ISSN : 1301-5680
e-ISSN : 2149-8156
TURKISH JOURNAL OF
THORACIC AND
CARDIOVASCULAR SURGERY
Turkish Journal of Thoracic and Cardiovascular Surgery     
Outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants
Ersin Erek1, Selim Aydın1, Bahar Temur1, Mehmet Akif Önalan1, Dilek Suzan2, Müzeyyen İyigün3, İbrahim Halil Demir4, Ender Ödemiş5
1Department of Cardiovascular Surgery, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Istanbul, Turkey
2Department of Cardiovascular Surgery, Marie Lannelongue Hospital, Paris, France
3Department of Anesthesiology and Reanimation, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Istanbul, Turkey
4Department of Pediatric Cardiac Surgery, Medicana International Hastanesi, Istanbul, Turkey
5Department of Pediatric Cardiology, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Istanbul, Turkey
DOI : 10.5606/tgkdc.dergisi.2020.18605
Background: In this study, we present the outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants.

Methods: In this study, a total of 97 pediatric patients who were operated due to hypoplastic left heart syndrome and its variants between March 2011 and October 2018 were retrospectively analyzed. Thirty-two of the patients (28 males, 4 females; median age 5 days; range, 1 to 25 days) underwent Norwood Stage I operation (Group N), while the remaining 65 patients (44 males, 21 females; median age 6 days; range, 1 to 55 days) underwent a hybrid procedure (Group H). Both treatment strategies were compared.

Results: The median body weight in Group H was significantly lower and the number of patients with a low birth weight (<2,500 g) was significantly higher than Group N (p=0.002 and 0.004, respectively). The postoperative early mortality rate was similar between the groups. Univariate and multivariate analyses revealed that the need for preoperative mechanical ventilation was a significant factor for mortality (p=0.004 and 0.003, respectively). Syndromic appearance was also a significant factor the multivariate analysis (p=0.03). There was a statistically significant difference between the groups in terms of the inter-stage mortality rates (p=0.0045). Second-stage procedure was performed in 32 patients. The early mortality rate after the Glenn operation was 7.6%. Six patients died after comprehensive Stage II operation. Five patients underwent biventricular repair and 8 patients had third-stage fenestrated extracardiac Fontan operation (Group N, n=7 and Group H, n=1). The Kaplan-Meier survival curve demonstrated that Group N had a higher survival rate at both one and five years than Group H, although the difference was not statistically significant (p=0.15). Subgroup analysis showed that the Norwood procedure with Sano modification had the highest survival rate with 40% at five years.

Conclusion: Our study results show that patients undergoing the Norwood procedure have a more uneventful course of inter-stage period and Stage II and III, despite drawbacks early after Stage I procedure. Based on our experiences, we recommend performing the hybrid intervention in patients with a poor clinical condition and a body weight of <2,500 g.

Keywords : Congenital heart anomalies, hypoplastic left heart syndrome, neonate, Norwood operation, palliative surgery
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