Özdemir Fatih, Korun Oktay, Dedemoğlu Mehmet, Çiçek Murat, Biçer Mehmet, Yurdakök Okan, Fırat Altın Hüsnü, Hekim Yılmaz Emine, Yurtseven Nurgül, Ali Aydemir Numan, Şaşmazel Ahmet
Department of Pediatric Cardiovascular Surgery, University of Health Sciences Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
Department of Pediatric Cardiovascular Surgery, University of Health Sciences Ümraniye Training and Research Hospital, Istanbul, Turkey.
Turk Gogus Kalp Damar Cerrahisi Derg. 2022 Jan 28;30(1):26-35. doi: 10.5606/tgkdc.dergisi.2022.22397. eCollection 2022 Jan.
In this study, we aimed to analyze the predictors and risk factors of mortality in patients who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome.
Between January 2009 and December 2020, a total of 139 patients (95 males, 44 females) who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome in our center were retrospectively analyzed.
The median birth weight was 3,200 (range, 3,000 to 3,350) g and the median age at the time of operation was seven (range, 5 to 10) days. Pulmonary flow was achieved with a Sano shunt in the majority (72%) of patients. Survival rate was 41% after the first stage. Reoperation for bleeding (p=0.017), reoperation for residual lesion (p=0.011), and postoperative peak lactate level (p=0.029), were associated with in-hospital mortality. Nineteen (33%) of 57 patients died before the second stage. Thirty-three (58%) patients underwent second stage, and survival after the second stage was 94%. Thirteen patients underwent third stage, and survival after the third stage was 85%. Estimated probability of survival at six months, and one, two, three, and four years were 33%, 33%, 25%, 25%, and 22% respectively.
Hospital and inter-stage mortality rates are still high and this seems to be the most challenging period in term of survival efforts of the patients with hypoplastic left heart syndrome. Early recognition and reintervention of anatomical residual defects, close follow-up in the inter-stage period, and the accumulation of multidisciplinary experience may help to improve the results to acceptable limits.
在本研究中,我们旨在分析诊断为左心发育不全综合征并接受诺伍德一期手术患者的死亡预测因素和风险因素。
回顾性分析2009年1月至2020年12月期间在我们中心诊断为左心发育不全综合征并接受诺伍德一期手术的139例患者(95例男性,44例女性)。
中位出生体重为3200(范围3000至3350)克,手术时的中位年龄为7(范围5至10)天。大多数(72%)患者通过桑诺分流实现肺血流。一期手术后生存率为41%。因出血再次手术(p=0.017)、因残余病变再次手术(p=0.011)和术后乳酸峰值水平(p=0.029)与院内死亡相关。57例患者中有19例(33%)在二期手术前死亡。33例(58%)患者接受了二期手术,二期手术后生存率为94%。13例患者接受了三期手术,三期手术后生存率为85%。六个月、一年、两年、三年和四年的估计生存概率分别为33%、33%、25%、25%和22%。
医院死亡率和阶段间死亡率仍然很高,这似乎是左心发育不全综合征患者生存努力中最具挑战性的时期。早期识别和再次干预解剖学残余缺陷、在阶段间密切随访以及积累多学科经验可能有助于将结果改善到可接受的限度。