Bostan Cem, Sinan Umit Yasar, Canbolat Polat, Abaci Okay, Munipoglu Sezer Karcier, Kucukoglu Serdar
Department of Cardiology, Institute of Cardiology, Istanbul University, Haseki, Istanbul/Turkey.
Echocardiography. 2014 Oct;31(9):1056-61. doi: 10.1111/echo.12537. Epub 2014 Feb 8.
In this study, we aimed to elucidate the factors affecting long-term all-cause mortality in patients with hypertrophic cardiomyopathy (HCM).
We retrospectively examined 31 patients (22 males and 9 females) diagnosed with HCM from 1999 to 2013. All subjects had sinus rhythm at the time of evaluation. Four patients had history of paroxysmal atrial fibrillation (PAF). In addition to echocardiographic examination plasma angiotensin-converting enzyme (ACE) activity and gene polymorphism were determined. The variables that were found to be significant in mortality were then included in multivariate analysis.
At the final follow-up examination, 12 patients had died, including 2 due to congestive heart failure and 10 due to sudden cardiac death. Patients with PAF had significantly higher mortality (P = 0.008). Moreover, left ventricular (LV) end-diastolic diameter (P = 0.04), LV systolic diameter (P = 0.001), LV mass index (P = 0.01), and left atrium diameter (P = 0.003) were found to be significantly correlated with mortality. However, no significant correlation was noted between mortality and age, type of HCM (familial/nonfamilial and obstructive/nonobstructive), ACE gene polymorphism, and plasma ACE level. In the multivariate analysis, left atrial (LA) diameter was still significantly associated with mortality. The LA diameter with a cutoff value of 4.1 cm predicted 13-year mortality with a sensitivity of 82% and specificity of 78%.
Instead of the ACE genotype and activity, echocardiographic evaluation findings such as LV systolic and diastolic diameters, LV mass index, and particularly LA dimension may predict long-term mortality in patients with HCM. PAF has also significant importance in the long-term mortality in patients with HCM.
在本研究中，我们旨在阐明影响肥厚型心肌病（HCM）患者长期全因死亡率的因素。
我们回顾性研究了1999年至2013年期间诊断为HCM的31例患者（22例男性和9例女性）。所有受试者在评估时均为窦性心律。4例患者有阵发性心房颤动（PAF）病史。除超声心动图检查外，还测定了血浆血管紧张素转换酶（ACE）活性和基因多态性。然后将在死亡率方面具有显著意义的变量纳入多变量分析。
在最后一次随访检查时，12例患者死亡，其中2例死于充血性心力衰竭，10例死于心源性猝死。PAF患者的死亡率显著更高（P = 0.008）。此外，发现左心室（LV）舒张末期直径（P = 0.04）、LV收缩期直径（P = 0.001）、LV质量指数（P = 0.01）和左心房直径（P = 0.003）与死亡率显著相关。然而，未发现死亡率与年龄、HCM类型（家族性/非家族性和梗阻性/非梗阻性）、ACE基因多态性和血浆ACE水平之间存在显著相关性。在多变量分析中，左心房（LA）直径仍与死亡率显著相关。截断值为4.1 cm的LA直径预测13年死亡率的敏感性为82%，特异性为78%。
与ACE基因型和活性不同，超声心动图评估结果如LV收缩期和舒张期直径、LV质量指数，特别是LA大小可能预测HCM患者的长期死亡率。PAF在HCM患者的长期死亡率中也具有重要意义。
