Fenestration in Extracardiac Conduits in Children After Modified Fontan Operation by Implantation of Stent Grafts

Abstract

Three patients (ages 3, 5, and 8 years) with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extracardiac conduit as a final reconstructive procedure. Failure of the Fontan circulation occurred immediately after surgery because of spontaneous closure of surgical fenestrations in two children and absent fenestration in one. As an emergency procedure, in all patients the conduit was perforated by transcatheter intervention in order to create a connection to the anatomical right atrium. Following balloon dilatation of the perforated conduit, in all three patients covered stent grafts were placed in the newly created defect to attain a reliable communication. Patency of the fenestration was demonstrated by angiogram and any leakage was ruled out. Cardiac output improved and severe pleural effusion and ascites subsided. Right-to-left shunt could be demonstrated by echocardiography at follow-up after 7 months (median) in all three patients. Oxygen saturation remained stable between 85 and 90%. These preliminary results suggest that stent graft fenestration can serve as a valuable tool in failing Fontan circulation, particularly in patients with an extracardiac conduit. Covered stents have the potential to reduce the acute risk of bleeding and they help to prevent early spontaneous closure of the newly created fenestration.

Unusual method of creation of a transcatheter fenestration in an extracardiac conduit fontan circulation

“…[34] As surgical creation of fenestration in Fontan circuit is more morbid, it is often done in the catheterization laboratory using Brokenbrough needle puncture of the conduit followed by stent placement. [567] If the angulation of the conduit from femoral venous route is unfavorable, or if there is lack of femoral venous access as in venous occlusion or left isomerism, transhepatic route gives a more perpendicular access to the conduit for a needle puncture. [89] In our patient, puncture was difficult because of tough ingrowth within the Dacron conduit as seen on the tomographic scans.…”

A computational study of the Fontan circulation with fenestration or hepatic vein exclusion

“…Different types of fenestrations have been clinically used for the extracardiac Fontan [9,13,52]. We consider a tube fenestration emanating from the extracardiac conduit (the synthetic conduit extending from the inferior vena cava to the pulmonary artery), as described in Ref.…”

“…[6,7]), but there is still uncertainty in both the mechanisms through which these complications develop and the best path for treatment. Surgeons have had some success in managing complications by introducing a fenestration or hepatic vein exclusion into the Fontan physiology [8–13]. …”

Creation of a transcatheter fenestration in children with failure of fontan circulation: Focus on extracardiac conduit connection

“…Second, bar metal stents can be used to create a diabolo‐ or butterfly‐shaped configuration for a sufficient communication with a diameter of ∼4–6 mm and without inducing hemorrhagic complications. Previously, we considered graft stent's to avoid bleeding complicaions . Our experience suggests that this approach can replace the relatively complicated sizing of the fenestration, as reported before .…”

“…Percutaneous creation of a Fontan fenestration in total cavopulmonary connection with extra‐cardiac tunnel was described previously in a small series and in case reports . We present a retrospective data analysis from the largest cohort of patients treated by transcatheter fenestration in a Fontan circulation with extracardiac conduit.…”