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Transcatheter Aortic Valve Replacement
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Table 4 The heterogeneous spectrum of bicuspid aortic value morphology
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Adapted and reprinted from Journal of Cardiovascular Computed Tomography, Vol 13/1, Philipp Blanke, Jonathan R.Weir-McCall, Stephan Achenbach, Victoria Delgado, Jörg Hausleiter, Hasan Jilaihawi, Mohamed Marwan, Bjarne L.Norgaard, Niccolo Piazza, Paul Schoenhagen, Jonathon A.Leipsic, Computed tomography imaging in the context of transcatheter aortic valve implantation (TAVI)/transcatheter aortic valve replacement (TAVR): An expert consensus document of the Society of Cardiovascular Computed Tomography, 1–20, Copyright 2019, with permission from Elsevier
“sh mouth” appearance and systolic doming of the valve apparatus with color doppler providing further information in differentiating tricuspid from bicuspid valves in the presence of calcica­tion and reduced leaet mobility [5]. However, anatomic assessment, including bicuspid valve
type, annular shape, and calcium distribution is limited by echocardiography [68] (Fig.23).
Given the diagnosis of severe, symptomatic AS and bicuspid aortic valve, a preprocedural CT scan was ordered and demonstrated an aortic annular area of 394mm2 with a bicuspid type 0
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Fig. 22 2D TEE (left panel) 2D TTE parasternal long axis (right panel) demonstrating severely calcied aortic valve
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d
Fig. 23 Post contrast Pre TAVR planning CT images with measurements performed in the systolic phase of the cardiac cycle. (a) Annular area of 394mm of 71.4mm. (b) The Left main coronary artery ostium is located 16.4mm and the (c) right coronary artery ostium
2
and perimeter
valve and severe nodular calcication at both leaets. There was moderate annular calcica­tion with a calcied nodule protruding into the annular lumen and a short intervalvular brosa. Risk of coronary artery obstruction was low with both right and left main coronary artery heights of 16mm, a sinus of Valsalva diameter averaged at 35 mm and an STJ height of 18 mm. The ascending aorta was dilated but less than 45mm. An orthogonal projection was possible at a left anterior oblique and caudal angulation of 10 and
e f
is 15.6mm above the annular plane suggesting low risk for coronary obstruction. (d) The sinotubular junction measures 28.3× 28.6mm in diameter and is located (e)
18.3 mm above the annular plane. (f) The Sinus of Valsalva is capacious and measures 35.3cm in diameter
21° respectively. Femoral access was feasible bilaterally.
CT is integral in the planning for the TAVR procedure particularly in bicuspid aortic valves due to the heterogeneity of the various bicuspid valve morphologies that has a potential to inuence the patients outcome [66]. MDCT dem­onstrates accurate correlation with surgical nd­ings and has improved diagnostic sensitivity when compared to TTE and TEE [68]. CT or MRI are recommended if echocardiographic
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assessment of the sinus of Valsalva and ascending aorta is suboptimal and inconclusive [4].
CMR provides good assessment of valve mor­phology with the ability to accurately determine velocities and gradients and annular size [68]. However assessment of calcication is limited [68].
Important features to be assessed by 3D imag­ing (ideally by CT but possible by CMR, or 3D TEE as well) include valve morphology [15] and the degree of raphe calcication assessed qualita­tively as mild, moderate and severe. A raphe par­ticularly if calcied may affect TAVR expansion and apposition at the annulus [66, 69] and increases pacemaker requirement risk, risk of device misplacement and regurgitation [69]. The bicuspid aortic valve annulus is relatively larger, often demonstrating an elliptical shape and more often exhibiting severe eccentric calcication when compared to tricuspid valves [65]. Note should be made that non raphe bicommissural bicuspid aortic valves demonstrates a larger sinus diameter but smaller annuli [15]. Landing zone conguration may be tubular, ared or tapered (classied by relative perimeters of the annulus and at the level of the free edge of the leaets) [69]. An aortic root with a tapered conguration may require THV under sizing or sizing at dimen­sions closer to the supra annular measurements [69]. There are two schools of thought regarding annular measurement and include sizing at the annulus and/or supra annular tracing (measuring perimeter and intercommissural distance approx­imately 4mm above annulus) [70]. These may be supplemented by balloon sizing [70]. The BAVARD registry states that annular measure­ments are suitable in almost 90% of cases [70]. Inappropriate oversizing can result in annular rupture and heart block [65]. The annular elliptic­ity, calcium burden and location should be assessed [67]. Heavy and asymmetric calcica­tions are a concern and may result in impaired valve expansion or aortic root injury following a countercoup injury from balloon ination and THV expansion [67]. Calcium located at the commissures can result in perforation whilst bulky calcication located at the tip of a leaet is a risk for coronary obstruction [69]. Spacious
sinuses in patients with BAV is often protective from coronary occlusion [69]. The ascending aorta should be assessed for aortopathy and dila­tation [15]. Optimal projection angles can be obtained using MDCT software providing coax­ial angles with optimal assessment of calcica­tion [70] (Fig.24).
In light of the patient’s likely suitability for TAVR, a pre-procedural angiogram was obtained to evaluate the coronary arteries and further evaluation of the aortic root. There was no sig­nicant coronary artery disease with aortic root calcication (Video 19). The ascending aorta was dilated. Femoral access had favorable imag­ing features bilaterally.
Dierential Diagnosis
Pre echocardiography differentials of lung pathology, mitral valve disease and congestive heart failure were excluded following imaging conrmation of severe aortic stenosis.
Heart Team Approach andDiscussion
Based on the diagnoses of severe AS in a patient with bicuspid aortic valve, reasonable root fea­tures and maximal aortic dimension of 41mm on CT and 42mm on TTE the decision was to pro­ceed with TAVR using a 23mm SAPIEN 3 Ultra.
Heart Team Decision
Bicuspid valves often have a larger annulus, an asymmetric valve orice, are heavily calcied, have a dilated and asymmetric aortic root and ascending aorta [40]. Complications such as sig­nicant PVL, device migration/embolization, non-uniform non circular valve deployment and annular rupture are more common [40]. Success rates are good with newer generation TAVR valves with improved outcomes in Sievers Type 1 L-R fusion [5]. Similar outcomes were achieved in low surgical risk patients with BAV (without raphe or leaet calcication), when compared with TAVR patients with similar clinical prole but with tricuspid valves and treated with SAPIEN 3 THV [71]. Patients with BAV and high surgical risk treated with EvolutR/Evolut PRO demonstrated similar outcomes in terms of 1year post procedural stroke and mortality rates
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Fig. 24 (a) Angiogram of the aortic root with mildly dilated ascending aorta and no signicant adverse root features and coronary ostial heights were at low risk for obstruction. (b–d) Coronary angiogram of the RCA and
when compared to their tricuspid aortic valve counterparts [72].
Denitive criteria between the choice of SAVR and TAVR is still to be decided with SAVR preferred in young patients, patients with aortop­athy and low risk patients [73]. Currently features conducive for TAVR in BAV include a favourable annular dimension, absence of severe leaet and raphe calcication especially in Sievers type 1 and with Sievers type 0 (in the absence of an extreme ellipsoidal annulus) [73]. Of note, in patients with bicuspid morphology treated with TAVR, there is a higher incidence of conduction abnormalities [5] and the presence of severe cal­cication of the raphe and leaets is associated with aortic injury and higher incidence of PVL with a higher 2year mortality especially if both leaet and raphe calcication were present [74]. There is however reduced incidence of PVL with newer generation devices [75].
LM and its branches showed patent vessels with no requirements for percutaneous intervention or bypass grafts. (e and f) patent iliofemoral arteries without signi­cant calcication, tortuosity or stenosis
Intra Procedural Assessment
The aortic valve was predilated using a 20mm balloon (Fig. 25, Video 20). The patient then demonstrated high degree A-V block requiring pacing. A 23mm Sapien 3 valve (2cc) was suc­cessfully deployed under rapid pacing (Fig. 25, Video 21) using uoroscopy.
Balloon Sizing andPre Dilatation inBicuspid Valves
Pre-dilatation is recommended for balloon annu­lar sizing in borderline dimensions, in fused right and non-coronary cusps, when considering sizing to the supra annular space and in the presence of heavily calcied bicuspid aortic valve with a cal­cium score >1000HU (to assist in crossing the native valve and prosthetic valve deployment) [69]. The region of balloon waisting will identify the position of best sealing whilst approximation of calcied leaets to the coronary ostia will
cd
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Fig. 25 (a) Pre deployment balloon valvuloplasty with balloon placed across the aortic valve and inated with displacement of leaet calcication. (b–d) A Sapien 3
assist in determining risk of coronary obstruction [69]. Absence of a leak supports congruent bal­loon and valve orice dimensions [69].
Post Procedural Assessment
Formal postprocedural TTE demonstrated a well seated aortic transcatheter heart valve with a mean gradient of 10 mmHg, Peak velocity of
2.3m/s and an aortic valve area of 1.4cm2 (Fig.
26). Mild paravalvular prosthetic regurgitation
was present and leaet motion was poorly visualized.
bioprosthetic valve was deployed at the annulus with min­imal tailoring in its mid portion
Postprocedural electrophysiology assessment
resulted in permanent pacemaker placement (Fig. 27) 30days post procedure echocardiogram revealed a well seated aortic valve with poor visualization of leaet motion. Mild paravalvular regurgitation persisted and was non-progressive. Aortic peak velocity had increased to 3.3 m/s with a mean gradient of 21 mmHg. There was mild mitral regurgitation with no systolic anterior motion of the mitral valve. Concentric hypertro­phy of the left ventricle remained stable. No peri­cardial effusion, septal defects or intracardiac
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abc
Fig. 26 2D TTE post procedure demonstrating normal valve position (a) with mild paravalvular regurgitation (a and b), mild mitral regurgitation and acceptable hemodynamic parameters (c)
ab c
Fig. 27 (a and b) 2D TTE 30days post TAVR procedure with (a and b) demonstrate persistence of mild paravalvular regurgitation. (c) Continuous wave doppler echo shows an elevated mean gradient at 21mmHg
shunts were present. Pacemaker leads identied in the right atrium and right ventricle.
Similar to the post-procedure evaluation of TAVR valves placed for severe, symptomatic AS in tricuspid aortic valves, post-procedure imaging is based initially on echocardiography. Postprocedural echocardiography should be
Valve dimensions demonstrated under-
expansion (Fig. 28) of the valve in its midportion (17mm diameter) with no thrombus or HALT.No VSD or pericardial effusion present and pace­maker leads were appropriately positioned. Aortic dimensions were stable with no aortic root injury.
focused on valve hemodynamics, the presence of aortic regurgitation, biventricular function, the function of the other three cardiac valves, and evi­dence of any intracardiac complications, such as cardiac perforation. Given the often-ovular nature of the bicuspid valve annulus, assessment for aor­tic valve regurgitation is of particular importance,
Complications inBicuspid Aortic Valves
Patients with BAV have a higher risk of malposi­tion [64], pacemaker rates [70] and paravalvular regurgitation with lower device success rates [15] above and beyond the standard TAVR complica-
tion prole. with posteriorly located regurgitant jets being most difcult to visualize due to metal shadowing from the valve frame. In the present case, an increase in 30-day TAVR gradients as compared to the post-procedure assessment suggested potential valvular dysfunction versus leaet abnormality and prompted a contrasted cardiac CT.
Paravalvular Regurgitation
Paravalvular leak occurs when the THV incom-
pletely approximates annulus and LVOT [47].
Incidence of moderate to severe paravalvular
regurgitation is higher in a bicuspid valve with a
calcied raphe and, higher still, if both the raphe
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Fig. 28 Post procedure post contrast CT performed to assess for valve expansion and exclude complications. (a) Sapien 3 valve 23mm that was underlled by 2cc demon­strates mild tailoring at the mid portion of the valve. (b) Short axis image of the valve at the mid portion demon-
Table 5
Bicuspid aortic valve multimodality imaging
Bicuspid valve ± aortopathy Echocardiography Computed tomography MRI
Pre procedure imaging
Intraprocedure imaging Postprocedure imaging
First line for valvular assessment with high accuracy especially on TEE. Accurate assessment may be limited in the presence of severe calcication. Limited assessment of co-existant aortopathy Similar to tricuspid aortic valves (see case 1) with the exception that TEE may play a larger role in device positioning in the absence of severe calcication to serve as a landmark on uoroscopy Similar to tricuspid aortic valves (see case 1)
and leaets are calcied [74]. Sievers type 1 BAV was associated with a relatively higher rate of PVL in comparison to other classications, how­ever there no signicant difference in paravalvu­lar regurgitation between the self and balloon expandable valves in all classication types [48]. Table5 showing role of Multimodality Imaging in TAVR in Bicuspid Aortic Valve.
strates a diameter of 17.12mm by 19.2mm. Conrming
mild under expansion with no leaet thickening or throm-
bosis. (c) Long axis view of the valve conrms mild under
expansion of the mid portion of the valve
Improved diagnostic sensitivity when compared to echocardiogram. Recommended in assessing sinus of Valsalva and ascending aorta if echo assessment is inconclusive
Good assessment of valve morphology and function as well as aortic morphology and pathology. Limited by calcication
system in self-expanding cases are thought to be
aetiological contributory factors [70].
Features predictive of conduction disturbances include calcication of the LVOT, especially below the non-coronary cusp and severe mitral annular calcication (>180°) [47]. The presence of a short membranous septum [47] less than 8 mm in length is a predictor for high degree AV-Block, especially if found in combination
Post Procedure Pacemaker Requirements
Higher pacemaker rates are documented in patients with bicuspid aortic valves post TAVI more so in balloon expandable instances which has a 23.5% post implantation pacemaker rate [70]. Lack of foreshortening of the stent frame of balloon expandable valves during oversizing and excessive force being applied to the conduction
with low valve implant and an underlying right bundle branch block [15]. Procedural risks include the use of a self expanding THV [47, 76], low valve placement of greater than 4mm below the annulus [47] and annular and LVOT oversizing [76]. Low deployment is the most frequent pre­dictor of LBBB with balloon and self- expandable valves [15]. Reduction of the implant depth sig-
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nicantly reduces mortality and pacemaker requirements [15]. The short term effects of edema, inammation, ischemia or direct trauma may demonstrate recovery thereby negating long term pacemaker dependency post TAVR [76].
Clinical Controversies andPearls
• Trials of FDA approved TAVR valves excluded patients with bicuspid aortic valves. As a result, randomized comparisons of TAVR vs. SAVR for bicuspid aortic valves is lacking.
• Bicuspid aortic valve disease is associated with aortopathy and other left sided cardiac lesions. As a result, careful evaluation of these structures is necessary when planning for treatment of severe symptomatic bicuspid aor­tic valve disease.
Key Points
– The degree of raphe calcication should
be assessed qualitatively as mild, moder­ate, and severe. A raphe especially when calcied may affect expansion and appo­sition of the TAVR at the annulus, risk of requiring pacemaker, device misplace­ment, root injury and regurgitation.
– Non raphe bicommissural bicuspid aor-
tic valve have a larger sinus diameter but smaller annuli.
– Calcium at the commissures can result
in perforation. Bulky calcication at the tip of the leaet is a risk for coronary obstruction.
Aortic Regurgitation
Case Study
Background andDenitions
Aortic regurgitation (AR) refers to the retrograde blood ow from the aorta into the left ventricle in diastole secondary to lack of aortic valve leaet coaptation [77].
Isolated (AR) is less common than symp­tomatic (AS) and affects approximately 13% of patients with pure left sided native valve dis­ease [78]. The estimated prevalence of at least moderate AR in those older than 70years is
2.2% [79]. Less than 1% of transcatheter aortic valve implantation is done for AR in the United States [80]. Chronic AR is clinically asymp­tomatic for prolonged periods. Thereafter patients develop congestive heart failure, sec­ondary to volume overload, left ventricular dysfunction and increased wall stress [81]. Valve replacement should be performed when regurgitation is severe and the patient is symp­tomatic, or when the left ventricle end systolic dimension is >50 mm (index left ventricular end systolic dimension is >25mm/m2) [82] or there is left ventricular systolic impairment [83].
The pathophysiology of AR differs from AS (degeneration and calcic disease [77]) and causes include:
• Congenital [81]
• Bicuspid Valve [80]
• Infectious [81]
• Rheumatic heart disease [80]
• Previous endocarditis [80]
• Vasculitis [81]
• Calcied/myxomatous leaets [77]
• Connective tissue diseases [80]
• Aortic root dilatation [80] secondary to
trauma, dissection, viruses or arteritides [77]
• Radiotherapy [81]
An 83-year-old male patient presented with NYHA class II symptoms without chest pain or syncope. He has had multiple prior hospitalizations for congestive cardiac failure. Physical examination revealed a grade 2 diastolic murmur loudest in the third intercostal space.
Diagnosis andPre-procedural Evaluation
A TTE demonstrated a trileaet, sclerotic aortic valve with mild reduction in cusp motion. There was moderate to severe aortic regurgitation with a regurgitant volume of 45 mL, an EROA of
0.23cm2, a P1/2T of 381ms and a vena contracta
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of 5mm. The left ventricle was severely dilated and globally hypokinetic with left ventricular hypertrophy and a reduced systolic function of 25%. The proximal ascending aorta was dilated, indexed at 20mm/m2.
Transthoracic Echo
TTE is the gold standard for workup [77] and assesses the mechanism and severity of regurgi­tation, valve type, and left ventricle remodelling and function [84]. Leaets are assessed for non­coaptation and prolapse and multiple measures are assessed and integrated to determine the severity of regurgitation [83]. Additionally, left ventricular end systolic dimension, ejection frac­tion, aortic root and ascending aorta are also assessed [83].
Severe AR:
• Ratio of regurgitant jet width to LVOT width 65% [84].
• Vena contracta >0.6cm [83].
• Effective regurgitant orice area ≥0.3 cm2 [84].
• Regurgitant volume >60mL and regurgitant fraction 50% [84].
• Deceleration time (pressure half-time) of the continuous wave aortic regurgitation doppler jet of <200ms [83].
• Reversal of ow in the descending aorta throughout diastole particularly when in con­junction with end diastolic velocity of >20cm/s [83].
Caveats:
• Linear measurements are based on the pre­sumption of a circular orice from a single plane of assessment. The accuracy may there­fore be increased with 3D TTE [83].
• Pressure half time varies with blood pressure and LV compliance and is often lower in acute AR [83].
Transoesophageal Echo
TEE is usually performed if a more detailed assessment of the aortic valve or aortic root is required [84]. Both 2D and 3D TEE provide
diagnostic value in dening mechanisms of AR [84]. Pre procedural TEE provides information for device sizing whilst intraprocedurally it is used to visualise bioprosthetic valve position­ing and assess for immediate complications [83]. TEE can also assess the valve morphology to enable valve preserving repair in some patients, especially those with aneurysms of the aortic root as well as regurgitation in non-calci­ed bicuspid valves [83]. Of note, doppler angles for assessing aortic regurgitation (for calculations such as aortic pressure ½ time or PISA EROA) are usually suboptimal in TEE as compared to TTE and necessitate deep trans­gastric imaging.
Cardiac Magnetic Resonance Imaging
CMR is the gold standard for the assessment of left ventricle volumes and ejection fraction [83]. In AR CMR may be used for valve planimetry, to determine the mechanism and quantify the sever­ity of aortic regurgitations and the presence of aortopathy [84]. CMR is indicated when the echo is suboptimal or when there is a discordance between either echo and clinical ndings or in determining AR severity between echo and dop­pler results [84]. CMR is also indicated when echo is limited in assessing aortopathy or in the presence of multiple valvular pathologies [84]. In addition, CMR is useful in follow up assessment with less variability then echo in determining the severity of AR and LV remodeling and is bene­cial in long term assessment of aortopathy in coexistent AR and a bicuspid aortic valve [84]. Aortic regurgitant volume and fraction is deter­mined by phase contrast velocity mapping of the aortic valve or in the absence of other valvular lesions by determining the difference between the aortic and pulmonic stroke volumes [84]. An aortic regurgitant orice of ≥48cm2 and a holo­diastolic ow reversal in the mid descending aorta where found to be sensitive in detecting and predicting severe AR [84]. Left ventricular dila­tion with end diastolic volume >246mL predicts symptom onset and possible requirement of valve surgery whilst a regurgitation fraction >33% on phase contrast predicts surgical need within 3years [83].
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In the present case, the patient had a sufcient
quality TTE to obviate the need for TEE or CMR.However, since the etiology of the patient’s LV dysfunction wasn’t clear, he underwent a cor­onary artery CT. There was no coronary artery disease, and the patient was categorized as a CAD-RADS 0.
Dierential Diagnosis
The possible diagnoses included coronary artery disease which was excluded with a CT coronary angiogram. Echocardiogram demonstrated fea­tures of severe aortic regurgitation and excluded leaet vegetation. Echocardiogram also excluded co existent signicant mitral valvular disease or ASD. In light of the patient’s worsening aortic regurgitation and the reduced left ventricular function, the work-up for TAVR was commenced (Fig. 29).
A pre TAVR CT was performed and demon-
strated a tricuspid non calcied valve with no annular or LVOT calcication. The systolic annu­lar area was measured at 405mm2. The risk for coronary ostial occlusion is low with the left main and the right coronary artery heights measuring
17.6mm and 17.9mm respectively. The average
SOV dimension is 31mm. The STJ diameter mea­sured 25.9mm. The orthogonal view was obtained
at LAO 16° and CAU 20°, centered onto the right cusp. There was dilatation of the left ventricle, measured as LVEDD of 66mm LVESD of 58mm, and both atria. The ascending aorta was dilated, and this with the arch and descending thoracic and abdominal aorta demonstrated no signicant calcications, stenosis or tortuosity.
Computed Tomography
Computed Tomography is used primarily for pre­procedural planning rather than diagnosis. However, absence of complete coaptation of aortic leaets during diastole can be used to detect aortic regurgitation [85]. An anatomic regurgitant area may be reliable to quantify severity with a value of
0.27 ± 0.16 cm2 suggestive of severe AR [85]. Regardless of symptoms, end—systolic diameter of 55mm or greater or an ejection fraction below 55% are indications for intervention [85]. Risk assessment for coronary obstruction, valve sizing and aortic dimension are assessed similarly to TAVR for aortic stenosis in the native valve.
Heart Team Approach andDiscussion
Only 1in 5 patients with severe AR and left ven­tricular ejection fraction between 30 and 50% are referred to surgery [81]. The co-existence of mul­tiple co-morbidities and the advanced patient age
abc
de
Fig. 29 (a) The annulus measures 405mm2 with a perim- eter of 73mm. (b–d) Low risk of coronary obstruction with coronary artery heights above 12mm and a capa-
f
cious sinus of Valsalva. (e) The RCC centered view pro­vided a plane orthogonal to the annulus. (f) The left ventricle is dilated measuring 66mm on end diastole