Stanton Christopher, Bruce Charles, Connolly Heidi, Brady Peter, Syed Imran, Hodge David, Asirvatham Samuel, Friedman Paul
Mayo Clinic, Rochester, Minnesota.
Am J Cardiol. 2009 Oct 15;104(8):1135-8. doi: 10.1016/j.amjcard.2009.05.062. Epub 2009 Aug 28.
Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy characterized by prominent trabeculae, deep intertrabecular recesses, and thickened myocardium with 2 distinct layers (compacted and noncompacted). Clinical characteristics, outcomes, and appropriate therapies remain poorly defined. Data were collected on patients diagnosed with ILVNC by echocardiographic criteria at the Mayo Clinic from 2001 through 2006. These data were entered prospectively into a clinical database and retrospectively analyzed. All-cause mortality, stroke, and development of atrial fibrillation (AF) were compared to community and nonischemic dilated cardiomyopathic (DC) controls. Implantable cardioverter-defibrillator (ICD) therapies were examined. Thirty patients with confirmed ILVNC were included in analyses (mean age at diagnosis 39 +/- 19.5 years, 60% men). Three patients with ILVNC died during follow-up (mean 2.5 +/- 1.2 years) compared to 5 DC and 1 community controls. No mortality difference was observed among these groups (p = 0.42 and 0.054, respectively). No ILVNC deaths were observed in patients with normal LV ejection fraction. New-onset AF was diagnosed in 2 patients with ILVNC, and none was observed in DC controls. Stroke occurred in 2 DC controls and none was observed in patients with ILVNC. ICDs were implanted in 11 patients with ILVNC. No appropriate therapies were identified during follow-up, but 2 patients underwent inappropriate therapies related to AF. In conclusion, mortality in patients with ILVNC is similar to that in DC patients. Deaths were observed only in patients with decreased LV ejection fraction, suggesting that ICD therapy may be reserved for this subgroup. New-onset AF may lead to inappropriate ICD discharges.
孤立性左心室心肌致密化不全（ILVNC）是一种罕见的先天性心肌病，其特征为显著的肌小梁、深陷的肌小梁间隐窝以及增厚的心肌，且心肌有两层不同结构（致密层和非致密层）。其临床特征、预后及合适的治疗方法仍未明确界定。收集了2001年至2006年在梅奥诊所根据超声心动图标准诊断为ILVNC的患者的数据。这些数据前瞻性地录入临床数据库并进行回顾性分析。将全因死亡率、中风和房颤（AF）的发生率与社区对照组和非缺血性扩张型心肌病（DC）对照组进行比较。对植入式心脏复律除颤器（ICD）治疗情况进行了检查。30例确诊为ILVNC的患者纳入分析（诊断时平均年龄39±19.5岁，60%为男性）。随访期间，3例ILVNC患者死亡（平均2.5±1.2年），而DC对照组有5例死亡，社区对照组有1例死亡。这些组间未观察到死亡率差异（分别为p = 0.42和0.054）。左心室射血分数正常的患者中未观察到ILVNC死亡病例。2例ILVNC患者诊断为新发房颤，而DC对照组未观察到房颤病例。2例DC对照组发生中风，ILVNC患者未观察到中风病例。11例ILVNC患者植入了ICD。随访期间未发现合适的治疗情况，但有2例患者因房颤接受了不适当的治疗。总之，ILVNC患者的死亡率与DC患者相似。仅在左心室射血分数降低的患者中观察到死亡病例，提示ICD治疗可能仅适用于该亚组患者。新发房颤可能导致ICD不适当放电。
