心脏肿瘤—心脏粘液瘤

---------------------------------------------------------------最新资料推荐------------------------------------------------------心脏肿瘤—心脏粘液瘤心脏肿瘤心脏粘液瘤中文：心脏粘液瘤是最常见的心脏原发肿瘤， 70%为良性肿瘤， 30%为恶性肿瘤，多数有瘤蒂，可发生于心脏各房、室腔，最常见于左心房。

病因和形态学：粘液瘤的的发病原因不清楚，一般认为肿瘤起源于心内膜下的多潜能的间质细胞，该细胞在形态学上与房间隔卵圆窝处胚胎残留细胞相似，可分化为内皮细胞、平滑肌细胞、成纤维细胞、骨及骨髓组织，产生无定形酸性粘多糖，构成粘液瘤的组织成分。

极少数粘液瘤可发生恶变，成为粘液肉瘤。

瘤体多为圆形或卵圆形，有深浅不一的切迹或分叶状，或呈葡萄状或息肉状。

外观多呈半透明胶冻状，表面光滑，常有散在大小不一的紫色、暗红和鲜红的出血区，偶有钙化。

瘤体大小可自 1cm 到 15cm，多数在 5cm~6cm。

质软而脆，容易破碎，脱落后可引起周围动脉栓塞或脑血管栓塞。

粘液瘤可发生于各个心腔，最常见于左心房，约占75%。

多数肿瘤有瘤蒂与心房壁相连， 90%的左房粘液瘤附着于心房间隔卵圆窝处。

1/ 14瘤体可随心脏的收缩、舒张而活动。

绝大多数为单发肿瘤，但也可为多发，常有家族遗传倾向。

临床表现：粘液瘤的临床表现主要有：心脏血流动力学紊乱、动脉栓塞和全身表现三个部分。

血流动力学紊乱。

心脏粘液瘤体积增大至相当程度，尤其是蒂长有活动度者，可阻塞二尖瓣口，引起血流障碍，产生瓣膜狭窄的症状和体征。

如心悸、气短、端坐呼吸、晕厥、咯血等。

如果这种梗阻为间歇性发作，患者可出现短暂的昏厥，且常与体位的变化有关，部分患者可能会发生猝死。

少数患者由于肿瘤梗阻或瓣叶损伤，而出现二尖瓣或三尖瓣口关闭不全。

肿瘤也可阻塞肺静脉和腔静脉开口，导致肺静脉和腔静脉引流障碍。

动脉栓塞的原因可以是肿瘤的碎片，也可能是整个肿瘤随血流漂动，引起体动脉栓塞，息肉状和葡萄状的粘液瘤较易脱落导致栓塞。

50%的体循环栓塞发生在脑血管，引起昏迷、偏瘫、失语等症状。

体循环栓塞还可引起急腹症，肢体疼痛、坏死等。

大约 30%的心脏粘液瘤患者可出现全身反应，表现为发热，---------------------------------------------------------------最新资料推荐------------------------------------------------------ 消瘦，食欲不振，乏力，关节或肌肉疼痛，杵状指(趾) ，贫血，血沉增快， C 反应蛋白阳性， g 球蛋白升高，肌酐激酶及转氨酶升高等。

瘤体较大的左房粘液瘤更易出现全身反应，这可能与粘液瘤的出血、变性、坏死，而引发的自身免疫反应有关，一般药物治疗无效。

手术摘除肿瘤后，症状通常可立即缓解或消失。

临床诊断：左房粘液瘤的临床症状酷似二尖瓣狭窄，大多数患者有心悸、气短、端坐呼吸、咯血等症状，发作时间较短，呈阵发性，常伴晕厥，病程进展较快，常出现心衰，药物治疗欠满意。

栓塞主要有脑血管意外及四肢末梢冷、痛等症状。

偶有因冠状动脉栓塞出现心绞痛或因肺动脉栓塞出现呼吸困难等症状，也可因腹腔干动脉栓塞出现急腹症。

体征不恒定，可随体位变动而变化。

左房粘液瘤听诊常有二尖瓣舒张期或双期杂音，个别只有收缩期杂音。

实验室检查一般正常。

心电图检查无特异性，可有心房纤颤、束支传导阻滞和异常 p 波。

X 线胸片无特异性，可以有心影扩大、肺瘀血及肺动脉高3/ 14压表现。

超声心动图是最简便可靠的诊断方法，诊出率可达 98%。

二维超声可显示肿瘤的大小、部位及活动情况; 多普勒超声可显示肿瘤引起瓣膜狭窄和关闭不全的程度。

鉴别诊断：左房粘液瘤需要与左房血栓和左房其他肿瘤相鉴别。

左房血栓的形成需要特定病因，例如心房纤颤或二尖瓣狭窄等，粘液瘤不需要上述因素。

血栓多固定在左心耳和左心房后壁，而且没有活动性或者活动性极差; 而粘液瘤则固定于房间隔，活动性很好，可经二尖瓣口进入左心室。

左心房其他肿瘤多位于心肌内或位于瓣缘，基本没有活动性; 粘液瘤则位于心腔内，活动性良好。

右房粘液瘤需要与心脏转移瘤和过度增生的肌小梁相鉴别。

转移肿瘤患者多较消瘦，肿瘤多起自下腔静脉，成条索状，与心房壁甚至心包相粘连;而粘液瘤患者状态良好，肿瘤多起自房间隔或右心房外侧壁，呈球形或分叶状，活动性良好，偶伴有肺栓塞。

个别过度增生的肌小梁会被误判为心房肿瘤，通过心脏 CT 扫描可以排除。

手术指征：左房粘液瘤一经诊断出来原则上就应立即手术治疗。

特别是有栓塞和晕厥病史的患者，应急诊手术治疗。

---------------------------------------------------------------最新资料推荐------------------------------------------------------ 手术方法：左房粘液瘤摘除术多经正中切口，在体外循环下切开右心房完成。

从右心房面在卵圆窝中点先做 1~2cm 纵切口，检查瘤蒂的附着部位，然后沿瘤蒂周围 5mm 切除瘤蒂，慢慢取出肿瘤，用涤纶布修补房间隔缺损。

术后处理：一般的处理原则与体外循环心内直视手术相同。

左房粘液瘤的病理生理改变非常近似二尖瓣狭窄患者，早期处理原则是切忌补充容量过渡，引起急性左心衰竭及肺水肿。

特别是术前已经出现左心衰的患者，术后应严格限制液体的输入量和输入速度，可适量应用白蛋白，同时应用少量多巴胺与多巴酚丁胺，提高心肌的收缩力和心排血量。

主要并发症 1. 栓塞：主要发生原因为探查时过渡搬动和挤压心脏，或切除时强行提拉瘤蒂发生瘤体破碎。

因此，切除肿瘤后应用盐水冲洗心腔，避免肿瘤碎片隐藏在心房、室的肌小梁内。

2. 急性心力衰竭：主要发生原因是对该病的病理生理认识不足，术后早期补充容量过多、过快，或对心功能较差的患者，未及时应用血管活性药5/ 14物等。

3. 术后心律失常：心房较大患者，手术后会出现房性心律失常; 个别患者可以发生传导阻滞，可能与手术损伤有关。

4. 心脏手术损伤：当肿瘤的蒂部位于左房顶、二尖瓣前叶附近、或传导束周围时，切除肿瘤时要十分小心，切除范围不能过大、过深，否则会损伤邻居结构。

可以通过缩小切除范围和深度避免损伤，然后通过电烧灼，精细清除可能残留的肿瘤组织。

预后：绝大多数患者预后良好，心功能可以恢复到NYHAⅠ ～Ⅱ 级。

文献报道手术死亡率在5%-10%之间，患者死亡多与粘液瘤手术本身无关，主要与手术时机、患者年龄和患者的心脏合并病变有关。

在染色体正常的患者中，肿瘤复发的几率仅为 1%-3%，可能与肿瘤种植、肿瘤切除不全和肿瘤再生有关; 不过，家族性粘液瘤的复发比例却很高，可以达到 30%-75%，平均复发时间为 30 个月。

英文：Cardiac tumor, cardiac myxoma Cardiac myxomas are the most common primary cardiac tumors, 70% benign tumors, 30% malignant tumors, most of Beattie, can occur in the heart---------------------------------------------------------------最新资料推荐------------------------------------------------------of the atrial, ventricular cavity, most commonly in the left atrium. Etiology and morphology: Myxoma of the etiology is not clear, is generally believed that the tumor originated in the subendocardial pluripotential mesenchymal cells, the cells in morphology and the atrial septum oval fossa residual embryos similar cells, can differentiate into endothelial cells, smooth muscle cells, fibroblasts, bone and bone marrow tissue, produced amorphous acid mucopolysaccharides polysaccharide, constitute a myxoma tissue components. A handful of myxoma can become malignant, mucus sarcoma. The tumor is round or oval, with shades of a notch or lobulated, or a grape-like or polypoidal. The appearance of multiple translucent jelly shape, smooth surface, often with scattered in different sizes of purple, red and red bleeding area, with occasional calcification. Tumor size from 1cm to 15cm, mostly in 5cm~6cm. Soft and brittle, easily broken, after shedding can cause peripheral arterial embolism or vascular embolism. Myxoma can occur in various heart cavity, most commonly in the left atrium, accounting for about 75% of. The majority of Beattie tumor and atrial wall connected, 90% left atrial myxoma attached to the atrial septal fossa ovalis.7/ 14The tumor with the contraction of the heart, blood and activities. For the vast majority of single tumor, but also can provide multiple, often familial and genetic tendency. Clinical manifestation. Myxoma of the main clinical manifestations: cardiac hemodynamic disorders, arterial embolization and systemic manifestations of three parts. Hemodynamic disorders. Cardiac myxoma volume increased to a considerable degree, especially the pedicle length of activity degree, can block the mitral orifice, causes of hemodynamic disorders, produce valvular stenosis symptoms and signs. Such as palpitations, shortness of breath, orthopnea, syncope, hemoptysis. If the obstruction is intermittent episodes, patients may present with transient syncope, and is often associated with postural changes related to part, can occur in patients with sudden death. A minority of patients due to tumor obstruction or leaflet injury, and the mitral or tricuspid regurgitation three mouth. The tumor can also be obstruction of the pulmonary veins and superior vena cava opening, leading to pulmonary vein and vena cava drainage obstacle. Causes for arterial embolism can bea tumor fragments, possibly the entire tumor with blood flow caused by floating, artery embolization, polypoid and---------------------------------------------------------------最新资料推荐------------------------------------------------------ grape-like myxoma is easy to fall off lead to embolism. 50%of the systemic embolism occurred in the cerebral vasculature, leading to coma, hemiplegia, aphasia and other symptoms. Systemic embolism can also cause acute abdomen, pain of the limbs, necrosis. Approximately 30% of patients with cardiac myxoma can occur in systemic reactions, manifested as fever, weight loss, loss of appetite, fatigue, joint or muscle pain, clubbing finger ( toe) , anemia, calcium, C reactive protein, G globulin elevated creatinine kinase, and transaminase elevation. The tumour is large left atrial myxoma prone to systemic reactions, which may be associated with myxoma of the bleeding, degeneration and necrosis, and caused its own immune response, general drug treatment is ineffective. Operation after tumor extirpation, symptoms usually can be immediately relieved or disappeared. Clinical diagnosis: Left atrial myxoma clinical symptoms mimicking mitral stenosis, the majority of patients with palpitations, shortness of breath, orthopnea, hemoptysis and other symptoms, onset time shorter, paroxysmal, often with syncope, progress is rapidder, often occurs in heart failure, drug treatment less satisfactory. Embolization9/ 14of cerebral vascular accident and main extremities cold, pain and other symptoms. Sometimes due to the onset of angina pectoris coronary artery embolization or caused by pulmonary embolism appearance of symptoms such as dyspnea, but also because of celiac artery embolism suffered from acute abdomen. Symptoms are not constant, with the position change and change. Left atrial myxoma auscultation often mitral diastolic or two phase noise, individual only systolic murmur. Laboratory examination of normal. Electrocardiogram examination no specificity, can have atrial fibrillation, bundle branch block and abnormal P wave. Chest X-ray is nonspecific, can have influence expanded, pulmonary congestion and manifestation of pulmonary hypertension. Ultrasonic heartbeat graph is the most simple and reliable methods of diagnosis, diagnostic rate of up to 98%. Two dimensional echocardiography showed tumor size, location and activities; Doppler ultrasound can show the tumor caused by valvular stenosis and regurgitation degree. Differential diagnosis: Left atrial myxoma and thrombus in left atrium and left atrial tumor differential diagnosis. Left atrial thrombus formation requires specific etiologies, such as atrial fibrillation or mitral stenosis, myxoma is not required for---------------------------------------------------------------最新资料推荐------------------------------------------------------the above factors. Thrombosis of the left atrial appendage and fixed on the posterior wall of the left atrium, and no activityor activity range; and the myxoma is fixed in the atrial septum, the activity is very good, but after mitral valve into the left ventricle. Left atrial tumor located in the myocardium or located on the valve edge, no activity; myxoma is located in the cavity of the heart, the activity is good. Right atrial myxoma with cardiac metastasis tumor and hyperplasia of the muscle of little Liang Xiang differential. Metastatic tumorin a patient with multiple relatively thin, tumors from the inferior vena cava, a funicular, and atrial wall and pericardium. ; and myxoma patients in good condition, the tumor from the atrial septum or right atria of the lateral wall, spherical or oval, activity, occasionally accompanied by pulmonary embolism. Individual hyperplasia of Ji Xiaoliang would be mistaken for atrial tumor, cardiac CT scans can be excluded by. Operation indication: Left atrial myxoma from diagnosis principle should be immediately operation treatment. Especially with embolization and history of syncope patients, should be treated by emergency operation. Operation method: Left atrial myxoma extirpation by median11/ 14sternotomy, cardiopulmonary bypass incision in right atrium complete. From the right atrium in the fossa ovalis midpointdo first 1~2cm longitudinal incision, check the pedicle attachment site, and then along the pedunculated tumor resection of pedunculated tumor around 5mm, slowly remove the tumor, with polyester fabric repair of atrial septal defect. Postoperative treatment: General treatment principles and open-heart operation the same. Left atrial myxoma pathophysiological changes are very similar in patients with mitral stenosis, early treatment principle is not added capacity transition, caused by acute left heart failure and pulmonary edema. Especially before surgery has emerged of left heart failure patients, after strict restriction of fluid input and input speed, amount of albumin, and apply a small amount of dopamine and dobutamine, enhance myocardial contractility and cardiac output. The main complications 1: the main cause of embolism for exploration when the transition to move and squeeze the heart, or resection when forced pulling pedunculated tumor occurred tumor shattering. Therefore, resection of the tumor after application of saline flushing chamber of the heart, prevent tumor fragments hiddenin the atrium, ventricular muscle trabeculae inside.---------------------------------------------------------------最新资料推荐------------------------------------------------------ Acute heart failure: 2 major causes is the lack of understanding the pathophysiology of the disease, early postoperative supplementary volume too much, too soon, or on the cardiac function of patients with poor, not timely application of vasoactive drugs. 3 postoperative arrhythmias: Atrial larger patients, operation after the emergence of atrial arrhythmias; individual patients can occur block, may be associated with operation injury. 4 heart operation injury: when the tumor was located in the left anterior leaflet of the mitral valve, near, or conduction bundle, resection of the tumor to be very careful, extent of resection can not be too large, too deep, otherwise it will damage the structure of neighborhood. Through the narrow resection extent and depth to avoid injury, and then through the electric cautery, fine removal may be residual tumor tissue. Prognosis: The vast majority of patients with good prognosis, heart function can be restored to the NYHAⅠ - Ⅱ. The operation mortality reported at between 5%-10%, death of patients with myxoma operation itself, mainly with the timing of operation, age of patients and patients with cardiac complications related to. In the chromosome in normal13/ 14patients, tumor recurrence is only 1%-3%, may be associated with tumor, excision of tumor and tumor related to incomplete regeneration; however, familial myxoma recurrence rate is very high, can reach 30%-75%, the average time to recurrence was 30 months. 本文由北京阜外医院网上挂号整理本文转载于：/news_show.asp?id=688。