Statistics from Altmetric.com
A boy with Ebstein's anomaly underwent uneventful, staged palliation to a fontan circulation. Fontan completion consisted of a 16 mm Gore-Tex extracardiac conduit between the inferior vena cava (IVC) and right pulmonary artery. A 6 mm polytetrafluoroethylene tube graft was anastamosed end-to-side between the extracardiac conduit and the right atrium to create a fenestration. Two surgical clips were placed in the mid-portion of the graft to reduce the fenestration lumen. Catheterisation 6 months postoperatively revealed spontaneous fenestration closure.
At routine follow-up at age 16 years, the patient presented with fatigue and exercise intolerance for 6–12 months. Physical examination was unremarkable and he completed 7 min of treadmill exercise testing. Echocardiography showed good function, trivial mitral regurgitation and an unobstructed outflow tract, with poor visualisation of the fontan circuit.
Catheterisation revealed a 9 mm Hg gradient across the extracardiac conduit with an IVC pressure of 17 mm Hg and pulmonary artery pressure of 8 mm Hg. An IVC angiogram revealed dense calcification causing subtotal obstruction at the mid-portion of the fontan conduit in online supplementary videos 1 a and b.
The patient underwent an uncomplicated fontan redo the following week. Intraoperative transoesophageal echocardiography and direct visualisation revealed an obstructive, calcified mass within the conduit with kinking at its mid-portion (figure 1). We postulate that this complication may be related to the tube graft fenestration, a finding not previously reported.1 It is plausible that, over time, thrombus extended from the tube graft into the conduit or that the altered geometry and flow dynamics in this region resulted in turbulence, causing thrombus formation and subsequent calcification.
Contributors All of the authors have contributed to the writing and editing of the text and selection, retrieval, and editing of the images seen in this report. Each of the authors approves of the content of this report and has approved of the final version submitted to Heart for peer review.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; internally peer reviewed.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.