左心房异构单心室姑息手术后的结果。

Outcomes following the Kawashima procedure for single-ventricle palliation in left atrial isomerism.

机构信息

Department of Congenital Heart Disease, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.

出版信息

Eur J Cardiothorac Surg. 2012 Mar;41(3):574-9. doi: 10.1093/ejcts/ezr003. Epub 2011 Oct 18.

DOI:10.1093/ejcts/ezr003

PMID:22011773

Abstract

OBJECTIVES

Patients with left atrial isomerism and interrupted inferior vena cava palliated with a superior cavopulmonary connection or Kawashima procedure (KP) have a high incidence of developing pulmonary arteriovenous malformations. The necessity for hepatic vein redirection (HVR) and its timing remains a controversy. We aimed to assess the clinical outcome of patients with left atrial isomerism following a KP. The main end points were death, requirement for HVR and the impact of HVR on oxygen saturation.

METHODS

Retrospective review of 21 patients with a diagnosis of left atrial isomerism, interruption of the inferior vena cava and single-ventricle physiology managed with a KP at a single centre between January 1990 and March 2010.

RESULTS

Twenty-one patients had a KP, with 12 subsequently undergoing HVR. There was relatively a constant monthly decrement in the proportion of patients who were free from death or HVR up until 60 months following the KP, with a dramatic increase in the hazard after this time. The Cox proportional hazards regression model demonstrated a reduced early risk for HVR or death in patients who underwent pulmonary artery banding versus arterial shunt as the primary procedure (hazard ratio: 0.10; P = 0.01), and an increased risk with bilateral superior vena cavas (SVCs) (hazard ratio: 3.4; P = 0.04) and age at KP (hazard ratio: 1.02 per month increase in age at KP; P = 0.02). HVR mortality was relatively high with 3 of 12 patients dying in the early postoperative period with profound cyanosis. The timing of HVR after the KP did not influence the postoperative rate of increase in oxygen saturation.

CONCLUSION

These findings confirm that the majority of patients who undergo a KP will require HVR. Patients who are older at the time of the KP or having an initial arterial shunt or bilateral SVCs are at higher risk of HVR or death. The relatively high mortality at HVR was characterized by severe postoperative cyanosis.

摘要

目的

接受上腔静脉-肺动脉吻合术（KP）或 Kawashima 手术（KP）姑息性治疗的左心房异构伴下腔静脉中断患者，肺动静脉畸形的发生率较高。需要进行肝静脉改道（HVR）及其时机仍存在争议。我们旨在评估接受 KP 治疗后的左心房异构患者的临床结果。主要终点为死亡、需要 HVR 以及 HVR 对氧饱和度的影响。

方法

回顾性分析 1990 年 1 月至 2010 年 3 月期间在一家中心接受 KP 治疗的左心房异构、下腔静脉中断和单心室生理学诊断的 21 例患者。

结果

21 例患者接受了 KP，其中 12 例随后进行了 HVR。在 KP 后 60 个月内，死亡或 HVR 无发生的患者比例逐月下降，但在此之后，风险急剧增加。Cox 比例风险回归模型显示，与动脉分流术相比，肺动脉带术作为主要术式的患者 HVR 或死亡的早期风险降低（风险比：0.10；P = 0.01），双侧上腔静脉（SVC）（风险比：3.4；P = 0.04）和 KP 年龄（风险比：KP 年龄每月增加 1.02 ；P = 0.02）增加了风险。HVR 死亡率相对较高，12 例患者中有 3 例在术后早期因严重发绀而死亡。KP 后 HVR 的时机并未影响术后氧饱和度增加的发生率。