Background: Early recognition of occlusion of any segment of a cavopulmonary connection (CPC) is mandatory, and efforts at immediate recanalization should be undertaken to optimise Fontan physiology
Patients and methods: Six consecutive patients (age range 3 months to 5 years) with clinical suspicion of occlusion of a CPC segment are presented. All had undergone a Norwood type of palliation, followed by a bidirectional Glenn shunt at between 3 and 5 months. The oldest patient had undegone Fontan completion using an extracardiac conduit. The interval from surgery to clinical presentation ranged between 1 day and 3 months. At emergency cardiac catheterization, total thrombotic occlusion was seen in the following segments: left pulmonary artery (n=5), superior vena cava (n=3), inferior vena cava (within the extracardiac conduit, n=1). All occluded segments could be crossed with a 4F endhole catheter and guidewire combination. Serial balloon dilation and stent implantation (n=11 stents, dilated to between 6mm and 15mm, depending on the size of the native vessel) were successfully undertaken.
Results: All occluded segments were reopened completely. Two patients died during further follow-up (2 to 10 days later), both due to multiple recurrent thrombotic obstructions, despite subsequent tPA therapy (and redilation/re-recanalization in one). The remaining patients are alive and well, and 2 of the survivors have had Fontan completion.
Conclusions: Early and aggresssive therapy can result in salvage of a significant proportion of acutely occluded CPC segments