1/27. Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt.A 21-year-old white woman, born with a univentricular heart, had undergone staged procedures before Fontan correction. She then began to develop edema, protein-losing enteropathy, and ascites refractory to diuretic therapy. Cardiac angiography showed a patent right Blalock-Taussig shunt, with turbulent cavopulmonary circulation. After undergoing an unsuccessful attempt at coil embolization she then underwent shunt ligation, with resolution of symptoms and normalization of protein levels. This report draws attention to the importance of cavopulmonary laminar flow to prevent the development of protein-losing enteropathy.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
2/27. Protein-losing enteropathy in congestive heart failure: diagnosis by means of a simple method.Protein-losing enteropathy is frequently overlooked in patients suffering from congestive heart failure. Although textbooks often quote heart failure as one of the causes of protein-losing enteropathy, this association has not been clearly documented in the literature. Furthermore, conventional diagnostic techniques for protein-losing enteropathy are both cumbersome and time-consuming. We report the first case of protein-losing enteropathy of a 79 year-old lady who suffered from dilated cardiomyopathy. The profound hypoalbuminemia could not be explained by urinary loss or impaired hepatic synthesis. Protein-losing enteropathy was confirmed by means of technetium-99m- (99mTc) labelled human serum albumin scintigraphy which showed diffuse activity in the entire colon. The use of this simple and non-invasive diagnostic technique in protein-losing enteropathy is discussed.- - - - - - - - - - ranking = 6keywords = heart (Clic here for more details about this article) |
3/27. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure.Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
4/27. heparin therapy and reversal of protein-losing enteropathy in a case with congenital heart disease.We describe a patient with tricuspid hypoplasia who developed a protein-losing enteropathy. Having failed to respond to medical and surgical treatments, a heparin regimen was started with immediate decrease in enteric protein loss.- - - - - - - - - - ranking = 4keywords = heart (Clic here for more details about this article) |
5/27. Reversal of protein-losing enteropathy in a child with Fontan circulation is correlated with central venous pressure after heart transplantation.We report on the reversal of protein-losing enteropathy (PLE) after heart transplantation (HTx) in a 10-yr-old boy with Fontan circulation, previously treated unsuccessfully with heparin for several months. The protein loss continued immediately after the Tx. During the following month, however, a gradual decrease in protein loss was observed, which correlated with a decrease in the inferior vena cava (IVC) pressure. The patient is doing well with a normal serum albumin level and a normal IVC pressure, 2 yr after Tx.- - - - - - - - - - ranking = 5keywords = heart (Clic here for more details about this article) |
6/27. Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus.A 38-year-old woman visited our hospital with edema on her face and conjunctivae. The underlying disease was not clarified, and she did not visit the hospital afterwards. She suffered from diarrhea, polyarthralgia, Raynaud's phenomenon, malar rash and hair loss in the subsequent two years, and was hospitalized because of hypoproteinemia. Her urine, liver and heart test results did not account for her hypoproteinemia. She was diagnosed as having protein-losing enteropathy (PLE) associated with SLE based on the 99mtechnetium-labeled human serum albumin scintigraphy findings, clinical findings and laboratory results of antinuclear and anti-Sm antibodies. This case report demonstrates a strong association between PLE and SLE because PLE was aggravated along with the appearance of SLE symptoms and PLE subsided with prednisolone treatment along with improvement of SLE.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
7/27. Right ventricular cardiomyopathy accompanied by protein-losing enteropathy and chylous effusion.Severe right-side heart failure developed in a 47-year-old Japanese woman who suffered from hypoalbuminemia and a massive right side chylous pleural effusion. She had been diagnosed as having protein-losing enteropathy with right ventricular cardiomyopathy. autopsy showed congenital anomalies of the lymph ducts and abnormal deposition of fibrous and fatty tissue in the right ventricular myocardium. The clinical and pathological findings are consistent with the nonarrythmogenic form of the arrythmogenic right ventricular dysplasia.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
8/27. 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 = 6keywords = heart (Clic here for more details about this article) |
9/27. Plastic bronchitis in children with Fontan palliation: analogue to protein losing enteropathy?We studied a 6-year-old boy and a 2-year-old girl with bronchitis fibroplastica following Fontan operation. Large endobronchial casts of rubber-like consistency resulted in life-threatening pulmonary failure. In one patient symptoms improved after optimizing heart function with diuretics, and in the other a dramatic improvement with the resolution of the clinical symptoms and normalized serum albumin followed subcutaneous high-molecular-weight heparin treatment. The severe relapse after discontinuation of the heparin medication and the once more successful treatment with heparin suggest that in addition to optimizing heart function, high-molecular-weight heparin might be a therapeutic option for this poorly understood condition.- - - - - - - - - - ranking = 2keywords = heart (Clic here for more details about this article) |
10/27. Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation.Protein-losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4% to 13% of patients after the fontan procedure. We report a case of PLE reversal after heart transplantation in a 14-year-old boy with Fontan circulation who previously was treated unsuccessfully with medical therapy. The protein loss continued after heart transplantation. We administered total parenteral nutrition to rest the bowel. After 16 months, we observed a gradual decrease in protein loss. The patient is doing well 5 years after heart transplantation and had has a normal serum albumin level.- - - - - - - - - - ranking = 3keywords = heart (Clic here for more details about this article) |
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