Abstract

Background: Ventricle-pulmonary artery connections in patients after the Fontan procedure lead to ineffective volume loading and can cause long term problems. In patients with a cavopulmonary shunt anterograde pulmonary blood flow is frequently maintained, but can cause significant volume loading of the heart or complicate the subsequent Fontan procedure.

Objective: To evaluate the use of transcatheter closure of a ventricle-pulmonary artery communication in the setting of a cavopulmonary shunt or after the Fontan procedure.

Patients and methods: Retrospective study at a tertiary referral centre. Eight patients (age 1.5–18 years, mean 7.8 years). Indications: cardiac failure or persistent pleural effusions after cavopulmonary shunt (n  =  2) or after Fontan (n  =  3) and abolishing the volume load of the single ventricle prior to Fontan completion (n  =  3).

Results: Devices used: Rashkind Umbrella (n  =  1), Amplatzer PDA (n  =  7) and Amplatzer ASD (n  =  1). One patient required two devices. There were no procedural complications. All 3 patients with prolonged pleural effusions (1 post CP shunt and 2 post Fontan) showed complete resolution between 4 and 10 days after catheter closure. Two patients underwent transcatheter occlusion for progressive ventricular dilatation and cardiac failure. The first patient was post Fontan and showed gradual improvement in ventricular function. The second patient (post CP shunt) was in end stage cardiac failure due to severe AV valve regurgitation. The patient died 48 hours after an uncomplicated procedure due to ventricular failure and electromechanical dissociation (non-procedure-related cardiac death). Three patients underwent catheter closure to off-load the systemic ventricle prior to the Fontan procedure. The device had to be removed prior to release in one patient, due to unsatisfactory position.

Conclusions: Transcatheter closure of ventricle-pulmonary artery communication is a safe and effective technique in the treatment of selected patients after cavopulmonary shunt or Fontan procedure with early or late complications due to inappropriate pulmonary blood flow. This intervention should also be considered in the preparation for the Fontan procedure in selected patients with ventricular overload.