Citations 2
Characteristics of Patients With the Arrhythmogenic Mitral Valve Prolapse Syndrome and Sudden Cardiac Arrest and Sustained Ventricular Arrhythmias
Chakrabarti, Apurba , Giudicessi, John R. , Delling, Francesca N. , Dixit, Shalini , Lee, Yoo Jin , Muser, Daniele , Magnani, Silvia , Wijngaarden, Aniek L. van , Miller, Marc A. , Gandhi, Jonathan, Font, Jonaz , Martins, Raphaël P. , McCaffrey, James A. , Chapagain, Himal , Mathew, Don , Kancharla, Krishna , Syed, Faisal F. , Abid, Ahad, Cerbin, Lukasz , Garg, Lohit , Natale, Andrea , Enríquez, Andrés , Bogun, Frank , Ackerman, Michael J. , Liang, Jackson J. , Ezzeddine, Fatima , Delling, Francesca N , Lee, Yoo Jin , Muser, Daniele , Magnani, Silvia , van Wijngaarden, Aniek , Marsan, Nina Ajmone , Trivieri, Maria G , Font, Jonaz , Martins, Raphael , McCaffrey, James A , Santangeli, Pasquale , Marchlinski, Francis E , Chapagain, Himal , Mathew, Don , Kancharla, Krishna , Syed, Faisal F , Abid, Ahad , Tzou, Wendy , Della Rocca, Domenico G , Natale, Andrea , Mohanty, Sanghamitra , Sheldon, Seth H. , Kuo, Ling , Haugaa, Kristina , Aabel, Eivind W , Enriquez, Andres , Maeda, Shingo , Deshmukh, Amrish , Ackerman, Michael J , Liang, Jackson , Ezzeddine, Fatima M. , Marsan, Nina Ajmone , Santangeli, Pasquale , Marchlinski, Francis E. , Tzou, Wendy S. , Rocca, Domenico G. Della , Haugaa, Kristina H. , Aabel, Eivind W. , Deshmukh, Amrish , Ghannam, Michael
Circulation: Arrhythmia and Electrophysiology | February 20, 2025 | Volume: 18 | Issue: 3 | Pages: No page available
BACKGROUND: Patients with arrhythmogenic mitral valve prolapse syndrome are at increased risk for life-threatening ventricular arrhythmias, but studies have been limited by small sample sizes. We sought to assemble an international arrhythmogenic mitral valve prolapse syndrome registry to delineate the clinical, imaging, and treatment characteristics of patients with arrhythmogenic mitral valve prolapse syndrome who survived sudden cardiac arrest (SCA) or had sustained ventricular tachycardia (VT) or ventricular fibrillation. METHODS: In this descriptive registry, we characterized patients with arrhythmogenic mitral valve prolapse syndrome who survived SCA, sustained VT, or ventricular fibrillation. Deidentified data were abstracted locally and combined centrally. RESULTS: We included 148 patients who had SCA or VT/ventricular fibrillation. Patients had a mean age of 43.7±15.4 years; 68% were women, 73% had bileaflet prolapse, 65% had mitral annular disjunction, 67% had nonsustained VT, and 59% had inferolateral T-wave inversions. Syncope (n=54, 48%) and anterolateral T-wave inversion (n=26, 22%) were relatively common. Catheter ablation was performed in 50 (35%) patients for premature ventricular complexes and in 18 (17.7%) patients for VT. Sites of origin for arrhythmias were commonly in the papillary muscles, fascicles, mitral annulus, and inferior/inferolateral left ventricle. CONCLUSIONS: In this international descriptive registry of patients with arrhythmogenic mitral valve prolapse syndrome and SCA, patients were young, women, and had bileaflet mitral valve prolapse, mitral annular disjunction, inferolateral T-wave inversions, and nonsustained VT. A history of syncope and anterolateral T-wave inversions was relatively common in patients who survived SCA or sustained VT/ventricular fibrillation.
Mitral valve prolapse: arrhythmic risk during pregnancy and postpartum
Sabbag, Avi , Aabel, Eivind W. , Castrini, Anna Isotta , Siontis, Konstantinos C. , Duthoit, Guillaume , Asirvatham, Samuel J. , Sehrawat, Ojasay, Kirkels, Feddo P. , Rav, Moshe , Peichl, Petr , Lim, H. , Martins, Raphaël P. , Font, Jonaz , Truong, Nguyen Ngoc Khoi , Estensen, Mette E , Laredo, Mikael , Duthoit, Guillaume , Kirkels, Feddo P , Beinart, Roy , Peichl, Petr , Sohns, Christian , Haugaa, Kristina , Asirvatham, Samuel J , Martins, Raphael , Aabel, Eivind W , Sabbag, Avi , Nizard, J. , Rosendael, Philippe J. van , Beinart, Roy , Haugaa, Kristina H.
European Heart Journal | May 14, 2024 | Volume: 45 | Issue: 20 | Pages: 1831-1839
Abstract Background and Aims Arrhythmic mitral valve prolapse (AMVP) is linked to life-threatening ventricular arrhythmias (VAs), and young women are considered at high risk. Cases of AMVP in women with malignant VA during pregnancy have emerged, but the arrhythmic risk during pregnancy is unknown. The authors aimed to describe features of women with high-risk AMVP who developed malignant VA during the perinatal period and to assess if pregnancy and the postpartum period were associated with a higher risk of malignant VA. Methods This retrospective international multi-centre case series included high-risk women with AMVP who experienced malignant VA and at least one pregnancy. Malignant VA included ventricular fibrillation, sustained ventricular tachycardia, or appropriate shock from an implantable cardioverter defibrillator. The authors compared the incidence of malignant VA in non-pregnant periods and perinatal period; the latter defined as occurring during pregnancy and within 6 months after delivery. Results The authors included 18 women with AMVP from 11 centres. During 7.5 (interquartile range 5.8–16.6) years of follow-up, 37 malignant VAs occurred, of which 18 were pregnancy related occurring in 13 (72%) unique patients. Pregnancy and 6 months after delivery showed increased incidence rate of malignant VA compared to the non-pregnancy period (univariate incidence rate ratio 2.66, 95% confidence interval 1.23–5.76). Conclusions The perinatal period could impose increased risk of malignant VA in women with high-risk AMVP. The data may provide general guidance for pre-conception counselling and for nuanced shared decision-making between patients and clinicians.