Long‐term follow‐up of patients with short QT syndrome: clinical profile and outcome
BackgroundShort QT syndrome (SQTS) is a rare inheritable disease associated with sudden cardiac death. Data on long‐term outcomes of families with SQTS are limited.Methods and ResultsSeventeen patients with SQTS in 7 independent families (48% men; median age, 42.4 years; corrected QT interval, 324.9...
Saved in:
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article (Journal) |
| Language: | English |
| Published: |
27 Nov 2018
|
| In: |
Journal of the American Heart Association
Year: 2018, Volume: 7, Issue: 23, Pages: 1-9 |
| ISSN: | 2047-9980 |
| DOI: | 10.1161/JAHA.118.010073 |
| Online Access: | Verlag, Volltext: https://doi.org/10.1161/JAHA.118.010073 Verlag, Volltext: https://www.ahajournals.org/doi/10.1161/JAHA.118.010073 
|
| Author Notes: | El‐Battrawy Ibrahim, Besler Johanna, Liebe Volker, Schimpf Rainer, Tülümen Erol, Rudic Boris, Lang Siegfried, Wolpert Christian, Zhou Xiaobo, Akin Ibrahim, and Borggrefe Martin |
MARC
| LEADER | 00000caa a2200000 c 4500 | ||
|---|---|---|---|
| 001 | 167163635X | ||
| 003 | DE-627 | ||
| 005 | 20240323101248.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 190820s2018 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1161/JAHA.118.010073 |2 doi | |
| 035 | |a (DE-627)167163635X | ||
| 035 | |a (DE-599)KXP167163635X | ||
| 035 | |a (OCoLC)1341237851 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rda | ||
| 041 | |a eng | ||
| 084 | |a 33 |2 sdnb | ||
| 100 | 1 | |a El-Battrawy, Ibrahim |d 1987- |e VerfasserIn |0 (DE-588)1033436917 |0 (DE-627)741219549 |0 (DE-576)38084673X |4 aut | |
| 245 | 1 | 0 | |a Long‐term follow‐up of patients with short QT syndrome |b clinical profile and outcome |c El‐Battrawy Ibrahim, Besler Johanna, Liebe Volker, Schimpf Rainer, Tülümen Erol, Rudic Boris, Lang Siegfried, Wolpert Christian, Zhou Xiaobo, Akin Ibrahim, and Borggrefe Martin |
| 264 | 1 | |c 27 Nov 2018 | |
| 300 | |a 9 | ||
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Gesehen am 20.08.2019 | ||
| 520 | |a BackgroundShort QT syndrome (SQTS) is a rare inheritable disease associated with sudden cardiac death. Data on long‐term outcomes of families with SQTS are limited.Methods and ResultsSeventeen patients with SQTS in 7 independent families (48% men; median age, 42.4 years; corrected QT interval, 324.9±40.8 ms) were followed up for 13.5±2.5 years. A history of sudden cardiac death was documented in 71% of families. A large number of them showed sudden cardiac deaths at a younger age, with a predominance of men (67%). Five patients had syncope (29%) and 9 (53%) had atrial fibrillation or atrial flutter. An SQTS‐related gene was found in 76% of the patients as follows: KCNH2 (SQTS 1) in 4, CACNA1C (SQTS 4) in 3, and CACNb2 (SQTS 5) in 6. Five patients (29%) received an implantable cardioverter‐defibrillator and 5 patients received long‐term prophylaxis with hydroquinidine. During follow‐up, 1 patient received an appropriate implantable cardioverter‐defibrillator shock attributable to ventricular fibrillation. The patient received no further implantable cardioverter‐defibrillator shocks after treatment with hydroquinidine.ConclusionsThe risk of sudden cardiac death in SQTS families is high. However, after appropriate risk assessment and individualized treatment options (hydroquinidine and/or implantable cardioverter‐defibrillator), the long‐term outcome is relatively benign when patients are seen at a reference center. | ||
| 700 | 1 | |a Liebe, Volker |d 1976- |e VerfasserIn |0 (DE-588)124795757 |0 (DE-627)366425102 |0 (DE-576)294506926 |4 aut | |
| 700 | 1 | |a Schimpf, Rainer |d 1966- |e VerfasserIn |0 (DE-588)1025920244 |0 (DE-627)725573767 |0 (DE-576)370912772 |4 aut | |
| 700 | 1 | |a Tueluemen, Erol |e VerfasserIn |0 (DE-588)1035228955 |0 (DE-627)746999585 |0 (DE-576)382840534 |4 aut | |
| 700 | 1 | |a Rudic, Boris |d 1982- |e VerfasserIn |0 (DE-588)1024726789 |0 (DE-627)720427134 |0 (DE-576)369410599 |4 aut | |
| 700 | 1 | |a Lang, Siegfried |d 1955- |e VerfasserIn |0 (DE-588)1026112613 |0 (DE-627)726109290 |0 (DE-576)371365473 |4 aut | |
| 700 | 1 | |a Wolpert, Christian |d 1967- |e VerfasserIn |0 (DE-588)122247965 |0 (DE-627)705830284 |0 (DE-576)293172900 |4 aut | |
| 700 | 1 | |a Zhou, Xiao-Bo |d 1958- |e VerfasserIn |0 (DE-588)1117113744 |0 (DE-627)871032457 |0 (DE-576)478766742 |4 aut | |
| 700 | 1 | |a Akın, Ibrahim |d 1978- |e VerfasserIn |0 (DE-588)132322293 |0 (DE-627)521039010 |0 (DE-576)299074366 |4 aut | |
| 700 | 1 | |a Borggrefe, Martin |e VerfasserIn |0 (DE-588)1025920546 |0 (DE-627)725574232 |0 (DE-576)370913426 |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |a American Heart Association |t Journal of the American Heart Association |d New York, NY : Association, 2012 |g 7(2018,23) Artikel-Nummer e10073, 9 Seiten |h Online-Ressource |w (DE-627)688605427 |w (DE-600)2653953-6 |w (DE-576)361844719 |x 2047-9980 |7 nnas |
| 773 | 1 | 8 | |g volume:7 |g year:2018 |g number:23 |g pages:1-9 |g extent:9 |a Long‐term follow‐up of patients with short QT syndrome clinical profile and outcome |
| 856 | 4 | 0 | |u https://doi.org/10.1161/JAHA.118.010073 |x Verlag |x Resolving-System |3 Volltext |
| 856 | 4 | 0 | |u https://www.ahajournals.org/doi/10.1161/JAHA.118.010073 |x Verlag |3 Volltext |
| 951 | |a AR | ||
| 992 | |a 20190820 | ||
| 993 | |a Article | ||
| 994 | |a 2018 | ||
| 998 | |g 1025920546 |a Borggrefe, Martin |m 1025920546:Borggrefe, Martin |d 60000 |d 61000 |e 60000PB1025920546 |e 61000PB1025920546 |k 0/60000/ |k 1/60000/61000/ |p 11 |y j | ||
| 998 | |g 132322293 |a Akın, Ibrahim |m 132322293:Akın, Ibrahim |d 60000 |d 61000 |e 60000PA132322293 |e 61000PA132322293 |k 0/60000/ |k 1/60000/61000/ |p 10 | ||
| 998 | |g 1117113744 |a Zhou, Xiao-Bo |m 1117113744:Zhou, Xiao-Bo |d 60000 |d 61000 |e 60000PZ1117113744 |e 61000PZ1117113744 |k 0/60000/ |k 1/60000/61000/ |p 9 | ||
| 998 | |g 122247965 |a Wolpert, Christian |m 122247965:Wolpert, Christian |d 60000 |d 61000 |e 60000PW122247965 |e 61000PW122247965 |k 0/60000/ |k 1/60000/61000/ |p 8 | ||
| 998 | |g 1026112613 |a Lang, Siegfried |m 1026112613:Lang, Siegfried |d 60000 |d 61000 |e 60000PL1026112613 |e 61000PL1026112613 |k 0/60000/ |k 1/60000/61000/ |p 7 | ||
| 998 | |g 1024726789 |a Rudic, Boris |m 1024726789:Rudic, Boris |d 60000 |d 61000 |e 60000PR1024726789 |e 61000PR1024726789 |k 0/60000/ |k 1/60000/61000/ |p 6 | ||
| 998 | |g 1035228955 |a Tueluemen, Erol |m 1035228955:Tueluemen, Erol |d 60000 |d 61000 |e 60000PT1035228955 |e 61000PT1035228955 |k 0/60000/ |k 1/60000/61000/ |p 5 | ||
| 998 | |g 1025920244 |a Schimpf, Rainer |m 1025920244:Schimpf, Rainer |d 60000 |d 61000 |e 60000PS1025920244 |e 61000PS1025920244 |k 0/60000/ |k 1/60000/61000/ |p 4 | ||
| 998 | |g 124795757 |a Liebe, Volker |m 124795757:Liebe, Volker |d 60000 |e 60000PL124795757 |k 0/60000/ |p 3 | ||
| 998 | |g 1033436917 |a El-Battrawy, Ibrahim |m 1033436917:El-Battrawy, Ibrahim |d 60000 |d 61000 |e 60000PE1033436917 |e 61000PE1033436917 |k 0/60000/ |k 1/60000/61000/ |p 1 |x j | ||
| 999 | |a KXP-PPN167163635X |e 3507650800 | ||
| BIB | |a Y | ||
| SER | |a journal | ||
| JSO | |a {"language":["eng"],"person":[{"role":"aut","given":"Ibrahim","display":"El-Battrawy, Ibrahim","family":"El-Battrawy"},{"display":"Liebe, Volker","family":"Liebe","role":"aut","given":"Volker"},{"family":"Schimpf","display":"Schimpf, Rainer","role":"aut","given":"Rainer"},{"role":"aut","given":"Erol","family":"Tueluemen","display":"Tueluemen, Erol"},{"display":"Rudic, Boris","family":"Rudic","given":"Boris","role":"aut"},{"family":"Lang","display":"Lang, Siegfried","given":"Siegfried","role":"aut"},{"role":"aut","given":"Christian","family":"Wolpert","display":"Wolpert, Christian"},{"family":"Zhou","display":"Zhou, Xiao-Bo","given":"Xiao-Bo","role":"aut"},{"family":"Akın","display":"Akın, Ibrahim","given":"Ibrahim","role":"aut"},{"given":"Martin","role":"aut","family":"Borggrefe","display":"Borggrefe, Martin"}],"id":{"doi":["10.1161/JAHA.118.010073"],"eki":["167163635X"]},"recId":"167163635X","relHost":[{"language":["eng"],"titleAlt":[{"title":"Journal of the American Heart Association, Cardiovascular and Cerebrovascular Disease"},{"title":"JAHA"}],"part":{"issue":"23","text":"7(2018,23) Artikel-Nummer e10073, 9 Seiten","year":"2018","pages":"1-9","extent":"9","volume":"7"},"corporate":[{"display":"American Heart Association","role":"aut"}],"physDesc":[{"extent":"Online-Ressource"}],"recId":"688605427","id":{"eki":["688605427"],"issn":["2047-9980"],"zdb":["2653953-6"]},"origin":[{"publisherPlace":"New York, NY","dateIssuedDisp":"2012-","dateIssuedKey":"2012","publisher":"Association"}],"pubHistory":["1.2012 -"],"note":["Gesehen am 09.01.14"],"title":[{"title":"Journal of the American Heart Association","subtitle":"JAHA","title_sort":"Journal of the American Heart Association"}],"disp":"American Heart AssociationJournal of the American Heart Association","type":{"media":"Online-Ressource","bibl":"periodical"}}],"physDesc":[{"extent":"9 S."}],"name":{"displayForm":["El‐Battrawy Ibrahim, Besler Johanna, Liebe Volker, Schimpf Rainer, Tülümen Erol, Rudic Boris, Lang Siegfried, Wolpert Christian, Zhou Xiaobo, Akin Ibrahim, and Borggrefe Martin"]},"type":{"bibl":"article-journal","media":"Online-Ressource"},"title":[{"title":"Long‐term follow‐up of patients with short QT syndrome","subtitle":"clinical profile and outcome","title_sort":"Long‐term follow‐up of patients with short QT syndrome"}],"origin":[{"dateIssuedDisp":"27 Nov 2018","dateIssuedKey":"2018"}],"note":["Gesehen am 20.08.2019"]} | ||
| SRT | |a ELBATTRAWYLONGTERMFO2720 | ||