Protein Losing Enteropathy in Post Fontan Patients

Protein losing enteropathy in post Fontan patients

Protein losing enteropathy is one of the troublesome long term complications of grossly elevated systemic venous pressures which can occur following a Fontan surgery. There is severe hypoproteinemia, refractory ascites, generalised edema and massive pleural effusion associated with this condition. The chance of developing protein losing enteropathy increases as time passes. The mechanism is a chronic protein loss from the digestive system induced by the chronically elevated venous pressure [1] in the inferior vena caval territory and the portal system. There is also secondary lymphangiectasia along with this.

Increased excretion of alpha 1 antitrypsin in stool is a marker of the condition, though the clinical features and hypoalbuminemia are the striking diagnostic findings. Angiotensin converting enzyme inhibitors and occasionally heparin [2] has been useful in the treatment, though sometimes revision of the Fontan circuit or a fenestration to produce an atrial septal defect to relieve the systemic venous congestion [3] may be needed.

References

  1. Davis CA, Driscoll DJ, Perrault J, Greenwood DH, Schaff HV, Puga FJ, Danielson GK, Feldt RH. Enteric protein loss after the Fontan operation. Mayo Clin Proc 1994;69:112-4.
  2. Kelly AM, Feldt RH, Driscoll DJ, Danielson GK. Use of heparin in the treatment of protein-losing enteropathy after Fontan operation for complex congenital heart disease. Mayo Clin Proc 1998;73:777-9.
  3. Mertens L, Dumoulin M, Gewillig M. Effect of percutaneous fenestration of the atrial septum on protein-losing enteropathy after the Fontan operation. Br Heart J 1994;72:591-2.