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Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome



شناسه مقاله
10.1016/j.jtcvs.2016.01.054
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انگلیسی

Title: Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome

Authors: Bahaaldin Alsoufi and Courtney McCracken and Brian Schlosser and Ritu Sachdeva and Andrew Well and Brian Kogon and William Border and Kirk Kanter

Journal: The Journal of Thoracic and Cardiovascular Surgery

Year: 2016

Abstract:

AbstractBackground Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac and extracardiac anomalies. We report current era palliation results. Methods Between 2002 and 2012, 67 infants with heterotaxy syndrome underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) and examined factors associated with survival. In addition, early and late outcomes following first-stage palliation surgery were compared with a matched contemporaneous control group of patients with nonheterotaxy single ventricle anomalies. Results Fifty-eight patients (87%) required neonatal palliation, including a modified Blalock-Taussig shunt (n = ۳۴; ۵۱%), Norwood operation (n = ۱۲; ۱۸%) or pulmonary artery band (n = ۱۲; ۱۸%), whereas 9 patients (13%) underwent a primary Glenn procedure. Competing risks analysis showed that at 1 year after first-stage palliation surgery, 29% of the patients had died or undergone transplantation and 63% had undergone a Glenn procedure. By 5 years after the Glenn procedure, 64% of patients had undergone a Fontan procedure. The overall 8-year survival rate was 66%. On multivariable analysis, factors associated with mortality were unplanned reoperation (hazard ratio [HR], 2.9; 95% confidence interval [CI], 1.1-7.3; P = .۰۰۵) and total anomalous pulmonary venous connection repair (HR, 2.3; 95% CI, 1.0-5.6; P = .۰۵۶). Comparison with the contemporaneous matched patients with nonheterotaxy single ventricle anomalies showed that first-stage palliation in the patients with heterotaxy was associated with a higher rate of in-hospital death (27% vs 10%; P = .۰۲۲), and significantly longer durations of ventilation and intensive care unit stay. Interstage mortality, survival after the Glenn procedure, and progression to the Fontan procedure were comparable in the 2 groups. Conclusions The management of infants with heterotaxy and a functional single ventricle remains challenging. First-stage palliation is associated with high operative mortality and increased resource utilization owing to surgical morbidity. Nonetheless, outcomes beyond hospital discharge are comparable to those for patients with other single ventricle anomalies. Efforts to improve survival in those patients should focus on perioperative care.

Keywords: single ventricle, heterotaxy syndrome, Glenn, Fontan

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عنوان: نتایج تسکین چند مرحله ای از نوزادان با بطن تک کاربردی و سندرم heterotaxy

نویسندگان: Bahaaldin Alsoufi and Courtney McCracken and Brian Schlosser and Ritu Sachdeva and Andrew Well and Brian Kogon and William Border and Kirk Kanter

ژورنال: The Journal of Thoracic and Cardiovascular Surgery

سال: ۲۰۱۶

چکیده:
نتیجه گیری مدیریت نوزادان با heterotaxy و یک بطن کاربردی تک باقی مانده است به چالش کشیدن تسکین مرحله اول است با مرگ و میر از عمل بالا و افزایش بهره برداری از منابع با توجه به عوارض جراحی با این حال مرتبط، نتایج فراتر از ترخیص از بیمارستان قابل مقایسه است برای بیماران مبتلا به دیگر تنها بطن ناهنجاری تلاش به منظور بهبود بقا در آن دسته از بیماران باید در مورد مراقبت بعد از عمل تمرکز
کلمات کلیدی:   تنها بطن، سندرم heterotaxy، گلن، فونتان

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