Medium-term outcomes of Kawashima and completion Fontan palliation in single-ventricle heart disease with heterotaxy and interrupted inferior vena Cava

Background We investigated medium-term outcomes in single-ventricle heart disease palliated with a Kawashima procedure (KP) compared with matched patients who had a standard Fontan procedure (FP). Methods Clinical characteristics, oxygen saturation as measured by pulse oximetry (Spo₂) trends, and survival in 18 KP cases were compared with 36 matched controls who underwent a standard FP. Records of surgical procedures, catheterizations, noninvasive imaging, and clinical follow-up were reviewed. Results The median duration of follow-up after KP was 6.7 years. The mean Spo₂ (%) in the KP group over the follow-up period before completion Fontan was 85.1 ± 6, and differed significantly from the control FP group (90.7 ± 5.1, p < 0.001). Thirteen of the 18 KP patients underwent completion Fontan at a median age of 3.3 years (1.1 to 8.6) and median interval of 2.61 years (0.61 to 7.35) after KP. Mean Spo₂ after completion Fontan following KP was 88.7 ± 7.6%. The Spo₂ in Fontan after KP was not as high as after control FP. Low Spo₂ with upward trend was noted throughout the postoperative follow-up. The overall survival in the KP group after Fontan estimated by the Kaplan-Meier method was 87% at 5 years and at 7 years. Conclusions The KP and subsequent Fontan completion results in acceptable medium-term survival, and remains the approach of choice for single-ventricle heart disease with interrupted inferior vena cava. The KP has to be undertaken with expectation for somewhat lower early Spo₂ relative to standard Fontan patients, but in anticipation that Spo₂ is likely to rise gradually with time.