The response to cardiac resynchronization therapy in LMNA cardiomyopathy

被引:16
作者
Sidhu, Kiran [1 ]
Castrini, Anna Isotta [2 ]
Parikh, Victoria [3 ]
Reza, Nosheen [4 ]
Owens, Anjali [4 ]
Tremblay-Gravel, Maxime [3 ]
Wheeler, Matthew T. [3 ]
Mestroni, Luisa [5 ]
Taylor, Matthew [5 ]
Graw, Sharon [5 ]
Gigli, Marta [6 ,7 ]
Merlo, Marco [6 ,7 ]
Paldino, Alessia [6 ,7 ]
Sinagra, Gianfranco [6 ,7 ]
Judge, Daniel P. [8 ]
Ramos, Hannia [8 ]
Mesubi, Olurotimi [9 ]
Brown, Emily [9 ]
Turnbull, Samual [10 ]
Kumar, Saurabh [10 ]
Roy, Darius [11 ]
Tedrow, Usha B. [11 ]
Ngo, Long [12 ]
Haugaa, Kristina [2 ]
Lakdawala, Neal K. [11 ]
机构
[1] Univ Manitoba, Max Rady Coll Med, Sect Cardiol, Winnipeg, MB, Canada
[2] Oslo Univ Hosp, Oslo, Norway
[3] Stanford Univ, Sch Med, Stanford, CA 94305 USA
[4] Univ Penn Hlth Syst, Philadelphia, PA USA
[5] Univ Colorado, Anschutz Med Campus, Denver, CO USA
[6] Azienda Sanit Univ Giuliano Isontina, Cardiovasc Dept, Trieste, Italy
[7] Univ Trieste, Trieste, Italy
[8] Med Univ South Carolina, Charleston, SC 29425 USA
[9] Johns Hopkins Univ, Sch Med, Baltimore, MD USA
[10] Univ Sydney, Westmead Hosp, Westmead Appl Res Ctr, Dept Cardiol, Sydney, NSW, Australia
[11] Brigham & Womens Hosp, 75 Francis St, Boston, MA 02115 USA
[12] Harvard TH Chan Sch Publ Hlth, Boston, MA USA
关键词
Lamin A; C; Cardiac resynchronization therapy; Dilated cardiomyopathy; Heart failure; HEART-FAILURE; VENTRICULAR-ARRHYTHMIAS; TRIAL; GUIDELINES; OUTCOMES; MILD; RISK;
D O I
10.1002/ejhf.2463
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Aims Cardiac implantable electronic device (CIED) therapy is fundamental to the management of LMNA cardiomyopathy due to the high frequency of atrioventricular block and ventricular tachyarrhythmias. We aimed to define the role of cardiac resynchronization therapy (CRT) in impacting heart failure in LMNA cardiomyopathy. Methods and results From nine referral centres, LMNA cardiomyopathy patients who underwent CRT with available pre- and post-echocardiograms were identified retrospectively. Factors associated with CRT response were identified (defined as improvement in left ventricular ejection fraction [LVEF] >= 5% 6 months post-implant) and the associated impact on the primary outcome of death, implantation of a left ventricular assist device or cardiac transplantation was assessed. We identified 105 patients (mean age 51 +/- 10 years) undergoing CRT, including 70 (67%) who underwent CRT as a CIED upgrade. The mean change in LVEF similar to 6 months post-CRT was +4 +/- 9%. A CRT response occurred in 40 (38%) patients and was associated with lower baseline LVEF or a high percentage of right ventricular pacing prior to CRT in patients with pre-existing CIED. In patients with a European Society of Cardiology class I guideline indication for CRT, response rates were 61%. A CRT response was evident at thresholds of LVEF <= 45% or percent pacing >= 50%. There was a 1.3 year estimated median difference in event-free survival in those who responded to CRT (p = 0.04). Conclusion Systolic function improves in patients with LMNA cardiomyopathy who undergo CRT, especially with strong guideline indications for implantation. Post-CRT improvements in LVEF are associated with survival benefits in this population with otherwise limited options.
引用
收藏
页码:685 / 693
页数:9
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