摘要目的 分析肥厚梗阻性心肌病合并二尖瓣病变的病理特点,探讨外科治疗策略.方法 1996年10月至2009年6月,外科手术治疗62例肥厚梗阻性心肌病病人,其中男41例,女21例;年龄6～68岁,平均(34.05±15.26)岁;体重27～83 kg,平均(60.42±12.71)kg.术前超声心动图(UCG)均提示二尖瓣SAM征,50例合并不同程度的二尖瓣关闭不全(MR).手术在全麻低温体外循环下完成,按常规经主动脉切口行室间隔心肌切除术,同期完成二尖瓣置换术(MVR)12例,二尖瓣成形术(MVP)9例.围术期常规UCG、心电图及X线胸片检查,评价左心房(LA)、左心室(LV)、左窒流出道流速及压差(LVOT)、左室射血分数(EF)、二尖瓣的结构和功能.结果 全组体外循环40～290 min,平均(104.23±47.14)min,主动脉阻断20～195 min,平均(66.76±36.32)min;气管插管5～21 h,平均(13.23±11.76)h.LA术前(43.46±7.21)mm,术后(34.56±5.23)mm;左室流出道压差术前(103.84±44.04)mm Hg(1 mm Hg=0.133 kPa),术后(23.54±17.78)mm Hg;室间隔厚度术前(26.93 ±5.23)mm,术后(17.12±5.67)mm,均显著下降(P＜0.05).术后MR和SAM症基本消失或显著减轻.手术死亡4例(6.4%,4/62例),主要死因为严重低心排综合征、严重心律失常及急性肾功能衰竭.主要并发症有完全左束支传导阻滞(33例)、室内传导阻滞(7例)、完全性房室传导阻滞(6例)、左前分支阻滞(5例)、Ⅰ度房室传导阻滞(5例)、心房颤动(4例).远期随访生存者症状消失,生活质量明显改善,心功能Ⅰ～Ⅱ级,二尖瓣结构功能良好;无远期死亡、并发症或再次手术.结论 肥厚梗阻性心肌病常常合并二尖瓣病变,室间隔肥厚心肌切除术,充分的疏通左心室流出道可基本消失MR和SAM征;如二尖瓣本身有严重先天性发育异常或继发性病变,首选MVP,疗效满意,因人工心脏瓣膜及术后抗凝治疗并发症,MVR不作为常规手术方式.

abstractsObjective analysis the pathogenesis and the treatment strategies of the hypertrophic obstructive cardiomyopathy (HOCM) with the concomitant mitral valve abnormalities. Methods From October 1996 to June 2009, 62 patients suffered from HOCM underwent surgical treatment. There were 41 males and 21 females with age ranging from 6 to 68 ( 34.05 ±15.26) years old. The body weight were 27 -83 kg [mean (60. 42 ± 12.71 ) kg]. Detected by preoperative echocardiography, all patients had the systolic anterior movement of the mitral leaflet ( SAM ) and 50 patients had mitral regurgitation (MR). Ventricular septal myomectomy was performed under general anesthesia and cardiopulmonary bypass (CPB) with a moderate systemic temperature and low volume blood flow. The concomitant operations included mitral valve replacement ( MVR 12 cases), mitral valve plasty ( MVP9 cases). During the perioperative period the patients were evaluated by echocardiography. Left ventricle ( LV ), left ventricular outflow tract ( LVOT), left atrium ( LA ), left ventricalar ejection fraction ( LVEF), mitral valve construction and function were evaluated. Results The time of CPB and the sortic occlusion were (104.23 ±47.14) (402290) min and (66.76 ±36.32) (20-195) min, respectively. The endotracheal intubation time was ( 13.23 ± 11.76 ) ( 5 -21 ) h and ICU stay was (42.53 ± 37.41 ) ( 11 ～ 183 ) h. Comparing with the parameters before operation: the sizes of the LA were (43.46 ± 7.21 ) mm vs. ( 34.56 ± 6.45 ) mm, pressure gradients through LVOT ( 103.84 ±44.04) mm Hg vs. (23.54±17.78) mm Hg and the thickness of the septal (26.93±5.23) mm vs. (17.12±5.67) mm.All parameters were significantly decreased( P ＜0.05 )postoperatively. All surviving patients had no or only trivial MR and SAM. Four patients (6.4% ,4/62 ) died during the perioperative period. The cause of death included severe low cardiac output, heart failure, severe ventricular arrhythmias and severe acute renal failure. The main complications were: left bandle branch block in 33 cases, intraventricular conduction block in 7, complete AV block in 6, anterior hemiblock in 5, type Ⅰ artioventricular conduction block in5, atrial fibrillation in 4. All surviving patients were in New York Heart Association functional class Ⅰ or Ⅱ during the follow-up from 1 month to 10 years. Cardiac symptoms were markedly relieved. Mitral valve construction and function were significantly improved. There were no death, no complications and none required additional mitral valve or myecomy surgery. Conclusion Ventricular septal myomectomy alone is sufficient to eliminate or significantly reduce the severity of MR and SAM. In patients with HOCM and MR due to the congenital mitral valve disease, concomitant MVP may be the first choice. Considering the inherent risks of prosthetic valves and anticoagulation complications, MVR should be the second choice.