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Multimodality Imaging ofRight Ventricular Outow Tract Disease inAdults withCongenital Heart Disease
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Fig. 12 Fluoroscopic still frame of stent positioning prior to delivery
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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 calcication
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Fig. 14 Post-procedural hemodynamics revealed gradient resolution
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Fig. 15 Doppler gradient showed minimal to no PS
Endocarditis: Prevention andRecognition
One of the most dreaded long-term complica­tions for patients with transcatheter valves is endocarditis. Higher residual gradient after implant appears to be a signicant 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 leaet 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 outow 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 andClinical Pearls
• During transcatheter replacement using bal­loon expandable prostheses, it is imperative to perform balloon ination 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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• 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 3–5years or prior to a planned intervention in the absence of acute clinical changes.
– Pulmonic stenosis is graded similar to aortic
stenosis, with <3m/s mild, 3m/s moderate, and4m/s severe.
– Severe bioprosthetic pulmonic regurgitation is
classied 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 ≥160mL/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 2.8m/s with mild pulmonic regurgitation. At what interval should imaging be performed to adequately interrogate valve function?
A. Every 3–5years B. Every 6months C. Once yearly D. Every 2years
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 5years and 10years following intervention if the patient is without symptoms or clinical exam changes. After 10years, 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
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regurgitation. She was closely followed by her previous cardiologist, who performed a cardiac MRI ~8months ago. This revealed a RVEF 49% with a RVEDVi 133mL/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 signicant 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 ~2years 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 sig­nicant 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
1. Wang JMH, Rai R, Carrasco M, Sam-Odusina T, Salandy S, Gielecki J, et al. An anatomical review of the right ventricle. Transl Res Anat. 2019;17:100049.
2. Gatzoulis MA, Webb GD, Daubeney PEF.Diagnosis and management of adult congenital heart disease, vol. 14. 3rd ed. Philadelphia: Elsevier; 2018. p.721.
3. Karl TR.Tetralogy of Fallot: current surgical perspec­tive. Ann Pediatr Cardiol. 2008;1(2):93–100.
4. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, etal. 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 with repair of tetralogy of Fallot: 36-year follow-up 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, etal. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientic 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 the American College of Cardiology/American Heart
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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 congenital heart disease in adults. Am J Roentgenol. 2009;193(2):388–96.
12. Rudski LG, Lai WW, Alalo J, Hua L, Handschumacher MD, Chandrasekaran K, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the 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, etal. 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 Appropriate use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for 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, etal. 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 by the American College of Cardiology Foundation, American Heart Association, European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography, and Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2009;22(9):975–1014.
16. Yoo SJ, Hussein N, Peel B, Coles J, van Arsdell GS, Honjo O, etal. 3D modeling and printing in congeni­tal heart surgery: entering the stage of maturation. Front Pediatr. 2021;9:621672.
17. Sanchez Ramirez CJ, Perez de Isla L.Tetralogy of Fallot: cardiac imaging evaluation. Ann Transl Med. 2020;8(15):966.
18. Gales J, Krasuski RA, Fleming GA. Transcatheter valve replacement for right-sided valve disease in congenital heart patients. Prog Cardiovasc Dis. 2018;61(3–4):347–59.
19. McElhinney DB, Sondergaard L, Armstrong AK, Bergersen L, Padera RF, Balzer DT, etal. Endocarditis after transcatheter pulmonary valve replacement. J Am Coll Cardiol. 2018;72(22):2717–28.
20. Corey KM, Campbell MJ, Hill KD, Hornik CP, Krasuski R, Barker PC, etal. Pulmonary valve endo­carditis: the potential utility of multimodal imaging prior to surgery. World J Pediatr Congenit Heart Surg. 2020;11(2):192–7.
Pre- andIntraprocedural Imaging
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Considerations inParavalvular Leak Closure
AdrianaPostolache, SimonaSperlongano, MathieuLempereur, RalucaDulgheru, FrançoisDamas, NilsDemarnee, andPatrizioLancellotti
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 efcient 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), conrming 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
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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 bileaet 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 signi­cant 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 conrm the diagnosis. A TEE showed normal functioning of the 2 mitral leaets, with a mild (non-signicant) 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, conrmed by color Doppler, due to a calcied 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 andDenitions
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 signicant aortic regurgitation in patients undergoing transcatheter aortic valve implantation (TAVI) has signicantly
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decreased in the last 20years, with 0.6–5.3% of patients undergoing TAVI having at least moder­ate PVL, in more recent trials [36]. 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 calcications, 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. Figure1 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 signi­cant 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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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 signi­cant 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 ofPre-procedural Assessment Before Transcatheter PVL Intervention
The evaluation of para-valvular regurgitation is difcult 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 andNumber oftheJet(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 theSeverity ofPVL
This is without a doubt the most difcult 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: