Percutaneous interventions in Fontan circulation

Abstract

Introduction and objectives: Different percutaneous interventional procedures are needed to reach and maintain adequate anatomical and physiological conditions for the Fontan circulation. We aim to describe the experience gained at a children's hospital in such interventions, and to analyze the clinical outcomes.

Methods: Retrospective study of all patients with Fontan circulation completed between 1995 and 2013. We analyzed the clinical characteristics and the different types of percutaneous interventions performed, considering three different periods of time: before Glenn surgery, between Glenn and Fontan surgeries, and after Fontan was completed. Survival and time to indication of percutaneous interventions in each period were analyzed, as well as the clinical situation at last follow-up.

Results: Of the 91 patients analyzed, 46 (50.5%) required percutaneous interventions. The most frequent procedures were pulmonary artery angioplasty and angioplasty of the Fontan conduit. Estimated survival at 10, 20 and 30 years of age was 96.2%, 94.7% and 89.4%, respectively. There were no significant differences in survival of patients undergoing percutaneous interventions or not. Overall survival and time to indication of percutaneous interventions were significantly lower in the group of patients with right morphology systemic ventricle. Patients with fenestrated Fontan required interventions more frequently. At the end of follow-up, 66 patients (72.5%) were asymptomatic, without significant differences between patients who underwent or did not undergo percutaneous interventions.

Conclusions: Interventional catheterization procedures are often necessary to reach and maintain the fragile Fontan circulation, mainly in patients with right morphology systemic ventricles and fenestrated Fontan conduits.

Keywords: CHD, congenital heart disease; Cardiac catheterization; Congenital heart disease; Fontan procedure; IVC, inferior vena cava; NYHA, New York Heart Association; PI, percutaneous intervention; Percutaneous intervention; SVC, superior vena cava; TCD, total cavopulmonary derivation.

Fig. 1

Temporal distribution Fontan operations (left) and interventional catheterizations (right) performed in the cohort of patients. For each year, the number of interventional catheterizations performed pre-Glenn surgery, between Glenn and Fontan surgeries, and post-Fontan surgery, is exposed. TCD: Total cavopulmonary derivation.

Fig. 2

Overall survival and survival after Fontan operation among patients undergoing or not undergoing PI.

Fig. 3

Overall survival and survival after Fontan operation in patients with right or left systemic ventricle.

Fig. 4

Time to first percutaneous intervention in patients with right or left systemic ventricle.

Fig. 5

Patients undergoing percutaneous interventions. Time to first percutaneous intervention along three different time periods: before Glenn surgery (pre-Glenn), between Glenn and Fontan surgeries, and after Fontan surgery (post-Fontan).

Fig. 6

Fenestration closure device in a patient who had undergone previous angioplasty in the left pulmonary artery with stent (solid arrow). A: Fontan conduit in initial angiography, where contrast flow through the fenestration (asterisk) to right atrium was observed. B: An Atrial Septal Occluder device (Amplatzer™) (dotted arrow) was implanted for fenestration closure, showing no residual shunt in control angiography.

Fig. 7

Fontan conduit angioplasty and fenestration closure. A: Fontan conduit stenosis (solid arrow) and permeable fenestration (dotted arrow) were observed in baseline angiography; previous embolization of the left superior vena cava had been done with a Duct Occluder device (Amplatzer™) and a Vascular Plug II device (Amplatzer™) (asterisk). B: Two open-cell stents were initially implanted in the Fontan conduit (solid arrow), with a mild luminal widening (but sufficient to eliminate the 4 mm Hg gradient recorded), and persistence of contrast flow through the fenestration (dotted arrow). C: Finally, a covered in-stent stent was implanted for fenestration sealing (key), without observing residual shunt in control angiogram.