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Perioperative risk factors for development of protein-losing enteropathy following a Fontan procedure

2001; Elsevier BV; Volume: 88; Issue: 10 Linguagem: Inglês

10.1016/s0002-9149(01)02066-5

1879-1913

Andrew J. Powell, Kimberlee Gauvreau, Kathy J. Jenkins, Elizabeth D. Blume, John E. Mayer, James E. Lock,

Cardiac Valve Diseases and Treatments

Protein-losing enteropathy (PLE) is a devastating complication after a Fontan procedure that often responds poorly to treatment. Its features include hypoalbuminemia, ascites, pleural effusions, diarrhea, malaise, and lymphocytopenia. The reported prevalence among patients who have undergone the Fontan procedure ranges from 2.5% to 11% 1 Gentles T.L. Gauvreau K. Mayer Jr, J.E. Fishberger S.B. Burnett J. Colan S.D. Newburger J.W. Wernovsky G. Functional outcome after the Fontan operation factors influencing late morbidity. J Thorac Cardiovasc Surg. 1997; 114: 392-403 Abstract Full Text Full Text PDF PubMed Scopus (186) Google Scholar , 2 Feldt R.H. Driscoll D.J. Offord K.P. Cha R.H. Perrault J. Schaff H.V. Puga F.J. Danielson G.K. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996; 112: 672-680 Abstract Full Text Full Text PDF PubMed Scopus (242) Google Scholar , 3 Mertens L. Hagler D.J. Sauer U. Somerville J. Gewillig M. Protein-losing enteropathy after the Fontan operation an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998; 115: 1063-1073 Abstract Full Text Full Text PDF PubMed Scopus (403) Google Scholar , 4 Kaulitz R. Luhmer I. Bergmann F. Rodeck B. Hausdorf G. Sequelae after modified Fontan operation postoperative haemodynamic data and organ function. Heart. 1997; 78: 154-159 PubMed Google Scholar with a 5-year postonset survival rate of 46% to 59%. 2 Feldt R.H. Driscoll D.J. Offord K.P. Cha R.H. Perrault J. Schaff H.V. Puga F.J. Danielson G.K. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996; 112: 672-680 Abstract Full Text Full Text PDF PubMed Scopus (242) Google Scholar , 3 Mertens L. Hagler D.J. Sauer U. Somerville J. Gewillig M. Protein-losing enteropathy after the Fontan operation an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998; 115: 1063-1073 Abstract Full Text Full Text PDF PubMed Scopus (403) Google Scholar Until recently, many clinicians have assumed that PLE results from chronically elevated systemic venous pressure that promotes the development of lymphangiectasis and protein loss into the gastrointestinal tract. However, Goff et al, 5 Goff D.A. Blume E.D. Gauvreau K. Mayer J.E. Lock J.E. Jenkins K.J. Clinical outcomes of fenestrated Fontan patients after closure the first ten years. Circulation. 2000; 102: 2094-2099 Crossref PubMed Scopus (106) Google Scholar from our institution, recently reported a surprisingly low prevalence of PLE (1 in 154, 0.6%) at follow-up (median 3.4 years) after Fontan fenestration closure, a procedure that produces a sustained increase in systemic venous pressure. 6 Bridges N.D. Lock J.E. Mayer Jr, J.E. Burnett J. Castaneda A.R. Cardiac catheterization and test occlusion of the interatrial communication after the fenestrated Fontan operation. J Am Coll Cardiol. 1995; 25: 1712-1717 Abstract Full Text PDF PubMed Scopus (64) Google Scholar This observation prompted us to explore the alternative hypothesis that PLE might be related to perioperative injury. Accordingly, by analyzing database information on the first 500 patients undergoing a Fontan procedure at our institution, we sought to identify perioperative risk factors for the development of PLE.