Naslov
Long Term Heparin Treatment of Protein-Losing Enteropathy after Fontan Procedure,
(Long Term Heparin Treatment of Protein-Losing Enteropathy after Fontan Procedure)

Autori
Jelušić, Marija ; Malčić, Ivan ; Kniewald, Hrvoje ; Šarić, Dalibor ; Mustapić, Željka ; Rojnić-Putarek, Nataša ; Dasović-Buljević, Andrea

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
4WCPCCS Abstract book / - , 2005

Skup
4th world congress of pediatric cardiology and cardiac surgery

Mjesto i datum
Buenos Aires, Argentina, 18.09.2005. - 22.09.2005

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Protein losing enteropathy; Fontan procedure

Sažetak
Protein-losing enteropathy (PLE) is a serious complication after Fontan. It is hypothesized that elevated systemic venous pressures associated with the Fontan circulation cause the intestinal protein loss by increased pressures in the enteric lymphatic system as well as association of the infection and inflammation with the onset of PLE. A recent reports noted dramatic improvement after subcutaneous administration of heparin. We report a 13-year old girl with heterotaxia syndrome (dextrocardia, pulmonary valve atresia, single ventricle of left ventricular type and haemyazigos continuity) who developed PLE four months after a TCPC and Kawashima procedure. After surgical enlargement of mild pulmonary artery stenosis performing RA-LA fenestration and low fat diet she showed improvement but few months later PLE developed again. Two years after a Fontan procedure, we started with high molecular weight heparin (HMWH) in dose of 8 000 i.j/sqm increasing the dose until significant improvement was achieved. A month later she was completely without any symptoms of PLE and with increased values of total protein, serum albumin, calcium and lymphocytes. After 8 years continuous treatment with HMWH the patient is generally in good condition, her values of total protein increased to 55 g/l, and calcium concentration is 2.22 mmol/l. In several occasions we tried to reduce HMWH dose but all symptoms of PLE developed soon after reduction. She is now treated with 15 000 i.j./m2 of HMWH, digitalis, diuretics, calcium, vitamins A, D, E, on low fat diet with MCT oils and is under control of osteopenia by densitometry

Izvorni jezik
Engleski

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