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Background: 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. Methods: 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. Results: 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. Conclusion: 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. / Amaç: bu çalışmada, hipoplastik sol kalp sendromu tanısıyla Norwood I işlemi uygulanan hastalarda mortalitenin öngörücüleri ve risk faktörleri incelendi. Ça¬lış¬ma pla¬nı: Ocak 2009 ve Aralık 2020 tarihleri arasında merkezimizde hipoplastik sol kalp sendromu tanısıyla Norwood I işlemi uygulanan toplam 139 hasta (95 erkek, 44 kadın) retrospektif olarak incelendi. Bulgular: medyan doğum ağırlığı 3200 (dağılım, 3000-3350) g ve ameliyat sırasında medyan yaş yedi (dağılım, 5-10) gün idi. Hastaların büyük kısmında (%72) pulmoner akım, Sano şant ile sağlandı. İlk aşama sonrası sağkalım %41 idi. Kanama nedeniyle tekrar ameliyat (p=0.017), rezidü anatomik lezyon nedeniyle tekrar ameliyat (p=0.011) ve ameliyat sonrası birinci gün pik laktat seviyesi (p=0.029) hastane mortalitesi ile ilişkili idi. Taburcu edilen 57 hastanın 19’u (%33) ikinci aşamaya ulaşamadan kaybedildi. Otuz üç (%58) hastaya ikinci aşama ameliyatı uygulandı ve ikinci aşama sonrası sağkalım %94 idi. On üç hastaya üçüncü aşama ameliyatı uygulandı ve üçüncü aşama sonrası sağkalım %85 idi. Altı ay ve birinci, ikinci, üçüncü ve dördüncü yılda tahmini sağkalım olasılığı sırasıyla %33, %33, %25, %25 ve %22 idi. So¬nuç: hastane ve aşamalar arası mortalite oranları halen yüksek olup, hipoplastik sol kalp sendromu tanılı hastaların sağkalma çabalarındaki en zorlayıcı zaman dilimi olarak görünmektedir. Anatomik rezidü defektlerin erken tanılanması ve erken yeniden girişim yapılması, aşamalar arası dönemde yakın takip ve multidisipliner birikim ve tecrübe sonuçların kabul edilebilir seviyelere ulaşmasına yardım edebilir., NA

Objectives: to evaluate the hemodynamicdynamic advantage of a new Fontan surgical template that is intended for complex single-ventricle patients with interrupted inferior vena cava-azygos and hemi-azygos continuation. The new technique has emerged from a comprehensive pre-surgical simulation campaign conducted to facilitate a balanced hepatic flow and somatic Fontan pathway growth after Kawashima procedure. Methods: for 9 patients, aged 2 to 18 years, majority having poor preoperative oxygen saturation, a pre-surgical computational fluid dynamics customization is conducted. Both the traditional Fontan pathways and the proposed novel Y-graft templates are considered. Numerical model was validated against in vivo phase-contrast magnetic resonance imaging data and in vitro experiments. Results: the proposed template is selected and executed for 6 out of the 9 patients based on its predicted superior hemodynamic performance. Pre-surgical simulations performed for this cohort indicated that flow from the hepatic veins (HEP) do not reach to the desired lung. The novel Y-graft template, customized via a right- or left-sided displacement of the total cavopulmonary connection anastomosis location resulted a drastic increase in HEP flow to the desired lung. Orientation of HEP to azygos direct shunt is found to be important as it can alter the flow pattern from 38% in the caudally located direct shunt to 3% in the cranial configuration with significantly reversed flow. The postoperative measurements prove that oxygen saturation increased significantly (P-value = 0.00009) to normal levels in 1 year follow-up. Conclusions: the new Y-graft template, if customized for the individual patient, is a viable alternative to the traditional surgical pathways. This template addresses the competing hemodynamic design factors of low physiological venous pressure, high postoperative oxygen saturation, low energy loss and balanced hepatic growth factor distribution possibly assuring adequate lung development., European Union (EU); Horizon 2020; European Research Council (ERC); Proof of Concept Grant; BloodTurbine; ERC Starting Grant; Scientific and Technological Research Council of Turkey (TÜBİTAK); 1003 Priority-Research Program Grant