Pol. Merkur. Lek (Pol. Med. J.), 2020, XLVIII/287: 335-338 Maximize

Pol. Merkur. Lek (Pol. Med. J.), 2020, XLVIII/287: 335-338

Title: Multimodality imaging of mitral annular disjunction 

Authors: Elikowski W, Małek-Elikowska M, Ganowicz-Kaatz T, Greberska W, Fertała N, Zawodna-Marszałek M, Baszko A.

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Multimodality imaging of mitral annular disjunction

Elikowski W1, Małek-Elikowska M2, Ganowicz-Kaatz T3, Greberska W3, Fertała N1, Zawodna-Marszałek M1, Baszko A4.

1Department of Internal Medicine, Józef Struś Hospital, Poznań, Poland: 22nd Department of Cardiology, Poznań University of Medical Sciences, Poland; 3Department of Cardiology, Józef Struś Hospital, Poznań, Poland; 4Radiology Unit; Józef Struś Hospital, Poznań, Poland

Mitral valve prolapse (MVP) is diagnosed by auscultation and echocardiography in about 2-3% of the general population and takes rather a benign course. However, in some patients, ventricular arrhythmia and sudden cardiac death (SCD) occur, which is linked to mitral annular disjunction (MAD). MAD is defined as distinct separation of the mitral valve annulus-left atrial wall continuum and the basal region of the posterolateral left ventricular (LV) myocardium. MAD results in disturbed inferior-posterior LV wall and posteromedial papillary muscle stretch giving rise to local fibrosis presenting in cardiac magnetic resonance (CMR) as late gadolinium enhancement (LGE) and posing a substrate for malignant arrhythmia. Multidetector-row computed tomography (MDCT) in MAD patients is still rarely used.
A case report
. The authors describe a 39-year old female, with MVP diagnosed several years ago, admitted due to recurrent palpitation and an episode of collapse. ECG showed polymorphic ventricular extrasystoles with right bundle branch block morphology. Echocardiography revealed bileaflet MVP, MAD with a 15 mm maximal distance (in systole), mild mitral regurgitation and posterior wall systolic curling corresponding to exaggerated longitudinal strain (-32%) in the middle segment of the posterior wall. Tissue doppler imaging (TDI) of the lateral mitral annulus revealed high-velocity mid-systolic spike (the pickelhaube sign). MAD was also distinctly seen in CMR and MDCT. Foci of LGE were found in CMR in the basal posterior LV segment. Multimodality MAD imaging, including different echocardiographic techniques, CMR and MDCT, allows selection of patients who should benefit from further procedures such as implantable cardioverter/defibrillator implantation, arrhythmia substrate ablation or MVP surgery.

Key words: mitral annular disjunction, mitral valve prolapse, malignant arrhythmia, sudden cardiac death, echocardiography, cardiac magnetic resonance, multidetector-row computed tomography

Pol Med J, 2020; XLVIII (287); 335–338