scholarly journals Mitral valve prolapse: American versus European guidelines - which one is better

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
J Brito ◽  
J Rigueira ◽  
T Rodrigues ◽  
I Aguiar-Ricardo ◽  
R Santos ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Clinical Presentation ◽  
Nyha Class ◽  
Daily Practice ◽  
Posterior Wall ◽  
Systolic Pulmonary Artery Pressure ◽  
Chi Square ◽  
Funding Sources ◽  
European Guidelines

Abstract Funding Acknowledgements Type of funding sources: None. Introduction According to the most recent recommendations of AHA, mitral valve prolapse (MVP) is defined as systolic displacement of the mitral leaflet into the left atrium (LA) of at least 2 mm from the mitral annular plane. The ESC recommendations define MVP, flail and billowing, according to the location of the leaflet tips in relation to the coaptation plan. Differences in outcomes considering these classifications are not established. Purpose To evaluate the differences in clinical presentation and outcomes of MVP considering AHA and ESC classifications. Methods Single-center retrospective study of consecutive patients with MVP (defined according to the AHA classification) documented in transthoracic echocardiogram between January 2014 and October 2019. Demographic, clinical, echocardiographic and electrocardiographic data were collected. The results were obtained using Chi-square and ANOVA tests. Results We included 247 patients (mean age 62.9 ± 18 years, 61% males) according to AHA classification; considering the ESC classification: 147 (59%) had prolapse, 30 (12%) flail and 67 (39%) billowing. In comparison to patients with flail and billowing, patients with MVP had less cordae rupture (p = 0.02). Prolapse was associated with better survival (p = 0.037) and was an independent predictor of survival (OR = 0.372, CI95% [0.148-0.935], p = 0.035) Patients with flail were older in comparison to the ones with prolapse and billowing (71 ± 14 vs 63 ± 17 vs 60 ± 21 years, respectively, p = 0.022). Patients with flail were mostly men (80%, p = 0.028), with more significant mitral regurgitation (p = 0.003) and higher NYHA class (p = 0.018). They also had higher systolic pulmonary artery pressure (SPAP) (48 ± 23 vs 38 ± 18 vs 36 ± 12mmHg, p = 0.015) and higher values of LV mass and posterior wall thickness (144 ±32 vs 125 ± 44 vs 114 ± 37g/m2, p = 0.005 and 11 ± 1,5 vs 10 ± 1,7 vs 9 ± 1.9 mm, p = 0.009, respectively). Women had more billowing (p = 0.04) than prolapse and flail. Conclusion The ESC classification adds information to the AHA classification in what concerns to clinical presentation and prognosis of mitral valve prolapse, so both classifications should be used in daily practice.

scholarly journals Mitral valve prolapse - it"s possible to stratify prognosis in these patients?

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
P Silverio Antonio ◽  
T Rodrigues ◽  
R Santos ◽  
A Nunes-Ferreira ◽  
N Cunha ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Nyha Class ◽  
Univariate Analysis ◽  
Developed Countries ◽  
Maximum Distance ◽  
Conduction Delay ◽  
Systolic Pulmonary Artery Pressure ◽  
Lv Mass

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Mitral valve prolapse (MVP) is one of the most frequent causes of mitral valve disease in developed countries, traditionally with a benign prognosis, however some patients develop arrythmias and significant mitral regurgitation (MR) with need of intervention. Herein our purpose was to establish clinical, electrocardiographic and echocardiographic predictors of arrythmias, mitral valve intervention (MVI) and hospitalization in MVP patients to better characterize the prognosis in these patients. Methods  Single-center retrospective study of consecutive patients with MVP documented in transthoracic echocardiogram between January 2014 and October 2019. MVP was defined as systolic displacement of the mitral leaflet into the left atrium ≥ 2 mm from the mitral annular plane. Demographic, clinical, echocardiographic, electrocardiographic data were collected as well as adverse events at follow-up. The results were obtained using Chi-square and Student-t tests; predictors were found with logistic regression. Results  247 patients were included (mean age 62.9 ± 18 years, 61% males), most with MVP involving the posterior leaflet (48.6%). 40% were symptomatic, 47.4% had more than moderate MR, and 25% had interventricular conduction delay in the ECG. During a mean follow-up of 30 ± 19 months, 38% had arrythmias, 27.1% needed mitral valve intervention (95% surgery and 5% percutaneous), 27.1% had atrial fibrillation (AF), 3.4% had ventricular arrythmias, 19.2% had ventricular premature beats, 13.3% had hospital admission for cardiovascular cause and 8.5% (n = 21) died. 9.3% of the patients had mitral annulus disjunction (MAD). Palpitations (p = 0.018), AF (p < 0.001), significant MR (p < 0.001), higher NYHA class (p = 0.016), systolic pulmonary artery pressure (SPAP) (p < 0.001), LV mass (p < 0.001), QTc (p = 0.01) and MAD maximum distance (p = 0.02) associated with MVI. MAD maximum distance value presented an excellent capacity to predict the MVI (AUC 0.85 p = 0.019); the best cut-off was 11,5 mm (Sens = 80%; Spec = 83%). AF was a predictor of hospitalization in univariate analysis (OR = 2.57, CI95% 1.15-5.75, p = 0.022). Regarding arrhythmic events, we found association with aortic root dilatation (p = 0.032), NYHA III-IV (p = 0.013), age and LV mass (both with p < 0.001). In multivariate analysis, LV mass (OR = 1.02, CI95% 1.005-1.027, p = 0.005) and age (OR = 1.038, CI95% 1.004-1.053, p = 0.021) were independent predictors of arrythmias. In this sample, MAD was not associated with arrythmias. Conclusion  Opposing to previous studies in our population, MAD was not associated with arrythmias but had an excellent capacity to predict MVI. Age and LV hypertrophy were independent predictors of arrythmias in our patients. Larger studies are needed to better stratify patients with MVP, as its association with arrhythmias, hospitalization and the need for intervention is not negligible.

scholarly journals Malignant Arrhythmic Mitral Valve Prolapse: A Continuum of Clinical Challenges from Diagnosis to Risk Stratification and Patient Management

2020 ◽  
Vol 8 (1) ◽  
pp. 2
Author(s):  
Idit Yedidya ◽  
Aniek L. van Wijngaarden ◽  
Nina Ajmone Marsan
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Clinical Decision Making ◽  
Clinical Presentation ◽  
Clinical Decision ◽  
Benign Condition ◽  
Long Period ◽  
Patients Experience ◽  
Hospital Cardiac Arrest

Mitral valve prolapse (MVP) is a common valvular disease, which may remain a benign condition for a long period of time. However, some patients experience malignant ventricular arrhythmias and sudden cardiac death (SCD). It is still largely unknown how to risk-stratify these patients, and no specific recommendations have been proposed to help the clinical decision-making. We present the case of a young man whose first clinical presentation was an out-of-hospital cardiac arrest and was subsequently diagnosed with MVP. We highlighted the possible risk factors for SCD and the challenges in the clinical management of these patients.

scholarly journals Status of the collagen metabolism and intracardiac hemodynamics in patients with mitral valve prolapse and diabetes mellitus type 1

2021 ◽  
Keyword(s):  
Diabetes Mellitus ◽  
Type 1 Diabetes ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Posterior Wall ◽  
Diabetes Mellitus Type 1 ◽  
Collagen Metabolism ◽  
Control Group ◽  
Destructive Processes

Summary. Mitral valve prolapse is a significant cardiovascular risk factor in young adults. Its combination with type 1 diabetes mellitus can influence the nature and development of the disease. Objective – a comparative analysis of free and peptide-bound oxyproline levels and basic echocardiographic parameters (ECP) in patients with mitral valve prolapse, type 1 diabetes and their combination. Materials and methods – 93 people aged 19–33 years were examined, including 24 people with mitral valve prolapse without concomitant pathology; 33 patients with mitral valve prolapse and type 1 diabetes; 36 patients with type 1 diabetes without mitral valve prolapse. Results. The level of free and peptide-bound oxyproline in blood serum and their ratio were assessed as a marker of collagen metabolism. The levels of free oxyproline were significantly higher only for the group of MVP patients with type 1 diabetes (p < 0.05) compared to the control group. Severity of destructive processes was demonstrated by a high level of peptide-bound oxyproline, both in combined pathology compared with control group, and compared with groups of patients with monomorbid diabetes and MVP (p < 0.05). In patients with mitral valve prolapse and type 1 diabetes for more than 10 years in anamnesis were found significant differences in the echocardiography parameters (ventricular septum thickness, posterior wall of the left ventricle thickness) compared with the subgroup of patients with less than 10 years of type 1 diabetes in anamnesis and the group with isolated mitral valve prolapse. Conclusions. The data obtained indicate an aggravation in collagen metabolism disorders in patients with mitral valve prolapse depending on the duration of type 1 diabetes, and demonstrate the effect of carbohydrate metabolism disorders on the risk of developing connective tissue degradation of the heart valve apparatus.

scholarly journals P1689 Mitral valve prolapse secondary to ischemic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
G Babur Guler ◽  
A Kilicgedik ◽  
H Zencirkiran Agus ◽  
G Kahveci
Keyword(s):  
Myocardial Infarction ◽  
Mitral Valve ◽  
Left Ventricle ◽  
Mitral Regurgitation ◽  
Transesophageal Echocardiography ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Systolic Function ◽  
Systolic Pulmonary Artery Pressure ◽  
Mitral Prolapse

Abstract Introduction Mitral valve prolapse is the most common form of degenerative mitral valve disease. However, ischemic mitral valve prolapse is a rare cause of mitral regurgitation. The mechanism was initially thought to be papillary muscle dysfunction, but more complex mechanisms were suggested recently. Purpose Try to understand the pathophysiology of ischemic mitral valve prolapse on a case example. Case Report A 42-year-old male with a history of inferoposterior myocardial infarction was admitted from outpatient clinic due to NYHA class 3 heart failure symptoms. On physical examination, a 4/6 holosystolic murmur was heard in the apex. He had a permanent pacemaker implanted for sick sinus syndrome. Transthoracic echocardiography showed 1-global dysfunction of the left ventricle (posterior segment akinetic and thinned), 2- prolapse of the posterior mitral leaflet (suspicion of ruptured chordae) 3-severe mitral regurgitation (with anterior eccentric jet), 4- moderate tricuspid regurgitation and high systolic pulmonary artery pressure (65 mmHg), 5- pacemaker lead in the right heart chambers. 6- normal right ventricular systolic function. Transesophageal echocardiography showed P2 scallop prolapse and chordae were intact, there were no redundant or myxamous components of the leaflets. It was observed that the posteromedial papillary muscle was elongated and did not contract. We commented that these echocardiographic findings represented ischemic mitral valve prolapse. Other echo findings in favour of this hypothesis were the posteromedial papillary muscle prolongation in systole and reduced the free strain of papillary muscle in the the apical long axis view. The patient underwent mitral ring anuloplasty and surgical neocord implantation. Surgery also reported the aetiology as ischemic mitral prolapse secondary to chordal extension in accordance with echocardiography. Conclusion(s): Ischemic mitral prolapse is a complex pathology involving multiple components of the mitral valve apparatus as left ventricle, papillary muscle, chordae, annulus, leaflets. The diagnostic criteria for ischemic mitral valve prolapse and its management are not defined. The presence of myocardial infarction and the exclusion of other possible valve pathologies with transesophageal echocardiography are important steps in the diagnosis. Abstract P1689 Figure.

scholarly journals Mitral Valve Prolapse In Patients With Benign Joint Hypermobility Syndrome (BJHS)

2019 ◽  
Vol 13 (1) ◽  
pp. 8-10
Author(s):  
Tariq Jassim Mohammed ◽  
Tariq Jassim Mohammed
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cross Sectional Study ◽  
Joint Hypermobility ◽  
Control Group ◽  
Hypermobility Syndrome ◽  
Joint Hypermobility Syndrome ◽  
Chi Square ◽  
Cross Sectional ◽  
Cardiac Assessment

Background: Joint hypermobility  was first mentioned by Hippocrates as an isolated feature, when he described the Celts' Incapacity to Pull a Bowstring or Throw a Dart, Due to The Slackness of Their Limbs Objective: to determine the prevalence of mitral valve prolapse(MVP)in patients with benign hypermobility syndrome (BJHS). Type of the study: Cross –sectional study. Methods: Ninety patients with BJHS were included in this study. Full cardiological assessment was done for all of them,  which  include clinical examination, electrocardiography and echocardiography. Cardiac assessment was done for another sixty age and sex matched (normal mobile) Individuals served as a control group. Statistical analysis was done by using T test or chi square as indicated. Results: Among 90 patients with BJHS, MVP was reported in 26 patients (28.9%) compared to four individuals (6.7%)of the control group on modern echocardiography studies (P=0.013). Conclusions: the prevalence of MVP was significantly higher among patients with BJHS compared to normal mobile individuals.    

Upright paradoxical posterior wall movement in mitral valve prolapse

1985 ◽  
Vol 56 (12) ◽  
pp. 804-806 ◽  
Author(s):  
Jack Krafchek ◽  
Mary Shaw ◽  
Joseph Kisslo
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Posterior Wall ◽  
Wall Movement

scholarly journals P199 Mitral valve annular disjunction prevalence and significance amongst patients with mitral valve prolapse

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
G Faia Carvalho Dias ◽  
B Faria ◽  
P Von Hafe ◽  
A F Cardoso ◽  
O Azevedo ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Statistical Significance ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Chamber View ◽  
Clinical Events ◽  
Echocardiographic Evaluation ◽  
Posterior Wall Thickness ◽  
The Difference

Abstract Background Mitral valve prolapse (MVP) is a common and usually benign entity. Occasionally, patients with MVP present with mitral annular disjunction (MAD), whose prevalence and clinical significance are still not clearly established. Purpose This study aimed to define the prevalence of MAD in a population of patients with MVP and study its echocardiographic and clinical implication. Methods A total of 31 patients with MVP who underwent echocardiographic evaluation in our laboratory were retrospectively evaluated. Echocardiographic, demographic and clinical variables were assessed. Disjunction amplitude (DA) was measured in parasternal long axis view (PLAX) and MAD was considered present if a separation of ≥ 5mm was verified. Annular diameter was measured in PLAX and apical four chamber view (A4C) both in systole and diastole. Results MAD was identified in 9 patients (29%), having a mean DA of 12.3 ± 3.2 mm. The group of patients with MAD was significantly younger than the group without MAD (mean age of 54 ± 18 vs 67 ± 15 years; p = 0.001), but there were no significant differences regarding gender, height, weight or cardiovascular risk factors. DA was inversely correlated with body surface area (r=-0.8, p = 0.009). Systolic annular diameters in PLAX and A4C views were increased in patients with MAD (4.2 ± 0.6 vs 3.6 ± 0.7 mm, p= 0.04 and 4.8 ± 0.7 vs 4.1 ± 0.7 mm, p = 0.025, respectively). The difference between systolic and diastolic diameters in PLAX was also greater in MAD (0.5 ± 0.2 vs 0.1 ± 0.3 mm, p = 0.007). Posterior wall thickness (8.5 ± 1.1 vs 9.7 ± 1.4 mm, p = 0.035), indexed left ventricular mass (89.0 ± 15.8 vs 110.6 ± 40,2 g/m2, p = 0.04) and ascending aortic dimensions (28.7 ± 6.7 vs 37.4 ± 3.6 mm, p = 0.018) were notably inferior in MAD patients, as was left ventricle ejection fraction (LVEF) (57.5 ± 5.8 vs 62.6 ± 4.9 %, p = 0.0023). No differences were found in chamber volumes. Similarly, eletrocardiographic parameters were identical in both groups. Five patients (56%) in the group with MAD had documentation of events (palpitations, dizziness, syncope or sudden cardiac death), in contrast with just 3 (21%) in the group without MAD, although statistical significance was not achieved (p = 0.078). Conclusion This study revealed that MAD is common among patients with MVP and is associated with altered annulus dynamics during the cardiac cycle. Its association with younger individuals, lower LVEF and, apparently, more clinical events highlight the importance of this entity.

P919Ventricular arrhythmias in patients with mitral valve prolapse and severe mitral regurgitation

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A L Van Wijngaarden ◽  
M De Riva Silva ◽  
Y L Hiemstra ◽  
J J Bax ◽  
V Delgado ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Mitral Valve Prolapse ◽  
Posterior Wall ◽  
Outflow Tract ◽  
Premature Ventricular Contractions ◽  
T Wave ◽  
Barlow’S Disease

Abstract Background Mitral valve prolapse (MVP) is known to be associated with ventricular arrhythmias (VA), from symptomatic premature ventricular contractions (PVCs) to malignant ventricular tachycardia (VT) and sudden cardiac death (SCD). Previous studies identified risk factors for VA in these patients such as young woman, bileaflet prolapse and inverted T-waves, but they included only specific cohort of patients who experienced SCD or who had only trivial or mild mitral regurgitation (MR). The prevalence of VA and their potential risk factors in a population of MVP patients with severe MR are unknown. Purpose The aim of our study was to describe the prevalence of VA in patients who underwent mitral valve surgery for moderate to severe MR due to MVP and to identify clinical, electrocardiographic and echocardiographic parameters associated with VA. Methods 797 patients (65±12 years, 65% male) who underwent surgery for MVP were included from 2000 until 2018. The presence of VA was defined as symptomatic and frequent PVCs (Lown grade ≥2), non-sustained ventricular tachycardia (nsVT), VT or ventricular fibrillation (VF) documented before surgery and without an ischemic cause. The echocardiogram and electrocardiogram (ECG) prior to operation were used for the specific analysis. ECGs were checked for rhythm, conduction times, QRS morphology and inferior T-wave inversion. The origin of the PVCs was derived from a 12-lead ECG when available and divided in 5 groups; mitral annulus, papillary muscle (PM), left ventricle (LV, including outflow tract), right ventricle (including outflow tract) and other. By echocardiography, several parameters such as LV thickness and volumes, LV ejection fraction (EF), global longitudinal strain (GLS) and MR grade were obtained. Results A total of 99 (12%) patients showed VA; 70% (69/99) of the patients had symptomatic PVCs, 36% (36/99) had nsVT, 12% (12/99) VT and 3% (3/99) VF. 21 patients had more than one type of VA, of which the combination PVCs and nsVT was the most common (19/99, 19%). In addition, 6 patients experienced an out of hospital cardiac arrest of a non-ischemic cause. When comparing the clinical characteristics (Table), patients with VA were significantly younger (and with better renal function), more frequently diagnosed with Barlow's disease and experienced more palpitations as compared to patients without VA. The ECG analysis showed that patients with VA more often had inferior T-wave inversions and that the PVCs predominantly originated from the PM whereas in patients without VA the PVCs originated from different regions. Echocardiography showed that patients with VA had a thinner posterior wall, reduced LV EF and worse GLS; more severe MR was not associated with VA. Conclusion In MVP patients with moderate to severe MR undergoing surgery, Barlow's disease, inferior T-wave inversions, thinner posterior wall and LV systolic dysfunction are associated with the presence and development of VA.

scholarly journals P302 Forgotten and neglected: LV hypertrophy and LV remodeling as important predictors in patients with mitral valve prolapse

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Trifunovic ◽  
O Petrovic ◽  
M Tomic-Dragovic ◽  
I Paunovic ◽  
V Tutus ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Left Ventricular ◽  
Secondary Outcome ◽  
Multivariable Model ◽  
Chi Square ◽  
Increased Risk ◽  
Lv Mass

Abstract Current ESC guidelines recommends left ventricular (LV) end-systolic diameter (ESD), LV ejection fraction (LV EF), systolic pulmonary arterial pressure (SPAP) as key parameters in a multifactorial treatment algorithm for chronic severe primary MR. However, LV hypertrophy (LVH) and LV remodelling during the process of adaptation to chronic MR can influence further clinical course. Aim of this study was to test whether LVH and distinctive LV geometry are coupled with increased risk for heart failure (HF) development and occurrence of major adverse cardiac event (MACE) among patients with MVP and can they improve power of statistical models for HF and MACE prediction based on parameters supported by the current guidelines. Methods 376 pts diagnosed with mitral valve prolapse (MVP) between 1. January 2014. and 31. December 2017 and with complete medical chart and follow-up data from central echo laboratory in the tertiary health center were enrolled in the study. Four types of LV geometry were identified: Type 1 (normal LV mass with normal geometry), Type 2 (normal LV mass with concentric remodeling), Type 3 (eccentric hypertrophy) and Type 4 (concentric hypertrophy). The primary outcome was HF and secondary outcome was MACE (HF development, myocardial infarction, myocardial revascularisation (both PCI and/or ACBG) and cardiac death). Results The distribution of patients was as follow: 51.2% (Group 1) vs 3.3% (Group 2) vs 41.4 % (Group 3) vs 4.1% (Group 4). In multivariable model the highest OR for HF development after adjustment for age, ESD and LVH, had concentric LVH (OR= 5.361, p= 0.004, 95% CI 1.696-16.648), then EF &lt; 60% (OR= 3.025, p = 0.004, 95% CI 1.427-6.411) and the lowest OR had SPAP &gt; 40 mmHg (OR = 2.274, p = 0.039, 95% 1.43-4.958). Adding LVH significantly increased model’s power to predict HF above traditional parameters (Chi-square from 19.386 to 23.640, p &lt; 0.001; Nagelkerke R square from 0.090 to 0.110), whereas addition of LV geometry increased it even more (Chi-square from 23.640 to 28.729, p &lt; 0.001; Negelkerke R square from 0.110 to 0.132). Independent MACE predictors in multivariable model were: EF &lt; 60% (OR 3.645, p &lt; 0.001, 95% CI 1.808- 7.50), new onset atrial fibrillation during the follow-up (OR =3.327, p = 0.012, 95% CI 0.305-8.484), concentric LVH (OR= 4.241, p = 0.015, 95% CI 1.327-13.550) and normal LV geometry without LVH (OR= 0.514, p = 0.002, 95% CI 0.288-0.918), even after adjustment for MV surgery. Adding LVH significantly improved model’s power (Chi-square from 29.026 to 35.112, p &lt; 0.001; Nagelkerke R square 0.121 to 0.146) to predict MACE and addition of type of LV geometry provided additional strength (Chi-square from 35.112 to 39.707, p &lt; 0.001; Nagelkerke R square from 0.146 to 0.164). Conclusion LVH and especially concentric LVH are independent predictors of heart failure development and MACE in mitral valve prolapse and significantly improves predictive powers of the models based on traditional parameters.

scholarly journals Myocardial extracellular volume by T1 mapping: a new marker of arrhythmia in mitral valve prolapse

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Pavon ◽  
D Arangalage ◽  
S Hugelshofer ◽  
T Rutz ◽  
AP Porretta ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Interstitial Fibrosis ◽  
Extracellular Volume ◽  
Holter Monitoring ◽  
Left Ventricular ◽  
Control Group ◽  
Funding Sources ◽  
Replacement Fibrosis ◽  
Additional Value

Abstract Funding Acknowledgements Type of funding sources: None. Background In MVP, MAD has been associated with myocardial replacement fibrosis and arrhythmia, but the importance of interstitial fibrosis remains unknown. We aimed to evaluate the relationship between mitral annular disjunction (MAD) severity and myocardial interstitial fibrosis at the left ventricular (LV) base in patients with mitral valve prolapse (MVP), and to assess the association between severity of interstitial fibrosis and the occurrence of ventricular arrhythmic events Methods Thirty patients with MVP and MAD (MVP-MAD) underwent Cardiac Magnetic Resonance (CMR) with assessment of MAD length, late gadolinium enhancement (LGE), and basal segments myocardial extracellular volume (ECV). The control group included 14 patients with mitral regurgitation but no MAD (MR-NoMAD) and 10 patients with normal CMR (NoMR-NoMAD). Fifteen MVP-MAD patients underwent 24h-Holter monitoring. Results LGE was observed in 47% of MVP-MAD patients and absent in controls. ECV was higher in MVP-MAD (30 ± 3% vs 24 ± 3% MR-NoMAD, p &lt; 0.0001 and vs 24 ± 2% NoMR-NoMAD, p &lt; 0.0001), even in MVP-MAD patients without LGE (29 ± 3% vs 24 ± 3%, p &lt; 0.0001 and vs 24 ± 2%, p &lt; 0.0001, respectively), Fig.1. MAD length was correlated with ECV (rho = 0.61, p = 0.0003), but not with LGE extent. Four patients had history of OHCA; LGE and ECV were equally performant to identify those high-risk patients (area under the ROC curve 0.81 vs 0.83, p = 0.84). Among patients with Holter, 87% had complex ventricular arrhythmia. ECV was above the cut-off value in all while only 53% had LGE. Conclusion Increase in ECV, a marker of interstitial fibrosis, occurs in MVP-MAD even in the absence of LGE, and was correlated with MAD length and OHCA. ECV should be part of the CMR examination of MVP patients in an effort to better assess fibrous remodelling as it may provide additional value beyond the assessment of LGE in the arrhythmic risk stratification.

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