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題名 | 單一心室循環的先天性心臟病的心衰竭=Perioperative Care of Congenital Heart Disease with Single Ventricle Physiology |
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作者 | 孫麗娟; 吳恩婷; 黃書健; 柯文哲; 王主科; 吳美環; | 書刊名 | 中華民國重症醫學雜誌 |
卷期 | 8:4 2007[民96] |
頁次 | 頁184-191 |
分類號 | 416.262 |
關鍵詞 | 先天性心臟病; 單一心室循環; 心衰竭; Congenital heart disease; Single ventricle; Perioperative care; Heart failure; |
語文 | 中文(Chinese) |
中文摘要 | 單一心室循環的先天性心臟病患者常伴隨心室功能不佳。在新生兒期階段性手術時心室衰竭的原因包括:肺循環過多血量造成右心室容積負荷,三尖瓣逆流,冠狀動脈血流不足及代謝性酸中毒等;周術期照顧的重要原則,就是要1.限制肺血流量,2.降低體循環阻力以增加全身循環,3.改善冠狀動脈的灌流,4.妥善使用強心劑及使用機械性支持。而最好的原則應是在肺循環量過多,全身循環下降及心室功能失調這些不利因素發生前,儘早進行手術。術後要安排心臟超音波以排除殘有的解剖問題。在藥物的使用方面,則建議使用milrinone及低劑量的epinephrine,因為他們可以增加心輸出量並減少肺部分流。在完成Fontan手術前先進行上腔靜脈肺動脈吻合術(bidirectional Glenn shunt),可保存心室功能減少心室擴大。目前術後的照顧策略可使用輕度的呼吸酸血症,如此可增加心輸出量,減少氧氣消耗增加動脈氧氣濃度。不過,此階段使用機械性支持(如ECMO)的存活率則比其他階段差。在完成Fontan術式後,許多病人會有進行性的心臟功能衰竭,原因可能包括:長期的心室容積壓力過大,低血氧,及經過多次體外心肺循環的心室損傷引起的變化;留下通乳及使用modified ultrafiltration,皆可減少死亡率,此外,接受心房肺動脈直接吻合(atriopulmonary Fontan)後合併心衰竭的病人,改開成lateral tunnel或是心外型Fontan並放置結律器後,有些心臟功能會改善,但有些病人仍需要心臟移植。新的針對長期心衰竭的治療策略包括Nesiritide, Levosimendan及機械型輔助,在成人病人似乎是有幫助的,但對單一心室循環的兒科病人,長期效果則未知。 |
英文摘要 | The patient with a functionally single ventricular heart is at increased risk for ventricular dysfunction for a variety of reasons. In neonatal palliation, the etiologies include volume overload from pulmonary over-circulation, tricuspid valvar regurgitation, coronary arterial insufficiency, and metabolic acidosis. To limit pulmonary over-circulation, manipulation of the pulmonary vasculature by hypercarbia and subambient oxygen may work. But the best policy is to perform the first stage palliation earlier before the development of marked pulmonary over-circulation and ventricular dysfunction. After operation, it is imperative to rule out any residual anatomic defects. Pharmacological support such as milrinone and low-dose epinephrine may be beneficial to increase systemic output and decrease the ratio of pulmonary to systemic flow as well. Performing a bidirectional superior cavopulmonary anastomosis (Glenn shunt), prior to completing the Fontan circulation may help to preserve ventricular function, decrease ventricular size, and increase the ratio of ventricular mass to volume. Recent work demonstrated that a mild respiratory acidosis postoperatively leads to an increased cardiac output, decreased consumption of oxygen, and improved arterial saturations. Survival in mechanical support after a bidirectional Glenn anastomosis is less than other stages. After completion of the Fontan circulation, many patients will experience a progressive decrease in functional status due to years of vlume and pressure overload, chronic hypoxemia, multiple episodes of cardiopulmonary bypass and increased wall stress. The use of a fenestration in the Fontan circuit and the use of modified ultrafiltration have both been associated with decreased mortality. The patient with a failing ventricle in the setting of atriopulmonary circuit may undergo conversion to the lateral tunnel or extracardaic Fontan with pacemaker insertion to achieve better cardiac output, but some still require heart transplantation. Several novel strategies have been proposed for congestive heart failure such as Nesiritide, Levosimendan and mechanical support. Their application on adult patients seems beneficial but long-term effects remain unknown in the patients with single ventricle physiology. |
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