Bordalo Alvaro D B, Alves Irina, Nobre Angelo L, Silva Fernanda, Lemos Alberto, Serpa Carlos, Fernandes Afonso, Cravino João
Serviço de Cirurgia Cardiotorácica, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisboa, Portugal.
Rev Port Cardiol. 2012 Sep;31(9):567-75. doi: 10.1016/j.repc.2012.05.008. Epub 2012 Jul 24.
Easy access to echocardiography and its extensive and repeated use (as is the case in Portugal) now facilitates the early diagnosis of cardiac myxoma (CM).
To re-evaluate the clinical and pathological profile of CM under current diagnostic conditions.
We performed a retrospective study of 40 patients consecutively referred for surgery (between January 2003 and January 2010) with a histologically-confirmed diagnosis of CM - 26 female (F) and 14 male (M), with a mean age of 64±12 years (range 12-81; 53% over 65, 43% over 70); 39 patients were operated (one was not operable due to major neurological deficit). Clinical characteristics, surgical protocols, follow-up records of survivors (range 1-76 months, with serial echocardiograms), and histological data were reviewed.
The apparent incidence was 2.6 cases/million/year; the overall F/M ratio was 1.9:1 (1.3:1 in those aged over 65, similar to the general population). The CM was located in the left atrium (LA) in 92.5%, with insertion in the fossa ovalis of the interatrial septum (IAS) in 53% (only 57% of LA myxomas), and outside the IAS in 30%. The mean size was 4.6 x 3.7cm. Asymptomatic tumors occurred in 48% of the total population (sessile and/or atypically inserted in 74%; 63% of large size, over 3 x 3cm), 61% were in patients referred in the last 25 months of the study; 23% of patients showed constitutional symptoms (all with very large CMs - mean 6.7 x 5.1cm), 35% had hemodynamic/obstructive symptoms, and 15% presented with embolic events. There was evidence of CM-related mitral valve (MV) disease in 20% of patients, resulting in moderate to severe mitral regurgitation requiring associated MV surgery in 13%. Significant comorbidities were present in 69%. Surgical procedures included simple excision in 74%; septoplasty/atrioplasty associated with extensive resection of the insertion site in 26%; and combined surgery (CM excision plus other procedures) in 28%. There were significant postoperative complications in 38%. In-hospital mortality was 10%; postoperative mortality was 7.7%. Mean follow-up was 30 months (100% of survivors, 44% for >2 years); late mortality was 5.6% and no CM recurrences were observed.
(1) CM has a higher incidence than described in the literature and mainly affects patients aged over 65; the reported predominance of female patients disappears after the age of 65. (2) Most CM cases are now asymptomatic at presentation as a result of earlier diagnosis. (3) CM is the cause of MV disease requiring surgical correction in more than 10% of cases, and is associated with significant postoperative mortality, mainly due to the presence of comorbidities.
如今,超声心动图检查易于进行且广泛反复应用(如在葡萄牙的情况),这有助于心脏黏液瘤(CM)的早期诊断。
在当前诊断条件下重新评估CM的临床和病理特征。
我们对2003年1月至2010年1月期间连续转诊接受手术且经组织学确诊为CM的40例患者进行了回顾性研究,其中女性26例,男性14例,平均年龄64±12岁(范围12 - 81岁;65岁以上者占53%,70岁以上者占43%);39例患者接受了手术(1例因严重神经功能缺损未进行手术)。回顾了临床特征、手术方案、幸存者的随访记录(范围1 - 76个月,有系列超声心动图检查)以及组织学数据。
明显发病率为2.6例/百万/年;总体男女比例为1.9:1(65岁以上者为1.3:1,与普通人群相似)。CM位于左心房(LA)的占92.5%,附着于房间隔(IAS)卵圆窝的占53%(仅占LA黏液瘤的57%),位于IAS以外的占30%。平均大小为4.6×3.7cm。无症状肿瘤占总病例数的48%(基底型和/或附着异常者占74%;63%为大尺寸,超过3×3cm),61%的患者是在研究的最后25个月转诊的;23%的患者有全身症状(均为非常大的CM,平均6.7×5.1cm),35%有血流动力学/梗阻症状,15%有栓塞事件。20%的患者有与CM相关的二尖瓣(MV)疾病证据,其中13%因中度至重度二尖瓣反流需要同时进行MV手术。69%的患者有显著合并症。手术方式包括单纯切除占74%;在26%的患者中,隔膜成形术/心房成形术联合广泛切除附着部位;28%的患者进行联合手术(CM切除加其他手术)。38%的患者有显著术后并发症。住院死亡率为10%;术后死亡率为7.7%。平均随访30个月(幸存者100%,超过2年者44%);晚期死亡率为5.6%,未观察到CM复发。
(1)CM的发病率高于文献报道,主要影响65岁以上患者;65岁以后女性患者占优势的情况消失。(2)由于早期诊断,目前大多数CM病例在就诊时无症状。(3)超过10%的病例中,CM是需要手术矫正的MV疾病的病因,且与显著的术后死亡率相关,主要是由于合并症的存在。