El Khourys Textbook of Aortic Valve Repair

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Library of Congress Cataloging-in-Publication Data

Names: Vohra, Hunaid A., editor. | deKerchcove, Laurent, editor. | El
Khoury, Gebrine, editor.
Title: El Khoury's textbook of aortic valve repair / Hunaid A. Vohra, MB,
BS, MRCS, MD, FRCS (CTh), FETCS, PhD, editor, Professor, Consultant
Cardiac Surgeon, Bristol Heart Institute, University of Bristol, UK,
University of West England, UK, Laurent deKerchcove, MD, PhD, editor,
Professor, Consultant Cardiac Surgeon, Saint-Luc University Hospital,
Brussels, Belgium, Gebrine El Khoury, MD, PhD, editor, Professor,
Consultant Cardiac Surgeon, Head of Department of Cardiac Surgery,
Saint-Luc University Hospital, Brussels, Belgium.
Other titles: Textbook of aortic valve repair
Description: New York : Nova Science Publishers, [2023] | Series:
Cardiology research and clinical developments | Includes bibliographical
references and index. |
Identifiers: LCCN 2023045963 (print) | LCCN 2023045964 (ebook) | ISBN
9798891132382 (hardcover) | ISBN 9798891132603 (adobe pdf)
Subjects: LCSH: Aortic valve--Surgery--Textbooks.
Classification: LCC RD598 .E38 2023 (print) | LCC RD598 (ebook) | DDC
617.4/12--dc23/eng/20231117
LC record available at https://lccn.loc.gov/2023045963
LC ebook record available at https://lccn.loc.gov/2023045964

Published by Nova Science Publishers, Inc. † New York

Contents

Foreword ..................................................................................................................... vii

Tirone David
Preface ...................................................................................................................... xi
Chapter 1 Applied Anatomy and Pathophysiology for Aortic Valve Repair ........... 1
Ruggero De Paulis, Andrea Salica and Raffaele Scaffa
Chapter 2 Echocardiography for Aortic Valve Repair ............................................ 23
Michel J. Van Dyck and Guillaume Lemaire
Chapter 3 Aortic Valve Sparing Root Replacement and Leaflet Repair in
Tricuspid Aortic Valves ............................................................................. 43
Jonathan C. Hong and Joel Price
Chapter 4 Bicuspid Aortic Valves: Anatomy, Echocardiographic
Assessment and Surgical Repair Techniques .......................................... 63
Habib Jabagi and Munir Boodhwani
Chapter 5 Aortic Valve-Preserving Surgery in Valve Endocarditis...................... 111
Shubhra Sinha, Massimo Caputo and Hunaid A. Vohra
Chapter 6 Aortic Valve Repair in Type A Aortic Dissection ................................. 127
Arnaldo Dimagli , Szabolcs Gergely and Umberto Benedetto
Chapter 7 The Ross Operation ................................................................................. 141
Serban Stoica
Chapter 8 Reoperative Aortic Root Repair ............................................................. 167
Stephanie N. Nguyen, David Blitzer and Hiroo Takayama
Chapter 9 Can Aortic Valve Repair Equal the Long-Term Results of
Modern Biological Valve Replacement? ................................................ 193
Michael Shang and John Elefteriades
Index ................................................................................................................... 221
About the Editors ................................................................................................................ 225
Foreword

In 1994 Robert Dion, the chief of cardiac surgery at the Cliniques Universitaires St-Luc in
Brussels, asked me to take one of his young cardiac surgeons to work with me for a semester.
I told him that his young surgeon would have to come as a clinical fellow if he wanted to
participate in patient care because of problems related to medical licensure and hospital bylaws,
and this would be perceived as a demotion to an already practicing surgeon. The humble
Gebrine El Khoury, an already accomplished young cardia surgeon, joined us in January 1995
and worked as a clinical fellow at Toronto General Hospital for 6 months. My associates and I
were highly impressed by this man’s technical abilities and quiet, modest, and respectful
character. Seven years later, Gebrine invited me to be the moderator of a live teleconference at
the Cliniques Universitaires St-Luc on newer operative techniques he had introduced in that
cardiac unit. He and his associates put on a spectacular show demonstrating innovative
operative techniques to a large audience of European surgeons. A variety of procedures such
as reimplantation of the aortic valve, remodeling of the aortic root, repair of mitral valve leaflet
prolapse with chordal replacement with Gore-Tex sutures, and the Ross procedure were
beautifully executed with echocardiographic evidence of perfect anatomic and functional
results. That was when I realized that a star surgeon was born and that he would elevate heart
valve surgery standards. Gebrine El Khoury is much more than an excellent technical surgeon,
innovator and mentor. He is one of most generous man I have met. Generous in every aspect
of human character. El Khoury’s Textbook of Aortic Valve Reconstruction is the proof that all
his trainees and friends feel as I do. They want to honour him by naming a surgical book after
him. He certainly deserves because he has done so much for us, for our patients, for our
specialty.
I read the entire book before writing this introduction. Students of aortic surgery should
have it on their personal bookshelf or computers. In my honorary address to the European
Cardio-Thoracic Association meeting in The Hague in 1994, I described the importance of
anatomy and physiology for cardiac surgeons who dedicated themselves to reconstructive
procedures of the heart. A sound knowledge of these two basic sciences is indispensable for
surgeons who correct pathological abnormalities that causes dysfunction. As a perpetual
student of anatomy, I prefer to use to term cusp to describe the parts that open and close the
semilunar valves and reserve the term leaflet to describe the parts that open and close the
atrioventricular valves. The normal aortic and pulmonary valves are tricuspid but it remarkable
to see that some patients with bicuspid valves (aortic or pulmonary valves) may go through life
without any clinically detectable physiological abnormality. This is, however, the exception
because anatomical abnormality frequent precedes functional abnormality, that is, “form
follows function”. Thus, an in-depth knowledge of functional anatomy of the semilunar valves
is indispensable to develop skills to repair the aortic valve or replace it with the native
pulmonary valve such as in the Ross procedure. The first chapter in this book is dedicated to
viii Tirone David

the functional anatomy of the aortic valve. The authors describe the functional anatomy of the
aortic root and provide the reader with their personal interpretation of the role of each
component of the aortic valve as it applies to reconstructive procedures of the aortic valve. The
following chapter is an excellent description of the role of echocardiography in aortic valve
surgery. I was fortunate to have intraoperative echocardiography soon after I started practicing
in early 1980’s, initially using an epicardial probe but soon after a transesophageal probe, and
a whole new world opened in front of me as a young surgeon because I could determine the
anatomical and functional quality of my reconstructive procedures in the operating room.
Transesophageal echocardiography has become the single most important diagnostic tool to
surgeons who repair heart valves and do other complex procedures such as reconstruction of
the mitral annulus and intervalvular fibrous body. The two following chapters are on aortic
valve sparing procedures and the information containing on bicuspid aortic valves is quite
comprehensive and informative. It should be mentioned that although Gebrine and his former
trainees use the Valsalva graft during reimplantation of the aortic valve and one cannot argue
with their successes, I remain skeptical about the practical importance of such graft. We now
have tens of patients who had reimplantation of the aortic valve into a straight tubular graft and
have been followed prospectively for over 20 years and the aortic valve continues to function
satisfactorily. If the presence of neo-aortic sinuses is important after reimplantation of the aortic
valve, it will take longer than 2 decades to prove it. I believe that the aortic valve cusps will
eventually fail after reimplantation procedure not because of lack of aortic sinuses but rather
because the operation that bears my name places the aortic valve inside a rigid, non-complaint
structure, the Dacron graft. Rigidity of the aortic root is likely one of the reasons a normal
tricuspid aortic valve calcifies in elderly patients.
The next two chapters of this textbook deal with more controversial subjects: valve repair
in patients with endocarditis and in type A aortic dissection. Both are quite comprehensive and
describes the state of art on such complex areas of aortic surgery. The following chapter is
dedicated to the Ross procedure, another controversial operation that is now seeing a third
revival since its introduction in late 1960’s. I was part of the second wave of Ross procedures
in the world. This is an excellent operation for young patients with aortic valve disease
particularly women during childbearing years and young men with aortic stenosis and relatively
normal aortic annulus. I have performed this operation throughout my career and in my
experience, it is not a valve for life. Regardless how well the pulmonary valve is transferred
from the pulmonary to the systemic circulation, either because of severance from its native
position (along with its blood supply and innervation), or because of lack of adaptation to the
systemic circulation, failure rates appear to accelerate after 15 to 20 years. Clearly more
longitudinal studies are needed for this old, but it is still useful operation to treat aortic valve
disease in the young. It is not a simple aortic valve replacement and experience is important to
provide excellent clinical outcomes.
A chapter in this book deals with reoperations in the aortic root. Surgery for infected
prosthetic aortic root and ascending aortic graft are associated with high operative mortality
and morbidity. Radical extirpation of all seemingly infected tissues is the only way to guarantee
bacteriological cure but it is often a formidable surgical endeavor. However, that is one the
reasons aortic surgery is becoming a sub-specialty within the domain of cardiovascular surgery.
The final chapter is a literature review of comparisons of outcomes of aortic valve replacement
with aortic valve repair. I doubt that will ever be randomized clinical trials comparing these
two approaches to aortic valve surgery.
 Foreword ix

Gebrine El Khoury has mastered all types of operations in the aortic root and proximal
thoracic aorta and taught us how to best perform them. Hats off to this a great cardiac surgeon
and mentor!

Tirone David, MD
Professor of Surgery, University of Toronto
Melanie Munk Chair of Cardiovascular Surgery,
Peter Munk Cardiac Centre at Toronto General Hospital, Toronto, Canada
Preface

The adoption of aortic valve repair is exponentially increasing, and patients and cardiologists
are demanding this with more enthusiasm. This is a current subject of great interest, and
contemporary results are already present, and more are awaited. This book describes the whole
journey through the set-up of an aortic valve repair program, pertinent investigations, patient
selection, different approaches, cardio-pulmonary bypass, re-operations, and its application
with combined surgery. This book is directed to all training cardiac surgeons and cardiologists,
consultant cardiac surgeons and cardiologists, anaesthetists, intensive care specialists and
perfusionists. This is the first book authored exclusively on this subject. The three editors
(especially Professor El Khoury) are leading authorities on this subject in the world and are
mentors for many surgical programs. Every chapter is authored by champions in this particular
aspect of aortic valve repair surgery. These are mainly from the USA and Europe.
Chapter 1

Applied Anatomy and Pathophysiology

for Aortic Valve Repair

Ruggero De Paulis*
Andrea Salica
and Raffaele Scaffa
Cardiac Surgery Department, European Hospital, Rome, Italy;
Unicamillus, International Medical University in Rome, Italy

Abstract

The aortic root represents a small anatomical region at the center of the heart with a
complex physiology that is important in regulating the normal function of the aortic valve.
The peculiar anatomical shape and interaction of the various components are instrumental
in assuring a life-long function of the thin valve leaflets. This chapter highlights the critical
anatomical components of this region, explains their complex interactions and indicates
how this knowledge is important for various procedure of aortic valve sparing or aortic
valve repair. A good surgical reconstruction necessarily takes advantage from a deep and
complete knowledge of the normal anatomy and physiology.

Keywords: aortic root, valve sparing, valve repair, anatomy

Introduction

Aortic valve sparing and aortic valve repair surgery represent a group of complex techniques
developed to treat aortic valve regurgitation or to spare the aortic valve in case of root aneurism.
Its practice started in the early 90's, [1, 2] and it has been progressively adopted by an increasing
number of experienced centers. Nowadays, the increased evidence of the clinically relevant
advantages of repair/sparing surgery in respect to replacement with prosthetic valves, has
increased the interest in aortic valve repair surgery. This applies especially in cases where the

*
Corresponding Author’s Email: [email protected].

In: El Khoury’s Textbook of Aortic Valve Repair

Editors: Hunaid A. Vohra, Laurent deKerchcove and Gebrine El Khoury
ISBN: 979-8-89113-238-2
© 2024 Nova Science Publishers, Inc.
2 Ruggero De Paulis, Andrea Salica and Raffaele Scaffa

aortic leaflets appear to be smooth and pliable, and with a particular focus on bicuspid aortic
valve that represents the category more frequently associated with severe aortic valve
insufficiency with or without or root aneurysm.
Despite the advantage of conservative aortic root surgery and aortic valve plasty represent
one of the most important recent advances in cardiac surgery, its complexity and the need for
relative long learning curve has somehow prevented a more rapid diffusion of the technique
and has slowed down its progressive adoption as a routine surgery.
A deep and codified knowledge of the root anatomy is the first fundamental step to
approach this type of operations. In fact, the morphology of anatomical components and their
intimate relationships are fundamental to achieve a normal valve function. Every aortic
aneurysm causes an alteration of the normal vessel's anatomy. However, the aneurysm of the
aortic root represents a peculiar form of aneurysm because the alteration of the normal anatomy
of this aortic segment has direct consequences for the normal mechanism of the aortic valve.
Indeed, the principle of valve sparing surgery is to achieve an anatomical reconstruction of the
root, not only with the aim of preventing acute aortic events due to the pathology, but also to
restore the normal dynamics of the aortic valve. When the surgeon is confronted with a root
surgery, the anatomical components might be distorted and the relationships between each
component are severely modified by the disease. The lack of anatomical reference point is very
frequent in this setting thus making the reconstruction more challenging. As an example,
suturing of the aortic commissures to a Dacron graft is a fundamental maneuver to restore the
aortic valve anatomy while at the same time achieving valve competence. Despite it represents
a key point of the procedure, the right height and mutual geometrical orientation of the
commissures which often relies on single surgeon experience, raises a great amount of
uncertainty in less experienced surgeons. Moreover, the potential but frequent needs to perform
an additional surgical maneuver on the leaflets to correct organic leaflet prolapse, might further
increases this amount of uncertainty. Aortic leaflet plasty, different form mitral leaflet plasty,
has paid for many years the lack of knowledge of a clear codification of the surgical steps. This
aspect has certainly been one source of controversies and has prevented a rapid worldwide
consensus on this type of approach.
A combination of various aspects has certainly limited the diffusion of valve sparing/aortic
valve repair, making this type of surgery limited to experienced and dedicated centers in a mood
of “more art than science” [3]. On the other hand, in patients with aortic root aneurysms, the
valve and root replacement, the Bentall procedure, represents a well codified and standardized
technique, easily reproducible with well-known results [4]. Despite the concerns for
anticoagulation therapy, for bio-prostheses deterioration and for the increased risk of
endocarditis the Bentall procedure is still preferred in many cardiac centers because of its
reproducibility, standardization and ease of execution.
Recently, a greater focus on the aortic valve pathophysiology, has fueled the interest on
aortic valve repair procedures around the world. Today, surgical indications for a remodeling
or reimplantation technique, as well as for aortic leaflets repair are well recognized.
Furthermore, a deeper knowledge of the surgical anatomy and physiology of the aortic
valve/root complex and the availability of new instruments and devices for the surgeon, has
allowed better standardization of these procedures, making good reproducibility possible across
different settings. It is therefore easier to understand how good comprehension of the
geometrical relationships and of the dynamics of the aortic valve and root is fundamental to a
successful approach in this field.
 Applied Anatomy and Pathophysiology for Aortic Valve Repair 3

Nowadays, aortic valve sparing/repair remains a complex, but increasingly codified

surgical procedure where three main aspects need to be emphasized in order to optimize the
final results. These three aspects are represented by 1) an anatomical approach to root
replacement, 2) an efficient strategy to achieve an aortic annuloplasty, and 3) a simple, rational
and standardized approach to aortic leaflets repair.
All these aspects could be considered together or separately, case by case, depending on
the presence or the absence of aortic aneurism and on the mechanism responsible for aortic
regurgitation, when present.

Normal and Functional Root Anatomy

The definition of “normal anatomy” when referring to the aortic root (as for the heart in general)
is not the same as when we are referring to most of the various organs of the human body. Not
only it could result quite difficult, but it might not be completely correct. In fact, anatomical
studies have always relied on a static interpretation of the human body resulting from refined
observation of cadaver studies. However, the cardiovascular system lacks the peculiarities to
be studied from a static point of view. Dimensions of the majority of its components are
different if measured in vivo with normal pressurized conditions or postmortem as in cadaver
specimens [5]. Normal anatomy of each valve is not exclusive of the open or closed position.
It is a dynamic anatomy of functional entities in which geometrical parameters, as annular
dimensions, leaflets position, or every other spatial coordinate change from the diastolic to the
systolic phase of the cardiac cycle. The physiologic interactions of the dynamic of every single
component result in a normal valve function and in a normal morphology [6)] In our opinion
and from the surgeon's point of view, definition of “normal functional anatomy” of the aortic
root represents the best way to describe the normal anatomy in vivo of a valvular apparatus [7].

Figure 1. The diameter of the sinotubular junction measured in cadaver roots (non-pressurized, left) are
significantly lower than when observed in alive subjects (echography evaluation, right).

One of the most important examples of this concept is represented by the bicuspid aortic
valve. Despite it represents a congenital anomaly, it can generally be considered from a
functional point of view like a normal valve, especially when no dysfunction in term of stenosis
4 Ruggero De Paulis, Andrea Salica and Raffaele Scaffa

or regurgitation is present. On the other hand, in a great portion of bicuspid aortic valve, the
annular or root dilatation are the main mechanisms of valve dysfunction. In other words, despite
the presence of a congenital disease, the functional anatomy of the root is responsible for the
valve dysfunction [8]. In these cases, “normal” aortic leaflets are frequently observed. Valve
repair and cusp coaptation is achieved first through the restoration of the correct anatomic
relationship between annular and root diameters and second by correcting the valve prolapse
that represent the consequences of the regurgitant flow onto the leaflets. Another good example
of functional anatomy are the different relationships between the sino-tubular junction (STJ)
and the virtual basal ring (VBR) when measured in vivo with respect to non-pressurized cadaver
specimens. In fact, diameters of STJ measured in cadaver roots are significantly smaller than
in the observations of alive subjects. However, in alive subjects the STJ is 1.3 times larger than
the VBR [9] [Figure 1]. These examples enhance the relevance of the functional anatomy as it
is represented in vivo, especially when seen in the perspective of an aortic valve sparing or
repair surgery.
The importance and the significance of the functional root anatomy has been extensively
described firstly by El Khoury and colleagues [10] and confirmed by other authors as results of
their clinical practice and their related studies [11].
It goes without saying that aortic valve function is strictly linked to the integrity of each
leaflet. In fact, aortic valve stenosis or regurgitation are frequently caused by leaflets
abnormalities. Cusp retraction, leaflets fibrosis or thickening, perforations or tears are examples
of leaflets diseases that are associated to acquired pathologies, as rheumatic disease, calcified
degeneration or endocarditis. In these cases, surgical approach is still oriented to the valve
replacement. On the other hand, aortic valve regurgitation can be also caused by aortic root
dilation despite the presence of normal aortic leaflets. Dilation of at least one of the root
components forming the functional aortic annulus (FAA), ventricular aortic junction (VAJ),
virtual basal ring (VBR), sino-tubular junction (STJ) may lead to aortic valve regurgitation of
various degree. In particular, a mismatch of the normal relationships among the components of
the aortic root is the cause of the lack of leaflet's coaptation and valvular dysfunction [10].
Since David and Yacoub first described valve sparing approaches [1, 2] and El Khoury and
colleagues [12] as well as Pethig and colleagues [13] described the initial techniques of aortic
leaflet repair, several efforts have been made to better standardize these surgical strategies and
many innovations have been suggested. Studies about repair-oriented classifications of the
aortic regurgitation and repair-oriented anatomy of the root have been carried out by the most
experienced cardiac center in this field [14]. Designs of new vascular prostheses [15, 16, 17]
and dedicated prosthetic rings [18, 19, 20, 21] have been introduced to promote surgical
reconstruction of the root. Introduction of new instruments to measure aortic leaflets height
have improved the opportunity to verify the decision-making process and the quality of leaflet
repair [22]. These additions to the complex field of aortic valve and root surgery have
contributed to achieve a higher level of standardization and to increase the durability of these
procedures and the reproducibility of the results. A detailed insight into the anatomy and
physiology of the aortic valve and root have certainly stimulated all these innovations and
contributed to the advancement in the field.
 Applied Anatomy and Pathophysiology for Aortic Valve Repair 5

Anatomy of Aortic Root and Aortic Leaflets

Anatomical Definition

The aortic root is a complex region at the center of the heart anatomy. It is in fact considered
the “centerpiece” of the heart, having relationships to all cardiac chambers [23]. It is positioned
immediately above the left ventricle acting as a bridge between the ventricle and the ascending
aorta. For this reason, it has two main roles in the physiology of the cardiovascular system.
First, it is the connection between the heart and the arterial system; second, it contributes to the
peculiar characteristics of the systemic blood flow that is instrumental for the normal function
of the aortic valve. Despite the aortic root is a well-defined anatomic unit, the best way to
describe it requires the description of each single components [24].

Aortic Annulus

Despite the aortic annulus represents one of the most studied anatomical regions of the heart, it
has been an enigmatic entity for a long time and still today there is a wide variability in its
characterization. In the daily surgical practice, it has more than one definition. In fact, when
considering an aortic valve replacement, it is commonly considered as the locus of the basal
attachment of the leaflets, just between the left ventricle and the sinuses of Valsalva [25].
Differently, in the setting of echocardiography, the VBR (a line passing along the nadir of the
three cusps) is generally used when providing measurements of the diameter of the aortic
annulus. Despite both definitions have been widely accepted because of their simplicity and the
ability to fulfilling a specific concept within a certain field (surgery or echocardiography), the
true anatomic description of the aortic annulus is much more complex.
In fact, if we want to describe the aortic annulus in a more complete functional way, we
should consider together a series of root components that not only identify the annulus but also
contribute to the normal function of the cardiac valve during the cardiac cycle. From this point
of view we need to visualize the aortic valve as working into a cage that is framed by two rings
(the virtual basal ring proximally and the sino-tubular junction distally) and connected by three
pillars represented by the three commissural posts. The diameters of the two rings change
continuously during the cardiac cycle and the positions of the commissural posts change
accordingly. In doing so, they regulate the dynamics of the valve leaflets. From a general point
of view if we want to optimize the description of this peculiar anatomical region characterized
by a clear three-dimensional morphology, we need to break it down into its several components:
1. Virtual basal ring, 2. Ventricular-aortic junction, 3. Sino-tubular junction, 4. Valve leaflets,
and 5. Crown-like attachment of the aortic leaflets and Inter-leaflets triangles [7].

Virtual Basal Ring

Despite the VBR is a virtual anatomic entity that has not a real histological counterpart, it
represents an exact anatomical locus [26, 27]. It is constructed by joining the nadir of the hinge
lines of the three aortic leaflets and can be clearly identified by direct surgical view and by CT
6 Ruggero De Paulis, Andrea Salica and Raffaele Scaffa

scan imaging methods with a very high level of accuracy [Figure 2] [28]. This region, despite
being a virtual ring, it is very important in the decision-making process of many clinical and
surgical procedures. The VBR represents the place where sutures are positioned to anchor the
Dacron graft during a reimplantation procedures and firmly producing a stable and complete
form of annuloplasty [Figure 3]. In the trans-catheter aortic valve implantation era, its
measurement represents one of the main parameters to accurately choose the proper size of the
valve prosthesis during the strategical planning.

Figure 2. ECG-gated contrast-enhanced computed tomography shows the progressive disappearing of

the nadirs of each leaflet (left to right). The last image represents the plane and the shape of the virtual
basal ring (VBR).

Figure 3. The position of 6 pledgets show the accurate location of the virtual basal ring (VBR) from the
surgical point of view. It also represents the place where sutures are positioned to anchor the Dacron
graft during reimplantation procedure or external ring for annuloplasty repair. The black arrows
indicate the position of the three commissure (base of interleaflet triangle) and the white arrow indicate
the nadirs of the three cusps.

Historically, the Virtual Basal Ring was first identified in the echo-lab and labeled as the
aortic ring. It was hypothesized as a circular line. Afterward, it was more exactly identified
with CT scan imaging study and better described as a bi-dimensional line with a more or less
elliptical shape. In fact, the shape of the aortic ring has been traditionally considered as a perfect
circular line, but during the last years the diffusion of trans-catheter aortic valve implantation
 Applied Anatomy and Pathophysiology for Aortic Valve Repair 7

and the increased adoption of aortic valve repair techniques, has contributed to a deeper
knowledge of this region of the aortic annulus. The precise characterization of the VBR shape
was highlighted by the studies of Rankin and colleagues [29] who described the ellipticity of
human awake aortic basal annulus in tricuspid aortic valve [Figure 4]. Based on these studies,
the Author devised an aortic prosthetic rigid ring, that was designed with an elliptical shape to
permanently restore the physiological annular geometry of a tricuspid aortic valve during the
repair procedures [30].

Figure 4. Major and minor diastolic diameters of the aortic annulus in a tricuspid aortic valve by ECG-
gated contrast-enhanced computed tomography. Ellipticity Index (EI): 1.40. EI is the ratio between a
major diameter and minor diameter. A ratio > 1.1 is usually considered “elliptic”.

Successively, further studies from our group evidenced that the VBR is characterized by
different grade of ellipticity between tricuspid (TAV) and bicuspid aortic valve (BAV). A
gradual spectrum of ellipticity of VBR has been observed from native symmetric (type 0)
BAVs, where it is circular, to more asymmetric BAVs phenotypes (type 1), to TAVs, where it
is increasingly more elliptic [31]. In further studies we have evidenced also a linear correlation
between the cusp orientation of a bicuspid aortic valve and the shape of the aortic annulus. In
particular, the aortic annulus (at the level of the VBR), follows a continuous spectrum of
ellipticity depending on the cusp orientation. It starts from a perfectly circular shape for
commissural orientation between 160°–180°, to an elliptical shape for commissural orientation
between 120°–139°, passing through an intermediate state for commissural orientation between
140°–159°. In other words, in the setting of bicuspid aortic valve, when the commissural
orientation approaches 120°, the morphology of the VBR approaches that of a tricuspid aortic
valve [32]. Interestingly, these observations related to the shape of the VBR match with the
classifications of bicuspid aortic valve proposed by De Kerchove and Schaefers [33]. These
authors proposed a new classification of bicuspid aortic valve based on cusp orientation. In
fact, they classified BAV in a symmetrical (type A), asymmetrical (type B) or very
asymmetrical (type C) phenotype depending on cusp orientation (160°–180°, 140°–159° and
120°–139°, respectively), which corresponds to the angle of non-fused cusp. When considering
potential analogies with the well-known Sievers classification [34], type A will more generally
correspond to the classical Sievers type 0 while type B or type C will both correspond to Sievers