Protein losing enteropathy after Fontan operation

This study examines the possible causes of protein losing enteropathy (PLE) as a late complication after the Fontan operation (FO). Between July 1974 and July 1994, 175 patients (100 male and 75 female) underwent the Fontan operation (Group 1, G1) in our institution aged between 4 mo-27.9 y (mean age 8.6 y). Twenty-one patients (12%) who developed PLE were selected from the total group within 1-127 months after the Fontan operation (median 10 mo) and they were resignated as group 1.1 (G 1.1). Seven children had tricuspid atresia (6 with normal and 1 with transposed connected great arteries), ten had single ventricle (6 LV type, 2RV type and 2 undetermined type of ventricle) and four had complex heart malformations. Group 1.2 (G1.2) as a control group, consisted of 20 patients without PLE, of the same sex and with the similar type of congenital heart disease and type of operation (p>>0.05). Eight had tricuspid atresia (6 with normal and 2 with transposed connected great arteries), 6 had single ventricle (5 LV and 1 RV type of ventricle) and 6 had complex heart malformations. Nine G1.1. patients and 5 G1.2 patients had pulmonary artery banding (PAB) before FO. The most important criterion in G1.1. was low serum protein (2-5.6 g/dl, mean 4.2). Beside low serum protein 4.2 g/dl, relevant findings were edema (95%), pleural effusions (93%), ascites (86%), chronic heart failure (66%), ECHO-ejection fraction 55% (90%), AV-valve insufficiency (40%), hypocalcemia (90%). Alfa1<^!>-AT clearance in stool and lymphocytopenia were not assessed. With the aim of estimating the risk factors for the onset of PLE, the different clinical and hemodynamic parameters in the whole group (G1) and the both of the subgroups, with PLE (G1.1.) and without PLE (G1.2.) were tested: the anatomy of congenital heart disease, the type of Fontan modification, the age at the time of the operation and mortality rate, as well as the condition of right atrial pressure, pulmonary vascular resistance, pulmonary artery pressure and ventricular end-diastolic pressure after FO. The size and pattern of blood appearance in the right atrium was assessed by angiocardiograph. When comparing the clinical and hemodynamic data in G1., G1.1. and G1.2. significant differences emerged: the development of PLE in the patients with single ventricle (10/21 vs. 52/175; p << 0.05) as compared to the others, as well as in those with complex FO (RA-PA+Damus-Stansel-Kaye) (4/21 vs. 6/175; p << 0.05); high mortality rate in G1.1., especially in late mortality (9/21 vs. 45/175 or 8/9 vs. 21/45, respectively; p << 0.01). Furthermore, there was significant correlation with PLE onset (G1.1. vs. G1.2): the interval between PAB and FO (mean 8.9 vs. 5.6 y; p << 0.05), significant difference by age at FO (mean 9.4 vs. 6.8; p << 0.05), significant by higher (p << 0.05) LVEDP, mean PAP and RAP after FO (7.5 ± 3.5 vs. 2.8 ± 1.5 mmHg; 15.7 ± 8.2 vs. 11.3 ± 4.9.5 mmHg; 14.9.3 ± 6.1 vs. 13.4 ± 5.5 mmHg). @SAZETAK = Summary: The risk for postoperative PLE is significantly related to the complexity of the heart malformation, older age at Fontan operation (>> 9y), long standing previous pulmonary artery banding, high postoperative ventricular end-diastolic pressure and turbulent flow in the postoperatively dilated right atrium.
Category: Original scientific paper
Volume: Vol. 42, No 2, april - june 1998
Authors: M. Biočić, M. Saraga, D. Budimir, J. Todorić, D. Furlan, I. Jurić, T. Šušnjar, D. Todorić, J. Meštrović
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