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Double-Outlet
Right Ventricle
KonstantinV.Shatalov
KonstantineM.Dzhidzhikhiya
Editors
123
Double-Outlet Right Ventricle
Konstantin V. Shatalov ·
Konstantine M. Dzhidzhikhiya
Editors
Double-Outlet Right
Ventricle
Editors
Konstantin V. Shatalov
A. N. Bakulev National Medical
Investigation Center for Cardiovascular
Surgery
Moscow, Russia
Konstantine M. Dzhidzhikhiya
A. N. Bakulev National Medical
Investigation Center for Cardiovascular
Surgery
Moscow, Russia
ISBN 978-3-031-49706-3 ISBN 978-3-031-49707-0 (eBook)
https://doi.org/10.1007/978-3-031-49707-0
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature
Switzerland AG 2024
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, specifically the rights of 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, 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 affiliations.
This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Paper in this product is recyclable.
Acknowledgement
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We gratefully acknowledge Dr. Khoren Abelian, Specialty Registrar in Clinical
Radiology, MRCGP, Liverpool, UK, for his help in revising and preparation the
manuscript.
v
Contents
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Description
History ........................................................... 3
S. P. Glyantsev
Anatomy ......................................................... 13
K. V. Shatalov, K. M. Dzhidzhikhiya, and M. V. Gordeeva
Classification ...................................................... 55
K. V. Shatalov and K. M. Dzhidzhikhiya
Arterial Hypoxemia ............................................... 61
A. K. Kade, P. P. Polyakov, S. A. Zanin, and Z. M. Dzhidzhikhiya
Myocardial Remodeling ............................................ 89
A. K. Kade, P. P. Polyakov, S. A. Zanin, and Z. M. Dzhidzhikhiya
Diagnostics
Echocardiography ................................................. 107
I. Y. Baryshnikova
Angiocardiography ................................................ 129
M. G. Pursanov and K. M. Dzhidzhikhiya
Computed Tomography ............................................ 149
K. M. Dzhidzhikhiya
3D Printing ....................................................... 163
K. M. Dzhidzhikhiya
Treatment
Surgery ........................................................... 175
K. V. Shatalov and K. M. Dzhidzhikhiya
vii
viii Contents
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Results ........................................................... 231
K. V. Shatalov and K. M. Dzhidzhikhiya
Index ............................................................. 243
Description
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History
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S. P. Glyantsev
Abstract Although the term «double-outlet right ventricle» was coined in 1957,
description of anatomical specimens of the hearts with aortic root connected to both
ventricles in literature accounts more than 200 years. The first anatomical correction
of DORVperformed in 1950s opened a new chapter in surgical correction of complex
cyanotic congenital heart defects. Nowadays, surgical techniques used for treatment
of DORV are the most variable compared to other congenital heart defects. This
chapter describes main historical milestones in surgical correction and understanding
of DORV since its first clinical description.
Keywords Double-outlet right ventricle
In 1814, the English physician J. R. Farre in his book «On Malformation of the Human
Heart» collected all clinical and anatomical cases on a congenital heart defect (CHD)
which he called «aortic ostium attached to both ventricles» published in the literature
over the previous 30 years. According to Farre, the first scientist who observed a
patient with this malformation and described the autopsy specimen in 1778 was
a professor of anatomy and surgery at Leiden University E. Sandifort in a thesis
entitled «Observationes Anatomico-pathologicæ». A 12-year-old child complained
on exertional shortness of breath, during which his face became swollen and cyanotic,
his eyes bulged, with visible pulsation of jugular veins on his neck. He suffered with
frequent colds, and in winter, he shivered from cold, even if he was near the hearth.
Sometimes he had nosebleeds, which brought relief. He developed these symptoms
in the 3rd year of his life. At the autopsy, the foramen ovale was open, the ostium
of the pulmonary valve was severely narrowed, which caused the right atrium and
ventricle to be significantly dilated, and the aorta was communicating with both
ventricles through the opening in the interventricular septum (IVS).
S. P. Glyantsev (B)
Department of Cardiac Surgery History, A. N. Bakulev National Medical Investigation Center for
Cardiovascular Surgery, Moscow, Russia
e-mail: spglyantsev@mail.ru
· History
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2024
K. V. Shatalov and K. M. Dzhidzhikhiya (eds.), Double-Outlet Right Ventricle,
https://doi.org/10.1007/978-3-031-49707-0_1
3
4 S. P. Glyantsev
https://t.me/medicina_free
A more detailed description of the malformation was published in 1784 by the
London anatomist W. Hunter. The child lived to the 13 years old. He was thin, with
a dark blue face. He had shortness of breath and palpitation. During the attack, the
child’s face turned almost black. He fell backwards and seemed to be dead. He
usually came out of such attacks, sobbing, yawning and feeling tired. Any haste or
rapid movement of the body, aggravated the symptoms, and in order to prevent it
or reduce its severity, the child lay down on the left side and remained motionless
for about ten minutes. On autopsy, the pulmonary artery originated from the right
ventricle, but it was so narrowed that it barely let in a thin probe. The upper part
of the IVS was perforated, which allowed the thumb to pass through it. The aortic
opening was located so close to this perforation that during contractions of the heart
it received blood from both ventricles.
A similar case was reported in 1785 by R. Pulteney. The appearance of this adolescent was the opposite of the patient described by W. Hunter. He often experienced a
feeling of despondency, dizziness and shortness of breath during physical exertion.
Gradually,these symptoms intensified so that he could not cross the room without the
skin of his face and hands becoming black. At this time, he was abruptly weakening,
and his breathing almost stopped. At the age of 13 years and 9 months, he died. On
autopsy, the ostium of the pulmonary valve was smaller than usual. When examining
the heart, the biventricular connection of the aorta was found, so it could let in the
tip of the finger to pass through the aorta to the left ventricle.
J. Abernethy, a surgeon at St. Bartholomew’s Hospital in London, described and
illustrated DORV in «Surgical and Physiological Essays» in 1793. He wrote about the
child who lived for two years. His skin was cyanotic and, as a rule, cold, breathing
was irregular due to paroxysms, during which he often lay face down. The heart
specimen was distinguished by the large foramen ovale and the aorta was located
almost exclusively above the right ventricle instead of being to the left to the IVS.
Also, pulmonary artery stenosis, dilation of the right heart and a hole in the upper
part of the IVS were noted.
In addition to E. Sandifort (1778), W. Hunter (1784), R. Pulteney (1785) and J.
Abernethy (1794), the aortic origin from both ventricles, according to J. R. Farre,
was described by professor of College de France J.-N. Corvisart (1806), who along
with his own observation mentioned two cases of professor Cailott, as well as A.
Burns (1809), Ring (1809) and Travers (1810). The last observation is particularly
noteworthy for the detailed description of the clinical picture of the malformation
and the autopsy specimen of the heart.
Mr. V., aged 14, visited Dr. Travers on August 4, 1809. He was tall, but very thin
and weak. There were practically no muscles on his arms and legs, the fingers and
toes were thin and long with thickened ends and nail clubbing. His dark blue skin was
covered with sticky sweat. The young man was suffering with shortness of breath
on moderate exertion, turning into yawning, a strong cough caused by running or
quickly climbing stairs, strong but regular heartbeats at rest and palpitations during
physical exertion. The radial pulse was 80 beats per minute, was regular, but weak.
Sometimes, the young man felt a sharp pain and burning in his chest. At night, he
often woke up with a feeling of suffocation, beginning in a dream, after which he
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