Displacement Of Leaflets Research Articles

Surgical Treatment of Unusual Anatomy of the Tricuspid Valve in Ebstein’s Anomaly

Ebstein’s anomaly is a congenital heart disease in which the septal and posterior leaflets of the tricuspid valve are displaced to the apex of the right ventricle, forming its atrialization. In contrast, the anatomical position of the anterior leaflet usually remains normal. The aim. To present and analyze a case of surgical treatment of Ebstein’s anomaly with a rare anatomy – isolated displacement of the anterior leaflet of the tricuspid valve. Case report. We present a rare clinical case of isolated displacement of the anterior leaflet of the tricuspid valve into the right ventricular cavity with the formation of a right atrial aneurysm. The diagnosis was made prenatally and confirmed after the birth of the child by echocardiography. Surgical intervention was postponed and successfully performed at the age of six years due to the progression of tricuspid insufficiency and increase in the right atrial aneurysm. We achieved a reduction in the size of the right atrium (diameter 31 mm, volume 25 ml and area 10 cm2) and minimized insufficiency at the tricuspid valve. The total duration of the surgical intervention was 270 minutes. The intra- and postoperative periods were uneventful. The duration of the patient’s stay in the intensive care unit was 4 days. We believe that this pathology of the anterior leaflet of the tricuspid valve should be interpreted as a variant of Ebstein’s anomaly. Conclusion. Isolated tricuspid valve anterior leaflet displacement with right atrial aneurysm is amenable to surgical correction with good immediate and long-term results.

Impact and limitations of 3D computational modelling in transcatheter mitral valve replacement-atwo-centre Dutch experience.

Transcatheter mitral valve replacement (TMVR) has emerged as aminimally invasive alternative to mitral valve surgery for patients at high or prohibitive operative risk. Prospective studies reported favourable outcomes in patients with annulus calcification (valve-in-mitral annulus calcification; ViMAC), failed annuloplasty ring (mitral valve-in-ring; MViR), and bioprosthetic mitral valve dysfunction (mitral valve-in-valve; MViV). Multi-slice computed tomography (MSCT)-derived 3D-modelling and simulations may provide complementary anatomical perspectives for TMVR planning. We aimed to illustrate the implementation of MSCT-derived modelling and simulations in the workup of TMVR for ViMAC, MViR, and MViV. For this retrospective study, we included all consecutive patients screened for TMVR and compared MSCT data, echocardiographic outcomes and clinical outcomes. Sixteen out of 41patients were treated with TMVR (ViMAC n = 9, MViR n = 3, MViV n = 4). Eleven patients were excluded for inappropriate sizing, 4for anchoring issues and 10for an unacceptable risk of left ventricular outflow tract obstruction (LVOTO) based on 3D modelling. There were 3procedure-related deaths and 1non-procedure-related cardiovascular death during 30days of follow-up. LVOTO occurred in 3ViMAC patients and 1MViR patient, due to deeper valve implantation than planned in 3patients, and anterior mitral leaflet displacement with recurrent basal septum thickening in 1patient. TMVR significantly reduced mitral mean gradients as compared with baseline measurements (median mean gradient 9.5(9.0-11.5) mm Hg before TMVR versus 5.0(4.5-6.0) mm Hg after TMVR, p = 0.03). There was no residual mitral regurgitation at 30days. MSCT-derived 3D modelling and simulation provide valuable anatomical insights for TMVR with transcatheter balloon expandable valves in ViMAC, MViR and MViV. Further planning iterations should target the persistent risk for neo-LVOTO.

Arrhythmic Mitral Valve Prolapse Phenotype: An Unsupervised Machine Learning Analysis Using a Multicenter Cardiac MRI Registry.

Purpose To use unsupervised machine learning to identify phenotypic clusters with increased risk of arrhythmic mitral valve prolapse (MVP). Materials and Methods This retrospective study included patients with MVP without hemodynamically significant mitral regurgitation or left ventricular (LV) dysfunction undergoing late gadolinium enhancement (LGE) cardiac MRI between October 2007 and June 2020 in 15 European tertiary centers. The study end point was a composite of sustained ventricular tachycardia, (aborted) sudden cardiac death, or unexplained syncope. Unsupervised data-driven hierarchical k-mean algorithm was utilized to identify phenotypic clusters. The association between clusters and the study end point was assessed by Cox proportional hazards model. Results A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 female, 230 male) with two phenotypic clusters were identified. Patients in cluster 2 (199 of 474, 42%) had more severe mitral valve degeneration (ie, bileaflet MVP and leaflet displacement), left and right heart chamber remodeling, and myocardial fibrosis as assessed with LGE cardiac MRI than those in cluster 1. Demographic and clinical features (ie, symptoms, arrhythmias at Holter monitoring) had negligible contribution in differentiating the two clusters. Compared with cluster 1, the risk of developing the study end point over a median follow-up of 39 months was significantly higher in cluster 2 patients (hazard ratio: 3.79 [95% CI: 1.19, 12.12], P = .02) after adjustment for LGE extent. Conclusion Among patients with MVP without significant mitral regurgitation or LV dysfunction, unsupervised machine learning enabled the identification of two phenotypic clusters with distinct arrhythmic outcomes based primarily on cardiac MRI features. These results encourage the use of in-depth imaging-based phenotyping for implementing arrhythmic risk prediction in MVP. Keywords: MR Imaging, Cardiac, Cardiac MRI, Mitral Valve Prolapse, Cluster Analysis, Ventricular Arrhythmia, Sudden Cardiac Death, Unsupervised Machine Learning Supplemental material is available for this article. © RSNA, 2024.

Primary Atriopathy in Mitral Valve Prolapse: Echocardiographic Evidence and Clinical Implications.

Prominent multi-scallop systolic leaflet displacement toward the left atrium (atrialization) is typically observed in bileaflet mitral valve prolapse (MVP) with mitral annular disjunction. We hypothesized that mitral leaflet atrialization is associated with an underlying left atrial (LA) myopathy characterized by progressive structural and functional abnormalities, irrespective of mitral regurgitation (MR) severity. We identified 334 consecutive patients with MVP, no prior atrial fibrillation, and comprehensive clinical and echocardiographic data. LA function was assessed by LA reservoir strain, LA function index, and LA emptying fraction. We also classified the stage of LA remodeling based on LA enlargement and LA reservoir strain (stage 1: no remodeling; stage 2: mild remodeling; stage 3: moderate remodeling; and stage 4: severe remodeling). The primary end point was the composite risk of sudden arrhythmic death, heart failure hospitalization, or the new onset of atrial fibrillation. Bileaflet MVP with no or mild MR had a lower LA reservoir strain (P=0.04) and LA function index (P<0.001) compared with other MVP subtypes. In multivariable linear regression adjusted for cardiovascular risk factors and MR ≥moderate, bileaflet MVP remained significantly associated with lower LA function parameters (all P<0.05). There was a significant increase in the risk of events as the LA reservoir strain and LA remodeling stage increased (P<0.001). In multivariable analysis, stage 4 of LA remodeling remained significantly associated with a higher risk of events compared with stage 1 (hazard ratio, 6.09 [95% CI, 1.69-21.9]; P=0.006). In a large MVP registry, bileaflet involvement is associated with reduced LA function regardless of MR severity, suggesting a primary atriopathy in this MVP subtype. Abnormal LA function, particularly when assessed through a multiparametric approach, is linked to a higher risk of cardiovascular events and may improve risk stratification in MVP, even in those without significant MR.

On the role of aortic valve architecture for physiological hemodynamics and valve replacement, Part II: Spectral analysis and anisotropy

Severe aortic valve stenosis can lead to heart failure and aortic valve replacement (AVR) is the primary treatment. However, increasing prevalence of aortic stenosis cases reveal limitations in current replacement options, necessitating improved prosthetic aortic valves. We investigate flow disturbances downstream of severe aortic stenosis and two bioprosthetic aortic valve (BioAV) designs using advanced energy-based analyses. Three-dimensional high-fidelity fluid–structure interaction simulations have been conducted and a dedicated and novel spectral analysis has been developed to characterise the kinetic energy (KE) carried by eddies in the wavenumber space. In addition, new field quantities, i.e. modal KE anisotropy intensity as well as normalised helicity intensity, are introduced. Spectral analysis shows kinetic energy (KE) decay variations, with the stenotic case aligning with Kolmogorov’s theory, while BioAV cases differing. We explore the impact of flow helicity on KE transfer and decay in BioAVs. Probability distributions of modal KE anisotropy unveil flow asymmetries in the stenotic and one BioAV cases. Moreover, an inverse correlation between temporally averaged modal KE anisotropy and normalised instantaneous helicity intensity is noted, with the coefficient of determination varying among the valve configurations. Leaflet dynamics analysis highlights a stronger correlation between flow and biomechanical KE anisotropy in one BioAV due to higher leaflet displacement magnitude. These findings emphasise the role of valve architecture in aortic turbulence as well as its importance for BioAV performance and energy-based design enhancement.

A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly.

Background: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient's prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. Methods and results: 71 pts (median age 53 years; range 24-84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (p = 0.9) and between ablated and non-ablated EA individuals (p = 0.89). Conclusions: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.

Anaesthetic management of a case of Ebstein’s anomaly undergoing a non cardiac surgery

Ebstein’s anomaly is a rare congenital heart disease characterized by apical displacement of the Tricuspid valve leaflets leading to Tricuspid Regurgitation. Here we report the successful management of a case of a patient with Ebstein’s anomaly who underwent Defunctioning Tranverse Colostomy. Given the complexity of Ebstein's anomaly and the potential for associated cardiac abnormalities, it is crucial to have a multidisciplinary team involved in the patient's care.

Stroke in mitral valve prolapse: risk factors and left atrial function in cryptogenic versus non-cryptogenic ischemic subtypes.

Mitral valve prolapse (MVP) has been associated with an increased risk of ischemic stroke. Older age, thicker mitral leaflets, and significant mitral regurgitation (MR) leading to atrial fibrillation have been traditionally considered risk factors for ischemic stroke in MVP. However, specific risk factors for MVP-stroke subtypes are not well defined. The aim of this study is to evaluate clinical and echocardiographic parameters, including left atrial (LA) function, in MVP with cryptogenic (C) vs. non-cryptogenic (NC) stroke. In this case-control matched study, MVPs were identified in consecutive echocardiograms obtained after a stroke from January 2013 to December2016 at the University of California, San Francisco. MVP was defined as leaflet displacement ≥2 mm in the parasternal long-axis view at end-systole. Age/gender matched MVPs without stroke and healthy controls without MVP were also identified. We analyzed LA end-systolic/diastolic volume index, emptying fraction (LAEF), function index (LAFI), and global longitudinal strain in all MVPs and controls. We also measured left ventricular (LV) volume indexes, mass index, ejection fraction (EF), degree of MR and leaflet thickness. We identified a total of 30 MVPs (age 70 ± 12, 50% females) with stroke (11 with C- and 19 with NC-stroke), 20 age/gender matched MVPs without a stroke and 16 controls. MVPs without stroke had lower BMI, less hypertension but more MR (≥moderate in 45% vs. 17%), more abnormal LA function (lower LAEF, LAFI) and larger LV volumes/mass (all p < 0.05) when compared to MVPs with stroke. Leaflet thickness was overall mild (<3 mm) and similar in the 2 groups. Within the MVP stroke group, NC-stroke had higher BMI, more hypertension and more atrial fibrillation compared to C-stroke. In the variables tested, patients with C-stroke did not differ from controls. MVP-related MR may be protective against stroke despite abnormal LA function. Risk of NC-stroke in MVP is related to common stroke risk factors rather than mitral valve leaflet thickness. The etiology of C-stroke in MVP warrants further studies.

Relationship between mitral valve echocardiographic parameters and interventional septal reduction therapy success in patients with hypertrophic obstructive cardiomyopathy - a pilot study

Abstract Funding Acknowledgements Type of funding sources: None. Background The mitral valve (MV) has an important contribution to left ventricular outflow tract obstruction (LVOTO) in patients with hypertrophic obstructive cardiomyopathy (HOCM). Several parameters were described to be significantly different between obstructive and non-obstructive forms of HCM, like the aortomitral angle, anterior mitral leaflet (AML) and LVOT size, anterior displacement of the coaptation line, annular related parameters, etc. The redundant length of the AML beyond the coaptation point and the entire AML length are important in the decision to also approach the mitral valve when surgical septal reduction therapy (SRT) is considered, to increase the success rate. Data is scarce regarding mitral valve related predictors of interventional SRT response. This is important as certain patients have a suboptimal response regardless of the proper coronary anatomy and branch targeting. Purpose To evaluate the possible differences in certain mitral valve parameters in interventional SRT responders versus non-responders. Methods We retrospectively included in the study a number of 21 consecutive HOCM patients treated with alcohol septal ablation (ASA). All the procedures were guided with contrast echocardiography besides fluoroscopy and all patients had a proper distribution of at least 1 septal branch. They were divided in two groups- responder group (15 patients) with a non-significant residual LVOT PG (&amp;lt;50mmHg/&amp;gt;50% reduction) and non-responder group (6 patients) with a significant residual LVOT PG (&amp;gt;50mmHg/&amp;lt;50% reduction) and/or early rebound. We accounted for the residual LVOT PG evaluated by continuous Doppler at least 6 months after SRT. The groups were compared regarding LV dimensions and ejection fraction and mitral valve annulus/ leaflet size and displacement of the coaptation line, evaluated by bidimensional transthoracic echocardiography, before the intervention. Results The most hypertrophied part of the LV was the basal interventricular septum (IVS). The two groups were similar regarding age, comorbidities, LV dimensions and ejection fraction and baseline LVOT PG. Also, there was no significant difference between AML length, posterior mitral leaflet (PML) length, AML/PML, MV annulus. The redundant length of the AML was significantly lower in responders whereas anterior displacement of the coaptation point (projection of AML/projection of PML on the MV annulus) was less intense in responders and almost reached statistical significance. (Table) Interestingly, we found a baseline AML/PML projection &amp;lt;1 in all non-responder patients and only in 2 responder patients (13.33%) (p&amp;lt;0.001). Conclusion So far, significant anterior displacement of the coaptation point and the residual AML length may have an important impact on the response to ASA in HOCM patients. This pilot study could be an important start point for larger prospective studies to confirm and evaluated the prediction value of these parameters among others.

Predictors and clinical implications of residual mitral regurgitation following left ventricular assist device therapy

BackgroundCorrection of mitral regurgitation (MR) at the time of left ventricular assist device (LVAD) implantation remains controversial. There is conflicting evidence regarding the clinical impact of residual MR, and studies...

P162 ARRHYTHMIC MITRAL VALVE PROLAPSE OCCURRING AS ABORTED SUDDEN CARDIAC DEATH

Abstract Mitral valve prolapse (MVP) is the common valvular heart disease, with a prevalence about 1–3% in the population. MVP is characterized on echocardiography by an abnormal systolic displacement of mitral valve leaflets into left atrium (LA) ≥ 2 mm above the mitral annulus. We described a clinical case of a 15 years–old boy with a first clinical presentation of out–of–hospital cardiac arrest related to ventricular fibrillation (VF) with a successful resuscitation after 2 DC shocks. Retrospectively, an episode of loss of awareness reported as post–minational. No familiar history of sudden cardiac death (SCD) or MVP was noticed. In the emergency room, the patient was hemodynamically unstable and was sedated and intubated. The surface electrocardiogram (ECG) showed sinus rhythm, QRS with fragmentation, and frequent monomorphic isolated premature ventricular contractions (PVCs) with RBBB/LAFB configuration.Frequent monomorphic PVCs with bigeminy was observed during continuous ECG monitoring, so the patient was treated with magnesium sulphate and metoprolol. The patient was subsequently weaned from ventilation support, presenting good gas exchange and without neurological sequalae. The 24h Holter monitoring showed an average of 53 bpm, extremely rare supraventricular extrasystoles, high incidence of monomorphic PVCs (23% of the total beats) with 4 couplets. Transthoracic echocardiography documented normal size and thickness with systolic function (GLS –21%, mild asynchrony of posterior basal segments), bi–leaflet prolapse associated to mild–moderate mitral regurgitation, and mitral annular disjunction (MAD) of 6.2 mm. The late gadolinium enhancement cardiac magnetic resonance showed bi–leaflet prolapse with mild insufficiency and maximum MAD 9 mm, mild LA dilatation, and slightly dilated both ventricles, without regional wall motion abnormalities; no scar or fibrosis at the late gadolinium enhancement was observed. According to guidelines, the patient received a subcutaneous implantable cardiac defibrillator for the secondary prevention of SCD and maintained under betablockers in order to control the arrhythmic burden. The genetic sampling did not show any pathogenetic mutation potentially related to cardiomyopathies or channelopathies. This case show an arrhythmic MVP complex with probably posterior papillary muscle source. The early PVCs with R/T constituted a possible trigger of VF. The betablocker therapy exhibited a full suppression of extrasystolic burden.

Abnormal Mechanics Relate to Myocardial Fibrosis and Ventricular Arrhythmias in Patients With Mitral Valve Prolapse.

The relation between ventricular arrhythmia and fibrosis in mitral valve prolapse (MVP) is reported, but underlying valve-induced mechanisms remain unknown. We evaluated the association between abnormal MVP-related mechanics and myocardial fibrosis, and their association with arrhythmia. We studied 113 patients with MVP with both echocardiogram and gadolinium cardiac magnetic resonance imaging for myocardial fibrosis. Two-dimensional and speckle-tracking echocardiography evaluated mitral regurgitation, superior leaflet and papillary muscle displacement with associated exaggerated basal myocardial systolic curling, and myocardial longitudinal strain. Follow-up assessed arrhythmic events (nonsustained or sustained ventricular tachycardia or ventricular fibrillation). Myocardial fibrosis was observed in 43 patients with MVP, predominantly in the basal-midventricular inferior-lateral wall and papillary muscles. Patients with MVP with fibrosis had greater mitral regurgitation, prolapse, and superior papillary muscle displacement with basal curling and more impaired inferior-posterior basal strain than those without fibrosis (P<0.001). An abnormal strain pattern with distinct peaks pre-end-systole and post-end-systole in inferior-lateral wall was frequent in patients with fibrosis (81 versus 26%, P<0.001) but absent in patients without MVP with basal inferior-lateral wall fibrosis (n=20). During median follow-up of 1008 days, 36 of 87 patients with MVP with >6-month follow-up developed ventricular arrhythmias associated (univariable) with fibrosis, greater prolapse, mitral annular disjunction, and double-peak strain. In multivariable analysis, double-peak strain showed incremental risk of arrhythmia over fibrosis. Basal inferior-posterior myocardial fibrosis in MVP is associated with abnormal MVP-related myocardial mechanics, which are potentially associated with ventricular arrhythmia. These associations suggest pathophysiological links between MVP-related mechanical abnormalities and myocardial fibrosis, which also may relate to ventricular arrhythmia and offer potential imaging markers of increased arrhythmic risk.

Prevalence and Clinical Implications of Tricuspid Valve Prolapse Based on Magnetic Resonance Diagnostic Criteria

BackgroundTricuspid valve prolapse (TVP) is an uncertain diagnosis with unknown clinical significance because of a scarcity of published data. ObjectivesIn this study, cardiac magnetic resonance was used to: 1) propose diagnostic criteria for TVP; 2) evaluate the prevalence of TVP in patients with primary mitral regurgitation (MR); and 3) identify the clinical implications of TVP with regard to tricuspid regurgitation (TR). MethodsForty-one healthy volunteers were analyzed to identify normal tricuspid leaflet displacement and propose criteria for TVP. A total of 465 consecutive patients with primary MR (263 with mitral valve prolapse [MVP] and 202 with nondegenerative mitral valve disease [non-MVP]) were phenotyped for the presence and clinical significance of TVP. ResultsThe proposed TVP criteria included right atrial displacement of ≥2 mm for the anterior and posterior tricuspid leaflets and ≥3 mm for the septal leaflet. Thirty-one (24%) subjects with single-leaflet MVP and 63 (47%) with bileaflet MVP met the proposed criteria for TVP. TVP was not evident in the non-MVP cohort. Patients with TVP were more likely to have severe MR (38.3% vs 18.9%; P < 0.001) and advanced TR (23.4% of patients with TVP demonstrated moderate or severe TR vs 6.2% of patients without TVP; P < 0.001), independent of right ventricular systolic function. ConclusionsTR in subjects with MVP should not be routinely considered functional, as TVP is a prevalent finding associated with MVP and more often associated with advanced TR compared with patients with primary MR without TVP. A comprehensive assessment of tricuspid anatomy should be an important component of the preoperative evaluation for mitral valve surgery.

Prevalence of Mitral Valve Prolapse and Its Adverse Sequelae in Healthy Adult Yemenis

Background: Mitral valve prolapse is a common cardiac disease with frequent complications.&#x0D; Objectives: To study the prevalence of mitral valve prolapse in healthy Yemenis associated with the prevalence of its adverse sequelae.&#x0D; Methods: We performed echocardiography for 1164 healthy Yemeni individuals: 615 women and 549 men (mean age ± SD 45.7 ± 10 years).Classical mitral valve prolapse was defined as a superior displacement of mitral valve leaflets at least 2 mm with a maximal leaflets thickness of at least 5 mm during diastasis. On the other hand, nonclassic prolapse was defined as displacement of more than 2 mm with a maximal thickness of less than 5 mm.&#x0D; Results: 74 subjects (6.3 percent) had mitral valve prolapse, 40 (3.4 percent) had classic prolapse and 34 (2.9 percent) had nonclassic prolapse. None of the patients with prolapse had a history of heart failure nor anyone had atrial fibrillation except one patient (1.3 percent) had cerebrovascular disease and two patients (2.7 percent) had syncope, as compared with unadjusted prevalence of these findings on the individuals without prolapse of 0.0, 0.0, 0.0 and 1.3 percent, respectively. Chest pain, dyspnea and electrocardiographic change frequencies were similar between two groups.&#x0D; Conclusion: The prevalence of mitral valve prolapse in adult Yemenis was 6.3 percent, which is similar to the results of other studies in different countries but with low prevalence of adverse sequelae in comparison to those studies. &#x0D;

Coronary access after repeat transcatheter aortic valve replacement in patients of small body size: A simulation study

BackgroundCoronary artery access after repeat transcatheter aortic valve replacement (TAV-in-TAV) is reportedly more difficult because leaflet displacement of the first transcatheter heart valve (THV) impairs coronary cannulation; however, its effects in small patients are unknown. This study aimed to simulate coronary accessibility after TAV-in-TAV in patients of small body size. MethodsWe retrospectively analyzed computed tomography scans after initial THV implantation and classified patients by THV and coronary artery location, valve-to-aorta distance, and valve-to-coronary distance. Risks were compared between the SAPIEN and CoreValve/Evolut series, among THV generations, and between bicuspid and tricuspid aortic valves in the CoreValve/Evolut series. ResultsA total of 254 patients (SAPIEN series, n = 164; CoreValve/Evolut series, n = 90) were enrolled. The average body surface area of the patients was 1.44 m2. Patients were classified as “feasible” (26%), “theoretically feasible with low risk” (19.7%), “theoretically feasible with high risk” (8.7%), or “unfeasible” (45.8%). The “unfeasible” rate was significantly higher in the CoreValve/Evolut series than in the SAPIEN series (78.9% vs 26.2%; P < .001). A significantly higher “unfeasible” rate was identified in the current model of SAPIEN (SAPIEN, 8.3%; SAPIENXT, 1.8%; SAPIEN3, 48.2%; P < .001), but not in the CoreValve/Evolut series (CoreValve, 83.3%; Evolut R, 80.0%; Evolut PRO, 71.4%; P = .587). Patients with a bicuspid aortic valve had a lower “unfeasible” rate compared to those with a tricuspid aortic valve (60.0% vs 86.2%; P = .014). ConclusionsPatients of small body size may have a high probability of “unfeasible” coronary access after TAV-in-TAV, especially when treated with current high-frame devices, suggesting the need for careful strategic planning for initial THV implantation.

Ebstein's anomaly of the tricuspid valve management – the importance of right ventricle assessment

Abstract Background Ebstein's anomaly (EA) is a rare congenital heart disease (CHD) characterized by well known echocardiographic (ECHO) features. The golden standard method for evaluating these patients is cardiac magnetic resonance (CMR) although not easily accessible. The aim of the study was to evaluate the correlation between ECHO and CMR parameters and to determine the optimal parameters for long-term follow-up. Performed was a retrospective cross-section analysis from our registry, which we consider national, as we serve as the only national adult CHD centre. Material and methods Of the 48 patients in the EA national registry, 28 non-operated adults (60% male, median age 39 (18–64) years) with performed CMR were included. Analyzed were ECHO and CMR right ventricular (RV) characteristics, NTproBNP and 6-minute walking distance (6MWD). ECHO parameters were evaluated as follows: 1. morphometric [tricuspid valve septal cusp displacement (mm), long-axis RV end-diastolic dimension (RVEDD,mm), long-axis end-diastolic right to left ventricle diameter ratio (RV/LV)] and 2. functional [tricuspid annular plane systolic excursion (TAPSE,mm), RV fractional area change (FAC,%), RV tissue Doppler myocardial velocity (m/s)]. CMR-derived analyzed paramaters were: ejection fraction (EF,%) and end-diastolic volume (EDV,ml) indexed per body surface area (BSA,m2). According to CMR-derived EDV, patients were divided into groups: 1. normal size RV (55–105ml/m2), 2. dilated RV (&amp;gt;105ml/m2) and 3. small RV (&amp;lt;55ml/m2). Results For ECHO morphometric measurements, Spearman's correlation (R) showed strong, statistically significant correlation of CMR-derived EDV with ECHO-based RV/LV ratio (R=0.6989, P&amp;lt;0.0001); and moderate, statistically significant correlation with ECHO-based RVEDD (R=0.55, P=0.004); No significant correlations between CMR-derived EF and ECHO parameters were found. CMR-derived EF and EDV were independent of the severity of septal leaflet displacement; CMR-derived EF was shown to be conditional to RV EDV, with mean EF in Groups 1/2/3 as follows: 57%/36.5%/25.8% (P=0.0013). CMR-derived Group 3 (small RV) compared to Groups 1 and 2 exhibited worst 6MWD (mean 416m) and highest NTproBNP (mean 1021ng/l) though without statistical significance. Conclusions The assessment of RV in EA seems more critical than the morphology of the tricuspid valve itself. Therefore, as in the context of our results, more emphasis needs to be placed on RV size. In particular, small-capacity RV, as we found, showed strong relation to reduced RV function and some (though not significant) to NTproBNP and 6MWD. CMR-derived EDV and EF are accepted as the gold standard method for RV evaluation. Since we detected significant correlation of CMR with simply gained ECHO parameters (RV/LV ratio and RVEDD), we assume that easily accessible ECHO remains a pertinent method in routine follow-up, able to reflect the complex hemodynamic situation in EA. Funding Acknowledgement Type of funding sources: None.

Ebstein anomaly, a rare congenital cause of heart failure coexisting with Chiari network

Abstract Ebstein’s anomaly is a rare congenital condition that presents with an apical displacement of the tricuspid valve leaflets. Much rarer is its coexistence with Chiari network (CN), and only a very few cases had been documented in the literature. We report a 30-year-old man who presented with a 5-month history of progressively worsening abdominal distension with associated weight loss, easy fatigability, early satiety, and loss of appetite. A chest X-ray revealed cardiomegaly with right ventricular preponderance. A 12-lead electrocardiogram showed atrial fibrillation. Transthoracic echocardiography revealed apical displacement of the tricuspid valve, severe right atrial enlargement, a small right ventricle, atrialization of the right ventricle, and CN. A diagnosis of Ebstein anomaly coexisting with CN in atrial fibrillation was made. To our knowledge, this is the first reported case of Ebstein’s anomaly coexisting with CN in Nigeria.

Ebstein Anomaly of the Tricuspid Valve with Intact Inter-Atrial Septum and Cor Triatriatum Dexter in an Asymptomatic Adult

Ebstein anomaly of the tricuspid valve is characterized by apical displacement of septal and posterior leaflets of the tricuspid valve leading to the atrialization of right ventricular cavity and severe low-pressure tricuspid regurgitation. Cor triatriatum dexter is a rare congenital cardiac anomaly in which a membranous structure divides the right atrium into two chambers. This combination is rare and very few cases have been described in the literature. We herein report an asymptomatic young female who was incidentally detected to have these anomalies with intact atrial septum. Echocardiographic examination showed tri-chambered right atrium consisting of atrialized right ventricle, anterior right atrial chamber, and the posterior right atrial sinus portion.

Mitral valve prolapse, mitral annular disjunction, left ventricular basal hypertrophy and ventricular repolarization abnormalities in Marfan patients

Mitral valve prolapse (MVP) has been associated with ventricular arrhythmia, originating from inferolateral left ventricle (LV) wall. However, its mechanism remains unclear. To take advantage of the high prevalence of MVP in Marfan syndrome (MFS) to study the relationship of MVP, mitral annular disjunction (MAD), LV basal hypertrophy and ECG abnormalities. We included all MFS patients (≥ 14 yo) without a history of thoracic surgery seen in our center between 2015 and 2017. MVP was identified on echocardiography according to Levine definition and the other cases of abnormal systolic mitral leaflet displacement were defined as billowing. Basal inferolateral hypertrophy (BILH) was defined as basal inferolateral thickness ≥ 12 mm and basal to mild wall thickness ratio ≥ 1.5. QTc was measured on rest 12-lead ECG ( Fig. 1 ). Two hundred and fifty MFS patients were included. Billowing or MVP (BMVP) was present in 187 (74.80%) patients. MAD was present in 52/235 (22.13%) and was associated with BMVP in all cases. End-systole mitral annular diameter was larger when BMVP was present (mean: 34.82 mm vs. 30.53 mm, P < 0.0001) and in MAD+ than in MAD− (mean: 37.14 mm vs. 32.46 mm, P < 0.0001) with a correlation between MAD length and end-systole mitral annular diameter (r = 0.395 P < 0.0001). Whereas mitral annular diameter decreased in systole in MAD−, it increased in MAD+ (mean mitral annular diameter (diastolic–systolic): 3.69 mm vs. −0.87 mm, P < 0.0001). BILH was present in 18/175 (10.29%) patients with BMVP vs. in 1/59 (1.69%) without ( P = 0.0367) and in 9/50 (18%) MAD+ vs. 10/174 (5.75%) MAD− ( P = 0.006). No electric abnormality on ECGs was associated with MAD or BMVP. In contrast, patients with BILH had a longer QTc than patients without HBIL (mean: 426ms vs. 411.4ms, P = 0.0220). In MFS population, MAD is associated with BMVP, systolic mitral annular dilatation, and BILH but not with ECG abnormalities. Only patients with BILH present a QTc prolongation.

An Abnormal Echocardiogram and Telemetry Tracing in a Late Preterm Infant.

An Abnormal Echocardiogram and Telemetry Tracing in a Late Preterm Infant.