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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3737_Библиотеки_им_академика_М_И_Перельмана
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Multimodality Imaging ofRight Ventricular Outow Tract Disease inAdults withCongenital Heart Disease
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Fig. 12 Fluoroscopic still frame of stent positioning prior to delivery
149
Fig. 13 Fluoroscopic image of stent during high-pressure balloon dilation. Note that a waist is still present, representing external compression due to heavy conduit calcication

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Fig. 14 Post-procedural
hemodynamics revealed
gradient resolution
T. Spates and R. A. Krasuski

Multimodality Imaging ofRight Ventricular Outow Tract Disease inAdults withCongenital Heart Disease
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Fig. 15 Doppler gradient showed minimal to no PS
Endocarditis: Prevention
andRecognition
One of the most dreaded long-term complications for patients with transcatheter valves is
endocarditis. Higher residual gradient after
implant appears to be a signicant risk factor
[19]. In addition to antibiotic prophylaxis with
dental procedures, all patients with prosthetic
valves (surgical or transcatheter) should also
receive a baby aspirin. Early valve failure may be
related to leaet thrombosis and antiplatelet therapy may be preventative.
PV endocarditis is particularly challenging to
identify. Symptoms may be indolent and valvular
vegetations can be challenging to visualize. An
acute increase in outow murmur intensity or
measured transpulmonary gradient should raise
the index of suspicion for endocarditis. Blood
cultures should be drawn whenever suspicion
exists. Transthoracic and transesophageal echocardiographic imaging may not be revealing; and
PET CT or cMRI may improve diagnostic sensitivity [20]. ICE can also be diagnostic: the catheter can be delivered across the tricuspid valve
and into the right ventricle and images can be
taken looking directly into the RVOT and pulmonic valve.
Clinical Controversies andClinical
Pearls
• During transcatheter replacement using balloon expandable prostheses, it is imperative to
perform balloon ination in the RVOT to evaluate for coronary compression or aortic root
deformation, as these features generally
preclude use of balloon expanding transcatheter valve.
• The use of self-expanding transcatheter
devices and the use of pre-stenting allows for
more inclusive RVOT anatomies and attenuates the concern for coronary compression.

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T. Spates and R. A. Krasuski
• An important step to diagnosis of pulmonic
valve endocarditis is having a high index of
suspicion. An unexplained rise in RVOT gradient is often seen. When transthoracic and
transesophageal echo are unrevealing, CT
PET, MRI or intracardiac echo should be
pursued.
Key Points
– Surgical and transcatheter interventions have
improved survival and quality of life in TOF;
though long-term follow-up is essential to
assess for infectious, structural, and arrhythmogenic sequelae.
– 2D echocardiography is a valuable tool for
serially monitoring. ACHD guidelines recommend cMRI be performed when quantitative
RV assessment is required to guide management, with appropriate use criteria justifying it
at an interval of every 3–5years or prior to a
planned intervention in the absence of acute
clinical changes.
– Pulmonic stenosis is graded similar to aortic
stenosis, with <3m/s mild, ≥3m/s moderate,
and≥4m/s severe.
– Severe bioprosthetic pulmonic regurgitation is
classied as a dense continuous wave Doppler
envelope, a jet width: annulus ratio>40%, and
regurgitant fraction >40% on
echocardiography.
– Pulmonic regurgitation and subsequent RV
dysfunction is one of the most common late
complications noted in these patients. In the
presence of ≥moderate pulmonic valve dysfunction (stenosis or regurgitation) with
symptoms of heart failure, decreased exercise
tolerance (as demonstrated by cardiopulmonary stress testing), or evidence of RV dilation
and enlargement, an intervention should be
considered.
– Using cMRI, right ventricular dilation sug-
gesting a need for pulmonic valve replacement for PR includes: RVEDVI ≥160mL/m2,
RVESVI ≥80 mL/m2, or RVEDV ≥2 ×
LVEDV.
– Transcatheter valve replacement should be
strongly favored if the patient is a poor surgi-
cal candidate, no additional operative interventions are necessary or to limit the number
of eventual sternotomies if the patient’s
anatomy is suitable.
Disclosures Dr. Krasuski serves as a consultant for
Actelion/Janssen Pharmaceuticals, Bayer, Gore Medical,
Medtronic and Neptune Medical. He receives research
funding from the Adult Congenital Heart Association and
Actelion/Janssen Pharmaceuticals. He is a principal investigator for trials with Artivion, Corvia, Edwards
Lifesciences, and Medtronic.
Chapter Review Questions
1. A 34-year-old gentleman with a history of
tetralogy of Fallot presents to clinic for routine evaluation. His history includes surgical
bioprosthetic valve placement at the age of
21. He is asymptomatic and had an echocardiogram performed a year ago that demonstrated normal biventricular function and
stable bioprosthetic pulmonic valve. The peak
velocity was measured at 2.8m/s with mild
pulmonic regurgitation. At what interval
should imaging be performed to adequately
interrogate valve function?
A. Every 3–5years
B. Every 6months
C. Once yearly
D. Every 2years
Answer: C
For patients with surgical bioprosthetic
valves, it is important that a post-operative
baseline echocardiogram is performed. The
2020 AHA ACC Guidelines for Valvular
Heart Disease states, echocardiograms should
then be performed at intervals of 5years and
10years following intervention if the patient
is without symptoms or clinical exam changes.
After 10years, patients should undergo annual
imaging given the increasing concern for progressive valve dysfunction.
2. A 47-year-old woman presents to your clinic
for new patient evaluation. She has a history
of tetralogy of Fallot repaired by transannular
patch with moderate to severe pulmonic

Multimodality Imaging ofRight Ventricular Outow Tract Disease inAdults withCongenital Heart Disease
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153
regurgitation. She was closely followed by
her previous cardiologist, who performed a
cardiac MRI ~8months ago. This revealed a
RVEF 49% with a RVEDVi 133mL/m2 and a
regurgitant fraction of 37%. She works from
home and is fairly sedentary. She notes mild
shortness of breath when walking her dog,
which she mainly attributes to decondition-
ing. What is the most appropriate next step?
A. Repeat cardiac MRI
B. Cardiopulmonary stress testing
C. Echocardiogram
D. Right heart catheterization
Answer: B
Cardiopulmonary stress testing to evaluate
for exertional intolerance is the most appropriate next step given her moderate to severe
regurgitation without signicant RV chamber
dilation or dysfunction. Should cardiopulmonary stress testing demonstrate cardiovascular
limitation, it would be reasonable to consider
surgical or transcatheter pulmonic valve
replacement with a class I recommendation as
per the ACC/AHA Guidelines for Adults with
Congenital Heart Disease.
3. A 31-year-old woman presents to clinic for
evaluation. She has a history of pulmonary
atresia for which she has undergone several
right ventricular to pulmonary artery conduit
replacements. Her last procedure was a transcatheter pulmonary valve replacement
~7 years ago. She was last seen in clinic
~2years ago. At that time, she had an echocardiogram revealing normal biventricular
systolic function and stable pulmonary valve
function. On exam today, there is a prominent
grade 3/6 systolic ejection murmur at the
LUSB with a grade 2 diastolic murmur. The
patient reports no exertional limitations, but
has experienced mild malaise, fatigue, and
lack of appetite over the last few weeks that
she attributes to “work stress.” What is the
most reasonable clinical approach?
A. Return to clinic in 1 year with
echocardiogram
B. Echocardiogram at the next available
appointment
C. Echocardiogram and draw 2 sets of blood
cultures today
D. Arrange for stat heart catheterization
Answer: C
Her physical exam is concerning for a signicant change in bioprosthetic valve function. While simple device degeneration is
possible, her symptoms of fatigue and malaise
raise concern for a possible subacute infectious process. Given these concerns, it is most
reasonable to perform both an echocardiogram to assess bioprosthetic function and to
draw blood cultures to evaluate for systemic
infection. It is important to note that the imaging recommendations for patients with transcatheter pulmonic valves suggest an
echocardiogram be performed yearly.
References
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T, Salandy S, Gielecki J, et al. An anatomical
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MD, Ilstrup DM, etal. Long-term outcome in patients
undergoing surgical repair of tetralogy of Fallot. N
Engl J Med. 1993;329(9):593–9.
5. Nollert G, Fischlein T, Bouterwek S, Bohmer C,
Klinner W, Reichart B.Long-term survival in patients
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of 490 survivors of the rst year after surgical repair.
J Am Coll Cardiol. 1997;30(5):1374–83.
6. Mavroudis C, Backer CL. Atlas of pediatric cardiac
surgery. 1st ed. London: Springer; 2015.
7. Cuypers JA, Witsenburg M, van der Linde D, RoosHesselink JW.Pulmonary stenosis: update on diagnosis and therapeutic options. Heart. 2013;99(5):339–47.
8. Feltes TF, Bacha E, Beekman RH 3rd, Cheatham JP,
Feinstein JA, Gomes AS, etal. Indications for cardiac
catheterization and intervention in pediatric cardiac
disease: a scientic statement from the American Heart
Association. Circulation. 2011;123(22):2607–52.
9. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B,
Broberg CS, Colman JM, et al. 2018 AHA/ACC
guideline for the management of adults with congenital heart disease: executive summary: a report of
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Association task force on clinical practice guidelines.
Circulation. 2019;139(14):e637–e97.
10. Baumgartner H, De Backer J. The ESC clinical practice guidelines for the Management of
Adult Congenital Heart Disease 2020. Eur Heart J.
2020;41(43):4153–4.
11. Wiant A, Nyberg E, Gilkeson RC. CT evaluation of
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12. Rudski LG, Lai WW, Alalo J, Hua L,
Handschumacher MD, Chandrasekaran K, et al.
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the right heart in adults: a report from the American
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European Association of Echocardiography, a registered branch of the European Society of Cardiology,
and the Canadian Society of Echocardiography. J Am
Soc Echocardiogr. 2010;23(7):685–713.
13. Sachdeva R, Valente AM, Armstrong AK, Cook
SC, Han BK, Lopez L, etal. ACC/AHA/ASE/HRS/
ISACHD/SCAI/SCCT/SCMR/SOPE 2020 appropriate use criteria for multimodality imaging during the follow-up Care of Patients with Congenital
Heart Disease: a report of the American College of
Cardiology Solution set Oversight Committee and
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Association, American Society of Echocardiography,
Heart Rhythm Society, International Society for Adult
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Cardiovascular Magnetic Resonance, and Society
of Pediatric Echocardiography. J Am Coll Cardiol.
2020;75(6):657–703.
14. Saremi F, Gera A, Ho SY, Hijazi ZM, SanchezQuintana D.CT and MR imaging of the pulmonary
valve. Radiographics. 2014;34(1):51–71.
15. Zoghbi WA, Chambers JB, Dumesnil JG, Foster E,
Gottdiener JS, Grayburn PA, etal. Recommendations
for evaluation of prosthetic valves with echocardiography and doppler ultrasound: a report From the
American Society of Echocardiography’s Guidelines
and Standards Committee and the Task Force on
Prosthetic Valves, developed in conjunction with
the American College of Cardiology Cardiovascular
Imaging Committee, Cardiac Imaging Committee
of the American Heart Association, the European
Association of Echocardiography, a registered
branch of the European Society of Cardiology, the
Japanese Society of Echocardiography and the
Canadian Society of Echocardiography, endorsed
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of Echocardiography. J Am Soc Echocardiogr.
2009;22(9):975–1014.
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Honjo O, etal. 3D modeling and printing in congenital heart surgery: entering the stage of maturation.
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18. Gales J, Krasuski RA, Fleming GA. Transcatheter
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in congenital heart patients. Prog Cardiovasc Dis.
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Bergersen L, Padera RF, Balzer DT, etal. Endocarditis
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20. Corey KM, Campbell MJ, Hill KD, Hornik CP,
Krasuski R, Barker PC, etal. Pulmonary valve endocarditis: the potential utility of multimodal imaging
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2020;11(2):192–7.

Pre- andIntraprocedural Imaging
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Considerations inParavalvular
Leak Closure
AdrianaPostolache, SimonaSperlongano,
MathieuLempereur, RalucaDulgheru,
FrançoisDamas, NilsDemarnee,
andPatrizioLancellotti
Abstract
Paravalvular regurgitation or leak (PVL) is not
an uncommon complication of prosthetic
valve disease and can be associated with
severe heart failure, hemolytic anemia, or
both. The management of these patients often
having multiple comorbidities is challenging
and the choice between surgical intervention,
transcatheter closure and medical treatment
should be decided by the Heart Team on a
case-by-case basis. Transcatheter PVL closure
has emerged as an attractive and efcient
treatment option for these patients and the
success of the procedure relies on a careful
pre-procedural evaluation, for selecting the
patients suitable for a transcatheter intervention, and expert imaging guidance during the
procedure. Pre-procedural evaluation should
determine the location and the number of the
jets and the severity of the
A. Postolache · M. Lempereur · R. Dulgheru ·
F. Damas · N. Demarneffe · P. Lancellotti (*)
Department of Cardiology, University of Liège
Hospital, Liège, Belgium
e-mail: adriana.postolache@chuliege.be; mathieu.
lempereur@chuliege.be; redulgheru@chuliege.be;
fdamas@chuliege.be; ndemarneffe@chuliege.be;
plancellotti@chuliege.be
S. Sperlongano
Division of Cardiology, Department of Translational
Medical Sciences, University of Campania Luigi
Vanvitelli, Naples, Italy
e-mail: simona.sperlongano@unicampania.it
PVL.Echocardiography holds a central role in
the pre-procedural evaluation of patients with
PVLs, but other imaging modalities, such as
cardiac magnetic resonance imaging (CMR)
and cardiac computed tomography (CT) can
offer useful adjunctive information for grading the severity of the regurgitation and for
selecting the type and the size of the prosthesis. The intervention is performed under uoroscopic and transesophageal/intra-cardiac
echocardiographic guidance. The use of fusion
imaging can facilitate the delivery of the
device. Echocardiography has an important
role on the procedural guidance, being especially useful for selecting the size of the
device(s), conrming the correct location of
the device(s) and its lack of interference with
the prosthetic valve function or adjacent structures (such as the coronary arteries for aortic
PVLs) and for evaluating the presence of complications. A good communication between
the echocardiographer and the interventional
cardiologist, at every step of the procedure, is
essential for the success of the intervention.
Keywords
Paravalvular leak · Pre-procedural planning ·
Intra-procedural guidance · Transcatheter
mitral paravalvular leak closure · Transcatheter
aortic paravalvular leak closure · Transcatheter
tricuspid paravalvular leak closure
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2024
A. M. Kelsey et al. (eds.), Cardiac Imaging in Structural Heart Disease Interventions,
https://doi.org/10.1007/978-3-031-50740-3_5
155

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A. Postolache et al.
Test your learning and check your understanding of this book’s contents: use the
“Springer Nature Flashcards” app to access
questions using ▶ https://sn.pub/ambACS.
To use the app, please follow the instructions in the chapter “Transcatheter Aortic
Valve Replacement.”
Learning Objectives
1. Describe the indications and the contraindications for transcatheter paravalvular leak
closure;
2. Describe how to evaluate the severity of PVLs
by using an integrative multiparametric and
multimodality approach;
3. Describe the principles of the procedural
guidance during percutaneous paravalvular
leak closure;
4. Describe the key points of pre- and intraprocedural evaluation for mitral, aortic and
tricuspid PVL closure.
Case Study on Transcatheter Mitral PVL
Closure
A 67-year-old female comes to our attention during a follow-up outpatient visit
reporting worsening of dyspnea over the
last few months. She is affected by type 2
diabetes mellitus on insulin therapy, permanent atrial brillation, and stage IIIA
chronic kidney disease. Her mobility is
poor due to a recent femur fracture with
endoprosthesis implantation. She had a
previous mitral valve surgical replacement
(1991) with a Carbomedics 25 mm bileaet
mechanical prosthetic valve. Blood tests
show chronic severe hemolytic anaemia
(which is partially responsible for the
symptoms) and no evidence of infection.
Transthoracic echocardiography (TTE) is
performed as the initial imaging test. On
TTE, left ventricle is not enlarged and its
systolic contractility is preserved, left
atrium is severely dilated, and moderate
aortic regurgitation and severe tricuspid
regurgitation are found. TTE also reveals
the presence of a mitral prosthesis paravalvular leak (PVL), which appears signicant on color Doppler analysis.
The TTE evaluation of our patient suggested the presence of mitral prosthesis
regurgitation, limited by acoustic artifacts
of the mechanical valve, so a transesophageal echocardiography (TEE) was performed to conrm the diagnosis. A TEE
showed normal functioning of the 2 mitral
leaets, with a mild (non-signicant) intraprosthetic washout. Multiple PVLs were
detected, the larger located anteriormedially. The “en face” view (surgical
view) of the mitral prosthesis on
3- dimensional (3D) TEE displays a large
antero-medial echo dropout area outside
the sewing ring, conrmed by color
Doppler, due to a calcied and brotic
annulus, resulting in disruption of the
sutures. The jet density and turbulence, the
wide vena contracta, the large proximal
isovelocity surface area (PISA) shell, and
the systolic retrograde ow into the pulmonary veins support the severity of the mitral
paravalvular regurgitation.
Background andDenitions
Prosthetic paravalvular regurgitation or paravalvular leak (PVL) is an abnormal communication
between the ring of a surgical or transcatheter
prosthesis, and the native valve annulus. A few
cases of PVL have also been described in patients
with mitral valve repair.
It is not an uncommon complication of pros-
thetic valve disease, occurring in 7–17% of
patients with mitral and 2–10% of patients with
surgical aortic prostheses [1, 2]. The incidence of
PVL is higher in transcatheter prosthetic valves,
even though the incidence of signicant aortic
regurgitation in patients undergoing transcatheter
aortic valve implantation (TAVI) has signicantly

Pre- andIntraprocedural Imaging Considerations inParavalvular Leak Closure
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157
decreased in the last 20years, with 0.6–5.3% of
patients undergoing TAVI having at least moderate PVL, in more recent trials [3–6]. Taking into
consideration the increase in transcatheter valvular replacements, the incidence of PVL is likely
to increase in coming years.
Paravalvular regurgitation may result from an
interaction of factors related to the intervention
(poor technique, use of sutures without pledgets,
use of continuous sutures for the mitral prostheses, supra-annular prostheses, inappropriate size
of a transcatheter valve) and factors related to the
local tissue (important annular calcications, tissue friability, the presence of infection).
The clinical presentation is highly variable,
ranging from an incidental nding on the follow up echocardiographic study to severe heart failure, hemolytic anemia, or infective endocarditis.
The vast majority of PVLs are mild and are considered benign, in the absence of infective endocarditis, with only a few cases being associated
with hemolysis. Approximately 2–5% of PVLs
on surgical valves are clinically relevant, being
associated with heart failure, hemolytic anemia,
or both [7].
The diagnosis, in particular, estimating the
severity of PVL, and the management of these
patients are challenging. Figure1 presents a proposed management plan for patients with
PVL. The choice between redo-surgery, transcatheter closure and medical palliative treatment
should be made by the Heart Team, on a case-bycase basis. According to the latest ESC and ACC/
AHA guidelines on valvular heart disease, surgical reintervention remains the rst treatment
option for patients with PVL associated with heart
failure or severe hemolytic anemia needing
repeated blood transfusions, whereas transcatheter intervention can be considered in patients at
high or prohibitive cardiac risk, with anatomically
suitable PVLs for a percutaneous closure [8, 9].
Transcatheter PVL closure has emerged as an
attractive treatment option in these patients who
often have many comorbidities and are at high
PVL
Is PVL associated with active infective endocarditis?
NO
Is PVL severe?
Is PVL clinically significant
YES
Heart Team discussion
High/prohibitive surgical risk
Transcatheter PVL closure
feasible
Good expertise
Transcatheter PVL closure Surgery
Fig. 1 Proposed management plan for patients with paravalvular regurgitation. The detection of paravalvular leak
(PVL) on an echocardiogram should prompt for the search
of infective endocarditis. In the absence of infective endocarditis, non-severe PVLs usually do not need and intervention as only few cases can be associated with severe
Active infective endocarditis
Dehiscence >1/4-1/3 of the valve ring circumference
Intracardiac thrombus
YES
NO
hemolytic anemia. Patients with severe, clinically signicant PVLs should be discussed in the Heart Team meeting, and the choice between surgical (re)intervention,
transcatheter closure and medical treatment should be
made on a case-by-case basis
Follow-up
Antibiotic treatement
Discussion of the
case on the
(Endocarditis) Heart
Te am
Inoperable patient
Transatheter PVL closure not
feasible
Medical treatment

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A. Postolache et al.
surgical risk. In centers with good expertise,
transcatheter PVL closure has a high feasibility
rate (>90%), a good success rate (reduction in
PVL to <mild in >70% of cases), which is associated with an improvement in heart failure symptoms, mid- and long-term survival and, in most
cases, a decrease in hemolytic anemia severity
[10, 11]. Although the success rate for the surgical reintervention is higher than for transcatheter
closure (<mild residual regurgitation in >90% of
cases), this comes with the price of a higher periprocedural mortality and complications risk, and
a risk of PVL recurrence, without an advantage
on long-term survival [10]. Nowadays, many
high-volume centers consider the transcatheter
intervention as the rst treatment option in feasible patients, with surgical reintervention being
reconsidered afterwards in case of failure of the
transcatheter procedure [10].
Based on current knowledge, transcatheter
closure is contraindicated in patients with active
infective endocarditis, in patients with a signicant dehiscence of the prosthetic valve ring,
involving >1/4–1/3 of the circumference, and in
patients with intracardiac thrombus [7]. It can
however be considered, after the resolution of the
infectious process and the disappearance of the
thrombus, in patients that are judged to be inoperable by the Heart Team.
The key steps for having a successful transcatheter PVL closure are:
• Careful pre-procedural evaluation for select-
ing the patients suitable for transcatheter
intervention and for planning the
intervention;
• Expert imaging guidance during the proce-
dure, with constant communication between
the operator(s) and the imager.
Principles ofPre-procedural
Assessment Before Transcatheter PVL
Intervention
The evaluation of para-valvular regurgitation is
difcult and should try to determine the following points:
• The location and the number of the jet(s);
• Estimate the severity of PVL.
Pre-procedural evaluation is helpful for planning
the procedure, by selecting the best approach for
delivering the device(s) in the individual patient,
and it offers an estimation of the type and size of
the device(s) that would best close the defect.
Location andNumber oftheJet(s)
Echocardiography is the rst and main imaging
modality for the diagnosis of prosthetic valve
dysfunction, and transthoracic (TTE) and transesophageal echocardiography (TEE) are usually
used together, as complementary exams. With
regards to the location of the regurgitant jets,
TTE is limited for the evaluation of mechanical
mitral PVLs, when often, the jet can only be visualized in an off-axis view (such as the sub-costal
view), and TEE is key for diagnosis. On the other
hand, for aortic PVLs, TTE and TEE are more
complementary, with TTE being useful for the
visualization of anteriorly located jets, whereas
posterior jets are better visualized by TEE [12].
3D echocardiography, in particular 3D TEE,
facilitates a more precise location of PVLs [12].
To improve the communication between the different actors involved in the patient’s management, it is recommended that the location of the
regurgitant jet(s) should be described on a clock
face or on anatomical criteria, as shown in Fig.2
[7, 12, 13].
Grading theSeverity ofPVL
This is without a doubt the most difcult part of
the evaluation of PVLs and an integrative, multiparametric, and, in many cases, a multimodality
imaging approach should be used [7, 12, 13].
Echocardiography is the main imaging modality used for estimating the severity of PVL and,
in a similar way to native valve regurgitation, an
integrated approach, which takes into consideration qualitative, semi-quantitative and quantitative parameters, from all echocardiographic
methods (2D, color, PW, CW Doppler, 3D echocardiography) is recommended for determining
the severity of PVLs [7, 12, 13]. We outline some
of the important points in the evaluation of PVLs:
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