心臟腫瘤—心臟粘液瘤

1、 心臟腫瘤心臟粘液瘤 心臟腫瘤心臟粘液瘤 中文： 心臟粘液瘤是最常見(jiàn)的心臟原發(fā)腫瘤， 70%為良性腫瘤， 30%為惡性腫瘤， 多數(shù)有瘤蒂，可發(fā)生于心臟各房、 室腔， 最常見(jiàn)于左心房。 病因和形態(tài)學(xué)： 粘液瘤的的發(fā)病原因不清楚， 一般認(rèn)為腫瘤起源于心內(nèi)膜下的多潛能的間質(zhì)細(xì)胞， 該細(xì)胞在形態(tài)學(xué)上與房間隔卵圓窩處胚胎殘留細(xì)胞相似， 可分化為內(nèi)皮細(xì)胞、 平滑肌細(xì)胞、 成纖維細(xì)胞、 骨及骨髓組織， 產(chǎn)生無(wú)定形酸性粘多糖， 構(gòu)成粘液瘤的組織成分。 極少數(shù)粘液瘤可發(fā)生惡變， 成為粘液肉瘤。 瘤體多為圓形或卵圓形， 有深淺不一的切跡或分葉狀， 或呈葡萄狀或息肉狀。 外觀多呈半透明膠凍狀， 表面光滑， 常有散在

2、大小不一的紫色、 暗紅和鮮紅的出血區(qū)， 偶有鈣化。 瘤體大小可自 1cm 到 15cm， 多數(shù)在 5cm6cm。 質(zhì)軟而脆， 容易破碎， 脫落后可引起周?chē)鷦?dòng)脈栓塞或腦血管栓塞。 粘液瘤可發(fā)生于各個(gè)心腔， 最常見(jiàn)于左心房， 約占75%。 多數(shù)腫瘤有瘤蒂與心房壁相連， 90%的左房粘液瘤附著于心房間隔卵圓窩處。 瘤體可隨心臟的收縮、 舒張而活動(dòng)。 絕大多數(shù)為單發(fā)腫瘤， 但也可為多發(fā)， 常有家族遺傳傾向。 臨床表現(xiàn)： 粘液瘤的臨床表現(xiàn)主要有： 心臟血流動(dòng)力學(xué)紊亂、 動(dòng)脈栓塞和全身表現(xiàn)三個(gè)部分。 血流動(dòng)力學(xué)紊亂。 心臟粘液瘤體積增大至相當(dāng)程度， 尤其是蒂長(zhǎng)有活動(dòng)度者， 可阻塞二尖瓣口， 引起血流障礙，

3、 產(chǎn)生瓣膜狹窄的癥狀和體征。 如心悸、 氣短、 端坐呼吸、 暈厥、 咯血等。 如果這種梗阻為間歇性發(fā)作， 患者可出現(xiàn)短暫的昏厥， 且常與體位的變化有關(guān)， 部分患者可能會(huì)發(fā)生猝死。 少數(shù)患者由于腫瘤梗阻或瓣葉損傷， 而出現(xiàn)二尖瓣或三尖瓣口關(guān)閉不全。 腫瘤也可阻塞肺靜脈和腔靜脈開(kāi)口， 導(dǎo)致肺靜脈和腔靜脈引流障礙。 動(dòng)脈栓塞的原因可以是腫瘤的碎片， 也可能是整個(gè)腫瘤隨血流漂動(dòng)， 引起體動(dòng)脈栓塞，息肉狀和葡萄狀的粘液瘤較易脫落導(dǎo)致栓塞。 50%的體循環(huán)栓塞發(fā)生在腦血管， 引起昏迷、偏癱、 失語(yǔ)等癥狀。 體循環(huán)栓塞還可引起急腹癥， 肢體疼痛、 壞死等。 大約 30%的心臟粘液瘤患者可出現(xiàn)全身反應(yīng)， 表現(xiàn)

4、為發(fā)熱， 消瘦， 食欲不振， 乏力， 關(guān)節(jié)或肌肉疼痛， 杵狀指(趾) ， 貧血， 血沉增快， C 反應(yīng)蛋白陽(yáng)性， g 球蛋白升高， 肌酐激酶及轉(zhuǎn)氨酶升高等。 瘤體較大的左房粘液瘤更易出現(xiàn)全身反應(yīng)， 這可能與粘液瘤的出血、 變性、壞死， 而引發(fā)的自身免疫反應(yīng)有關(guān)， 一般藥物治療無(wú)效。 手術(shù)摘除腫瘤后， 癥狀通?？闪⒓淳徑饣蛳?。 臨床診斷： 左房粘液瘤的臨床癥狀酷似二尖瓣狹窄， 大多數(shù)患者有心悸、 氣短、 端坐呼吸、 咯血等癥狀， 發(fā)作時(shí)間較短， 呈陣發(fā)性， 常伴暈厥， 病程進(jìn)展較快， 常出現(xiàn)心衰， 藥物治療欠滿意。 栓塞主要有腦血管意外及四肢末梢冷、 痛等癥狀。 偶有因冠狀動(dòng)脈栓塞出現(xiàn)心絞痛或

5、因肺動(dòng)脈栓塞出現(xiàn)呼吸困難等癥狀， 也可因腹腔干動(dòng)脈栓塞出現(xiàn)急腹癥。 體征不恒定， 可隨體位變動(dòng)而變化。 左房粘液瘤聽(tīng)診常有二尖瓣舒張期或雙期雜音， 個(gè)別只有收縮期雜音。 實(shí)驗(yàn)室檢查一般正常。 心電圖檢查無(wú)特異性， 可有心房纖顫、 束支傳導(dǎo)阻滯和異常 p 波。 X 線胸片無(wú)特異性， 可以有心影擴(kuò)大、 肺瘀血及肺動(dòng)脈高壓表現(xiàn)。 超聲心動(dòng)圖是最簡(jiǎn)便可靠的診斷方法， 診出率可達(dá) 98%。 二維超聲可顯示腫瘤的大小、部位及活動(dòng)情況; 多普勒超聲可顯示腫瘤引起瓣膜狹窄和關(guān)閉不全的程度。 鑒別診斷： 左房粘液瘤需要與左房血栓和左房其他腫瘤相鑒別。 左房血栓的形成需要特定病因， 例如心房纖顫或二尖瓣狹窄等，

6、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， 而且沒(méi)有活動(dòng)性或者活動(dòng)性極差; 而粘液瘤則固定于房間隔， 活動(dòng)性很好， 可經(jīng)二尖瓣口進(jìn)入左心室。 左心房其他腫瘤多位于心肌內(nèi)或位于瓣緣， 基本沒(méi)有活動(dòng)性; 粘液瘤則位于心腔內(nèi)， 活動(dòng)性良好。 右房粘液瘤需要與心臟轉(zhuǎn)移瘤和過(guò)度增生的肌小梁相鑒別。 轉(zhuǎn)移腫瘤患者多較消瘦， 腫瘤多起自下腔靜脈， 成條索狀， 與心房壁甚至心包相粘連;而粘液瘤患者狀態(tài)良好， 腫瘤多起自房間隔或右心房外側(cè)壁， 呈球形或分葉狀， 活動(dòng)性良好，偶伴有肺栓塞。 個(gè)別過(guò)度增生的肌小梁會(huì)被誤判為心房腫瘤， 通過(guò)心臟 CT 掃描可以排除。 手術(shù)指征： 左房粘液瘤一經(jīng)診斷出來(lái)原

7、則上就應(yīng)立即手術(shù)治療。 特別是有栓塞和暈厥病史的患者，應(yīng)急診手術(shù)治療。 手術(shù)方法： 左房粘液瘤摘除術(shù)多經(jīng)正中切口， 在體外循環(huán)下切開(kāi)右心房完成。 從右心房面在卵圓窩中點(diǎn)先做 12cm 縱切口， 檢查瘤蒂的附著部位， 然后沿瘤蒂周?chē)?5mm 切除瘤蒂， 慢慢取出腫瘤， 用滌綸布修補(bǔ)房間隔缺損。 術(shù)后處理： 一般的處理原則與體外循環(huán)心內(nèi)直視手術(shù)相同。 左房粘液瘤的病理生理改變非常近似二尖瓣狹窄患者， 早期處理原則是切忌補(bǔ)充容量過(guò)渡， 引起急性左心衰竭及肺水腫。 特別是術(shù)前已經(jīng)出現(xiàn)左心衰的患者， 術(shù)后應(yīng)嚴(yán)格限制液體的輸入量和輸入速度， 可適量應(yīng)用白蛋白，同時(shí)應(yīng)用少量多巴胺與多巴酚丁胺， 提高心肌的收

8、縮力和心排血量。 主要并發(fā)癥 1. 栓塞： 主要發(fā)生原因?yàn)樘讲闀r(shí)過(guò)渡搬動(dòng)和擠壓心臟， 或切除時(shí)強(qiáng)行提拉瘤蒂發(fā)生瘤體破碎。 因此， 切除腫瘤后應(yīng)用鹽水沖洗心腔， 避免腫瘤碎片隱藏在心房、 室的肌小梁內(nèi)。 2. 急性心力衰竭： 主要發(fā)生原因是對(duì)該病的病理生理認(rèn)識(shí)不足， 術(shù)后早期補(bǔ)充容量過(guò)多、 過(guò)快， 或?qū)π墓δ茌^差的患者， 未及時(shí)應(yīng)用血管活性藥物等。 3. 術(shù)后心律失常： 心房較大患者， 手術(shù)后會(huì)出現(xiàn)房性心律失常; 個(gè)別患者可以發(fā)生傳導(dǎo)阻滯， 可能與手術(shù)損傷有關(guān)。 4. 心臟手術(shù)損傷： 當(dāng)腫瘤的蒂部位于左房頂、 二尖瓣前葉附近、 或傳導(dǎo)束周?chē)鷷r(shí)， 切除腫瘤時(shí)要十分小心， 切除范圍不能過(guò)大、 過(guò)深，

9、 否則會(huì)損傷鄰居結(jié)構(gòu)。 可以通過(guò)縮小切除范圍和深度避免損傷， 然后通過(guò)電燒灼， 精細(xì)清除可能殘留的腫瘤組織。 預(yù)后： 絕大多數(shù)患者預(yù)后良好， 心功能可以恢復(fù)到 NYHA 級(jí)。 文獻(xiàn)報(bào)道手術(shù)死亡率在5%-10%之間， 患者死亡多與粘液瘤手術(shù)本身無(wú)關(guān)， 主要與手術(shù)時(shí)機(jī)、 患者年齡和患者的心臟合并病變有關(guān)。 在染色體正常的患者中， 腫瘤復(fù)發(fā)的幾率僅為 1%-3%， 可能與腫瘤種植、 腫瘤切除不全和腫瘤再生有關(guān); 不過(guò)， 家族性粘液瘤的復(fù)發(fā)比例卻很高， 可以達(dá)到 30%-75%，平均復(fù)發(fā)時(shí)間為 30 個(gè)月。 英文： Cardiac tumor, cardiac myxoma Cardiac myxom

10、as are the most common primary cardiac tumors, 70% benign tumors, 30% malignant tumors, most of Beattie, can occur in the heart 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 orig

11、inated 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

12、 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

13、 different sizes of purple, red and red bleeding area, with occasional calcification. Tumor size from 1cm to 15cm, mostly in 5cm6cm. 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

14、 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. The tumor with the contraction of the heart, blood and activities. For the vast majority of single tumor, but also can provide mu

15、ltiple, 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, especiall

16、y 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 tran

17、sient 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 an

18、d superior vena cava opening, leading to pulmonary vein and vena cava drainage obstacle. Causes for arterial embolism can be a tumor fragments, possibly the entire tumor with blood flow caused by floating, artery embolization, polypoid and grape-like myxoma is easy to fall off lead to embolism. 50%

19、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,

20、 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 t

21、he 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 m

22、ajority 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. Embolization of cerebral vascular accident and main extremi

23、ties 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

24、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 nonspec

25、ific, 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 ult

26、rasound 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 ste

27、nosis, myxoma is not required for the above factors. Thrombosis of the left atrial appendage and fixed on the posterior wall of the left atrium, and no activity or activity range; and the myxoma is fixed in the atrial septum, the activity is very good, but after mitral valve into the left ventricle.

28、 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 tumor in a patien

29、t 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

30、. 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 treat

31、ed by emergency operation. Operation method: Left atrial myxoma extirpation by median sternotomy, cardiopulmonary bypass incision in right atrium complete. From the right atrium in the fossa ovalis midpoint do first 12cm longitudinal incision, check the pedicle attachment site, and then along the pe

32、dunculated 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 patie

33、nts 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

34、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. Therefo

35、re, resection of the tumor after application of saline flushing chamber of the heart, prevent tumor fragments hidden in the atrium, ventricular muscle trabeculae inside. 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 aft