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原發性心臟腫瘤的外科治療

目的 總結原發性心臟腫瘤的外科治療經驗，以提高手術療效。 方法 回顧性分析1980年6月至2008年12月中國醫科大學附屬第一醫院收治的263例原發性心臟腫瘤患者的臨床資料，其中男90例，女173例；年齡10～79歲（41±13歲）。良性腫瘤241例，其中良性粘液瘤240例，纖維瘤1例；惡性腫瘤22例，其中惡性粘液瘤7例，惡性間皮瘤4例，血管肉瘤5例，橫紋肌肉瘤1例，肺動脈內膜肉瘤1例，平滑肌肉瘤1例，炎癥性惡性纖維組織細胞瘤1例，惡性淋巴瘤1例，滑膜肉瘤1例。手術完整摘除腫瘤252例（惡性腫瘤11例），局部切除腫瘤5例（均為惡性腫瘤），開胸探查取病理活組織檢查6例（均為惡性腫瘤）。同期行冠狀動脈旁路移植術（CABG）5例，二尖瓣置換術5例，二尖瓣成形術4例，三尖瓣成形術9例，三尖瓣生物瓣置換術1例，主動脈根部及肺動脈成形術1例，肺動脈瓣置換術1例，肺動脈主干及左右肺動脈人工血管加肺動脈瓣置換術1例，肺動脈切開取栓術1例，經股動脈取瘤栓術5例。 結果 圍術期死亡7例（良性粘液瘤6例、惡性粘液瘤1例），其中術中不能停體外循環2例，術后發生低心排血量、心室顫動3例，呼吸、循環衰竭1例，大面積腦出血1例；其余患者無并發癥發生。隨訪247例（良性腫瘤229例，惡性腫瘤18例），隨訪時間3個月～28年，失訪9例（良性腫瘤6例、惡性腫瘤3例）。隨訪期間良性粘液瘤復發4例，均再次手術治療；良性腫瘤患者死亡13例（心源性猝死6例、腦卒中2例、肺癌1例、不明原因4例），其余216例均生存。隨訪期間惡性腫瘤患者死亡15例，術后生存時間為1～4年，死于腫瘤復發或轉移11例，心力衰竭和惡病質各2例。 結論 原發性心臟腫瘤一經確診應盡早手術治療，良性腫瘤手術效果好，惡性腫瘤術中應盡量切除腫瘤。

Clinical Outcomes of Left Atrial Myxoma Resection via Right Anterolateral Minithoracotomy

ObjectiveTo investigate clinical outcomes and safety of minimally invasive left atrial myxoma (LAM) resection via right anterolateral minithoracotomy (ALMT). MethodsClinical data of 9 patients who underwent minimally invasive LAM resection via right ALMT in the Affiliated Hospital of Luzhou Medical College from January 2011 to October 2013 were retrospectively analyzed. There were 2 male and 7 female patients with their age of 37-62 (51±9) years. The operation was performed through a small (4-6 cm) right ALMT incision. Femoral artery and vein and superior vein were cannulated to establish cardiopulmonary bypass (CPB). Transthoracic clamp was used for ascending aortic clamping. Antegrade cold blood cardioplegia was infused for myocardial protection. LAM was resected through right atriotomy trans-septal approach. ResultsAll the operations were successfully performed without in-hospital death. Operation time was 210-310(260±33) minutes, aortic cross-clamping time was 23-50(37±9) minutes, CPB time was 60-87(71±9) minutes, postoperative mechanical ventilation time was 6-14(9.0±2.5) hours, and length of ICU stay was 17-26(20±3) hours. Postoperative mediastinum drainage was 100-650(376±190) ml. Mean length of right ALMT was 4.5-6.0 (5.3±0.6) cm. All the patients were followed up for 1 to 30 months,and echocardiography showed no LAM recurrence. ConclusionMinimally invasive LAM resection via right ALMT is safe and feasible with satisfactory clinical outcomes.

101例心臟粘液瘤的外科治療體會

目的總結101例心臟粘液瘤的診斷和治療經驗，以提高療效。方法回順性分析101例心臟粘液瘤患者的臨床資料，其中左心房粘液瘤94例，右心房粘液瘤6例，雙心房粘液瘤1例，均在體外循環下行粘液瘤摘除術；同期行二尖瓣成形術5例，二尖瓣機械瓣置換術1例，三尖瓣成形術32例，冠狀動脈旁路移植術1例，激光心肌打孔血運重建術1例。結果術后死亡1例，100例患者經治療痊愈出院。隨訪84例，隨訪率84％（84／100）；隨訪時間3個月～5年，復發1例。結論心臟粘液瘤術前超聲心動圖檢查診斷準確率高，一經確診應盡早手術；手術的關鍵是保持瘤體完整，將瘤體連同蒂部周圍部分組織一并切除。

Diagnosis and Treatment of Traumatic Rapture of Diaphragm (Report of 32 Cases)

目的探討腹膜假性粘液瘤的診斷和治療。方法對本院1988~2002年收治的5例腹膜假性粘液瘤的臨床資料進行回顧性分析，并結合文獻加以討論。結果該5例患者的主要臨床表現為腹脹、腹痛、腹部包塊，其中4例行減瘤手術治療，并在術后輔以腹腔內化療。術后隨訪，1例3年內死亡，1例7年內死亡，另2例現已分別存活9年零5個月和3個月。結論腹膜假性粘液瘤是一種少見疾病，其來源多見于闌尾，術前診斷困難，多次針吸和血清癌胚抗原檢查對診斷可能有幫助。多數患者術中探查可見腹腔內廣泛病變，一次清除很困難，往往需采取多次減瘤術。外科手術治療仍是目前有效的治療手段。

Clinical features and research progress of Carney complex

Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas and multiple endocrine tumors. The disease is caused by inactivating mutations or large deletions of the PRKAR1A gene located at 17q22–24 coding for the regulatory subunit type Ⅰ alpha of protein kinase A (PKA) gene. Most recently, components of the complex have been associated with defects of other PKA subunits, such as the catalytic subunits PRKACA (adrenal hyperplasia) and PRKACB (pigmented spots, myxomas, pituitary adenomas). We reviewed CNC’s clinical features, diagnosis, treatment and molecular etiology.

Clinical analysis of cardiac myxoma leading to embolic events

ObjectiveTo investigate the clinical manifestations of patients with cardiac myxoma and the factors affecting the occurrence of embolic events. Methods A retrospective study of 38 patients with cardiac myxoma diagnosed and surgically removed from January 2010 to December 2017 was performed. There were 11 males and 27 females at age of 32-75 (50.00±16.12) years. The patients were divided into a non-embolized group and an embolized group. The clinical manifestations of the patients were summarized and the factors leading to embolism were analyzed. RseultsOf the 26 patients in the non-embolized group, 22 patients (84.62%) had dyspnea, 14 patients (53.85%) had palpitations, 4 patients (15.38%) had angina pectoris, and 1 patient (3.85%) had heart failure. Of the 12 patients in the embolized group, 4 patients (33.33%) had dyspnea, 3 patients (25%) had palpitations, and 1 patient (8.33%) had angina pectoris. The mean diameter of the non-embolized group was 5.71±1.63 cm, and the maximum diameter of the tumor in the embolized group was 4.52±1.88 cm. There was no significant difference between the maximum diameter of the tumor in the embolized group and the maximum diameter of the non-embolized group (P>0.05). Atrial fibrillation occurred in 2 patients in the non-embolized group before operation. Atrial fibrillation occurred in 5 patients in the embolized group. Atrial fibrillation was more likely to occur in the embolized group (P<0.05). Conclusion Atrial fibrillation in the patients with cardiac myxoma is closely related to embolic events. The size of myxoma is not related to the occurrence of embolic events.

非粘液瘤性心臟良性腫瘤的診斷與手術治療

目的 總結非粘液瘤性心臟良性腫瘤的診斷和外科治療經驗。 方法 1982年9月～2005年10月手術治療5例非粘液瘤性心臟良性腫瘤，其中男2例，女3例；年齡2～46歲（33.4±17.8歲）。所有腫瘤均被完整切除，同時將其附著的心內膜和心肌組織一并切除，用心包或補片修補缺損，術畢反復沖洗心腔并吸凈。 結果 5例患者均治愈出院。術后均獲得隨訪，隨訪時間3個月至11年，未發現嚴重心律失常，并恢復正常工作、學習；其中1例5年后復發，再次手術無法切除。 結論 非粘液瘤性心臟良性腫瘤的臨床表現、病理類型多樣，手術治療效果好，術后應重視復查。

心臟粘液瘤的診斷與外科治療

目的 總結心臟粘液瘤的外科治療經驗。 方法 回顧性分析1997年6月～2006年6月我院收治的113例心臟粘液瘤患者的臨床資料，其中左心房粘液瘤98例,左心室粘液瘤2例,右心房粘液瘤8例,右心室粘液瘤4例,雙心房粘液瘤1例。所有患者均在中度低溫體外循環下行粘液瘤摘除術,同期行二尖瓣機械瓣置換術2例,二尖瓣成形術3例,三尖瓣成形術7例,腹主動脈取栓術1例。 結果 本組圍術期死亡5例（4.4%）,其中2例死于多器官功能衰竭，1例死于低心排血量綜合征，1例死于左心衰竭，1例死于惡性心律失常。其余患者均順利脫離呼吸機，痊愈出院。108例患者獲得隨訪,隨訪時間2～110個月,心功能Ⅰ級63例,Ⅱ級31例,Ⅲ級12例;復發3例,2例再次手術后痊愈出院；遠期死亡2例，均為惡性粘液瘤。 結論 心臟粘液瘤患者被確診后應盡快手術治療,其效果滿意,彩色超聲心動圖對診斷及隨訪均具有重要作用。

Thoracoscopic Surgery versus Median Sternotomy Surgery for Left Atrium Myxoma Excision: A Case Control Study

Objective To examine the effect and safety of thoracoscopic surgery for left atrium myxoma excision. Method Sixty-nine left atrial myxoma patients underwent excision of left atrial myxoma in our hospital between January 2012 and August 2014 year. The patients were divided into two groups according to the procedure. Thirty patients under-went thoracoscopic surgery, as a thoracoscopic group, with 8 males and 22 females, aged 47.36±13.02 years. Thirty-nine patients received median sternotomy surgery, as a median sternotomy group, with 10 males and 29 females, aged 49.17±13.09 years. The effect and safety between the two groups were compared. Results All patients survived after surgery without death and other serious complications. Compared with the median sternotomy surgery group, longer cardiopul- monary bypass and aortic cross clamp time, shorter ICU stay, ventilator support, and postoperative drainage time, shorter hospital stay time, less postoperative drainage, lower cost, and more higher rate of returning to work in 1 month after surgery were found in the thoracoscopic group with P value less than 0.05. There was no complication of stroke and other neurological complication in the two groups. All patients were followed up for 11 months to 4 years and 7 months, average age of 38.5±12.7 months. There was no recurrence in both groups. Conclusions The thoracoscopic left atrial myxoma excision cardiopulmonary is effective and safe. It can be used as a surgical treatment of left atrial myxoma preferred.

起源于二尖瓣瓣環的心臟粘液瘤一例