Treatment of Children with Protein - Losing Enteropathy After Fontan and Other Complex Congenital Heart Disease Procedures in Condition with Limited Human and Technical Resources

Bejiqi, Ramush; Retkoceri, Ragip; Zeka, Naim; Bejiqi, Hana; Vuqiterna, Armend; Maloku, Arlinda

doi:10.5455/msm.2014.26.39-42

Cited by 8 publications

(5 citation statements)

References 9 publications

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“…The symptoms, signs, and laboratory abnormalities of PLE can be ameliorated by pharmacological, cardiaccatheter interventional techniques, and surgery including heart transplantation [1,8]. Nonetheless, the liver abnormalities induced by Fontan physiology may persist.…”

Section: Discussionmentioning

confidence: 98%

Fontan Outcomes and Pulmonary Blood Flow at Birth

et al. 2015

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We previously noted, in a small group of post-Fontan patients, a possible association between hepatic fibrosis scores and the status of pulmonary blood flow at birth. To further explore this observation, we examined data from all Fontan patients seen in our center from July 2010 to March 2015. We identified 200 patients for analysis. Of the 200 patients, 56 underwent transvenous-hepatic biopsy. Of the 200 patients, 13 (6.5%) had protein-losing enteropathy. We divided both the 56 biopsy patients and the entire cohort of 200 patients into 4 groups: (1) unobstructed pulmonary blood flow at birth with functional left ventricles, (2) unobstructed pulmonary blood flow at birth with functional right ventricles, (3) obstructed pulmonary blood flow at birth with functional left ventricles, and (4) obstructed pulmonary blood flow at birth with functional right ventricles. Analysis of the 56 liver-biopsy patient groups showed median hepatic total-fibrosis scores for the 4 groups of 2 (0-6), 2 (0-8), 3 (2-6), and 4 (1-8), respectively, with statistical significance between groups 4 and 1 (p = 0.031). For the entire cohort of 200 patients, we analyzed the incidence of protein-losing enteropathy for each of the four groups and found protein-losing enteropathy percent occurrences of 0, 2.9, 8.8, and 16.1, respectively, with statistical significance between groups 4 and 2 (p = 0.031) and between groups 4 and 1 (p = 0.025). A history of obstructed pulmonary blood flow at birth, coupled with a functional right ventricle, may predict a poorer long-term Fontan outcome.

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Section: Discussionmentioning

confidence: 98%

Fontan Outcomes and Pulmonary Blood Flow at Birth

et al. 2015

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“…9 Longterm response to this type of diet therapy was registered in 11 of the 18 patients. 10 Obstructive lesions, if any, in the Fontan circuit must be evaluated for and, if present, relieved by means of transcatheter or surgical therapy. Similarly, aortopulmonary connections, naturally-occurring or prior surgical shunts, should be sought out and closed by either transcatheter methodology (coils, devices, etc.)…”

Section: Discussionmentioning

confidence: 99%

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

2019

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Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

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“…Trace element abnormalities and early identification of immune-compromised states can aid in reducing morbidity in children after cardiac surgery. Use of feeding protocols and a home surveillance system for hypoplastic left heart syndrome improves outcomes of those children [ 4 , 9 ].…”

Section: Discussionmentioning

confidence: 99%

Kosovo’s Experience for Children with Feeding Difficulties after Cardiac Surgery for Congenital Heart Defect

Retkoceri¹,

Bejiqi²,

Maloku³

et al. 2017

Open Access Maced J Med Sci

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BACKGROUND:A feeding disorder in infancy and during childhood is a complex condition involving different symptoms such as food refusal and faddiest, both leading to a decreased food intake.AIM:We aimed to assess the prevalence and predictor factors of feeding difficulties in children who underwent cardiac open heart surgery in neonatal period and infancy. We address selected nutritional and caloric requirements for children after cardiac surgery and explore nutritional interdependence with other system functions.METHODS:This was a retrospective study in a tertiary referral hospital, and prior approval from the institutional ethics committee was obtained. Information for 78 children (42 male and 36 female) was taken from patients charts. Data were analysed with descriptive statistics and logistic regression.RESULTS:From a cohort of analysed children with feeding problems we have occurred in 23% of such cases. At the time of the study, refusal to eat or poor appetite was reported as a significant problem in 19 children and subnormal height and weight were recorded in 11 children. Early neonatal intervention and reoperation were identified as risk factors for latter feeding difficulties or inadequate intake. Children with feeding problems also tended to eat less than children without feeding problems. There was a trend towards more feeding problems in patients with chromosomal abnormalities or other associated anomalies.CONCLUSION:Feeding disorder is often and a frequent long-term sequel in children after neonatal or early infancy heart surgery. Patients with chromosomal and associated anomalies who underwent multiple cardiac surgeries are at risk of developing feeding difficulties.

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Treatment of Children with Protein - Losing Enteropathy After Fontan and Other Complex Congenital Heart Disease Procedures in Condition with Limited Human and Technical Resources

Cited by 8 publications

References 9 publications

Fontan Outcomes and Pulmonary Blood Flow at Birth

Fontan Outcomes and Pulmonary Blood Flow at Birth

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

Kosovo’s Experience for Children with Feeding Difficulties after Cardiac Surgery for Congenital Heart Defect

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