Alongi Ashlyn M, Kirklin James K, Deng Luqin, Padilla Luz, Pavnica Jozef, Romp Robb L, Mauchley David C, Cleveland David C, Dabal Robert J
University of Alabama at Birmingham School of Medicine, AL, USA.
Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, AL, USA.
World J Pediatr Congenit Heart Surg. 2020 Mar;11(2):166-176. doi: 10.1177/2150135119893650.
Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies.
We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016.
Of the 103 patients (50 males and 53 females), 31 had left atrial isomerism, 64 had right atrial isomerism (RAI), and 8 patients' isomerism was indeterminate (IND), with first cardiac operation at a mean 1.0 year (standard deviation ±3.0 years) of age. Kaplan-Meier overall survival estimate was 83.1% at six months, 77.8% at one year, 65.9% at five years, and 52.1% at ten years. Survival was particularly low among RAI following repair of total anomalous pulmonary venous connection (TAPVC) at first operation, with one- and five-year survival of 57% and 46%, respectively. By multivariable analysis, the only risk factor for death during the early phase (hazard model) was repair of TAPVC at the first cardiac operation (hazard ratio [HR]: 4.4, = .01), and risk factors during the longer term constant phase were atrioventricular valve (AVV) regurgitation (HR: 4.2, < .01), male gender (HR: 3.7, < .01), and two-ventricle repair (HR: 3.0, = .02). Patients with heterotaxy undergoing the Fontan procedure had excellent subsequent survival (85% at ten years).
This analysis of over 100 patients with heterotaxy identified TAPVC requiring initial repair as the major risk factor for early death and important AVV regurgitation as the major risk factor in the longer term. Survival with RAI and early repair of TAPVC were poor, with one-year mortality exceeding 40%. Patients with single ventricle completing the Fontan operation enjoyed outstanding ten-year survival (85%). Initial management of RAI requiring early repair of TAPVC remains challenging. For this high-risk subset, alternative strategies such as early referral for cardiac transplantation evaluation warrant consideration.
即使在当今时代，内脏反位综合征在外科治疗中仍面临独特挑战。我们推测某些解剖亚组需要新的治疗策略。
我们分析了1990年1月1日至2016年5月31日期间在单一机构连续接受心脏手术的103例内脏反位患者的形态学细节、手术情况、合并症、后续住院情况及生存率，采用Kaplan-Meier方法和多变量风险模型进行分析。
103例患者（50例男性和53例女性）中，31例为左房异构，64例为右房异构（RAI），8例异构情况不确定（IND），首次心脏手术时平均年龄为1.0岁（标准差±3.0岁）。Kaplan-Meier法估计的总体生存率在6个月时为83.1%，1年时为77.8%，5年时为65.9%，10年时为52.1%。首次手术修复完全性肺静脉异位连接（TAPVC）后，RAI患者的生存率特别低，1年和5年生存率分别为57%和46%。多变量分析显示，早期（风险模型）死亡的唯一危险因素是首次心脏手术时修复TAPVC（风险比[HR]：4.4，P = 0.01），长期稳定期的危险因素是房室瓣（AVV）反流（HR：4.2，P < 0.01）、男性（HR：3.7，P < 0.01）和双心室修复（HR：3.0，P = 0.02）。接受Fontan手术的内脏反位患者后续生存率良好（10年时为85%）。
对100多例内脏反位患者的分析表明，需要初次修复的TAPVC是早期死亡的主要危险因素，重要的AVV反流是长期的主要危险因素。RAI患者及早期修复TAPVC的患者生存率较差，1年死亡率超过40%。完成Fontan手术的单心室患者10年生存率出色（85%）。需要早期修复TAPVC的RAI患者的初始治疗仍然具有挑战性。对于这个高危亚组，诸如早期转诊进行心脏移植评估等替代策略值得考虑。
