Outcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience.

Abstract

Background: Previous reports have demonstrated the safety and efficacy of performing early Kawashima procedure (KP). Despite this, more recent studies have shown that the average age at the time of operation remains greater than one year of age. We report our experience with performing KP at an earlier age than previously reported.

Methods: A retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined.

Results: Initial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort.

Conclusions: The KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit.