Role of caval venous manipulation in treatment of protein-losing enteropathy

Abstract

Protein-losing enteropathy has been associated with the Fontan operation, restrictive cardiomyopathy, constrictive pericarditis, tricuspid valvar stenosis and insufficiency, and superior caval venous obstruction. The mechanism of development of this complication of cardiac disease likely is multifactorial. We report here our experience with 3 patients with protein-losing enteropathy. Two had superior caval venous occlusion. The third patient had undergone an extracardiac Fontan operation, with stenosis of the extracardiac conduit placed to the right pulmonary artery. In all three cases, the complication was resolved by restoring unobstructed flow of blood in the superior or inferior caval vein. Since the aetiology of protein-losing enteropathy associated with cardiac disease is multifactorial, reduction of pressure in the tributaries of the superior caval vein may, in some cases, be curative. It is more difficult to cure the problem when it is associated with the Fontan operation.

Complications of central venous stenosis due to permanent central venous catheters in children on hemodialysis

“…Relief of the venous hypertension as performed here was found in previous reports to alleviate PLE [15]. 7.…”

Protein‐Losing Enteropathy and the Fontan Operation

“…Obstructions to systemic venous flow (from the vena cavae through the pulmonary arteries) are particularly problematic (because they elevate venous pressure) and must be eliminated, preferably by balloon angioplasty and intravascular stents. Relief of significant venous obstructions can improve PLE symptoms in up to 60% of patients 24 . Specific treatment strategies for the Fontan patient with PLE are summarized below and in Table 2.…”

Protein losing enteropathy secondary to a pulmonary artery stent

“…Previous studies have demonstrated that PLE is associated with a significantly worse outcome,[34] and every effort should be made to manage these patients aggressively, using a combination of optimal pharmacological therapy and surgical or catheter based interventions to relieve mechanical problems affecting the Fontan circuit. [25–7] In the patient presented here, we postulate a new mechanical problem as being causative for PLE, with intermittent sharp increases in intrathoracic pressure accounting for elevation of the systemic venous pressure, and concomitant intestinal protein loss.…”