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 (SpO(2)) 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 SpO(2) (%) 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 SpO(2) after completion Fontan following KP was 88.7 ± 7.6%. The SpO(2) in Fontan after KP was not as high as after control FP. Low SpO(2) 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 SpO(2) relative to standard Fontan patients, but in anticipation that SpO(2) is likely to rise gradually with time.