What Do We Know So Far About Ventricular Arrhythmias and Sudden Cardiac Death Prediction in the Mitral Valve Prolapse Population? Could Biomarkers Help Us Predict Their Occurrence?

PURPOSE OF THE REVIEW: To summarize currently available data on the topic of mitral valve prolapse (MVP) and its correlation to the occurrence of atrial and ventricular arrhythmias. To assess the prognostic value of several diagnostic methods such as transthoracic echocardiography, transesophageal echocardiography, cardiac magnetic resonance, cardiac computed tomography, electrocardiography, and electrophysiology concerning arrhythmic episodes. To explore intra and extracellular biochemistry of the cardiovascular system and its biomarkers as diagnostic tools to predict rhythm disturbances in the MVP population. RECENT FINDINGS: MVP is a common and mainly benign valvular disorder. It affects 2-3% of the general population. MVP is a heterogeneous and highly variable phenomenon with three structural phenotypes: myxomatous degeneration, fibroelastic deficiency, and forme fruste. Exercise intolerance, supraventricular tachycardia, and chest discomfort are the symptoms that are often paired with psychosomatic components. Though MVP is thought to be benign, the association between isolated MVP without mitral regurgitation (MR) or left ventricle dysfunction, with ventricular arrhythmia (VA) and sudden cardiac death (SCD) has been observed. The incidence of SCD in the MVP population is around 0.6% per year, which is 6 times higher than the occurrence of SCD in the general population. Often asymptomatic MVP population poses a challenge to screen for VA and prevent SCD. Therefore, it is crucial to carefully assess the risk of VA and SCD in patients with MVP with the use of various tools such as diagnostic imaging and biochemical and genetic screening.

Quantification of Mitral Regurgitation in Mitral Valve Prolapse by Three-Dimensional Vena Contracta Area: Derived Cutoff Values and Comparison With Two-Dimensional Multiparametric Approach.

BACKGROUND: Echocardiographic grading of mitral regurgitation (MR) in mitral valve prolapse (MVP) is challenging. Three-dimensional (3D) vena contracta area (VCA) has been proposed as a valuable method. However, data defining the cutoff values of severity and validation in the subset of patients with MVP are scarce. The aim of this study was to validate the 3D VCA by 3D color-Doppler transesophageal echocardiography (TEE) in patients with MVP and to define the cutoff values of severity grading. The secondary aim was to compare 3D VCA to the effective regurgitant orifice area estimation by proximal isovelocity surface area (EROA-PISA) method. METHODS: A total of 1,138 patients with at least moderate MR who underwent TEE were included. Three-dimensional VCA was measured, and the cutoff value and area under the curve (AUC) for the prediction of severe MR were estimated by receiver operating characteristic curve using a guideline-suggested multiparametric approach as the reference standard. In a subgroup of patients, 3D regurgitant volume (RV) and 3D fraction were calculated from mitral and left ventricular outflow tract stroke volumes to further validate 3D VCA against a 3D volumetric reference standard. RESULTS: The optimal 3D VCA cutoff value for predicting severe MR was 0.45 cm2 (specificity, 0.87; sensitivity, 0.90) with an AUC of 0.95 using a multiparametric approach as reference. Three-dimensional VCA had a good linear correlation with EROA-PISA (r = 0.62, P < .05) with larger values compared to EROA-PISA (0.63 cm2 vs 0.44 cm2, P < .05). A cutoff of 0.50 cm2 (AUC of 0.84; sensitivity, 0.78; specificity, 0.78) predicts an EROA-PISA of 0.40 cm2. Three-dimensional VCA had a good linear correlation with 3D RV (r = 0.56, P < .01), with an AUC of 0.86 to predict a 3D fraction >50%. CONCLUSIONS: The present study suggests 0.45 cm2 as the best cutoff value of 3D VCA to define severe MR in patients with MVP, showing an optimal agreement with the reference standard multiparametric approach and 3D RV.

Determinants of Ventricular Arrhythmias in Mitral Valve Prolapse.

BACKGROUND: Mitral valve prolapse (MVP) may be associated with ventricular arrhythmias (VA) even in the absence of significant valvular regurgitation. Curling, mitral annulus disjunction (MAD) and myocardial fibrosis (late gadolinium enhancement [LGE]) may account for arrhythmogenesis. OBJECTIVES: This study investigated the determinants of VA in patients with MVP without significant regurgitation. METHODS: This study included 108 patients with MVP (66 female; median age: 48 years) without valve regurgitation. All patients underwent 12-lead electrocardiography, 12-lead 24-hour electrocardiographic Holter monitoring, exercise stress test, and cardiac magnetic resonance. Patients were divided into 2 groups (arrhythmic and no-arrhythmic MVP), according to the presence of VA with a right bundle branch block pattern. RESULTS: The 62 patients (57%) with arrhythmic MVP showed: 1) higher MAD (median length: 6.0 vs 3.2 mm; P = 0.017); 2) higher prevalence of curling (79% vs 52%; P = 0.012); and 3) higher prevalence of left ventricular LGE (79% vs 52%; P = 0.012). Mediation analysis showed that curling had both a direct (P = 0.03) and indirect effect mediated by LGE (P = 0.04) on VA, whereas the association between MAD and VA was completely mediated by LGE. Patients with severe VA showed more pronounced morphofunctional alterations, in terms of MAD (7.0 vs 4.6 mm; P = 0.004) and presence and severity of curling (respectively, 91% vs 64%; P = 0.010; and 4 vs 3 mm; P = 0.004), compared to those without severe VA. CONCLUSIONS: In patients with MVP the occurrence of VA with right bundle branch block morphology is the expression of more severe morphologic, mechanical, and tissue alterations. Curling has both a direct and an indirect effect on VA.

Usefulness of cardiac magnetic resonance images for prediction of sudden cardiac arrest in patients with mitral valve prolapse: a multicenter retrospective cohort study.

BACKGROUND: An association has been identified between mitral valve prolapse (MVP) and sudden cardiac arrest (SCA), and ventricular arrhythmias (VA). This study aimed to elucidate predictive factors for SCA or VA in MVP patients. METHODS: MVP patients who underwent cardiac magnetic resonance (CMR) were retrospectively included. Patients with other structural heart disease or causes of aborted SCA were excluded. Clinical characteristics (sex, age, body mass index, histories of diabetes, hypertension, and dyslipidemia) and electrocardiographic (PR interval, QRS duration, corrected QT interval, inverted T wave in the inferior leads, bundle branch block, and atrial fibrillation), echocardiographic [mitral regurgitation grade, prolapsing mitral leaflet, and right ventricular systolic pressure (RVSP)], and CMR [left atrial volume index, both ventricular ejection fractions, both ventricular end-diastolic and systolic volume indexes, prolapse distance, mitral annular disjunction, systolic curling motion, presence of late gadolinium enhancement (LGE), LGE volume and proportion] parameters were analyzed. RESULTS: Of the 85 patients [age, 54.0 (41.0-65.0) years; 46 men], seven experienced SCA or VA. Younger age and wide QRS complex were observed more often in the SCA/VA group than in the no-SCA/VA group. The SCA/VA group exhibited lower RVSP, more systolic curling motion and LGE, greater LGE volume, and higher LGE proportion. The presence of LGE [hazard ratio (HR), 19.8; 95% confidence interval (CI) 2.65-148.15; P = 0.004], LGE volume (HR 1.08; 95% CI 1.02-1.14; P = 0.006) and LGE proportion (HR 1.32; 95% CI 1.08-1.60; P = 0.006) were independently associated with higher risk of SCA or VA in MVP patients together with systolic curling motion in each model. CONCLUSIONS: The presence of systolic curling motion, high LGE volume and proportion, and the presence of LGE on CMR were independent predictive factors for SCA or VA in MVP patients.

Comparison of electrocardiographic parameters in dogs with different stages of myxomatous mitral valve disease.

This study evaluated changes in electrocardiographic (ECG) parameters according to the stage of myxomatous mitral valve disease (MMVD) in dogs, as well as the utility of ECG parameters as prognostic indicators for congestive heart failure (CHF). Medical records of dogs with MMVD were retrospectively searched. Dogs with MMVD (N = 101) were classified into stages B [B1 (n = 52) and B2 (n = 23)] and C (n = 26) according to the American College of Veterinary Internal Medicine guidelines. Baseline variables were collected; these included signalment, radiographic, echocardiographic, and ECG parameters. Corrected QT intervals (QTc) were calculated using the logarithmic (QTc1) and Fridericia (QTc2) formulas. The P wave duration, QTc1, and QTc2 were significantly longer in stage C than in stage B. The P wave duration cutoff of 43.5 ms had a diagnostic accuracy of 65% for differentiating CHF, with a sensitivity of 63% and a specificity of 90%. A cutoff value of 307.8 ms for QTc1 yielded a sensitivity of 62%, a specificity of 76%, and a diagnostic accuracy of 78%, and a cutoff value of 239.2 ms for QTc2 yielded a sensitivity of 62%, a specificity of 83%, and a diagnostic accuracy of 77% for diagnosing CHF. Therefore, prolonged P wave and QTc in dogs with MMVD may facilitate the prediction of CHF. Electrocardiography could provide clinicians with a readily available and cost-effective screening tool for predicting CHF, if the usefulness of ECG parameters can be verified.

Cette étude a évalué les changements des paramètres électrocardiographiques (ECG) en fonction du stade de la maladie de la valve mitrale myxomateuse (MMVD) chez le chien, ainsi que l'utilité des paramètres ECG en tant qu'indicateurs pronostiques de l'insuffisance cardiaque congestive (ICC). Les dossiers médicaux des chiens atteints de MMVD ont été consultés rétrospectivement. Les chiens atteints de MMVD (N = 101) ont été classés en stades B [B1 (n = 52) et B2 (n = 23)] et C (n = 26) selon les directives de l'American College of Veterinary Internal Medicine. Les variables de base ont été collectées; celles-ci comprenaient le signalement, ainsi que les paramètres radiographiques, échocardiographiques et ECG. Les intervalles QT corrigés (QTc) ont été calculés à l'aide des formules logarithmiques (QTc1) et Fridericia (QTc2). La durée de l'onde P, QTc1 et QTc2 étaient significativement plus longues au stade C qu'au stade B. Le seuil de durée de l'onde P de 43,5 ms avait une précision diagnostique de 65 % pour différencier l'ICC, avec une sensibilité de 63 % et une spécificité de 90 %. Une valeur seuil de 307,8 ms pour QTc1 a donné une sensibilité de 62 %, une spécificité de 76 % et une précision diagnostique de 78 %, et une valeur seuil de 239,2 ms pour QTc2 a donné une sensibilité de 62 %, une spécificité de 83 %, et une précision diagnostique de 77 % pour le diagnostic d'ICC. Par conséquent, une onde P et un QTc prolongés chez les chiens atteints de MMVD peuvent faciliter la prédiction de l'ICC. L'électrocardiographie pourrait fournir aux cliniciens un outil de dépistage facilement disponible et rentable pour prédire l'ICC, si l'utilité des paramètres ECG peut être vérifiée.(Traduit par Docteur Serge Messier).

Influence of Prolapse Volume in Mitral Valve Prolapse.

Mitral valve prolapse (MVP) is characterized by excessive leaflet tissue leading to a wide spectrum of mitral regurgitation (MR) ranging from trivial to severe. The prolapse volume (PV) below the prolapsing leaflets in end-systole was suspected to impact both chamber remodeling and MR grading in MVP. Based on 157 consecutive patients (45 women; mean age 62±15) referred for CMR assessment of MR, either from MVP (n = 91; 58%) or fibroelastic disease (FED) (n = 66; 42%), we sought to study (i) the interaction between PV and cardiac chamber geometry (ii) to study the impact of PV on MR quantification in MVP. Despite similar left ventricular (LV) size, PV was larger in MVP (11±9ml) than in FED (2±2ml). PV progressively increased with the severity of MR in MVP but not in FED. Despite a low regurgitant volume (32±18ml), some MVP patients with less than moderate MR exhibit significant cardiac chambers remodeling compared to 52 age and sex-matched controls. PV correlated significantly (r = 0.52) with the LV dilatation in severe MR but also in less than moderate MR. In MVP, PV>14ml was associated with a significant underestimation (Bias=-26±32ml) of regurgitant volume by PISA compared to CMR. In conclusion, in MVP, PV may play a role in left cardiac chambers remodeling, even in patients without severe MR, and in discordant grading of MR between echocardiography and CMR.

Arrhythmic mitral valve prolapse: Diagnostic parameters for high-risk patients: A systematic review and meta-analysis.

BACKGROUND: Although frequently considered a benign condition, new evidence has shown that mitral valve prolapse (MVP) is associated with complex ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Therefore, we conducted a systematic review and meta-analysis of the relevant studies to investigate the parameters that could identify MVP patients at higher risk of experiencing complex VAs. METHOD: We performed a systematic literature search of PubMed for potential studies between January 2010 and January 2021. Our meta-analysis included studies comparing MVP patients with complex VAs (A-MVP) and those without (NA-MVP). We used the fixed-effects model to obtain the odds ratio (OR), risk ratio (RR), or mean difference (MD) and 95% confidence interval (CI) for each analyzed parameter. RESULTS: Six studies with 848 individuals were included in the meta-analysis. As compared to the NA-MVP patients, A-MVP patients had a higher prevalence of inverted T-wave (OR: 2.73; 95% CI: 1.85-4.02; p < .00001) and longer QTc interval on the resting ECG (MD: 14.73; 95% CI: 9.39-20.08; p < .00001), longer anterior mitral leaflet length (MD: 2.67; 95% CI: 2.02-3.31; p < .00001), bi-leaflet prolapse (OR: 1.65; 95% CI: 1.22-2.24; p = .001), and mitral annulus disjunction (MAD) on echocardiogram (RR: 1.90; 95% CI: 1.50-2.40; p < .00001), and late gadolinium enhancement (LGE) on cardiac magnetic resonance (RR: 4.38; 95% CI: 1.77-10.86; p = .001). CONCLUSION: Our comprehensive meta-analysis suggests that risk factors related to A-MVP are T-wave inversion, longer QTc interval, bi-leaflet prolapse, longer anterior mitral valve leaflet, MAD, and LGE.

Mitral Valve Prolapse and Its Motley Crew-Syndromic Prevalence, Pathophysiology, and Progression of a Common Heart Condition.

Mitral valve prolapse (MVP) is a commonly occurring heart condition defined by enlargement and superior displacement of the mitral valve leaflet(s) during systole. Although commonly seen as a standalone disorder, MVP has also been described in case reports and small studies of patients with various genetic syndromes. In this review, we analyzed the prevalence of MVP within syndromes where an association to MVP has previously been reported. We further discussed the shared biological pathways that cause MVP in these syndromes, as well as how MVP in turn causes a diverse array of cardiac and noncardiac complications. We found 105 studies that identified patients with mitral valve anomalies within 18 different genetic, developmental, and connective tissue diseases. We show that some disorders previously believed to have an increased prevalence of MVP, including osteogenesis imperfecta, fragile X syndrome, Down syndrome, and Pseudoxanthoma elasticum, have few to no studies that use up-to-date diagnostic criteria for the disease and therefore may be overestimating the prevalence of MVP within the syndrome. Additionally, we highlight that in contrast to early studies describing MVP as a benign entity, the clinical course experienced by patients can be heterogeneous and may cause significant cardiovascular morbidity and mortality. Currently only surgical correction of MVP is curative, but it is reserved for severe cases in which irreversible complications of MVP may already be established; therefore, a review of clinical guidelines to allow for earlier surgical intervention may be warranted to lower cardiovascular risk in patients with MVP.

Replacement Myocardial Fibrosis in Patients With Mitral Valve Prolapse: Relation to Mitral Regurgitation, Ventricular Remodeling, and Arrhythmia.

BACKGROUND: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders, and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance in patients with MVP. METHODS: Four hundred patients (53±15 years of age, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE cardiac magnetic resonance, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). RESULTS: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 with myocardial wall including 71 with basal inferolateral wall, 29 with papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate MR, and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45% versus 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (odds ratio, 1.01 [95% CI, 1.002-1.017], P=0.009) and moderate-severe MR (odds ratio, 2.28 [95% CI, 1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ versus 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (hazard ratio, 2.6 [1.4-4.9], P=0.002) were associated with poor outcome. CONCLUSIONS: LV replacement myocardial fibrosis is frequent in patients with MVP; is associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia; and is independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease. Last, cardiac magnetic resonance provides additional information to echocardiography in MVP.

Strain patterns in primary mitral regurgitation due to rheumatic heart disease and mitral valve prolapse.

OBJECTIVE: Left atrial (LA) and left ventricular (LV) remodelling are the adaptive changes that occur in primary mitral regurgitation (MR) and are related to its clinical outcomes. Despite the pathophysiological differences in MR in rheumatic heart disease (RHD) and mitral valve prolapse (MVP), whether the pattern of LV and LA remodelling is different between the two conditions remains unknown. Hence, we compared the LA and LV strain pattern in MR due to RHD, the predominant etiology in developing countries topatients with MVP and age and sex-matched controls. METHODS: A total of 50 patients of severe MR which included 30 MVP MR and 20 RHD MR were assessed by strain imaging by speckle tracking echocardiography (STE) and were compared with age and sex-matched controls. 2D STE was used for LA and 3D STE was used for LV strain analysis. LA and LV strain parameters were compared between MVP MR and RHD MR groups. RESULTS: 30 patients with MVP and 20 with RHD were studied. 60% (n = 30) were symptomatic. Mean GLS was -17.2 ± 4.4% compared to -20 ± 3.2% among controls and mean LA strain was 17.35 ± 10.3% compared to 51.34 ± 11.5% among controls which were significantly lower (both p < 0.01). No significant difference in LA strain and GLS was found between MVP and RHD subgroups (LA strain 20.45 ± 11.9% and 14.63 ± 8.85%; p = 0.08; GLS - 18.25 ± 4.3% and-16.2 ± 4.6%; p = 0.12). PALS in the RHD group was lower compared to MVP(p = 0.08) which showed a trend towards significance. LV strain parameters showed no significant difference among the MVP and RHD groups. CONCLUSION: LA and LV strain parameters showed no significant difference in MR due to either RHD or MVP. There was a trend towards lower LA strain in RHD which needs validation with large multicentric studies. The current strain parameters from MVP with the prognostic value may be applied to MR of RHD etiology, pending confirmation of our results by other groups.

Risk factors and progression of systolic anterior motion after mitral valve repair.

OBJECTIVES: The phenomenon of systolic anterior motion (SAM) of the mitral valve (MV) was discovered 50 years ago, but to date only a few studies have identified risk factors for SAM following mitral repair. There are limited data on the necessity of surgical reintervention on the MV once SAM is discovered by intraoperative transesophageal echocardiography. We sought to identify predictors of SAM in a large cohort of consecutive patients, assess the rate of early reintervention on the MV to address SAM, and follow the progression of SAM postdischarge. METHODS: Analysis of electronically stored echocardiographic exams of adults who underwent MV repair in a recent decade. RESULTS: Following MV repair, the incidence of SAM immediately after cardiopulmonary bypass was 13% (98 of 761 patients). Multivariable analysis revealed several preoperative risk factors of SAM development and progression, including a lower ratio of anterior to posterior leaflets heights, younger age, lower end-systolic left ventricular volume, presence of bileaflet prolapse, and male sex. SAM was managed conservatively in 91 patients (93%) and surgically in 7 patients (7%). In a majority of patients (70 of 98 patients [71%]) SAM resolved before hospital discharge. CONCLUSIONS: Transesophageal echocardiography findings associated with SAM were excessive height of posterior to anterior mitral leaflet, smaller left ventricular end-systolic volume, and bileaflet prolapse. Conservative management of SAM was usually successful, and persistent hemodynamically significant SAM was uncommon. Prophylactic modification of the surgical technique to avoid SAM seems unnecessary for all but those at highest risk for developing SAM.

A novel cross-species model of Barlow's disease to biomechanically analyze repair techniques in an ex vivo left heart simulator.

OBJECTIVE: Barlow's disease remains challenging to repair, given the complex valvular morphology and lack of quantitative data to compare techniques. Although there have been recent strides in ex vivo evaluation of cardiac mechanics, to our knowledge, there is no disease model that accurately simulates the morphology and pathophysiology of Barlow's disease. The purpose of this study was to design such a model. METHODS: To simulate Barlow's disease, a cross-species ex vivo model was developed. Bovine mitral valves (n = 4) were sewn into a porcine annulus mount to create excess leaflet tissue and elongated chordae. A heart simulator generated physiologic conditions while hemodynamic data, high-speed videography, and chordal force measurements were collected. The regurgitant valves were repaired using nonresectional repair techniques such as neochord placement. RESULTS: The model successfully imitated the complexities of Barlow's disease, including redundant, billowing bileaflet tissues with notable regurgitation. After repair, hemodynamic data confirmed reduction of mitral leakage volume (25.9 ± 2.9 vs 2.1 ± 1.8 mL, P < .001) and strain gauge analysis revealed lower primary chordae forces (0.51 ± 0.17 vs 0.10 ± 0.05 N, P < .001). In addition, the maximum rate of change of force was significantly lower postrepair for both primary (30.80 ± 11.38 vs 8.59 ± 4.83 N/s, P < .001) and secondary chordae (33.52 ± 10.59 vs 19.07 ± 7.00 N/s, P = .006). CONCLUSIONS: This study provides insight into the biomechanics of Barlow's disease, including sharply fluctuating force profiles experienced by elongated chordae prerepair, as well as restoration of primary chordae forces postrepair. Our disease model facilitates further in-depth analyses to optimize the repair of Barlow's disease.

Complex sympathetic regulation in adolescent mitral valve prolapse.

Mitral valve prolapse (MVP) belongs to cardiac disorders characterized by impaired closure of mitral leaflets. We studied adolescent group of patients with MVP suffering from symptomatology that cannot be explained by mitral regurgitation alone. Several studies suggested that symptoms can be explained by autonomic, in particular sympathetic-linked dysfunction. Thus, we assessed non-invasive sympathetic indices of blood pressure and heart rate variability and electrodermal activity (EDA). Fifty-three adolescents with MVP (age: 15.1+/-0.4 years) and 43 healthy age- and gender-matched adolescents (age: 14.9+/-0.4 years) were examined. Blood pressure, heart rate and EDA were continuously recorded during 6-min rest. Evaluated parameters were: low frequency band of systolic blood pressure variability, systolic, diastolic and mean blood pressure, mean RR interval, cardiac sympathetic indices: symbolic dynamics (0V%), left ventricular ejection time (LVET), pre-ejection period (PEP), and EDA. Our findings revealed significantly higher systolic, diastolic, and mean blood pressure values, shortened mean RR interval, increased 0V%, and shortened LVET in MVP patients vs. controls (p=0.028, p<0.001, p=0.002, p<0.001, p=0.050, p<0.001; respectively). Our study revealed enhanced cardiovascular sympathetic regulation in adolescent MVP patients. We suggest that evaluation of non-invasive sympathetic parameters could represent potential biomarkers for early diagnosis of cardiovascular complications associated with MVP already at adolescent age.

Cardiac magnetic resonance imaging with standard imaging planes for mitral valve scallop pathology: interrater agreement and comparison with echocardiography.

Magnetic resonance imaging (CMR) is applied in mitral valve regurgitation (MR) to quantify regurgitation volume/fraction and cardiac volumes, but individual scallop pathology is evaluated by echocardiography. To evaluate CMR for determination of individual scallop pathology, interrater variability on evaluation of scallop pathology from echocardiography and a standard clinical CMR protocol including a transversal stack was compared. 318 mitral scallops from 53 patients with primary MR were evaluated by two cardiologists evaluating echocardiography scans and two other cardiologists evaluating CMR scans (blinded). Inter-rater variability was determined with percentage agreement and Cohen's kappa. In evaluable scallops, interrater agreement on the diagnosis of a prolapsing and/or flail scallop was 77-87% and kappa values of 0.27-0.67, irrespective of physician or modality. Important differences between modalities were primarily related to CMR-evaluators judging the A3 and the P3 to be normal when echocardiography demonstrated prolapsing or even flail scallops; poor imaging of calcification; and flailed scallops occasionally being undetected with CMR since the flow-voids may mask the scallop. Inter-rater agreement for scallop pathology in primary MR is comparable for echocardiography and standard magnetic resonance imaging scans, but CMR has important pitfalls relating to evaluation of A3 and P3 scallops, and suffers from poor visualization of calcification and lower spatial resolution than echo. CMR with standard planes cannot replace CMR with longitudinal planes or echo for the evaluation of specific scallop pathology in severe primary MR.

Influence of chest conformation on myocardial strain parameters in healthy subjects with mitral valve prolapse.

Chest shape might affect myocardial strain parameters. However, the relationship between myocardial strain parameters and chest conformation has not been previously investigated in subjects with mitral valve prolapse (MVP). Between April 2019 and May 2020, 60 healthy subjects (50.1 ± 8.6 year/old, 46.6% females) with MVP and mild-to-moderate mitral regurgitation, and 60 controls matched by age, sex, and cardiovascular risk factors were consecutively studied. Participants underwent modified Haller index (MHI) assessment (ratio of chest transverse diameter over the distance between sternum and spine), and transthoracic echocardiography implemented with 2D-speckle tracking analysis. MHI was significantly greater in MVP group than controls (2.6 ± 0.35 vs 2.1 ± 0.23, p < 0.0001). Left ventricular (LV) ejection fraction was similar in MVP and controls (63.5 ± 3.7% vs 64.3 ± 3.9%, p = 0.25). LV regional and global longitudinal (GLS), circumferential (GCS) and radial strain (GRS) parameters and LV peak twist were all significantly lower in MVP compared to controls (all p < 0.0001). MVP subjects with a tight chest (MHI > 2.5, n = 30), and those with MHI ≤ 2.5 (n = 30) were then separately analyzed. A significant impairment in myocardial strain parameters and LV peak twist was documented in MVP subjects with MHI > 2.5, but not in those with MHI ≤ 2.5. MHI showed a strong inverse correlation with LV-GLS (r = - 0.85), GCS (r = - 0.84), GRS (r = - 0.84) and LV peak twist (r = - 0.94). In MVP subjects, impairment of myocardial strain parameters is not due to intrinsic reduction of cardiac contractility function, but it appears to be related to the degree of chest deformity.

Evaluation of aortic distensibility in patients with mitral valve prolapse using echocardiography and applanation tonometry.

OBJECTIVE: Mitral valve prolapse (MVP) is a heart valve anomaly that affects a considerable segment of the population. Studies of patients with isolated MVP have shown that aortic distensibility decreased as the aortic diameter increased. The aim of this study was to compare evaluations of aortic distensibility in MVP patients using both applanation tonometry and the conventional echocardiographic examination. METHODS: A total of 36 consecutive patients with MVP (16 male and 20 female) and 23 healthy controls (11 male and 12 female) were included in this study. The difference in aortic diameter and distensibility was examined using echocardiography and pulse wave velocity (PWV) was measured with applanation tonometry. RESULTS: According to the echocardiographic measurements, the aortic distensibility was lower in the MVP patients than in the control group (6.2±4.0 cm².dyn⁻¹.10⁻6 vs. 10.0±5.2 cm². dyn⁻¹.10⁻6; p=0.02). The PWV measured with applanation tonometry was significantly higher in the MVP patients than in the control group (9.0±2.4 m/s vs. 7.2±1.4 m/s; p=0.006). CONCLUSION: The results of this study showed that aortic distensibility was reduced in patients with isolated MVP compared with a healthy control group. There was a moderate negative correlation between the results of both methods.

Relations of Augmented Systolic Annular Expansion and Leaflet/Papillary Muscle Dynamics in Late-Systolic Mitral Valve Prolapse Evaluated by Echocardiography with a Speckle Tracking Analysis.

The mechanism of systolic annular expansion in mitral valve prolapse (MVP) is not clarified. Since annular expansion is systolic outward shift of MV leaflet/chorda tissue complex at superior and outer ends, annular expansion could be related to inward (superior) shift of the complex at another inferior and inner end of the papillary muscle (PM) tip and/or systolic lengthening of the tissue complex, especially MV leaflets.MV annulus systolic expansion, PMs' systolic superior shift, and MV leaflets' systolic lengthening were evaluated by echocardiography with a speckle tracking analysis in 25 normal subjects, 25 subjects with holo-systolic MVP and 20 subjects with late-systolic MVP.PMs' superior shift, MV leaflets' lengthening, MV annular area at the onset of systole and subsequent MV annulus expansion were significantly greater in late-systolic MVP than in holo-systolic MVP (4.6 ± 1.6 versus 1.5 ± 0.7 mm/m2, 2.5 ± 1.4 versus 0.6 ± 2.0 mm/m2, 6.8 ± 2.5 versus 5.7 ± 1.0 cm2/m2 and 1.6 ± 0.8 versus 0.1 ± 0.5 cm2/m2, P < 0.001, respectively). Multivariate analysis identified MV leaflets' lengthening and PMs' superior shift as independent factors associated with MV annular expansion.Conclusions: These results suggest that systolic MV annular expansion in MVP is related to abnormal MV leaflets' lengthening and PMs' superior shift.