Hidden Danger Behind the Prolapse
2019; Lippincott Williams & Wilkins; Volume: 12; Issue: 10 Linguagem: Inglês
10.1161/circimaging.119.009639
ISSN1942-0080
AutoresMassimo Slavich, Anna Palmisano, Luigi Pannone, Eustachio Agricola, Alberto Margonato, Antonio Esposito,
Tópico(s)Cardiovascular Issues in Pregnancy
ResumoHomeCirculation: Cardiovascular ImagingVol. 12, No. 10Hidden Danger Behind the Prolapse Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessCase ReportPDF/EPUBHidden Danger Behind the Prolapse Massimo Slavich, MD, Anna Palmisano, MD, Luigi Pannone, MD, Eustachio Agricola, MD, Alberto Margonato, MD and Antonio Esposito, MD Massimo SlavichMassimo Slavich Massimo Slavich, MD, Division of Cardiology, San Raffaele Scientific Institute, Via Olgettina 60, Milan, Italy. Email E-mail Address: [email protected] Division of Cardiology (M.S., L.P., E.A., A.M.), IRCCS Ospedale San Raffaele, Milan, Italy. , Anna PalmisanoAnna Palmisano Division of Radiology (A.P., A.E.), IRCCS Ospedale San Raffaele, Milan, Italy. , Luigi PannoneLuigi Pannone Division of Cardiology (M.S., L.P., E.A., A.M.), IRCCS Ospedale San Raffaele, Milan, Italy. , Eustachio AgricolaEustachio Agricola Division of Cardiology (M.S., L.P., E.A., A.M.), IRCCS Ospedale San Raffaele, Milan, Italy. , Alberto MargonatoAlberto Margonato Division of Cardiology (M.S., L.P., E.A., A.M.), IRCCS Ospedale San Raffaele, Milan, Italy. and Antonio EspositoAntonio Esposito Division of Radiology (A.P., A.E.), IRCCS Ospedale San Raffaele, Milan, Italy. Originally published1 Oct 2019https://doi.org/10.1161/CIRCIMAGING.119.009639Circulation: Cardiovascular Imaging. 2019;12:e009639A 25-year-old woman came to our attention because of palpitations and a systolic murmur. ECG (EKG) was normal (Figure 1A), and 24-hour holter monitoring performed on an asymptomatic day was unremarkable too. Transthoracic echocardiography showed bileaflet mitral valve prolapse (MVP; Figure 1B; Movies I through III in the Data Supplement) with mild regurgitation (B). High-velocity systolic signal with tissue doppler imaging was documented on the lateral mitral valve annulus (Pickelhaube sign; Figure 1C). Because of the presence of these echocardiographic risk markers for malignant MVP and the referred palpitation, cardiac magnetic resonance was performed. On cardiac magnetic resonance, the presence of mild mitral regurgitation due to a bileaflet prolapse was confirmed and a hypermobility of the mitral valve apparatus with paradoxical systolic increase of the mitral annulus diameter was detected (Movies IV through VI in the Data Supplement). Detachment of the roots of the mitral annulus from the ventricular myocardium (mitral annulus disjunction) was found (Figure 2A, red arrow). Although the distance cutoff is not established, a length >4.8 mm is often associated to myocardial fibrosis and >8.5 mm to higher risk of arrhythmic events. Moreover, the myocardial function adjacent to the prolapsed leaflets (especially the posterior) was increased, with lateral basal segment focal hypertrophy (curling; Figure 2B, red arrow). Late gadolinium enhancement images showed mesocardial late gadolinium enhancement involving inferolateral, inferior, and septal basal segments (arrows; Figure 3A through 3C) associated to native T1 values higher than normal (1137 ms), suggesting a diffuse increase of interstitial fibrosis (Figure 3D). After the multimodality imaging evaluation, the patient was considered at high risk for significant arrhythmic events. Therefore, the patient underwent electrophysiological study that was positive for ventricular fibrillation inducibility with 2 extrastimuli. Implantable cardioverter defibrillator (ICD) was implanted for primary prevention. After 2 years, spontaneous ventricular fibrillation was promptly detected and treated by a single ICD shock after antitachycardia pacing failure (Figure 4).Download figureDownload PowerPointFigure 1. EKG and echocardiography findings. Normal EKG: mitral valve prolapse (MVP) patients with an abnormal EKG (especially with negative or biphasic T waves in the inferior leads) need to be further stratified. However, the presence of a normal EKG in MVP patients symptomatic for palpitation should alert clinicians too (A). Bilifleat MVP: it is a known high risk feature for sudden cardiac death, but its relevance increases in the presence of other risk factors (B). Pickelhaube sign: the presence of a peak systolic lateral mitral annulus velocity >15 cm/s is associated to a higher risk of malignant ventricular arrhythmic events (C).Download figureDownload PowerPointFigure 2. Cardiac Magnetic Resonance findings. Mitral annulus disjunction: it is localized near to the posterior leaflet and represents the detachment of the roots of the annulus from the ventricular myocardium (A, red arrow). Curling: lateral basal focal hypertrophy (B, red arrow).Download figureDownload PowerPointFigure 3. Cardiac magnetic resonance. Late gadolinium enhancement in the left ventricular inferolateral, inferior, and septal basal segments are reported (A–C). T1 mapping with native T1 values higher than normal (D).Download figureDownload PowerPointFigure 4. Implantable cardioverter defibrillator (ICD) interrogation: spontaneous ventricular fibrillation promptly detected and interrupted by a single ICD shock (not shown).MVP affects 1% to 3% of the population with an annual risk of death estimated between 0.2% and 1.9%. Nowadays, several risk factors for sudden cardiac death have been identified, but specific guidelines in arrhythmic MVP management are still missing. Our patient, despite the absence of negative T waves on the EKG, which is present in >75% of malignant MVP, had other high risk features: Pickelhaube sign, mitral annulus disjunction, systolic curling, and severe left ventricular fibrosis on late gadolinium enhancement; moreover, the patient was symptomatic for palpitations. For these reasons, electrophysiological study was performed and ICD implanted. Therefore, we suggest to perform electrophysiological study for risk stratification when after clinical and multimodality imaging evaluation, risk factors are demonstrated. Sustained ventricular tachycardia or ventricular fibrillation induction should warrant ICD implantation.In this case, a complete clinical and multimodality imaging assessment was used for sudden cardiac death stratification and effective primary prevention of sudden cardiac death.DisclosuresNone.FootnotesThe Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.119.009639.Massimo Slavich, MD, Division of Cardiology, San Raffaele Scientific Institute, Via Olgettina 60, Milan, Italy. Email slavich.[email protected]it Previous Back to top Next FiguresReferencesRelatedDetailsCited By Nagata Y, Bertrand P and Levine R (2022) Malignant Mitral Valve Prolapse: Risk and Prevention of Sudden Cardiac Death, Current Treatment Options in Cardiovascular Medicine, 10.1007/s11936-022-00956-3, 24:5, (61-86), Online publication date: 1-May-2022. Marano P, Lim L, Sanchez J, Alvi R, Nah G, Badhwar N, Gerstenfeld E, Tseng Z, Marcus G and Delling F (2020) Long-term outcomes of ablation for ventricular arrhythmias in mitral valve prolapse, Journal of Interventional Cardiac Electrophysiology, 10.1007/s10840-020-00775-1, 61:1, (145-154), Online publication date: 1-Jun-2021. Gatti M, Palmisano A, Esposito A, Fiore S, Monti C, Andreis A, Pistelli L, Vergara P, Bergamasco L, Giustetto C, De Cobelli F, Fonio P and Faletti R (2021) Feature tracking myocardial strain analysis in patients with bileaflet mitral valve prolapse: relationship with LGE and arrhythmias, European Radiology, 10.1007/s00330-021-07876-z, 31:10, (7273-7282), Online publication date: 1-Oct-2021. October 2019Vol 12, Issue 10 Advertisement Article InformationMetrics © 2019 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.119.009639PMID: 31569957 Originally publishedOctober 1, 2019 Keywordshumansmitral valve prolapseadultattentiondeath, sudden, cardiacPDF download Advertisement SubjectsEchocardiographyMagnetic Resonance Imaging (MRI)Sudden Cardiac Death
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