TY - JOUR
T1 - Prevalence and clinical phenotype of concomitant long QT syndrome and arrhythmogenic bileaflet mitral valve prolapse
AU - Giudicessi, John R.
AU - Rohatgi, Ram K.
AU - Bos, Johan M.
AU - Ackerman, Michael J.
N1 - Publisher Copyright:
© 2018 Elsevier B.V.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - Background: Mitral valve prolapse (MVP), including the recently described arrhythmogenic bileaflet MVP syndrome (ABiMVPS), is associated with repolarization abnormalities and may represent an underestimated cause of sudden cardiac death. The impact of concomitant MVP or ABiMVPS on long QT syndrome (LQTS) clinical severity is unknown. Methods and results: Retrospective review of 754 LQTS patients [445 females (58%) and mean QTc 471 ± 41 ms] with available echocardiographic data was performed to identify LQTS patients with not only MVP, but also a pro-arrhythmic ABiMVPS phenotype defined as bileaflet MVP, inferolateral T-wave inversions, and frequent complex ventricular ectopy/arrhythmia. As expected, 18/754 (2%) LQTS patients had concomitant MVP. Of these, 5/18 (28%) LQTS patients with MVP satisfied ABiMVPS diagnostic criteria. No difference in symptomatology, degree of QT prolongation, or clinical management was observed between LQTS patients with and without MVP. In contrast, LQTS plus ABiMVPS resulted in a severe cardiac phenotype as illustrated by symptomatic status (LQTS-ABiMVPS; 5/5; 100%; vs LQTS: 279/736; 39%; p =.008), degree of baseline QTc prolongation (LQTS-ABiMVPS: 536 ± 58 ms; vs LQTS: 470 ± 40 ms; p =.009), and number of patients experiencing ≥1 on-therapy break-through cardiac event (LQTS-ABiMVPS: 4/5; 80%; vs LQTS: 48/736; 7%; p <.001]. Lastly, individuals with LQTS plus ABiMVPS were more likely to experience appropriate ICD therapies post-cardiac denervation (LQTS-ABiMVPS: 2/3; 67% vs LQTS: 4/49; 8%; p =.03]. Conclusions and relevance: The co-existence of LQTS and ABiMVPS may lead to a rare, but malignant, clinical entity characterized by potentially life-threatening arrhythmias despite maximal LQTS therapy.
AB - Background: Mitral valve prolapse (MVP), including the recently described arrhythmogenic bileaflet MVP syndrome (ABiMVPS), is associated with repolarization abnormalities and may represent an underestimated cause of sudden cardiac death. The impact of concomitant MVP or ABiMVPS on long QT syndrome (LQTS) clinical severity is unknown. Methods and results: Retrospective review of 754 LQTS patients [445 females (58%) and mean QTc 471 ± 41 ms] with available echocardiographic data was performed to identify LQTS patients with not only MVP, but also a pro-arrhythmic ABiMVPS phenotype defined as bileaflet MVP, inferolateral T-wave inversions, and frequent complex ventricular ectopy/arrhythmia. As expected, 18/754 (2%) LQTS patients had concomitant MVP. Of these, 5/18 (28%) LQTS patients with MVP satisfied ABiMVPS diagnostic criteria. No difference in symptomatology, degree of QT prolongation, or clinical management was observed between LQTS patients with and without MVP. In contrast, LQTS plus ABiMVPS resulted in a severe cardiac phenotype as illustrated by symptomatic status (LQTS-ABiMVPS; 5/5; 100%; vs LQTS: 279/736; 39%; p =.008), degree of baseline QTc prolongation (LQTS-ABiMVPS: 536 ± 58 ms; vs LQTS: 470 ± 40 ms; p =.009), and number of patients experiencing ≥1 on-therapy break-through cardiac event (LQTS-ABiMVPS: 4/5; 80%; vs LQTS: 48/736; 7%; p <.001]. Lastly, individuals with LQTS plus ABiMVPS were more likely to experience appropriate ICD therapies post-cardiac denervation (LQTS-ABiMVPS: 2/3; 67% vs LQTS: 4/49; 8%; p =.03]. Conclusions and relevance: The co-existence of LQTS and ABiMVPS may lead to a rare, but malignant, clinical entity characterized by potentially life-threatening arrhythmias despite maximal LQTS therapy.
KW - Arrhythmia
KW - Long QT syndrome
KW - Mitral valve prolapse
KW - Out-of-hospital cardiac arrest
KW - Sudden cardiac death
UR - http://www.scopus.com/inward/record.url?scp=85053128247&partnerID=8YFLogxK
U2 - 10.1016/j.ijcard.2018.09.046
DO - 10.1016/j.ijcard.2018.09.046
M3 - Article
C2 - 30219255
AN - SCOPUS:85053128247
SN - 0167-5273
VL - 274
SP - 175
EP - 178
JO - International Journal of Cardiology
JF - International Journal of Cardiology
ER -