Assistant Professor of Internal Medicine University of Texas Health Science Center at Dallas Chief, Pulmonary Section, Veterans Administration Medical Center Dallas, TX Warren R. Summer, M.D. Professor of Medicine Chief, Pulmonary-Critical Care Division Louisiana State University Medical Center New Orleans, LA Wayne E. Taylor, M.D. Assistant Professor of Pathology University of Texas Health Science Center at Dallas Dallas, TX Galen S. Wagner, M.D. Associate Professor of Medicine Cardiology Division Duke University School of Medicine Durham, NC PREFACE Responsibility for the diagnosis and management of disorders of the pulmonary circulation has become the shared domain of the pulmonologist, cardiologist, surgeon, radiologist, pathologist, and, perhaps most important of all, the internist. It is the general internist who is most likely to care for the majority of patients with lung diseases that secondarily give rise to pulmonary heart disease, and it is the internist who will first evaluate the patient with primary pulmonary hyperten sion or recurrent pulmonary thromboembolism who presents with nonspecific complaints and may manifest subtle and nondiagnostic findings on preliminary evaluation. The burgeoning medical literature concerning aspects of the pulmonary circula tion, both clinical and investigative, is a reflection of the reawakening of great interest in this field and has led to many new developments, both in our understand ing of cardiopulmonary pathophysiology and in the diagnosis and treatment of pulmonary vascular diseases. This book is an attempt to provide the clinician with a comprehensive overview of pulmonary heart disease from the perspective of experts representing a variety of disciplines. It is intended to be thorough yet clinically relevant. Individuals familiar with some facets of pulmonary heart disease may gain insight into other aspects of this condition, whereas those unfamiliar with this disorder may find this work useful as a general reference or as a resource to address a specific question. Since there are few incontrovertible facts concerning the approach to patients with pulmonary heart disease, emphasis has been placed on general principles and guidelines rather than dogmatic recommendations. xiii