Venovenous malformation: a common finding after Kawashima operation

Abstract

Our findings suggest that systemic VVM can occur frequently after Kawashima operation and can produce significant desaturation postoperatively, and hence we support hepatic incorporation. Performing detailed angiographic studies of the supra- and infra-diaphragmatic systemic veins in routine assessment of patients before Kawashima operation is, probably, warranted.

Staged Kawashima operation with cavopulmonary connection

“…4 She did not have venovenous malformations (between the hemiazygos and hepatic veins) because the hemiazygos system was not pressurized as in a high-risk Kawashima procedure, in which it would be draining to the pulmonary circulation. 5 At the time of the secondstage procedure, the hemiazygos vein could be connected to the left pulmonary artery without much technical difficulty.…”

Progressive cyanosis following Kawashima operation: slow resolution after redirection of hepatic veins

“…However, the prevalence following total cavopulmonary connection (TCPC) has been described as relatively low [12,13], except for patients with left atrial isomerism in whom the hepatic venous flow is not incorporated directly in the TCPC. In these patients pulmonary arteriovenous malformations develops in 21-75 percent [3,7,9,14]. The large range in patients developing pulmonary arteriovenous malformations may be a combination of lack of early awareness of the problem, a wide range in the clinical presentation, additional venous-venous collaterals and a time factor, since these malformations take several years to develop.…”

Magnetic Resonance Angiography in Paced Complex Heterotaxy Syndrome with Fontan Conduit Obstruction and Venovenous Collateral Decompression

“…Cardiac ultrasound has limitations for evaluation of extracardiac vascular anatomy in this patient population. A recently published study of patients with complicated complex extra cardiac shunts (Kawashima) suggested that detailed angiography is warranted before further surgical review 1 . Catheter angiography is, however, invasive and similar to computed tomography, exposes the child to ionizing radiation and iodinated contrast with limited functional information.…”