Bavishi Aakash, Soutar Marybeth, Kurnides Margaret, Speer Heather, Oyarce Glenda, Bryde Robyn, Lander Bradley, Richards Karen, DeMarco Karen, Martinez Matthew W
Division of Cardiology, University of Illinois-Chicago, Chicago, Illinois, USA.
Division of Cardiology, Atlantic Health, New Jersey, USA.
JACC Adv. 2025 Jan 8;4(2):101531. doi: 10.1016/j.jacadv.2024.101531. eCollection 2025 Feb.
The Valsalva maneuver is essential in evaluating left ventricular outflow tract (LVOT) obstruction in patients with hypertrophic cardiomyopathy (HCM). Traditionally, a self-directed Valsalva (SDV) maneuver is taught to patients using vague instructions such as "bear down." SDV is often not performed correctly leading to variable results and underestimation of the true provocable LVOT gradient. Alternatively, a standardized, goal-directed Valsalva (GDV) approach by maintaining an intraoral pressure 40 mm Hg for 10 seconds or more provides a more objective, reproducible result. Accurate and reproducible LVOT gradient is key in the mavacamten algorithm for dose titration.
The objective was to evaluate the clinical impact of the GDV compared to SDV in patients with obstructive HCM on mavacamten.
In this prospective study, patients with obstructive HCM on mavacamten performed both an SDV and GDV. Peak LVOT gradient (pLVOTg) was measured at rest and using both provocative SDV and GDV maneuvers.
A total of 69 patients were included with 203 total transthoracic echocardiograms (TTEs). Among initial postmavacamten TTEs, mean pLVOTg was significantly higher with GDV vs SDV (31 mm Hg vs 24 mm Hg, < 0.01). When compared to SDV, GDV was associated with fewer patients maintaining pLVOTg ≤20 mm Hg (29 [43%] vs 43 [63%], < 0.01) which decreased medication reductions and reduced TTE follow-up in the GDV cohort.
GDV can significantly alter patient management in patients with obstructive HCM on cardiac myosin inhibitor therapy by reclassifying disease severity, preventing unnecessary dose reductions in medications, and reducing the burden of frequent TTEs.
瓦尔萨尔瓦动作对于评估肥厚型心肌病（HCM）患者的左心室流出道（LVOT）梗阻至关重要。传统上，向患者传授的自主瓦尔萨尔瓦（SDV）动作使用诸如“用力下压”等模糊指令。SDV动作常常执行不正确，导致结果多变且低估了真正可诱发的LVOT压差。另外，通过维持口腔内压力40毫米汞柱达10秒或更长时间的标准化、目标导向性瓦尔萨尔瓦（GDV）方法可提供更客观、可重复的结果。准确且可重复的LVOT压差是mavacamten算法中剂量滴定的关键。
目的是评估在接受mavacamten治疗的梗阻性HCM患者中，GDV与SDV相比的临床影响。
在这项前瞻性研究中，接受mavacamten治疗的梗阻性HCM患者同时进行了SDV和GDV动作。在静息状态以及使用诱发SDV和GDV动作时测量LVOT峰值压差（pLVOTg）。
共纳入69例患者，总计进行了203次经胸超声心动图（TTE）检查。在初始mavacamten治疗后的TTE检查中，与SDV相比，GDV时的平均pLVOTg显著更高（31毫米汞柱对24毫米汞柱，<0.01）。与SDV相比，GDV时维持pLVOTg≤20毫米汞柱的患者更少（29例[43%]对43例[63%]，<0.01），这减少了GDV队列中药物减量并减少了TTE随访。
GDV可通过重新分类疾病严重程度、防止不必要的药物减量以及减轻频繁TTE检查的负担，显著改变接受心肌肌球蛋白抑制剂治疗的梗阻性HCM患者的治疗管理。
