Pinede L, Duhaut P, Loire R
Department of Internal Medicine, Edouard Herriot Hospital, 69437 Lyons, France.
Medicine (Baltimore). 2001 May;80(3):159-72. doi: 10.1097/00005792-200105000-00002.
We analyzed a series of 112 consecutive cases of left atrial myxoma diagnosed in a single French hospital (72 women and 40 men; age range, 5-84 yr) over 40 years, from 1959 to 1998. Symptoms of mitral valve obstruction, the first arm of the classic triad of myxoma presentation, were present in 75 patients (67%), with mostly cardiac failure or malaise. Symptoms of embolism, the second frequent presentation in the classic triad, were observed in 33 cases (29%) with 1 or several locations, essentially cerebral emboli with stroke. Males are statistically at greater risk than females of developing embolic complications. The third arm of the classic triad consists of constitutional symptoms (34%) with fever, weight loss, or symptoms resembling connective tissue disease, due to cytokine (interleukin-6) secretion. Younger and male patients have more neurologic symptoms, and female patients have more systemic symptoms. Seventy-two patients (64%) had cardiac auscultation abnormalities, essentially pseudo-mitral valve disease (53.5%) and more rarely the suggestive tumor plop (15%). The most frequent electrocardiographic sign was left atrial hypertrophy (35%), whereas arrhythmias were uncommon. The greater number of myxoma patients (98) diagnosed preoperatively after 1977 reflects the introduction of echocardiography as a noninvasive diagnostic procedure. However, there was no significant reduction in the average time from onset of symptoms to operation between patients seen in the periods before and after 1977. The tumor diameter ranged from 1 to 15 cm with a weight of between 15 and 180 g (mean, 37 g). The myxoma surface was friable or villous in 35% of the cases, and smooth in the other 65% cases. Myxomas in patients presenting with embolism have a friable surface; those in patients with cardiac symptoms, pseudo-mitral auscultation signs, tumor plop, and electrocardiogram or radiologic signs of left atrium hypertrophy and dilatation are significantly the larger tumors. The long-term prognosis is excellent, and only 4 deaths occurred among our 112 cases over a median follow-up of 3 years. The recurrence rate is low (5%), but long-term follow-up and serial echocardiography are advisable especially for young patients.
我们分析了1959年至1998年40年间在法国一家医院连续诊断的112例左心房黏液瘤病例(72例女性和40例男性;年龄范围5 - 84岁)。二尖瓣梗阻症状是黏液瘤典型三联征的首要表现,75例患者(67%)出现该症状,主要为心力衰竭或不适。栓塞症状是典型三联征的第二常见表现,33例(29%)患者出现,栓塞部位为1处或多处,主要是脑栓塞伴中风。从统计学角度看,男性发生栓塞并发症的风险高于女性。典型三联征的第三个表现是全身症状(34%),包括发热、体重减轻或类似结缔组织病的症状,这是由细胞因子(白细胞介素-6)分泌所致。年轻患者和男性患者神经症状更多,女性患者全身症状更多。72例患者(64%)有心脏听诊异常,主要是假性二尖瓣疾病(53.5%),较少见提示性的肿瘤扑落音(15%)。最常见的心电图表现是左心房肥大(35%),心律失常不常见。1977年后术前诊断的黏液瘤患者数量较多(98例),这反映了超声心动图作为一种非侵入性诊断方法的引入。然而,1977年前后就诊的患者从症状出现到手术的平均时间并无显著缩短。肿瘤直径为1至15厘米,重量在15至180克之间(平均37克)。35%的病例黏液瘤表面易碎或呈绒毛状,其余65%表面光滑。出现栓塞症状的患者的黏液瘤表面易碎;有心脏症状、假性二尖瓣听诊体征、肿瘤扑落音以及心电图或放射学表现为左心房肥大和扩张的患者的黏液瘤明显更大。长期预后良好,在我们的112例病例中,中位随访3年仅有4例死亡。复发率较低(5%),但建议进行长期随访并定期做超声心动图检查,尤其是对年轻患者。