1/4. Restrictive interatrial communication with protein-losing enteropathy and coagulopathy in hypoplastic left heart syndrome after Norwood palliation.The Norwood procedure is one option for neonates born with hypoplastic left heart syndrome. We describe a case of an infant with hypoplastic left heart syndrome, palliatively repaired with the Norwood procedure. The infant developed restriction of the interatrial communication, despite atrioseptectomy at the first stage of palliation. Consequently, a protein-losing enteropathy with severe coagulopathy developed which resolved after a repeat atrioseptectomy.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
2/4. Transcatheter fenestration of autologous pericardial extracardiac Fontan via the transhepatic approach.We describe a case of a patient with hypoplastic left heart syndrome (HLHS) who developed protein-losing enteropathy (PLE) following autologous pericardial extracardiac Fontan (APEF) operation with successful resolution of PLE following transcatheter fenestration via the transhepatic approach.- - - - - - - - - - ranking = 0.2keywords = enteropathy (Clic here for more details about this article) |
3/4. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan.A 9.5-year-old girl after fontan procedure for hypoplastic left heart syndrome had recurrent protein-losing enteropathy (PLE) develop 2 months after partial catheter closure of the Fontan fenestration. Despite satisfactory hemodynamic measurements under general anesthesia, we postulated that she suffered from increased pulmonary vascular reactivity and commenced her on Sildenafil treatment. After 6 weeks of oral Sildenafil treatment, her serum albumin and the fecal alpha-1-antitrypsin levels normalized, and her exercise tolerance had increased. There was also an improvement of the mesenteric arterial flow patterns on Doppler studies. Sildenafil should be considered in the treatment of PLE after the fontan procedure.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
4/4. Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration.A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |