Haemodynamic consequences following closure of an Abernethy malformation in a patient following a total cavopulmonary shunt

Abstract

A 17-year-old girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone Kawashima operation 10 years back. She had significant desaturation because of a large Abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein. It was closed with a vascular plug, with improvement in oxygen saturation. She developed extensive inferior caval vein thrombus following the procedure, which was managed conservatively by anti-coagulation.

Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation

“…Whether Abernethy malformation patients should use anticoagulants after surgery is still controversial, and most patients do not use anticoagulants postoperatively. 9,13 We did not prescribe anticoagulants following surgical ligation closure of this Abernethy malformation. One month after the operation, a thrombus at the site of portal vein ligation was observed.…”

“…Sasikumar et al found an Abernethy malformation patient developed a significant inferior cava vein and portal vein thrombus one week postoperatively that showed good resolution at two months' follow-up, after the patient received anticoagulation therapy. 13 In addition, studies have shown that oral rivaroxaban can effectively treat portal vein thrombosis. 14 Since the coagulation function of this patient was normal, he was also managed conservatively with anticoagulants (heparin infusion) and started on a single oral anticoagulant drug (rivaroxaban).…”

Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome

“…The implications of congenital extrahepatic portosystemic shunt for the development of pulmonary arteriovenous malformations in children with polysplenia syndrome and univentricular heart still remain to be seen. After literature search, nine cases of congenital extrahepatic portosystemic shunt associated with interrupted inferior vena cava and univentricular heart were identified (six cases reported by McElhinney et al ,4 one case each by Sasikumar et al ,14 Nakamura et al 15 and Ikeda et al 16). In all these cases, the extrahepatic portosystemic shunt was recognised after Kawashima operation.…”