先天性心臟病常見的神經學併發症

Neurological Complications after Open Heart Surgery in Neonates and Infants

吳焜烺(Kun-Lang Wu) ； 吳恩婷(En-Ting Wu) ； 黃書健(Shu-Chien Huang) ； 柯文哲(Wen-Je Ko) ； 李旺祚(Wang-Tso Lee) ； 王主科(Jou-Kou Wang) ； 吳美環(Mei-Hwan Wu)

《重症醫學雜誌》 9卷3期 (2008/09) Pp. 181-190

https://doi.org/10.30035/TCCM.200809.0005

摘要

隨著先天性心臟病手術的進步，同時心臟超音波診斷技術的發展，加護病房照顧的進步，及引用prostaglandin及介入性心導管的發展，手術的死亡率已大幅降低，手術年齡也逐漸下降。目前有愈來愈多的患者可活到青少年甚至成年他們可能伴隨的神經學合併症，和學業及行爲方面的表現，是一個令人關注的問題。新生兒期或嬰兒期接受開心手術的患者，有較高比例有學習障礙、行爲異常、發育遲緩、動作協調不佳的問題、以及言語遲緩、注意力不集中、或過動的現象。造成這樣的神經行爲學的原因很複雜，可能和原本的心臟疾病有關、可能和手術處理有關，或法因的問題，也可能和太晚診斷以致缺氣有關，或是開完刀的低心輸出量症候群有關。大致上可分爲：（一）先天性腦疾患：因爲中樞神經系統和心血管系統在胎兒期的發育是同時的，所以這些先天性心臟病患童的腦部有先天異常的比例較高。此外，先天性心臟病可能改變了腦部的血流、氣氣輸送、導致中樞神經受損。（二）周術期的因素：大部分複雜性先天性心臟病的新生兒一出生即需要接受靜脈注射，可能因氣泡或其他物質造成奇異性栓塞(Paradoxical emboli)到腦部造成中風。這些病人的血氣濃度也低於正常人，造成氣氣輸送不足。另外，心肺體外循環或其他開心手術時支持循環的因素，包括：氣泡或其他栓塞、核心體溫降低的速度及程度、深度低溫循環中止(Deep Hypothermia Circulatory Arrest, DHCA)、再灌流傷害與發炎反應、升溫的速度／高溫反應、高血糖、使用高氧氣(hyperoxia)，及體外循環時的pH值及血比容的變化等都可能造成傷害。（三）術後的因素：開心手術後的低心輸出量症候群會造成腦部灌流不足。（四）基因及環境因素：父母的社會經濟地位可能是影響最後的神經學發展最大的一個因素，因爲它反映出基因及環境兩大方面的差異。還有一些法因異常如22q11缺失、唐氏症或是威廉氏症候群等同時也合併了中樞神經異常。必須有大規模的研究來釐清造成嬰幼兒開心術後常見的神經學合併症的原因才能進一步預防。

關鍵字

先天性心臟病 ； 神經學 ； 併發症

並列摘要

The nearly simultaneous advances in congenital cardiac surgery, echocardiography, and intensive care, with the availability of prostaglandin have resulted in a dramatic fall in surgical mortality, and also complex repairs taking place at increasingly younger ages. As a result, an increasing number of children with congenital heart disease lived into school age and even adulthood. Many have a significantly higher incidence of academic difficulties, behavioral abnormalities, fine and gross motor delays, problems with visual-motor integration and executive planning, speech delays, inattention, and hyperactivity. The causes of these neurological complications are multifactorial, including (1) congenital cerebral disease: Given that the central nervous and cardiovascular systems form nearly simultaneously in early gestation, it is not surprising that there is an increased incidence of structural abnormalities of the brain in children with structural cardiac abnormalities. (2) perioperative contributors: Neonates with complex congenital cardiac disease frequently require intravenous infusions which carry risks to the central nervous system, especially the potential for paradoxical embolus to the brain of air or particulate matter in children with intracardiac right-to-left shunts. Also, the conduct of cardiopulmonary bypass, and other support techniques used during open-heart surgery, including air emboli, degree of low core temperature, the speed of rewarming, hypoglycemia and the use of hyperoxia have also received considerable attention. (3) postoperative contributors: low cardiac output syndrome after open heart surgery may cause poor perfusion in the brain, which is especially vulnerable to secondary insults of decreased oxygen delivery, particularly after circulatory arrest. (4) genetic and environmental factors: Socioeconomic status is perhaps the strongest predictor of eventual neurodevelopmental outcome, and is a reflection of both the environment of the child and the genetic factors for development inherited from his or her parents. Besides, chromosome anomalies, including 22q11 deletion syndrome, Down's syndrome and Williams' syndrome, have coexisting neuropsychological problems. Much more work needs to be done to understand the modifiable risk factors in the perioperative period, the influences of the timing of surgery and whether or not improved monitoring of the central nervous system in the intensive care unit setting will improve neurological and developmental outcomes.

並列關鍵字

Congenital heart disease ； Neurological complications ； Cardiopulmonary bypass