Successful Palliation via Kawashima Procedure of an Infant With Heterotaxy Syndrome and Left-Atrial Isomerism.

Hannah Lively-Endicott,Diego A Lara

doi:10.31486/toj.18.0042

Abstract

Heterotaxy is a condition of abnormal lateralization of organs across the body's left-right axis, causing multiple congenital malformations. The anatomic manifestations of heterotaxy syndrome generally follow one of two patterns, referred to as right atrial isomerism (with two similar right atria and duplication of right-sided features of multiple organs) and left atrial isomerism (with two similar left atria and duplication of left-sided features of multiple organs). Cardiac surgical intervention for patients with heterotaxy syndrome depends on ventricular physiology and circulatory balance. For patients with single-ventricle physiology, a Fontan operation, which directs systemic venous return to the pulmonary arteries, is the definitive intervention. Prior to a Fontan operation, many patients require one or more palliative surgeries (eg, a Blalock-Taussig-Thomas shunt or bidirectional Glenn/Kawashima procedure) to prepare them for definitive correction. We present the case of a term female neonate who was transferred to our pediatric cardiovascular intensive care unit for management of suspected congenital cardiac disease. Echocardiography confirmed the diagnosis of heterotaxy syndrome with left atrial isomerism, an interrupted inferior vena cava with azygos continuation, and a hypoplastic left ventricle with single-ventricle physiology. At 11 months of age, she underwent a Kawashima procedure with subtotal pulmonary artery ligation. She tolerated the procedure well and is anticipated to remain stable for the near future, possibly without the need for further cardiac surgery. Patients with heterotaxy syndrome have congenital malformations in several organ systems, requiring lifelong coordination of care among health providers across multiple disciplines.

Highlights

Heterotaxy syndrome is a condition of abnormal lateralization of organs across the body’s left-right axis.[1,2] Establishment of asymmetric tissue differentiation is a unique feature of vertebrate embryogenesis and occurs early in embryonic development.[2,3] When asymmetric tissue differentiation fails, organ tissue usually specific to one-half of the body can be duplicated, while tissue from the other half can fail to develop
Multiple genes believed to play a role in establishment of the left-right axis during embryonic development have been identified as possible contributors to the pathogenesis: ZIC3, NODAL, CFC1, ACVR2B, LEFTY2, CITED2, and GDF1.3 Growth factors responsible for establishing asymmetric left-sided tissue differentiation in mouse embryos have been shown to be interrupted in high-glucose media.[6]
We present the case of a term neonate who had heterotaxy syndrome with left atrial isomerism, an interrupted inferior vena cava with azygos continuation, and mild pulmonary stenosis who was successfully palliated with a Kawashima procedure with subtotal pulmonary artery ligation at 11 months of age

Summary

Background

Heterotaxy is a condition of abnormal lateralization of organs across the body’s left-right axis, causing multiple congenital malformations. Cardiac surgical intervention for patients with heterotaxy syndrome depends on ventricular physiology and circulatory balance. For patients with single-ventricle physiology, a Fontan operation, which directs systemic venous return to the pulmonary arteries, is the definitive intervention. Prior to a Fontan operation, many patients require one or more palliative surgeries (eg, a BlalockTaussig-Thomas shunt or bidirectional Glenn/Kawashima procedure) to prepare them for definitive correction. At 11 months of age, she underwent a Kawashima procedure with subtotal pulmonary artery ligation. She tolerated the procedure well and is anticipated to remain stable for the near future, possibly without the need for further cardiac surgery. Conclusion: Patients with heterotaxy syndrome have congenital malformations in several organ systems, requiring lifelong coordination of care among health providers across multiple disciplines

INTRODUCTION

Findings

DISCUSSION

CONCLUSION