P6490Paradoxycal restricted motion in diastole is a frequent finding in mitral valve prolapse/dystrophy patients

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Le Tourneau ◽  
C Cueff ◽  
N Piriou ◽  
R Capoulade ◽  
S Le Scouarnec ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Aortic Valve ◽  
Clinical Examination ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Mitral Annulus ◽  
Filamin A ◽  
Familial Form ◽  
Frequent Finding ◽  
Valve Annulus

Abstract Background Filamin-A mitral valve prolapse/dystrophy (FLNA-MVP) phenotype associates MVP and a paradoxical restricted motion in diastole. Purpose We aim to assess the association of mitral valve prolapse to restricted motion in diastole in MVP patients (restricted MVP). Methods We prospectively enrolled 475 MVP probands (64±13 years) and controls relatives. Patients underwent a clinical examination and a comprehensive echocardiographic analysis of mitral valve apparatus. Results Among 475 consecutive probands, 48 (10.1%, 95% CI 7.7–13.3) had both a MVP and a doming aspect in diastole. Patients with restricted MVP exhibited shorted chordae tendinaes, and a shorter distance between papillary muscle tip and mitral annulus. Compared with controls, mitral valve leaflets were lenghtened, thickened and mitral valve annulus was enlarged. The prevalence of polyvalvular disease and bicuspid aortic valve was not increased in restricted MVP patients compared with conventional MVP. Familial form of restricted MVP was identified even in the absence of Filamin-A mutation. Conclusion Restricted MVP is a quite frequent finding in MVP patients and is associated with unique features of the MV apparatus. Restricted MVP can be regarded as a third type of MVP beside myxomatous Barlow disease and fibro-elastic deficiency MVP. Acknowledgement/Funding PHRC I Mitral, Fédération Française de Cardiologie, Fondation Coeur et recherche

scholarly journals P756 Paradoxycal restricted motion in diastole associated to mitral valve prolapse/dystrophy: a frequent finding

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
T Le Tourneau ◽  
C Cueff ◽  
M Marrec ◽  
N Piriou ◽  
R Capoulade ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Mitral Annulus ◽  
Valve Leaflet ◽  
Filamin A ◽  
Mitral Valve Leaflet ◽  
Frequent Finding ◽  
Valve Annulus ◽  
Leaflet Prolapse ◽  
Funding Acknowledgements

Abstract Funding Acknowledgements PHRCI mitral 2012 Background Filamin-A mitral valve prolapse/dystrophy (FLNA-MVP) phenotype associates moderate MVP and a paradoxical restricted motion in diastole. Purpose We aimed to assess the association of MVP with restricted motion in diastole in MVP patients (restricted MVP). Methods We prospectively enrolled 433 MVP probands (57 ± 16 years). Patients underwent a clinical examination and a comprehensive echocardiographic analysis of mitral valve apparatus. Results Among the 433 probands, 27 (6.2%, 95% CI 3.9-8.5) had both a MVP and a doming aspect in diastole. Patients with restricted MVP exhibited shorter posterior chordae tendinaes (24.8 ± 6.3 vs 27.2 ± 5.9 mm, P = 0.037), and a shorter distance between papillary muscle (PM) tips and mitral annulus (anterior PM: P = 0.0001; posterior PM: P = 0.009). Anterior mitral valve leaflet was lengthened (15.5 ± 2.4 vs 14.3 ± 2.6 mm/m², P = 0.018), but leaflet thickness, leaflet prolapse, and mitral valve annulus did not differ between the 2 groups. Bicuspid aortic valve was more frequent in patients with restricted phenotype (14.8 vs 2.9%, P < 0.05). Familial recurrence of restricted MVP was identified even in the absence of Filamin-A mutation. Conclusion Restricted MVP is a quite frequent finding in MVP patients and is associated with PM tips location closer to mitral annulus. Restricted MVP can be regarded as a third type of MVP beside myxomatous Barlow disease and fibro-elastic deficiency MVP.

Excessive Papillary Muscle Traction and Dilated Mitral Annulus in Mitral Valve Prolapse Without Mitral Regurgitation

1996 ◽  
Vol 78 (4) ◽  
pp. 482-485 ◽  
Author(s):  
Tsung-Ming Lee ◽  
Sheng-Fang Su ◽  
Tsuei-Yuen Huang ◽  
Ming-Fong Chen ◽  
Chiau-Suong Liau ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Mitral Annulus

scholarly journals 407 Is Pickelhaube Sign really the hallmark of arrhythmogenic MVP in athletes? And does MVP really cause sudden death? A case report

2020 ◽  
Vol 22 (Supplement_N) ◽  
pp. N131-N131
Author(s):  
Massimo Bolognesi
Keyword(s):  
Mitral Valve ◽  
Sudden Death ◽  
Mitral Valve Prolapse ◽  
Sports Medicine ◽  
Cardiac Death ◽  
Stress Test ◽  
Mitral Annulus ◽  
Unexpected Death ◽  
Premature Ventricular Contractions ◽  
Military Helmet

Abstract The Pickelhaube Sign is today recognized as a novel Echocardiographic Risk Marker for Malignant Mitral Valve Prolapse Syndrome. Mitral Valve Prolapse (MVP) has long been recognized to be a relatively common valve abnormality in the general population. Patients with relatively non-specific symptoms and asymptomatic athletes who have MVP still represent an important clinical conundrum for any physician involved in preventive medicine and sports screening. Although cardiac arrhythmias and/or cardiac death are an undesirable problem in MVP patients, when these subjects were studied with Holter Electrocardiogram (ECG) monitoring a prevalence of ventricular arrhythmias up to 34% was observed, with premature ventricular contractions as the most common pattern (66% of cases). At this regard a paper by Anders et al. described a series of cases that suggest that even clinically considered benign cases of MVP in young adults may cause sudden and unexpected death. However, cardiac arrest and Sudden Arrhythmic Cardiac Death (SCD) resulted in rare events only in patients with MVP based on data from a community study. A middle-aged athletic male who has been practicing competitive cycling for about 20 years came to our Sports Medicine Centre to undergo screening of sports preparation for competitive cycling and the related renewal of certification for participation in sports competitions. This athlete was always considered suitable in previous competitive fitness assessments performed in other sports medicine centers. His family history was unremarkable, as well as his recent and remote pathological anamnesis. The physical examination revealed a 3/6 regurgitation heart murmur with a click in the mid late systole. Previous echocardiographic examinations revealed a MVP which was considered benign with mild not relevant mitral regurgitation. He did not complain of symptoms such as dyspnoea or heart palpitations during physical activity. The resting ECG showed negative T waves in the inferior limb leads, and the stress test showed sporadic premature ventricular beats (a couple) with right bundle branch block morphology. An echocardiogram confirmed the presence of a classic mitral valve prolapse with billowing of both mitral leaflets, associated with a mild to moderate valve regurgitation. The TDI exam at the level of the lateral mitral annulus showed a high-velocity mid-systolic spike like a Pickelhaube sign, i.e. spiked German military helmet morphology. Consequently, an in-depth diagnostic imaging with cardiac magnetic resonance imaging was proposed, but the athlete refused it, both because he was totally asymptomatic and above all because he would be forced to pay a considerable amount of money as the examination is not guaranteed by the Italian National Health Service. In conclusion, the athlete remained sub judice as for competitive suitability, Finally, the question is: does MVP really cause sudden death? Is it enough to detect the Pickelhaube signal by echocardiography to stop this athlete? Let us bear in mind that this athlete was asymptomatic, and he had not had any trouble during exercise and maximal effort for many years. Why must we declare him unsuitable to do competitive sports?

Prevalence of Calcification of the Mitral Valve Annulus in Patients Undergoing Surgical Repair of Mitral Valve Prolapse

2014 ◽  
Vol 113 (11) ◽  
pp. 1867-1873 ◽  
Author(s):  
Laura Fusini ◽  
Sarah Ghulam Ali ◽  
Gloria Tamborini ◽  
Manuela Muratori ◽  
Paola Gripari ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Surgical Repair ◽  
Valve Annulus

scholarly journals P436Abnormal papillary muscle signal on cine CMR images in mitral valve prolapse

2019 ◽  
Vol 20 (Supplement_2) ◽  
Author(s):  
A Scatteia ◽  
C E Pascale ◽  
P Guarini ◽  
S Dellegrottaglie
Keyword(s):  
Mitral Valve ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Cine Cmr

Arrhythmic Mitral Annulus Disjunction and Mitral Valve Prolapse

2019 ◽  
Vol 73 (6) ◽  
pp. 739
Author(s):  
Hui-Chen Han ◽  
Paul Calafiore ◽  
Andrew W. Teh ◽  
Omar Farouque ◽  
Han S. Lim
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Mitral Annulus

scholarly journals Mitral and tricuspid annulus disjunction frequently coexist

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
E Aabel ◽  
M Chivulescu ◽  
L A Dejgaard ◽  
M Ribe ◽  
E Gjertsen ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Right Ventricular ◽  
Mitral Valve Prolapse ◽  
Odds Ratio ◽  
Ventricular Arrhythmias ◽  
Mitral Annulus ◽  
Ventricular Free Wall ◽  
Free Wall ◽  
Tricuspid Annulus ◽  
Right Ventricular Free Wall

Abstract Background Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral annulus, frequently found in patients with high-risk arrhythmogenic mitral valve prolapse syndrome. It is unknown whether the annulus disjunction extends to the right side of the heart as tricuspid annulus disjunction (TAD), and whether it is associated with right ventricular electrical instability. Purpose We aimed to explore the presence of TAD, and if extended annulus disjunction was associated with ventricular arrhythmias. Methods We included patients with previously described MAD assessed by cardiac magnetic resonance imaging (CMR) in an ambispective cohort study. MAD and TAD was defined as ≥1 mm separation between the respective atrial wall-valve leaflet junction and the top of the ventricular myocardium. TAD was assessed in the lateral and inferior right ventricular free wall by means of the 4-chamber and right ventricular 2-chamber views, respectively. MAD circumference was assessed by a CMR study protocol with six left ventricular long axis views separated by 30 degrees. Mitral valve prolapse was defined as ≥2 mm superior displacement of any part of the mitral leaflets beyond the mitral annulus. Ventricular arrhythmias were defined as aborted cardiac arrest or non-sustained/sustained ventricular tachycardias recorded by electrocardiogram (ECG), stress ECG or Holter monitoring. Results We included 92 patients with MAD (62% female, age 47±16 years, 71% mitral valve prolapse). TAD was found in 48 (52%) patients, both in the lateral (n=40, 83%) and inferior (n=30, 63%) right ventricular free wall. Patients with TAD were older (age 51±16 years vs. 43±14 years, p=0.01), had greater MAD circumference (168±56° vs. 117±62°, p=0.001) and greater MAD distance (9.2±2.9 mm vs. 6.4±2.8 mm, p<0.001). Additionally, patients with TAD had more frequently mitral valve prolapse (40 patients [85%] vs. 25 patients [57%], p=0.003), whereas similar frequency of bileaflet prolapse (17 patients [39%] vs. 10 patients [39%], p=0.99). Ventricular arrhythmias had occurred in 38 (41%) patients, who were younger (age 40±14 years vs. 52±15 years, p<0.001) and had less frequently TAD (14 patients [37%] vs. 34 patients [63%], p=0.01; univariate odds ratio 0.34 [0.15–0.81], p=0.02). However, TAD was not associated with ventricular arrhythmias when adjusted for age (multivariate odds ratio 0.46 [0.18–1.15], p=0.10). Conclusions TAD by CMR was highly prevalent in patients with MAD and was a marker of severe annulus disjunction and mitral valve prolapse. TAD was not associated with more ventricular arrhythmias. This novel marker warrants further research to explore the clinical implications of right-sided annulus disjunction. FUNDunding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Norwegian Research Council

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