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Surgical Treatment of
Chronic Constrictive
Pericarditis
Ujjwal K. Chowdhury
Lakshmi Kumari Sankhyan
123
Surgical Treatment of Chronic Constrictive
Pericarditis
UjjwalK.Chowdhury • LakshmiKumariSankhyan
Surgical Treatment
of Chronic Constrictive
Pericarditis
UjjwalK.Chowdhury
Dept. of Cardiothoracic Surgery
All India Institute of Medical Sciences
New Delhi, Delhi, India
LakshmiKumariSankhyan
Department of Cardiothoracic Surgery
All India Institute of Medical Sciences
New Delhi, Delhi, India
ISBN 978-981-99-5807-8 ISBN 978-981-99-5808-5 (eBook)
https://doi.org/10.1007/978-981-99-5808-5
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Singapore
Pte Ltd. 2023
This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether
the whole or part of the material is concerned, specically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microlms 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 specic 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, expressed or implied, with respect to the material contained herein or for any
errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional
claims in published maps and institutional afliations.
This Springer imprint is published by the registered company Springer Nature Singapore Pte Ltd.
The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721,
Singapore
Paper in this product is recyclable.
Dedicated to my mentors in
https://t.me/medicina_free
Cardiac Surgery
The late Dr. Stanley John, MS, MCh,
FIACS, FACS, who educated me in the
science and art of the surgical profession,
and taught me much about surgical
operations. He gifted me unrestricted
opportunities to practise and develop my
skills and abilities.
Prof. Robert H.Anderson, BSc, MD, PhD
(Hons), FRCPath, FRCS Edin. (Hons), who
continues to provide his unrestricted
guidance in my scientic writing.
Prof. Panangipalli Venugopal, MS, MCh,
who invited me to work in an environment
where honesty and doing the right thing are
the principles that guide all personal and
professional relationships.
and
The surgeons and staff of All India Institute
of Medical Sciences, New Delhi, upon whom
its future depends.
Foreword
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Pericardiectomy—not a procedure to be taken lightly.
“There is a pericardiectomy on the schedule tomorrow. The cardiologist told me
that he was reasonably convinced that the patient had constriction by haemodynamics”, the resident said reassuringly to his attending. The attending thought about it
and decided not to check the haemodynamic tracings himself. He would leave the
decision to the cardiologist, and furthermore he really wasn’t facile with haemodynamic tracings to distinguish pericardial constriction from restrictive cardiomyopathy. What could go wrong with that plan?
In fact, a lot could go wrong.
Pericardial constriction is a very uncommon condition in developed countries,
and apart from surgeons in centres with a specic interest and matching referrals
with pericardial disease, pericardiectomy for constriction will, for most surgeons,
be a rare procedure. The little narrative above is not meant to be pejorative but to
express the concern that a case of “stripping the pericardium for constriction” could
be interpreted as unexacting both diagnostically and surgically. This can be far from
reality for several reasons and the following are important considerations for cardiac surgeons, and are outlined to provide a knowledge acquisition “checklist” for
reading this book which I hope, at a minimum, will give surgeons a much greater
degree of condence dealing with pericardial disease:
• while tuberculosis is the commonest cause of pericardial constriction worldwide,
that would be an extremely rare cause in developed countries where the mecha-
nisms are increasingly dominated by iatrogenic causes that can make the diagno-
sis and management even more difcult—post-mediastinal irradiation, following
cardiac surgery, cardiac transplantation, and lung transplantation, uraemia,
trauma, neoplasms, connective tissue disorders, infection, and idiopathic.
• the surgeon must be aware of the pathology and natural history of the three sub-
types of pericardial constriction—transient constriction, chronic constriction,
and effusive constriction, and this distinction is important as it predicates which
patients require pericardiectomy and which patients can be placed on medical
treatment to reduce inammation with the possibility of resolution of the process
and proceed with surgery if this strategy proves unsuccessful.
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Foreword
• making the distinction between pericardial constriction and restrictive cardiomy-
opathy is vitally important as the therapeutic directions are very different. A
pericardiectomy performed in a patient with restriction misdiagnosed as con-
striction may well have a fatal outcome, or a challenging postoperative course
with no prospect of improvement.
• multimodal imaging of the pericardium and the heart provides important clues to
the distinction between constriction and restriction as well as sub-typing the type
of constriction.
• it is very important that surgeons can independently identify the haemodynamic
phenomena characteristic of constriction—exaggerated ventricular interdepen-
dence and dissociation of intracardiac and intrathoracic pressures—and under-
stand the precision with which these hemodynamic measurements must be made
at catheterization to deliver the most sensitive and specic markers of constric-
tion. It is important to be aware that these haemodynamic phenomena can at
times be quite subtle and the nal diagnosis will depend on the interpretation of
all available clinical and investigational information.
• the distinction between constriction and restriction becomes particularly tricky
when features of both coexist, which is a situation that may be seen particularly
after cardiac transplantation and mediastinal radiation.
• the arguments for the surgical approach (median sternotomy versus anterolateral
thoracotomy), the extent of pericardial removal (“phrenic to phrenic” pericardi-
ectomy versus total pericardiectomy), and the reasons for occasionally requiring
cardiopulmonary bypass must be appreciated by surgeons undertaking this
procedure.
• the postoperative management of patients after pericardiectomy is usually quite
straightforward but on occasions can be very challenging, especially when there
is coexisting cardiac disease and some degree of underlying restrictive
cardiomyopathy.
• the long-term outcome after pericardiectomy is usually determined by the under-
lying cause of the constriction.
Every so often, the cardiac surgical community is fortunate to have a genuine expert in
a particular disease and its surgical management wrap up what is known about the subject, underpinned by a unique surgical experience and make this available to us all in a
book from which all cardiac surgeons, and cardiologists for that matter, can benet. That
is what Dr. Chowdhury and his co-authors have done. The book covers all aspects of the
disease and its management and will be an invaluable, authoritative reference, particularly for surgeons faced with some of the diagnostic and surgical conundrums outlined
above (all addressed in this book), which can make this disease challenging. Having
surgical videos as part of the book is especially useful, not only for surgeons facing an
unfamiliar procedure but particularly for cardiac surgical trainees. We should be very
grateful that Dr. Chowdhury has gone to considerable trouble to write this book.
DavidMcGifn, MBBS, FRACS, DMedHS
Cardiothoracic Surgery & Transplantation
Professor of Cardiothoracic Surgery
Monash University, Australia
Preface
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First described 300 years ago as concertio cordis, chronic constrictive pericarditis
commands substantial clinical interest because the disease continues to elude clinicians, mimicking restrictive cardiomyopathy, endomyocardial brosis, and chronic
liver disease. Unlike other diseases linked to underdevelopment and inammation,
such as rheumatic heart disease, aortoarteritis, and endomyocardial brosis, which
have shown a decrease in the prevalence with socio-economic development, constrictive pericarditis has not shown a declining trend.
This condition has posed a diagnostic dilemma since it was rst recognized clinically. Although many diagnostic approaches have become available subsequently,
the diagnostic challenge remains. Now, with two-dimensional and Doppler echocardiography, other causes of right heart failure can be diagnosed or excluded. Imaging
methods such as computed tomography and magnetic resonance imaging can measure pericardial thickness, which is usually increased in patients with constrictive
pericarditis. Constrictive pericarditis can, however, occur in a substantial percentage of patients with normal pericardial thickness as well.
The evolving aetiology of chronic constrictive pericarditis in the past few decades
has led to diagnostic uncertainties. Specic major causes to be ruled out are tubercular pericarditis, neoplastic pericarditis, and pericarditis associated with a systemic
disease including autoimmune disease. Tuberculosis continues to be the leading
cause of chronic constrictive pericarditis in developing countries, with a reported
incidence of 38–83%. Due to the emergence of drug-resistant strains of tuberculosis
in association with AIDS, the prevalence has increased to more than 90%. Tubercular
pericarditis may present with dense brosis without direct evidence of tuberculosis,
similar to other aetiologies of chronic constrictive pericarditis. The advent of antitubercular chemotherapy brought down the mortality from 90% to 40%. While proven
tubercular pericarditis may present with dense brosis without direct evidence of
tuberculosis, such brosis may follow other aetiologies of chronic constrictive pericarditis as well.
In developed countries, other causes such as mediastinal radiation and previous
open heart surgery continue to dominate. Emerging additional causes include iatrogenic origins such as percutaneous coronary interventions, pacemaker insertion,
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Preface
catheter ablation, and following cardiac transplantation. The prevalence of idiopathic chronic constrictive pericarditis varied from 24% to 61% in Indian studies,
depending on the criteria used to diagnose chronic constrictive pericarditis.
Thus, patients today have symptoms and signs of right-sided heart failure that are
disproportionate to left ventricular dysfunction or valvular heart disease. The challenge is to determine whether abnormalities are caused by pericardial restraint,
myocardial restriction, or both.
The precise pathogenesis of chronic constrictive pericarditis remains debatable
and is scantly investigated. Limited evidence-based data are available to guide the
management of pericardial diseases. Diagnostic efforts are worthy if they affect
subsequent treatments and prognosis. A targeted aetiology search directed to the
commonest causes on the basis of clinical background, epidemiological issues, or
specic presentations will prove benecial.
The diagnosis and management of pericardial diseases in general, and chronic
constrictive pericarditis in particular, remain challenging because of the vast spectrum of clinical manifestations, coupled with inadequate numbers of patients and
clinical data. The American College of Cardiology and the American Heart
Association have been silent on the management of pericardial diseases. In 2004,
and subsequently in 2015, the European Society of Cardiology published guidelines
for the diagnosis and management of pericardial diseases.
This condition has posed a diagnostic dilemma since it was rst recognized.
Misdiagnosis with other disease entities has also not been adequately addressed. No
single approach can be used to diagnose all cases of constrictive pericarditis. The
diagnostic approach taken should be individualized for every patient. Diagnosis
may be made on the basis of history, physical examination, chest radiograph, echocardiography, computed tomography, cardiac magnetic resonance imaging, cardiac
catheterization, and visualization of the pericardium. The key diagnostic tool is the
clinical suspicion of constrictive pericarditis in a patient with signs and symptoms
of right-sided heart failure that are disproportionate to pulmonary or left-sided heart
disease.
Clinically it is necessary to differentiate constrictive pericarditis from other
causes of right-sided heart failure, such as pulmonary embolism, pulmonary hypertension, right ventricular infarction, mitral stenosis, and left ventricular systolic dysfunction. Kussmaul’s sign may be positive but it lacks specicity, as it is also seen
in patients with restrictive cardiomyopathy, endomyocardial brosis, right ventricular failure, and tricuspid stenosis. In constrictive pericarditis, ascites appears rst
followed by pedal oedema, known as “ascites precox”. This sequence is among the
cardinal features in chronic constrictive pericarditis.
Despite improved accuracy of diagnosis with echocardiography, Doppler colour
ow mapping, cardiac catheterization, aggressive preoperative stabilization,
improvements in cardiac anaesthesia, and intensive care, the surgical mortality of
pericardiectomy continues to be high, with reports ranging from 6% to 19%. In
2005, we reported worse outcomes of pericardiectomy in patients with preoperative
high right atrial pressure higher than 24 mmHg, hyperbilirubinemia, renal dysfunction, atrial brillation, and pericardial calcication.
Preface
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xi
The idea of resecting the pericardium for constrictive pericarditis dates back to
1898, when Delorme rst suggested it. The German group Rehn and Sauerbruch in
1913 performed successful pericardial resection for constrictive pericarditis through
a left anterolateral thoracotomy approach. The operative approaches used by
Churchill, and later by Harrington, are now of historical interest. Surgical approaches
for pericardiectomy include left anterolateral thoracotomy, median sternotomy, a
U-incision with the base of “U” at the left sternal border (Harrington’s approach),
and bilateral anterolateral thoracotomy.
Despite experience spanning over 100 years, there is no foolproof formula in
the published literature that can be used in selecting an optimal approach for a
given patient. The literature is rife with examples of patients with constrictive pericarditis having been treated by pericardiectomy by either left anterolateral thoracotomy or median sternotomy. Despite the effectiveness of surgical therapy for the
treatment of constrictive pericarditis, there are disparate opinions regarding the
role of corticosteroids in the treatment of tubercular pericarditis, the timing of the
operation, the issue of a surgical approach, extent of decortication, and the requirement of cardiopulmonary bypass. The operative tactics and techniques applied to
pericardial excision do not as yet match the pleomorphic pathology and pathophysiology presented by individual patients. This is illustrated by the variety of
surgical approaches advanced, suggesting a degree of inconsistency in surgical
management.
The efcacy of pericardiocentesis in preventing chronic constrictive pericarditis
in pericardial effusion (serous or haemorrhagic) has been inadequately investigated.
The problems of perioperative diagnostic error have also not been adequately
addressed in the surgical literature, despite known difculties in differentiating
patients with restrictive cardiomyopathy from those with constriction.
Reports addressing the issue of surgical approach, the extent of pericardiectomy, and postoperative haemodynamics are limited and controversial. The terms
“radical”, “total”, “extensive”, “complete”, “subtotal”, “adequate”, “near-total”,
and “partial” pericardiectomy also have been variably used in the literature to
describe the procedure to be performed, often without precise denition of the
limits of pericardial resection. Published reports attest to the unpredictable and
variable pattern of constrictive pericarditis, and lend support to radical
decortication.
In view of the multitude of surgical approaches and strategies, it rst seemed
attractive to ask for contributions from authorities in the eld. We prefer, however,
to present a unied concept for the non-operative, operative, and perioperative management of pericardial constriction.
The decision to create this monograph was based on the belief that surgery for
chronic constrictive pericarditis is safe and reproducible. It is, therefore, teachable.
We recognize that the techniques and concepts of others may be different from ours
and may give results that are as good. Yet we are condent that the concepts and
techniques reected in this monograph, learned carefully and followed meticulously, will deliver excellent results from the surgical treatment of chronic constrictive pericarditis in the hands of any surgeon who wishes to apply them.
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