Pseudo-Fontan Physiology From Venous Lead Obstruction: A Rare Case of Acquired Right Atrial Obstruction

Venous obstruction involving the axillary, subclavian, and brachiocephalic veins, and less commonly the superior vena cava, is a significant complication of transvenous pacemaker leads. Lead-related venous obstruction may be asymptomatic but can result in significant morbidity in individuals who develop superior vena cava syndrome.

We present the first reported case of a 54-year-old man with lead-related venous obstruction at the level of the right atrium, manifesting as pseudo-Fontan physiology, including hepatic fibrosis, lymphatic leakage, and protein-losing enteropathy. Ultimately, the patient underwent surgical extraction of the retained pacemaker leads and reconstruction of the involved portion of the right atrium and tricuspid valve, leading to the resolution of his symptoms.

This case underscores the importance of cardiac device selection and understanding potential lead-related complications including venous obstructions.

Lead-related venous obstructions at the level of the right atrium can lead to chronically elevated bicaval pressures, resulting in late complications often observed in individuals with prior Fontan procedures.