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Introduction: Protein-losing enteropathy manifests as a loss of serum proteins through the gastrointestinal tract, resulting in hypoproteinemia, extravascular fluid retention, and edema. Management consists of nutritional maintenance in conjunction with interventions targeted at treating the underlying etiology., Materials and Methods: This report describes a patient with protein-losing enteropathy from a central conducting lymphatic obstruction who was treated with percutaneous extra-anatomic lymphovenous bypass creation., Results: A modified gun-sight technique was used to create a lymphovenous bypass between an occluded terminal thoracic duct and the left internal jugular vein., Conclusion: A percutaneous technique to reconstruct the terminal thoracic duct via lymphovenous bypass creation was feasible.

Introduction: Protein-losing enteropathy and spontaneous isolated superior mesenteric artery dissection are both rare clinically. Protein-losing enteropathy due to superior mesenteric artery dissection is extremely rare., Patient Concerns: A 46-year-old male with acute abdominal pain and hematochezia was diagnosed with a complete occlusion of the superior mesenteric artery because of dissection. He suffered from diarrhea and hypoproteinemia after an emergency thromboendarterectomy., Diagnoses: Based on laboratory tests and capsule endoscopy inspection, a diagnosis of protein-losing enteropathy was made., Interventions: Endovascular treatment was provided., Outcomes: After stent placement, he quickly recovered without a recurrence of symptoms., Conclusion: Protein-losing enteropathy is a serious complication of an isolated superior mesenteric artery dissection. Restoring the patency of the superior mesenteric artery is keyed for the treatment of this complication.

Mechanical circulatory support in failing Fontan patients with Fontan circuit failure remains challenging. Herein, we describe a failing fenestrated Fontan patient who underwent systemic ventricular assist device support leaving the fenestration open. Perioperative course and hemodynamics during mechanical support are described in detail.

Secondary protein-losing enteropathy (PLE) is a rare complication following pediatric liver transplantation (LT), mostly related to venous outflow obstruction of the liver. Here, we discuss a thus far unknown cause of secondary PLE following pediatric LT. A 7-month-old boy underwent LT with biliary anastomosis using a Roux-en-Y jejunal loop. Eleven months later he developed PLE. Routine diagnostic workup was negative. No hepatic outflow obstruction was detected during catheterization. Although the hepatic venous pressure gradient was slightly increased (10 mm Hg), there were no clinical signs of portal hypertension. Albumin scintigraphy with specific early recordings suggested focal albumin intestinal entry in the jejunal Roux-en-Y loop. Local bacterial overgrowth or local lymphangiectasia, possibly due to (venous) congestion, was considered. Treatment with metronidazole did not improve albumin loss. Next, surgical revision of the jejunal Roux-en-Y loop was performed. The explanted loop contained a small abnormal area with a thin hyperemic mucosa, near the former anastomosis. Histopathological analysis showed changes both in the blood vessels and the lymphatic vessels with focal deeper chronic active inflammation resulting in congestion of vessels, hampering lymphatic outflow leading to lymphangiectasia and patchy distortion of lymphatic vessels. Following surgical revision, secondary PLE disappeared, up to now, 1.5 year post revision., (© 2019 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Background: Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement., Methods: We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography., Results: Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well., Conclusions: Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options., Level of Evidence: IV., (Copyright © 2018. Published by Elsevier Inc.)

Rationale: Nonocclusive mesenteric ischemia (NOMI) is a life-threatening disorder; prompt diagnosis is vital. Surgical treatment is often required, but some cases can be treated conservatively. We herein report an extremely rare case wherein protein-losing enteropathy (PLE) developed after conservative treatment of NOMI., Patient Concerns: The patient was a 66-year-old man. He underwent laparoscopic super low anterior resection and temporary ileostomy for sigmoid colon cancer and rectum cancer. During the postoperative course, he developed ileus. Subsequently, he developed shock. On examination, the possibility of NOMI could not be denied, but intestinal necrosis was absent. Conservative treatment was initiated. His general condition improved, but the ileus persisted. Therefore, we performed a stoma closure. Ten days after stoma closure, he developed continuous unexplained diarrhea. The serum albumin and total protein levels were low. The symptoms improved after administration of an antidiarrheal drug, but the root cause was yet untreated., Diagnosis: The patient's alpha-1 antitrypsin clearance was increased. A CT scan revealed an enhanced hypertrophied wall of the short segment of the small intestine, and 99m Tc-labeled human serum albumin scintigraphy revealed protein leakage into the thickened wall of the small intestine. We arrived at a definitive diagnosis of PLE secondary to NOMI., Interventions: Partial resection of the affected small intestine was performed., Outcomes: The patient recovered uneventfully and was discharged on the 30th postoperative day., Lessons: NOMI has a high mortality rate, often requiring intestinal resection immediately after onset. To our knowledge, there is no report of PLE developing after conservative treatment, as in our case. Further study of cases is necessary to determine the reversibility of the condition, which will influence the therapeutic plan. We herein present an extremely rare case of PLE after conservative treatment for NOMI. The possibility of PLE also needs to be considered when hypoalbuminemia occurs after conservative treatment of NOMI.

Managing protein-losing enteropathy after Fontan surgery is challenging. We report a case of protein-losing enteropathy associated with Fontan circulation in a 14-year-old boy. He was treated medically for 2 years, without improvement until Fontan takedown and pulsatile bidirectional cavopulmonary shunting were performed.

Rationale: Although systemic lupus erythematosus (SLE) can be complicated by various gastrointestinal tract diseases, it is rarely associated with lupus enteritis and protein-losing enteropathy (PLE). We report here the successful surgical treatment of lupus enteritis and therapy-resistant and refractory PLE in a patient with SLE. We also provide a review of relevant literature., Patient Concerns: A 16-year-old girl presenting with polyarthritis, malar rash, and palmar erythema was indicated for steroid therapy on the basis of positive results for antinuclear, anti-Smith, and antiphospholipid antibodies, which confirmed the diagnosis of SLE. During the course of steroid therapy, the patient developed acute abdomen and hypoalbuminemia., Diagnoses: Computed tomography and Tc-labeled human serum albumin scintigraphy revealed abnormal findings, and a diagnosis of lupus enteritis and PLE was made. Steroid treatment was continued but no significant improvement was observed, and the patient was referred and admitted to our hospital. Double-balloon enteroscopy revealed multiple ischemic stenoses and mucosal necroses in the small intestine, suggesting that PLE was associated with ischemic enteritis due to antiphospholipid syndrome. The patient received steroids, immunosuppressive drugs, and antithrombotic therapy, with no improvement in symptoms. Thus, the disease was judged to be refractory and resistant to medical therapy, and the patient was indicated for surgical treatment., Interventions: Partial small intestinal resection was performed by removing the segment of the small intestine presenting PLE lesions, and a double-end ileostomy was created., Outcomes: Multiple stenotic lesions were confirmed in the resected segment. Histopathology evaluation revealed marked inflammatory cell infiltration in the intestinal tract wall and recanalization of the vessels, suggesting a circulatory disorder caused by vasculitis and antiphospholipid syndrome. Postoperatively, the clinical course was good. Serum albumin levels and body weight increased as nutritional status improved significantly. Secondary enteroenterostomy with ileostomy closure could be performed at 2 months after the initial surgery., Lessons: Timely surgical treatment can be successful in managing therapy-resistant and refractory PLE in patients with SLE.

Background: Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected., Objectives: The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE., Methods: This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE., Results: Liver lymphangiography was performed in 8 patients (5 males, 3 females; median age, 21 years), 7 of whom demonstrated leakage of liver lymph into the duodenum through abnormal hepatoduodenal lymphatic communications. This was confirmed by duodenoscopy with simultaneous injection of isosulfan blue dye into the liver lymphatics in 6 of 7 patients. Liver lymphatic embolization with ethiodized oil in 2 patients resulted in a temporary increase in albumin blood level and symptom improvement in 1 patient, but was complicated by duodenal bleeding in both patients. Of the remaining 6 patients, liver lymphatic embolization with n-butyl cyanoacrylate glue resulted in sustained improvement of the serum albumin level and symptoms in 3 patients, temporary improvement in 2 patients, and no change in 1 patient with median follow-up of 135 days (range, 84 to 1,005 days)., Conclusions: The authors demonstrated liver lymph leakage as a cause of PLE in patients with congenital heart disease and elevated central venous pressure. Lymphatic embolization led to improved albumin levels and relief of symptoms. Further experience with the technique is needed to determine long-term outcome of this procedure., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative procedure in 2 patients with failing Fontan circulation with PLE refractory to optimized medical therapy., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis., Objectives: The aim of this study was to review outcomes in patients with PLE following the Fontan operation., Methods: From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively., Results: Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m(2) vs. 2.7 ± 0.7 l/min/m(2); p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%])., Conclusions: PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Lipogranulomatous lymphangitis is inflammation of the intestinal lymphatic vessels and surrounding tissues caused by chronic leakage of lipid-laden chyle. Grossly, lipogranulomas are typically disseminated small masses on the serosa and surrounding lymphatic vessels and consist of epithelioid macrophages, multinucleated giant cells, and cholesterol. Lipogranulomatous lymphangitis is occasionally seen in patients with lymphangiectasia and protein-losing enteropathy (PLE)., Objectives: To characterize the historical features, clinical signs, treatment, histopathology, and outcome of dogs with focal lipogranulomatous lymphangitis., Animals: Six dogs with ultrasonographic evidence of focal, regional small intestinal masses, often with involvement of the adjacent mesentery, and a diagnosis of focal lipogranulomatous lymphangitis based on histopathology of biopsied masses., Results: The median age of dogs was 6.9 years (range 3-10 years). All dogs had total protein, globulin, and albumin concentrations within the reference range at initial presentation and had intestinal masses identified on abdominal ultrasound examination. Histopathologic evaluation of lesions identified severe mural and mesenteric lipogranulomatous lymphangitis. Lymphangiectasia was noted in 5 cases and only in sections within the mass-like lesion; tissue without lipogranulomas had minimal lymphangiectasia, suggesting a localized phenomenon. Postoperative outcomes ranged from remission of clinical signs with no subsequent treatment for 10-12 months in 2 dogs, postoperative management with medical and nutritional management in 3 dogs, and no outcome for 1 case., Conclusions and Clinical Importance: This case series describes a unique mass-like manifestation of intestinal lipogranulomatous lymphangitis and should be considered as a possible differential diagnosis in dogs with an intestinal mass., (Copyright © 2013 by the American College of Veterinary Internal Medicine.)

PFIC 1 is a genetic disorder characterized by hepatic and gastrointestinal disease, often requiring LT during childhood. Extrahepatic symptoms, such as diarrhea and malabsorption, do not improve or may be aggravated after LT, as graft steatosis or steatohepatitis as consequences of the interaction between transplanted liver and native bowel. We describe a patient with PFIC 1 who presented with cholestasis in infancy, who developed intractable pruritus and liver fibrosis. The child underwent living donor LT at 3.6 yr of age, and he early developed severe refractory diarrhea, secondary malabsorption with protein-losing enteropathy, and an early fatty liver disease trough graft steatohepatitis. As the response to cholestyramine was unsatisfactory, we decided to perform an EBD by using the jejunal loop used for the cholangiojejunostomy. Diarrhea resolved rapidly after surgery. He remained well after six months following biliary diversion, with normal stool output and no protein loss. We documented a dramatic improvement of graft steatosis at histology as well as normalization of liver function test. EBD can be considered a valuable treatment option to avoid organ disfunction and loss in PFIC 1 transplanted patients who develop graft steatohepatitis., (© 2011 John Wiley & Sons A/S.)

Among primary immunodeficiencies, common variable immunodeficiency (CVID) is defined by an impaired production of immunoglobulins characterized by low levels of plasma immunoglobulins and an altered antibody response. The case reported here was initially interpreted as a CVID. A 20 year old male suffered from diarrhea, weight loss, and malnutrition. Accurate diagnostic assessment uncovered a protein-losing enteropathy. Conventional oil contrast lymphangiography accurately documented the underlying problem and established the appropriate therapeutic approach. The operation consisted of multiple antigravitational ligatures of dilated and incompetent chylous vessels and chylous vessel-mesenteric vein microanastomoses. Serum albumin and leukocyte counts normalized by 1 week after operation and remained stable with time. There were no more episodes of diarrhea, and the patient regained weight. Accurate diagnostic assessment and particularly lymphangiography may be necessary to properly define difficult cases of immunodeficiency due to intestinal protein loss and to plan a corrective therapeutic functional approach.

Protein-losing enteropathy in children is caused by intestinal metabolic, inflammatory, or infectious processes, or by lymphatic obstruction (intestinal lymphangiectasia). In this report, a 17-month-old child is presented with protein-losing enteropathy due to intestinal malrotation and chronic midgut volvulus causing lymphatic obstruction and spillage of lymph in the intestine and the peritoneum. This report should alert the pediatrician that intestinal malrotation should be added to the wide list of possible causes of protein-losing enteropathy in children.

Primary intestinal lymphangiectasia is a rare cause of protein-losing enteropathy and usually presents with intermittent diarrhea or malnutrition. Diagnosis depends largely on its pathologic condition demonstrating greatly dilated lymphatics mainly in the lamina propria of the mucosa. We report a case of primary intestinal lymphangiectasia, of the diffuse type, presenting with abdominal pain and voluminous diarrhea in a previously healthy 8-year-old boy. He had periumbilical pain for 3 months before presentation. He was managed by segmental bowel resections and end-to-end anastomoses. The histopathologic condition of the resected small intestine showed lymphatic dilation limited mainly to the subserosa and mesentery but was not prominent in the mucosa. Abdominal pain and diarrhea subsided postoperatively. The present case is the fourth report describing a response to operative resection.

Protein-losing enteropathy is a rare but life-threatening complication that occurs in some patients who develop intestinal lymphangiectasis secondary to increased systemic venous pressure. Although different forms of treatment have been tried, with varying results, the majority were reported to be unsuccessful. The aim of this study was to demonstrate that heart transplantation may be an appropriate therapeutic option for patients who do not respond to medical treatment. At our center, we performed heart transplantations in three patients with this condition. The mean follow-up period was 11+/-2 months. No patient died and the enteropathy regressed in all three.

Background: The working hypothesis for a one and a half ventricle repair has been that the benefits of a pulsatile pulmonary circulation may negate some of the late complications of the Fontan procedure. Those benefits are thought to outweigh the downside risk of having retrograde pulsatility in the superior vena cava. We sought to define the long-term fate of this strategy., Methods: One hundred fourteen patients who underwent a superior vena cava to pulmonary artery anastomosis as an adjunct to biventricular repair were identified for the years 1965 to 2003. Median follow-up was 92.3 months (range, 1 month to 38 years)., Results: The long-term outcome for operative survivors was 83.4%, 80.1%, and 69.3% at 5, 10, and 20 years, respectively. The survival in the most recent 10 years is 91.8% (p = 0.063). Of the late deaths, 69.6% (16 of 23) were known cardiac deaths or sudden. Patients with chronic right ventricular dysfunction demonstrated the best 10-year survival (91.6%). Of the late survivors, 98.8% of patients are in New York Heart Association class I or II. Arterial O(2) saturation increased significantly from before to late after repair. (83.5% to 94.5%, p < 0.001; n = 82). Freedom from new atrial arrhythmia was 92.2% at 20 years. The superior vena cava to pulmonary artery anastomosis was taken down in 3. There was no patient with clinically evident protein-losing enteropathy., Conclusions: The most common cause of late mortality is cardiac. Atrial and ventricular arrhythmias occur, but no protein-losing enteropathy was identified. The serious complication risk related to pulsatility in the superior vena cava was 2.6%.

We report a 25-year-old Malay man with Subutex-related endocarditis, complicated by protein-losing enteropathy from severe tricuspid regurgitation and congestive heart failure. The intestinal protein loss was reversed with surgical valvular intervention. This case highlights the importance of recognising the rare association between protein-losing enteropathy and congestive heart failure in the setting of endocarditis.

A male patient with chronic bloody stool was diagnosed as juvenile polyposis at the age of 28. He had thirty to forty colonic polyps and some were removed endoscopically, while gastric polyps were too numerous to intervene. As the polyposis advanced gradually, the patient developed intractable anemia and serious hypoproteinemia. Albumin scintigram revealed protein losing gastropathy due to progressive gastric polyposis. Total gastrectomy was carried out at the age of 34 and the patient has achieved remarkable and sustainable improvement.

Protein-losing enteropathy (PLE) is an uncommon manifestation associated with systemic lupus erythematosus (SLE). Here, a case with SLE and concomitant hypoalbuminemia is reported. Technetium-99m albumin scintigraphy demonstrated a localized lesion in the ascending colon, and the diagnosis of SLE-related PLE was established. Due to a poor response to medical treatment, this patient received surgical resection, but relapse still developed later on. Recurrent protein-lose from the remaining of the colon was documented by repeated images. This report discusses the management of SLE-related PLE and the role of nuclear medicine scintigraphy in the investigation of PLE.

IPEX syndrome is a rare, inherited condition characterized by immune dysfunction, polyendocrinopathy, enteropathy, and X-linked recessive inheritance. Patients typically present in infancy with severe diarrhea and failure to thrive. Most children die by 1 year of age without therapy. The diagnosis is established by genetic analysis, which often takes several weeks to complete and can sometimes delay crucial immunosuppressive treatment. We attempted to develop a screening tool that allows rapid identification of patients with IPEX syndrome using immunocytochemical staining of FOXP3+ cells in bowel biopsies. We found that 2 patients with classic IPEX syndrome due to protein-truncating mutations in FOXP3 had markedly decreased staining of FOXP3+ T cells in the lamina propria and lymphoid aggregates. One patient with a mild, late-onset presentation and a missense mutation in FOXP3 had intact staining of FOXP3+ cells. This screening test provides a valuable tool for diagnosing IPEX syndrome in extremely ill patients who may not tolerate a delay in therapeutic intervention.

The application of radiofrequency (RF) technologies in the treatment of congenital heart defects has provided a safe and effective alternative to conventional therapies in the restoration of vascular patency for a variety of arterial and venous occlusions. This report concerns an 8-year old girl that developed protein-losing enteropathy and elevated central venous pressure after occlusion of a surgically redirected anomalous draining left superior vena cava (SVC). Cardiac catheterization revealed complete obstruction of the anastomosis of the SVC into the coronary sinus. Transcatheter recanalization by RF perforation and subsequent stent implantation led to the restoration of upper venous blood flow and the resolution of her symptoms., (Copyright 2006 Wiley-Liss, Inc.)

Although acute pericarditis is a common and usual benign disorder, sometimes evolution to constrictive pericarditis may occur. We present a case of constrictive pericarditis late after coronary bypass grafting, complicated by right sided heart failure. Edema formation was aggravated due to protein-losing enteropathy, resulting in hypoalbuminemia. Imaging of constrictive pericarditis was done by ultrasound as well as simultaneous pressure recording of the right and left ventricle. Imaging of intestinal protein loss was possible using intravenous Technetium-99m-labelled human serum albumin.

A 27-year-old man who was diagnosed with arrhythmogenic right ventricular dysplasia (ARVD) underwent the total right ventricular (RV) exclusion procedure: the RV free wall was resected and an extracardiac total cavopulmonary connection (TCPC) was created using a 24-mm-diameter polytetrafluoroethylene (PTFE) graft. After an uneventful period of 7 years, he began to develop protein-losing enteropathy (PLE), which was resistant to medical therapy, moderate mitral regurgitation (MR) and right femoral arteriovenous fistula due to heart catheterization. Therefore, fenestration of TCPC, mitral annuloplasty and ligation of arteriovenous fistula were performed. After the operation, symptoms and levels of total protein and albumin were immediately improved and normalized.

The patient investigated was a 43-year-old woman with primary pulmonary hypertension (PPH) and refractory protein-losing enteropathy (PLE). She underwent living-donor lobar lung transplantation (LDLLT), which led to remarkable improvement in both pulmonary hypertension and PLE. Although there have been no reports, to our knowledge, that have demonstrated PLE as a complication of PPH, the present case clearly shows how PLE could complicate PPH. In addition, and more importantly, hypo-proteinemia due to PLE should not necessarily be an exclusion criterion for lung transplantation when PPH is involved, because it could markedly improve after transplantation.

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.

Cronkhite-Canada syndrome is a rare form of nonhereditary gastrointestinal polyposis associated with ectodermal change and protein-losing enteropathy. Here we report a 63-year-old male presenting with diffuse gastrointestinal polyposis, onychodystrophy, cutaneous pigmentation, alopecia, diarrhea, hypoalbuminemia and lower leg edema. Technetium-99m-labeled human serum albumin scan confirmed the patient to have protein-losing enteropathy, which originated from the transverse and descending colon. Subtotal colectomy was performed. Albumin level and ectodermal change were gradually improved during three years of outpatient clinic follow-up. Based on our finding, Technetium-99m-labeled human serum albumin scintigraphy is helpful to localize the protein-losing origins and surgery is an effective treatment for Cronkhite-Canada syndrome with protein-losing enteropathy.

Objective: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with Fontan conversion and concomitant arrhythmia surgery., Methods: Between January 1996 and February 2004, 16 patients underwent Fontan conversion and arrhythmia surgery. Mean age at the initial Fontan operation was 5.1+/-3.5 (range: 2-15) years and mean age at Fontan conversion was 17.0+/-5.8 (range: 6-30). The initial Fontan operations were atriopulmonary connections in 14 patients, extracardiac lateral tunnel in 1, and intracardiac lateral tunnel in 1. The types of arrhythmia included atrial flutter in 10 patients and atrial fibrillation in 3. Fontan conversion operation was performed with intracardiac lateral tunnel in 5 patients and extracardiac conduit in 11. Arrhythmia surgery included isthmus cryoablation in 10 patients and right-sided maze in 3., Results: There has been no mortality. At Fontan conversion operation, 7 patients required permanent pacemaker. All patients have improved to New York Heart Association class I or II. With a mean follow-up of 26.9+/-30.6 (range:1-87) months, 16 patients had sinus rhythm, 2 patients had transient atrial flutter which was well controlled, and 2 patients required permanent pacemaker during follow-up., Conclusions: Fontan conversion with concomitant arrhythmia surgery and permanent pacemaker placement is safe, improves New York Heart Association functional class, and has a low incidence of recurrent arrhythmias. In most patients, concomitant permanent pacemakers are needed.

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.

Protein losing enteropathy (PLE) is a gastrointestinal disorder that is associated with excessive loss of plasma protein into the gut resulting from abnormal mucosal permeability. The disease is usually caused by inflammation. The loss of protein in PLE is a nonselective process affecting albumin, globulin and transferrin. Abdominal scintigraphy with human serum albumin marked by Tc99m seems to be an easy and sensitive method for diagnosing PLE. An 4-year-old girl was presented to an outside Pediatric Department due to hypoproteinemia and recurrent pneumonia which had caused several prior hospitalizations. The laboratory tests revealed hypoproteinemia, hypoalbuminemia, low level of IgG, sideropenia, and a decreased level of T lymphocytes. The loss of protein into the gut was confirmed by fecal clearance of alfa-1 antitrypsin. Only nonspecific inflammation was detected by biopsy of the small intestine. These clinical and laboratory findings, quickly decreasing IgG and albumin levels in spite of i.v. supplementation and the lack of proteinuria permitted PLE diagnosis. The abdominal scintigraphy was planned to assess and localise protein losing through GIT and for strategy of possible surgical treatment. Abdominal dynamic scintigraphy was performed immediately after the injection of 300 MBq Tc99m human albumin. 90 images were taken within 180 minutes. Delayed abdominal images were obtained 6 and 24 hours after the tracer injection. Anterior abdominal scintigraphy showed pathological activity of Tc99m-albumin in small bowel in the upper left segment of the abdomen in the 40th minute after injection. Extensive accumulation of albumin was seen in the 160th minute. Delayed images, after 3 and 6 hours, revealed translocation of the tracer into the lower right abdominal segment. The further passage and tracer concentration was detected in ascendant and transverse colon. Based on the laboratory tests and scintigraphic images the girl was suspected to have segmental lymphangiectasia of small intestine and was qualified for laparatomy and, possibly, for surgical resection of the pathologically changed bowel. However, diffuse pathological changes revealed in the guts during the operation, rendered the operation impossible. Tc99m-labelled human serum albumin scintigraphy may be considered the method-of-choice in the diagnosis of protein-losing enteropathy. However, in this test the assessment of the enteropathy dimension is difficult and surgical treatment should be planned with caution.

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.

Intestinal involvement by endometriotic tissue occurs in up to 37% of patients with endometriosis. The vast majority of patients do not experience symptoms related to the gastrointestinal tract. In particular, the complications of intestinal obstruction and malabsorption secondary to endometriosis are exceedingly uncommon. We present a 42-year-old woman with intestinal obstruction, protein-losing enteropathy, and anasarca secondary to endometriosis. She had a 1-year history of watery diarrhea, bloating, and abdominal pain with a 30-lb weight-loss over 3 months. She had no previous history of endometriosis, and laboratory investigations showed severe hypoalbuminemia, hypokalemia, and metabolic acidosis. Abdominal x-rays revealed air-fluid levels and dilated loops of small bowel. She underwent surgical resection with primary anastomosis. Pathologic evaluation showed extensive endometriosis of the small bowel and appendix, which resulted in complete obstruction. Segments of ileum also demonstrated moderate-to-marked blunting of the villi. Postoperatively, the patient had a slow recovery with resolution of anasarca and a gradual increase in her weight. This report illuminates the rare, yet significant, complications of intestinal endometriosis, including small bowel obstruction, the development of a protein-losing enteropathy, and anasarca. One should consider the possibility of intestinal endometriosis in the differential diagnosis of bowel obstruction in women of childbearing age.

A 64-year-old man presented with leg edema and hypoproteinemia. His alpha-1 antitrypsin clearance rate of 174 mL/day indicated the presence of protein-losing enteropathy (PLE). Computed tomographic scans demonstrated thickened ileal wall and mesenteric edema. Angiography revealed occlusion in a peripheral branch of the superior mesenteric vein. Furthermore, both the patient and his son had low protein C levels. The patient was treated successfully with partial resection of the ileum. Histologic examination of the resected ileum revealed multiple erosions and submucosal fibrosis with organized venous thrombi in the mesenteric veins. This is the first case report of PLE caused by mesenteric venous thrombosis, and our findings suggest that serum protein was lost through erosion of the ileum caused by ischemia due to mesenteric venous thrombosis.

Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. Prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the Fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified Fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation.

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.