The mitral annulus disjunction as a risk marker in mitral valve prolapse

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?

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Pickelhaube Spike, a High-Risk Marker for Bileaflet Myxomatous Mitral Valve Prolapse: Sonographer's Quest for the Highest Spike

Journal of the American Society of Echocardiography ◽

10.1016/j.echo.2020.02.004 ◽

2020 ◽

Vol 33 (5) ◽

pp. 639-640

Author(s):

Denise Ignatowski ◽

McKenzie Schweitzer ◽

Kelly Pesek ◽

Renuka Jain ◽

Lakshmi Muthukumar ◽

...

Keyword(s):

High Risk ◽

Mitral Valve ◽

Mitral Valve Prolapse ◽

Risk Marker

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Arrhythmic Mitral Annulus Disjunction and Mitral Valve Prolapse

Journal of the American College of Cardiology ◽

10.1016/j.jacc.2018.10.090 ◽

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

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

The American Journal of Cardiology ◽

10.1016/0002-9149(97)00002-7 ◽

1996 ◽

Vol 78 (4) ◽

pp. 482-485 ◽

Cited By ~ 10

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

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Mitral and tricuspid annulus disjunction frequently coexist

European Heart Journal ◽

10.1093/eurheartj/ehab724.0232 ◽

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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Late gadolinium enhancement predicts adverse clinical outcome in patients with mitral valve prolapse/mitral annulus disjunction

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeab090.048 ◽

2021 ◽

Vol 22 (Supplement_2) ◽

Author(s):

S Figliozzi ◽

G Georgiopoulos ◽

GD Aquaro ◽

K Bauer ◽

L Monti ◽

...

Keyword(s):

Heart Disease ◽

Late Gadolinium Enhancement ◽

Magnetic Resonance ◽

Mitral Valve ◽

Clinical Outcome ◽

Myocardial Fibrosis ◽

Mitral Valve Prolapse ◽

Mitral Annulus ◽

Gadolinium Enhancement ◽

Unexplained Syncope

Abstract Funding Acknowledgements Type of funding sources: None. OnBehalf Mitral vAlve prolapse and disjunction by cardiac maGnetIC resonance (MA-GIC) registry Backgroung Mitral valve prolapse (MVP) is 2-3% prevalent in the general population with good prognosis. However, some patients develop complex ventricular arrhythmias (CVAs), sudden cardiac death (SCD), or severe mitral regurgitation (MR). Previous studies suggested that bi-leaflet involvement, mitral annulus disjunction (MAD), and myocardial fibrosis (MF) are associated with adverse outcome. Notwithstanding, these findings were limited to autopsic series or single-centre studies involving highly selected patients. Moreover, MF has been scantly investigated as predictor of clinical outcome. Purpose To investigate the prognostic significance of MF in an international multicentre study of MVP patients studied by cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods From October 2007 to June 2020 patients undergoing LGE-CMR were screened in 14 European centres. Inclusion criteria were: i) age > 18 years; ii) full clinical history and cardiac rhythm monitoring at baseline; iii) MVP (leaflet displacement ≥ 2 mm beyond the annulus). Exclusion criteria were: i) ischemic heart disease; ii) primary cardiomyopathy; iii) inflammatory heart disease; iv) congenital heart diseases; v) moderate-to-severe valvular heart disease. CVAs at the study outset was defined as one of the following: i) ventricular ectopic beats >10000/24h; ii) ≥ 1 episode of non-sustained ventricular tachycardia (VT); iii) sustained VT; iv) aborted SCD. Primary end-point was a composite of SCD, unexplained syncope, and mitral valve repair/replacement. Secondary end-point was a composite of SCD and unexplained syncope. Results Four-hundred-fifty-eight MVP patients were eventually included (46 ± 16 years old, 51% males) of whom 68% had MAD. LGE was detected in 103 (22%) of subjects with mid-wall pattern (46%) in left ventricular (LV) lateral wall (66%) as the most prevalent feature. At baseline, 37% of LGE-positive patients vs. 18% of LGE-negative individuals had CVAs (P < 0.001). SVT and/or aborted SCD were more prevalent in LGE-positive than in LGE-negative patients (9% vs 2%, P < 0.001). By multivariable Cox-regression analysis, LGE presence or extent were strong independent predictors of the primary (HR = 4.02, P = 0.003 and HR = 4.76 per 10% increase, P = 0.032, respectively) and secondary (HR = 5.39, P = 0.008 and HR = 8.78 per 10% increase, P = 0.012, respectively) endpoints after correction for major confounders including LV volumes, left atrial size and MAD presence. Conlusion Myocardial fibrosis by LGE is the strongest independent predictor of clinical outcome in MVP. In contrast, MAD per se does not harbinger worse prognosis.

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Morphofunctional Abnormalities of Mitral Annulus and Arrhythmic Mitral Valve Prolapse

Circulation Cardiovascular Imaging ◽

10.1161/circimaging.116.005030 ◽

2016 ◽

Vol 9 (8) ◽

Cited By ~ 71

Author(s):

Martina Perazzolo Marra ◽

Cristina Basso ◽

Manuel De Lazzari ◽

Stefania Rizzo ◽

Alberto Cipriani ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Mitral Annulus

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Cerebral Ischemia with Mitral Valve Prolapse and Mitral Annulus Calcification

Clinical Medicine and the Nervous System - The Heart and Stroke ◽

10.1007/978-1-4471-3129-8_5 ◽

1987 ◽

pp. 63-100 ◽

Cited By ~ 1

Author(s):

S. Lauzier ◽

H. J. M. Barnett

Keyword(s):

Cerebral Ischemia ◽

Mitral Valve ◽

Mitral Valve Prolapse ◽

Mitral Annulus ◽

Mitral Annulus Calcification

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ECG modifications, life-threatening ventricular arrhythmias and relation to mitral valve apparatus phenotype in mitral valve prolapse

European Heart Journal ◽

10.1093/ehjci/ehaa946.1885 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

D Stevant ◽

M Plessis ◽

J.B Gourraud ◽

C Cueff ◽

N Piriou ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias ◽

Qt Dispersion ◽

Mitral Annulus ◽

Exercise Stress ◽

Stress Tests ◽

T Wave ◽

Wave Inversion ◽

Ecg Abnormalities

Abstract Background Mitral valve prolapse (MVP) is a common condition in the general population, which can be associated to non-specific ECG abnormalities described initially as ST segment depression, T waves flattening or inversion, especially in the inferior leads. Lately, this type of ECG abnormalities has been reported in patients with MVP and ventricular arrhythmias (VA) or sudden death (SD). However, the prevalence of ECG abnormalities has never been studied in a large series of patients, and the link between ECG abnormalities, VA and SD to echocardiography examination has never been prospectively assessed. Objective To study the prevalence of ECG abnormalities including ventricular arrhythmias in MVP patients and their link with echocardiographic characteristics. Methods All patients (n=731, MVP = 486, Controls = 245) were prospectively enrolled and underwent a comprehensive echocardiography and ECG. In MVP patients 81 had minimal systolic displacement (MSD), 92 isolated MVP, 108 mild-moderate MR, and 196 severe MR. A comprehensive qualitative and quantitative analysis of ECG obtained from rest ECG, 24-hours ECG recording or exercise stress tests, was carried out. Mean follow-up was 4.4 years. Results The mains ECG abnormalities were an inversion of T wave in the inferior leads found in 12 MVP vs 1 control (2.5 vs 0.4%, P=0.047) or a QRS notch (5.1 vs 2.9%, P=0.13). In bileaflet MVP T wave inversion was more frequent as compared with other MVP patients (3.8 vs 0.8%, P=0.039). In addition there was a progressive prolongation of PR interval, QRS duration and increase QT dispersion associated with worsening of MR and heart chambers remodeling. None of ECG findings were significantly linked with the presence of MVP only. Out of 731 individuals, 27 (3.7%) had an history of VA or SD. In a multivariate analysis, bileaflet prolapse and mitral annulus disjunction were associated with VA or SD, whereas none of ECG criterion was associated with. Conclusion In this large prospective series of MVP patients, prevalence of inferior leads ECG abnormalities is very low. Prolongation of atrio-ventricular and ventricular conduction, as well increased QT dispersion is associated with worsening of MR and LV remodeling. Bileaflet prolapse and mitral annulus disjunction are associated with VA or SD. Funding Acknowledgement Type of funding source: None

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