Arrhythmic mitral valve prolapse
Updates to Article Attributes
Arrhythmic or arrhythmogenic mitral valve prolapse is an entity that describes the presence of frequent or complex ventricular arrhythmias and mitral valve prolapse, possibly associated with mitral annular disjunction1-3.
The following two major phenotypic subtypes are recognised 1:
degenerative mitral valve prolapse with severe mitral regurgitation
severe myxomatous mitral valve prolapse irrespective of the degree of mitral regurgitation
Epidemiology
Arrhythmogenic mitral valve prolapse seems to be more common in young females 2.
Diagnosis
According to an expert consensus statement, the diagnosis can be established if the following findings are present 1:
mitral valve prolapse possibly associated with mitral annular disjunction
frequent or complex ventricular arrhythmias
absence of any other well-known arrhythmic substrates
Clinical presentation
Common symptoms include palpitations, chest pain and dyspnoea but patients might also be completely asymptomatic or show mild symptoms such as atypical chest discomfort or anxiety.
Syncope is a more alarming sign and should be investigated further 1.
The presence of either frequent premature ventricular contractions (≥5% total burden) or complex ventricular arrhythmias such as nonsustained ventricular tachycardia (NSVT), ventricular tachycardia or ventricular fibrillation (VS) are required as a criterion for the diagnosis 1.
Some ECG features associated with arrhythmias are T-wave abnormalities such as T-wave inversion and biphasic T-waves, especially in inferior and lateral leads 1,2.
Complications
Complications of arrhythmogenic mitral valve prolapse include cardiac arrest and sudden cardiac death 1-3.
Radiographic features
Main imaging features include the demonstration of mitral valve prolapse defined as displacement of the mitral leaflets >2 mm above the mitral annular plane3.
Echocardiography
Echocardiography displays mitral valve prolapse often associated with mitral annular disjunction involving the insertion of the posterior leaflet on the mitral annulus possibly leading to mechanical dispersion and postsystolic shortening of the inferobasal segments of the left ventricle.
MRI
In addition to mitral valve prolapse and mitral annular disjunction MRI might show myocardial fibrosis of the papillary muscles and the inferobasal segments and the periannular region 1-4, demonstrated as non-ischaemic pattern of late gadolinium enhancement suggesting replacement fibrosis or increased T1 mapping values 1,3.
Radiology report
The radiology report should include a description of the following features 1-4:
-
affected leaflets
prolapse depth, leaflet length
myxomatous valve, leaflet thickness
mitral regurgitation with degree
-
mitral annular disjunction (if present)
extent and severity
mitral annulus systolic enlargement (ap and commissural diameter in end-systole and end-diastole)
the ratio of basal to midventricular wall thickness
left ventricular remodelling
-
myocardial fibrosis (focal or diffuse)
papillary muscles
periannular region
Treatment and prognosis
Treatment options are aimed at alleviating symptoms and improving survival and include medical therapy, ICD implantation, catheter ablation and mitral valve surgery, the latter being controversial as a stand-alone treatment 1.
- +<p><strong>Arrhythmic</strong> or <strong>arrhythmogenic mitral valve prolapse </strong>is an entity that describes the presence of frequent or complex <a href="/articles/ventricular-arrhythmia" title="Ventricular arrhythmias">ventricular arrhythmias</a> and <a href="/articles/mitral-valve-prolapse" title="Mitral valve prolapse">mitral valve prolapse</a>, possibly associated with <a href="/articles/mitral-annular-disjunction-1" title="Mitral annular disjunction">mitral annular disjunction</a> <sup>1-3</sup>.</p><p>The following two major phenotypic subtypes are recognised <sup>1</sup>:</p><ul>
- +<li><p>degenerative mitral valve prolapse with severe <a href="/articles/mitral-valve-regurgitation" title="Mitral regurgitation">mitral regurgitation</a></p></li>
- +<li><p>severe myxomatous mitral valve prolapse irrespective of the degree of mitral regurgitation</p></li>
- +</ul><h4>Epidemiology</h4><p>Arrhythmogenic mitral valve prolapse seems to be more common in young females <sup>2</sup>.</p><h4>Diagnosis</h4><p>According to an expert consensus statement, the diagnosis can be established if the following findings are present <sup>1</sup>:</p><ul>
- +<li><p>mitral valve prolapse possibly associated with mitral annular disjunction</p></li>
- +<li><p>frequent or complex ventricular arrhythmias</p></li>
- +<li><p>absence of any other well-known arrhythmic substrates</p></li>
- +</ul><h4>Clinical presentation</h4><p>Common symptoms include palpitations, chest pain and dyspnoea but patients might also be completely asymptomatic or show mild symptoms such as atypical chest discomfort or anxiety. </p><p>Syncope is a more alarming sign and should be investigated further <sup>1</sup>.</p><p>The presence of either frequent premature ventricular contractions (≥5% total burden) or complex ventricular arrhythmias such as nonsustained ventricular tachycardia (NSVT), ventricular tachycardia or ventricular fibrillation (VS) are required as a criterion for the diagnosis <sup>1</sup>.</p><p>Some ECG features associated with arrhythmias are T-wave abnormalities such as T-wave inversion and biphasic T-waves, especially in inferior and lateral leads <sup>1,2</sup>.</p><h5>Complications</h5><p>Complications of arrhythmogenic mitral valve prolapse include cardiac arrest and sudden cardiac death <sup>1-3</sup>.</p><h4>Radiographic features</h4><p>Main imaging features include the demonstration of mitral valve prolapse defined as displacement of the mitral leaflets >2 mm above the <a href="/articles/mitral-annulus" title="Mitral annulus">mitral annular plane</a> <sup>3</sup>.</p><h5>Echocardiography</h5><p>Echocardiography displays mitral valve prolapse often associated with mitral annular disjunction involving the insertion of the posterior leaflet on the mitral annulus possibly leading to mechanical dispersion and postsystolic shortening of the inferobasal segments of the left ventricle.</p><h5>MRI</h5><p>In addition to mitral valve prolapse and mitral annular disjunction MRI might show <a href="/articles/myocardial-fibrosis" title="Myocardial fibrosis">myocardial fibrosis</a> of the <a href="/articles/papillary-muscles" title="Papillary muscles">papillary muscles</a> and the inferobasal segments and the periannular region <sup>1-4</sup>, demonstrated as non-ischaemic pattern of <a href="/articles/late-gadolinium-enhancement-2" title="Late gadolinium enhancement">late gadolinium enhancement</a> suggesting replacement fibrosis or increased <a href="/articles/t1-mapping-myocardium" title="T1 mapping - myocardium">T1 mapping</a> values <sup>1,3</sup>.</p><h4>Radiology report</h4><p>The radiology report should include a description of the following features <sup>1-4</sup>:</p><ul>
- +<li>
- +<p><a href="/articles/mitral-valve-prolapse" title="Mitral valve prolapse">mitral valve prolapse</a></p>
- +<ul>
- +<li><p>affected leaflets</p></li>
- +<li><p>prolapse depth, leaflet length</p></li>
- +<li><p>myxomatous valve, leaflet thickness</p></li>
- +</ul>
- +</li>
- +<li><p><a href="/articles/mitral-valve-regurgitation" title="Mitral regurgitation">mitral regurgitation</a> with degree</p></li>
- +<li>
- +<p><a href="/articles/mitral-annular-disjunction-1" title="Mitral annular disjunction">mitral annular disjunction</a> (if present)</p>
- +<ul>
- +<li><p>extent and severity</p></li>
- +<li><p>mitral annulus systolic enlargement (ap and commissural diameter in end-systole and end-diastole)</p></li>
- +<li><p>the ratio of basal to midventricular wall thickness</p></li>
- +</ul>
- +</li>
- +<li><p>left ventricular remodelling</p></li>
- +<li>
- +<p><a href="/articles/myocardial-fibrosis" title="Myocardial fibrosis">myocardial fibrosis</a> (focal or diffuse)</p>
- +<ul>
- +<li><p>papillary muscles</p></li>
- +<li><p>periannular region</p></li>
- +</ul>
- +</li>
- +</ul><h4>Treatment and prognosis</h4><p>Treatment options are aimed at alleviating symptoms and improving survival and include medical therapy, ICD implantation, catheter ablation and mitral valve surgery, the latter being controversial as a stand-alone treatment <sup>1</sup>.</p>
References changed:
- 1. Sabbag A, Essayagh B, Barrera J et al. EHRA Expert Consensus Statement on Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction Complex in Collaboration with the ESC Council on Valvular Heart Disease and the European Association of Cardiovascular Imaging Endorsed Cby the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society. Europace. 2022;24(12):1981-2003. <a href="https://doi.org/10.1093/europace/euac125">doi:10.1093/europace/euac125</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/35951656">Pubmed</a>
- 2. Miller M, Dukkipati S, Turagam M, Liao S, Adams D, Reddy V. Arrhythmic Mitral Valve Prolapse: JACC Review Topic of the Week. J Am Coll Cardiol. 2018;72(23 Pt A):2904-14. <a href="https://doi.org/10.1016/j.jacc.2018.09.048">doi:10.1016/j.jacc.2018.09.048</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/30522653">Pubmed</a>
- 3. Korovesis T, Koutrolou-Sotiropoulou P, Katritsis D. Arrhythmogenic Mitral Valve Prolapse. Arrhythm Electrophysiol Rev. 2022;11:e16. <a href="https://doi.org/10.15420/aer.2021.28">doi:10.15420/aer.2021.28</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/35990107">Pubmed</a>
- 4. Deng Y, Liu J, Wu S et al. Arrhythmic Mitral Valve Prolapse: A Comprehensive Review. Diagnostics (Basel). 2023;13(18):2868. <a href="https://doi.org/10.3390/diagnostics13182868">doi:10.3390/diagnostics13182868</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/37761235">Pubmed</a>
- 5. Essayagh B, Iacuzio L, Civaia F, Avierinos J, Tribouilloy C, Levy F. Usefulness of 3-Tesla Cardiac Magnetic Resonance to Detect Mitral Annular Disjunction in Patients With Mitral Valve Prolapse. Am J Cardiol. 2019;124(11):1725-30. <a href="https://doi.org/10.1016/j.amjcard.2019.08.047">doi:10.1016/j.amjcard.2019.08.047</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/31606191">Pubmed</a>
Tags changed:
- mitral valve
Systems changed:
- Cardiac