Embolic Stroke After Total Cavopulmonary Connection for Complex Congenital Heart Disease - A Case Report

Fontan 手術後可能發生血栓等併發症。其發生的時間差異相當大，可以在手術後的第一天　發生，也可以在手術超過十年以後才發生。我們在此報告一位複雜型的先天性心臟病病患，他在出生二個月大時先接受肺動脈縮小手術；在四歲大時接受完全上下腔靜脈－肺動脈的連接手術。手術十一年後，病患出現頭痛症狀，但並無神經學檢查之缺陷。第一次的腦部電腦斷層檢查並無異常，心導管檢查發現有明顯的右至左分流，由下腔靜脈經肝靜脈再流入右心房。在右側肺動脈及心房內人造血管處有疑似血栓的存在。經阿斯匹林抗血栓治療及頭痛之症狀治療，病患症狀逐漸改善後返家。不幸的，病患返家六週後，突然再次出現頭痛且發生意識狀態改變及左側半身輕癱的現象。第二次腦部電腦斷層檢查發現右大腦中動脈栓塞性中風，經給予降腦壓處理及抗凝血劑藥物 (warfarin) 治療仍無效，在入院第三天後死亡。雖然Fontan 式手術後血栓的形成並不是少見的併發症，但是，抗凝血劑使用的策略及原則，一直到現在都沒有一致的共識。經過此案例的痛苦經驗，我們建議：(一) 任何Fontan 式手術後的病患，都應接受長期甚至是終身的抗凝血劑治療，以期預防血栓併發症的發生，並且需要定期檢測抗凝血劑治療的效果；(二) 定期追蹤經胸前或是經食道的心臟超音波檢查，除了評估心臟功能外，也可以儘早偵測血栓的存在，以預防手術後的併發症。

關鍵字

完全上下腔靜脈－肺動脈連接術； Fontan 式手術；單一心室；中風；血栓；抗凝血劑

並列摘要

Thrombosis has been reported to occur variably from 1 day to more than 10 years after Fontan-type operation. We herein describe a case with heterotaxy syndrome with situs ambiguous, dextrocardia, single ventricle, complete atrioventricular canal defect, malposition of great arteries and bilateral superior vena cava who had received pulmonary artery banding and total cavopulmonary connection at the ages of 2 months and 4 years, respectively. However, he was lost to follow-up. Eleven years after the operation, the patient suffered from acute cerebral infarction of right middle cerebral artery territory, which was documented by brain computed tomography. Multiple venous thrombi were detected by suprasternal approach of two-dimensional transthoracic echocardiography and cardiac angiography during hospitalization. The patient finally expired in spite of aggressive treatment.We suggest that transthoracic and/or even transesophageal echocardiography should be performed as a routine surveillance for cardiac function evaluation as well as for thrombus detection in patients after cavopulmonary connection. In addition, long-term use of oral anticoagulant may be necessary, especially when patients have low cardiac output and/or high atrial pressure.

並列關鍵字

Cavopulmonary connection ； Fontan-type operation ； Stroke ； Cerebral infarction ； Thrombosis ； Anticoagulant

國際替代計量

Embolic Stroke After Total Cavopulmonary Connection for Complex Congenital Heart Disease - A Case Report

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