In-hospital mortality in dogs with protein-losing enteropathy and associated risk factors.

BACKGROUND: Risk factors associated with negative outcomes in dogs with protein-losing enteropathy (PLE) are well documented. However, mortality before hospital discharge and associated risk factors are not well described. HYPOTHESIS/OBJECTIVES: Report the percentage of dogs with PLE that do not survive to hospital discharge and identify associated risk factors. ANIMALS: One-hundred and seven dogs presented to a referral hospital and diagnosed with PLE caused by inflammatory enteritis, intestinal lymphangiectasia or both. METHODS: Retrospective cross-sectional study assessing hospital records. Data on in-hospital mortality and cause were assessed, and presenting signs, treatments prescribed, neutrophil count, lymphocyte count, serum albumin, globulin, and C-reactive protein (CRP) concentrations, and histopathologic findings were compared between survivors and non-survivors. RESULTS: In-hospital mortality was 21.5% with the most common causes including financial limitations, failure to improve and aspiration pneumonia. Factors associated with mortality during hospitalization included longer duration of hospitalization (P = .04), longer duration of clinical signs (P = .02) and an increase in serum CRP concentration after 1-3 days of in-hospital treatment (P = .02). Higher mortality was identified in Pugs (odds ratio [OR], 4.93; 95% confidence interval [CI], 1.41-17.2; P = .01) and was a result of presumptive aspiration pneumonia in 5/6 of these dogs. CONCLUSIONS AND CLINICAL IMPORTANCE: Protein-losing enteropathy in dogs has substantial mortality during hospitalization. Monitoring for improvement in CRP concentration after treatment during hospitalization may help predict survival to discharge. Pugs have increased in-hospital mortality because of aspiration pneumonia; measures to prevent, recognize, and promptly treat this complication may improve outcomes in this breed.

Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients.

BACKGROUND: Fontan physiology results in multiorgan dysfunction, most notably affecting the liver and kidney. We evaluated the utility of Model for End-Stage Liver Disease Excluding INR (MELD-XI) score, a score evaluating the function of both liver and kidney to identify Fontan patients at increased risk for morbidity and mortality post-heart transplant. METHODS: The Pediatric Heart Transplant Society database was queried to identify Fontan patients listed for heart transplant between January 2005 and December 2018. MELD-XI scores were calculated at listing and heart transplant. A multivariable analysis was conducted to identify risk factors for post-heart transplant mortality. Demographic, clinical characteristics, and survival differences were evaluated and compared between the high and low MELD-XI score cohorts. The impact of changing MELD-XI scores during the waitlist period on post-heart transplant outcomes was also evaluated. RESULTS: Of 565 Fontan patients who underwent transplantation, 524 (93%) had calculable MELD-XI scores at the time of heart transplant: 421 calculable at listing and 392 calculable at listing and at heart transplant. On multivariable analysis, only MELD-XI score (squared) (hazard ratio, 1.007), history of protein-losing enteropathy (hazard ratio, 2.1), and ventricular assist device use at transplant (hazard ratio, 3.4) were risk factors for early phase post-heart transplant mortality. Patients with high MELD-XI scores at heart transplant had inferior survival post-heart transplant (P = .02); those in the high MELD-XI score cohort at wait listing and heart transplant tend to have the worst post-heart transplant survival; however, this was not significant (P = .42). CONCLUSIONS: The MELD-XI, an easily calculated score, serves as a valuable aid in identifying pediatric Fontan patients at increased risk for post-heart transplant mortality.

Protein-losing enteropathy and plastic bronchitis after the Fontan procedure.

OBJECTIVES: Protein losing enteropathy and plastic bronchitis are severe complications in Fontan circulation, with 5-year survival ranging from 46% to 88%. We report risk factors and outcomes of protein losing enteropathy and plastic bronchitis in patients undergoing the Fontan. METHODS: We performed a retrospective analysis of 1561 patients from the Australia New Zealand Fontan Registry. Two end points were death and cardiac transplantation examined with Cox regression (if no competing risks) or cumulative incidence curves and cause-specific Cs regression. RESULTS: A total of 55 patients with protein losing enteropathy/plastic bronchitis were included. Their median age at the Fontan was 5.7 years, and time to onset after the Fontan for protein losing enteropathy was 5.0 years and plastic bronchitis was 1.7 years. Independent predictors for developing protein losing enteropathy/plastic bronchitis were right-ventricular morphology with hypoplastic left-heart syndrome (hazard ratio, 2.30; confidence interval, 1.12-4.74), older age at Fontan (hazard ratio, 1.13; confidence interval, 1.03-1.23), and pleural effusions after Fontan (hazard ratio, 2.43; confidence interval, 1.09-5.41); left-ventricular morphology was protective (hazard ratio, 0.36; confidence interval, 0.18-0.70). In the protein losing enteropathy/plastic bronchitis population, freedom from death or transplantation after protein losing enteropathy/plastic bronchitis diagnosis at 5, 10, and 15 years was 70% (confidence interval, 58-85), 65% (confidence interval, 51-83), and 43% (confidence interval, 26-73), respectively; only older age (hazard ratio, 1.23; confidence interval, 1.01-1.52) was an independent predictor. Twenty-six surgical interventions were performed in 20 patients, comprising Fontan revisions (n = 5), fenestrations (n = 11), Fontan conversions (n = 5), atrioventricular valve repairs (n = 3), and hepatic vein diversion (n = 2). CONCLUSIONS: Protein losing enteropathy and plastic bronchitis remain severe complications, preferably affecting patients with dominant right single ventricle, with older age at Fontan being a predictor of developing protein losing enteropathy/plastic bronchitis and poorer prognosis. Heart transplantation remains the ultimate treatment, with 30% dying or requiring transplantation within 5 years, and the remaining being stable for long periods.

Association of chronic enteropathy activity index, blood urea concentration, and risk of death in dogs with protein-losing enteropathy.

BACKGROUND: Malnutrition is associated with increased risk of premature death in humans with inflammatory bowel disease. HYPOTHESIS/OBJECTIVE: To determine if historical, clinical, and laboratory markers of malnutrition in dogs at the time of histologic diagnosis of protein-losing enteropathy (PLE) caused by chronic enteropathy (CE) or lymphangiectasia are associated with increased risk of death. ANIMALS: Seventy-one client-owned dogs diagnosed with PLE. METHODS: The medical records were retrospectively searched for cases of PLE, diagnosed with CE or lymphangiectasia on the basis of histopathology of intestinal biopsies at a referral hospital. For each case, various variables at the time of diagnostic investigation were recorded and follow-up obtained by telephone contact with the referring veterinarian. RESULTS: A multivariable cox model indicated that canine chronic enteropathy activity index (CCEAI) and blood urea concentration were significantly associated with death (P values <.01). For each unit increase in CCEAI, the hazard of death increased by 22.9% (confidence interval [CI]: 6.9%-41.2%). Dogs with a CCEAI of ≤8 and dogs with urea ≤7 mmol/L survived 256 days longer (P = .001, CI: 106.7-405.4 days) and 279 days longer (P = .009, CI: 70.0-488.7 days) than those with a CCEAI of >8 and urea >7 mmol/L on average, respectively, when followed up for 647 days. CONCLUSIONS AND CLINICAL IMPORTANCE: Increased CCEAI and blood urea concentration at the time of diagnosis might be predictive of death in dogs with PLE caused by CE or lymphangiectasia.

Comparison of Clinical Profiles in Patients with Protein-Losing Enteropathy With and Without Fontan Circulation.

Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs. 8 ± 1 years, p = 0.02), while there was no difference in PLE onset age between groups. Furthermore, there were no differences in prevalence of clinically relevant arrhythmias, cardiac output, or central venous pressure between the two groups at PLE onset. Percentage of structural lesions (valve regurgitation and/or stenotic lesions) responsible for development of PLE and ventricular end-diastolic pressure were higher in the BVR group than in the Fontan group (93 vs. 50%, p < 0.01), (13.4 ± 6.3 vs. 7.5 ± 4.1, p < 0.0001). Catheter intervention was applied in 2Fontan and 6BVR patients, while surgical intervention was required in 8BVR and 7Fontan patients. Of these, catheter intervention was effective in 2 (25%, 1Fontan, 1BVR) and surgical intervention was effective in 4 (26.7%, 1Fontan, 3BVR). Only one patient (5.3%) improved without intervention. Complete PLE remission rate was higher in the BVR group than in the Fontan group (38 vs. 7%, p = 0.02). During follow-up, death of 2 BVR and 8 Fontan patients occurred. There were no group differences in 5- to 10-year survival rates after PLE onset (81 vs. 81%, BVR, 81 vs. 66%, Fontan). Although BVR patients may have greater chance of PLE remission when compared with those exhibiting Fontan pathophysiology, mortality in PLE-CHD patients was significantly high regardless of postoperative hemodynamics.

Gastrointestinal system involvement in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy (99mTc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.

Hypovitaminosis D is associated with negative outcome in dogs with protein losing enteropathy: a retrospective study of 43 cases.

BACKGROUND: Hypovitaminosis D has previously been shown to be prevalent amongst dogs with protein losing enteropathy (PLE). The hypothesis of this study was that Low 25-hydroxyvitamin D (25(OH) D) serum concentrations could be a risk factor for negative outcome in dogs with PLE. Forty-three dogs diagnosed with PLE (2005-2014) and which serum Vitamin D serum concentrations were collected and archived at -80 Degrees C were analyzed. Post-diagnostic communication with referring veterinarians was made to determine outcome of PLE dogss: Dogs which died due to PLE within 4 months after diagnosis (negative outcome group, n = 22) and dogs alive or which died due to another disease at the end point of the study (1 year after diagnosis, good outcome group, n = 21). Serum samples taken at the time of diagnosis were analysed for ionized calcium (iCa) concentrations and serum 25(OH) D concentration. RESULTS: Clinical (CCECAI) scores, age at PLE diagnosis, and iCa concentrations were not significantly different between dog groups. A significantly greater (p < 0.001) number of PLE dogs treated with hydrolyzed or elimination diet alone showed good outcome as compared to the PLE negative outcome group. Median serum 25(OH) D concentration was significantly (p = 0.017) lower in dogs with negative outcome versus PLE dogs with good outcome. Using logistic regression analysis, 25(OH) D serum concentration was shown to be a statistically significant factor for outcome determination. Cox regression analysis yielded a hazard ratio of 0.974 (95% CI 0.949, 0.999) per each one nmol/l increase in serum 25(OH) D concentration. CONCLUSIONS: Low serum 25(OH) D concentration in PLE dogs was significantly associated with poor outcome. Further studies are required to investigate the clinical efficacy of Vitamin D (cholecalciferol) as a potential therapeutic agent for dogs with PLE.

Clinicopathologic and prognostic factors in short- and long-term surviving dogs with protein-losing enteropathy.

INTRODUCTION: The aim of the present study was to investigate the differences in the characteristics of short- and long-term surviving dogs with protein-losing enteropathy (PLE) and to identify factors that predict its outcome. We retrospectively reviewed the medical records of 59 client- owned dogs with PLE diagnosed at three different hospitals between January 2009 and November 2013. The dogs were classified as either short-term (= 6 months; STs) or long-term (> 6 months; LTs) survivors. Clinical and clinicopathological variables were investigated between the groups and receiver operating characteristic (ROC) curve analysis was performed. Nineteen dogs were classified as STs and 40 as LTs. Body weight and blood urea nitrogen concentrations were significantly higher in the STs at diagnosis (P < 0.05). At 1 month after initiation of immunosuppressive therapy (data- driven cut-off, T1), chronic canine enteropathy clinical activity index (CCECAI) scores were higher (P < 0.01) and albumin, serum total protein and total cholesterol concentrations were lower (P < 0.01) in the STs. ROC curve analysis showed that CCECAI > 5 evaluated at T1 was the best predictor of poor outcome. Although the severity of clinical signs and the majority of clinicopathological findings at diagnosis did not influence the outcome, survival time was shorter in the dogs with high CCECAI scores at T1 and which did not respond to therapy.

INTRODUCTION: Le présent travail avait pour buts d'étudier quels sont les différences de symptômes chez les chiens survivant à court et à long terme à une d'entéropathie exsudative (PLE) et d'identifier les facteurs ayant une valeur pronostique. On a étudié pour cela les dossiers médicaux de 59 chiens sur lesquels une entéropathie exsudative avait été diagnostiquée dans trois cliniques différentes entre janvier 2009 et novembre 2013. Les chiens ont été classés comme survivants à court terme (= 6 mois; STs) respectivement à long terme (= 6 mois; LTs). Les variations cliniques et clinico-pathologiques entre les groupes ont été relevées et une courbe ROC a été établie. Dixneuf chiens ont été classés comme STs et 40 comme LTs. Le poids corporel et la concentration sanguine d'urée était significativement plus élevée (P < 0.05) chez les STs que chez les LTs. Un mois après le début d'une immunosuppression (cut-off établi sur la base des données disponibles, T1), le score clinique d'activité pour une entéropathie chronique chez le chien (CCEAI) était plus élevé chez les STs que chez les LTs(P < 0.01), les valeur sanguines d'albumine, de protéines totales et de cholestérine totale par contre plus basses (P < 0.01). Dans l'analyse par la courbe ROC, un CCEAI > 5 à T1 s'est avéré être un indice fiable quant à une évolution de courte ou de longue durée. Bien que l'étendue des symptômes cliniques et la quantité des découvertes clinico-pathologiques n'aient pas influencé le pronostic, le taux de survie des chiens avec un CCEAI élevé à T1 et de ceux qui n'avaient pas répondu au traitement a été plus faible.

Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation.

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.

Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment.

Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.

[Protein-losing enteropathy in Rottweilers]. / Protein-losing enteropathy bij de Rottweiler.

OBJECTIVES: To describe the signs, histopathological features, and treatment outcome of a not previously described protein-losing enteropathy (PLE) in Rottweiler dogs. METHODS: A retrospective study involving 17 Rottweilers referred for PLE. Data on sex, age, presenting signs, histopathological diagnosis, and treatment outcome were collected. The canine inflammatory bowel disease activity index (CIBDAI) score was calculated, to quantify disease severity. Endoscopic intestinal biopsies were evaluated according to the guidelines of the World Small Animal Veterinary Association (WSAVA) gastrointestinal standardization group. RESULTS: Presenting signs were watery diarrhoea and weight loss. In all dogs with PLE in this study, the abnormalities found were consistent with a form of inflammatory bowel disease. Some of the dogs had a secondary infection with Giardia or Cyniclomyces guttulatus. In 10 dogs (59%) the CIBDAI score was 9 or higher, indicative of severe disease. Histopathological findings revealed lymphoplasmacellular enteritis, with lymphangiectasia in 14 (82%) dogs and eosinophil infiltration in 10 (59%) dogs. Eleven (65%) dogs were euthanized or died because of the intestinal disease. Kaplan-Meier analysis revealed a median survival time of 5 months, with a 1-year survival rate of 47%. Seven (4%) dogs were disease-free after treatment with immunosuppressants and dietary measures, but some relapsed (median disease-free interval 21 months). CLINICAL SIGNIFICANCE: In Rottweilers presenting with chronic diarrhoea and weight loss, clinicians should consider the presence of severe PLE, which has a poor prognosis.

Superior vena cava to pulmonary artery anastomosis as an adjunct to biventricular repair: 38-year follow-up.

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%.

Usefulness of heparin therapy in protein-losing enteropathy associated with single ventricle palliation.

This retrospective study was designed to evaluate the effectiveness of subcutaneous heparin therapy for the treatment of protein-losing enteropathy (PLE) associated with single-ventricle palliation and to evaluate the side effects of long-term heparin use. PLE affects 4% to 13% of Fontan operative survivors. Five-year survival after onset of PLE is only 46% to 59%. We studied a cohort of patients with single-ventricle palliation who developed PLE and were treated with subcutaneous heparin. Seventeen patients were included in the study. Symptoms of PLE appeared on average 43 months after surgical palliation. At diagnosis of PLE, mean albumin level was 2.0 +/- 0.4 g/dl. At cardiac catheterization, mean systemic venous pressure was 11.6 mm Hg. Subjective symptomatic improvement on heparin therapy occurred in 13 patients (76%). Three patients (18%) went into clinical remission. Compared with the period before initiation of heparin, there was no significant difference in the number of hospital admissions (p = 0.99) or albumin infusions (p = 0.88) during the first year of heparin therapy. Five patients had x-rays of their thoracolumbar spine, and 9 patients had bone mineral analyses; all scans were grossly abnormal. In conclusion, subcutaneous heparin therapy leads to subjective improvement of PLE symptoms in most patients; however, it does not change the need for frequent albumin infusions and does not increase the rate of remission above that for standard medical therapy.

Early death in two sisters with Hennekam syndrome.

We report on two sisters with facial anomalies, protein-losing enteropathy, and intestinal lymphangiectasia consistent with the diagnosis of Hennekam syndrome. Both patients had a number of other anomalies not previously described in this autosomal recessive disorder, i.e., primary hypothyroidism, hypertrophic pyloric stenosis, and an early fatal outcome. These cases support the autosomal recessive transmission and the expansion of the phenotype of the Hennekam syndrome.

Pharmacological protection of NSAID-induced intestinal permeability in the rat: effect of tempo and metronidazole as potential free radical scavengers.

Recently, NSAID-induced changes in both the structure and function of the distal intestine have been found to occur more frequently and with greater toxicological significance than previously thought. We have previously validated a suitable animal model to evaluate intestinal permeability changes using orally administered 51Cr-EDTA that correlates with intestinal ulceration. In this study we investigated the suitability of metronidazole and the nitroxide stable free radical scavenger (tempo) as protective agents against NSAID-induced intestinal permeability. Male Sprague-Dawley rats were dosed with two doses of metronidazole (50 mg/kg, 12 and 1 h pre-NSAID) or a single 100 mg/kg dose of tempo 1 h prior to NSAIDs. The urinary excretion of the orally administered marker 51Cr-EDTA was measured. Both tempo and metronidazole dramatically reduced indomethacin (20 mg/kg) and flurbiprofen (10 mg/kg)-induced intestinal permeability. All the animals exposed to indomethacin alone died within 48-96 h and presented with histological evidence of drug-induced enteropathy, ulceration and frank peritonitis. Protection by tempo and metronidazole suggests that free radicals and/or bacteria may be important mediators in the pathogenesis of intestinal mucosal damage induced by NSAIDs. Nitric oxide donor compounds used concomitantly with NSAIDs may protect gastrointestinal tract.

Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis.

After modified Fontan procedures with atriopulmonary anastomoses or right atrium-right ventricle conduits, some patients have progressive exercise intolerance, effusions, arrhythmias, or protein-losing enteropathy. Theoretic advantages of a lateral atrial tunnel cavopulmonary anastomosis and published clinical results suggest that conversion of other Fontan procedures to the lateral atrial tunnel may afford clinical improvement for some patients. Eight patients (8 to 25 years old) with tricuspid atresia (n =4), double-inlet left ventricle (n = 3), and double-outlet right ventricle (n=1) underwent conversion to a lateral tunnel procedure between December 1990 and November 1994. An arbitrary clinical score was assigned before the lateral tunnel procedure and at follow-up. Before conversion, patients had decreased exercise tolerance (n = 8), arrhythmias (n = 6), effusions (n = 4), and protein-losing enteropathy (n = 8). At catheterization, all had a low cardiac index (1.9 +/- 0.7 L x min(-1) x M(-2), five had elevated pulmonary vascular resistance (>3 Wood units), and three had right pulmonary venous return obstruction by compression of an enlarged right atrium. Fenestrated lateral tunnel construction was undertaken 7.3 +/- 3.6 years after atriopulmonary anastomosis, with one early death related to low cardiac output. After the lateral tunnel procedure, two patients had no clinical improvement (no change in clinical score) but five patients had either marked or partial improvement. The right pulmonary vein compression present in three patients was resolved after conversion. The mean clinical scores improved from 4.5 +/- 1 to 3.0 +/- 2 (p < 0.04). In conclusion, conversion to a lateral tunnel procedure led to clinical improvement in five of eight patients at short-term follow-up and may be particularly indicated for patients with giant right atria or pulmonary vein compression who have symptoms. Pulmonary vein compression should be looked for in patients after modified Fontan procedures and can be relieved by conversion to the lateral tunnel procedure.

Gastro-intestinal protein loss in elderly patients with cardiac cachexia.

Undernutrition resulting from chronic congestive heart failure (cardiac cachexia, CC) increases morbidity and mortality particularly in elderly people. The aetiology of CC is thought to be multifactorial. We have assessed the presence of gastro-intestinal protein loss in a group of patients with CC and a group of healthy age-and sex-matched controls. Gastro-intestinal protein loss was measured using the chromic chloride test in 29 patients with CC [mean age 76.1 (SD4.4) years] and 29 healthy controls [mean age 74.9 (SD 4.8) years]. The patients were undernourished in terms of anthropometric measurements compared to controls. The patients had a significantly lower mean ejection fraction [41.5(18.3)% vs. 65.5(2. 2)%] and higher mean pulmonary artery pressure [89.4(19.9)mmHg vs. 19.3(8.1) mmHg]. The recovery of radioactivity in a 5-day stool collection was similar in the two groups [patients vs. controls: 1. 0(0.7)% vs. 0.98(0.6)%, p=0.9]. These values are within the expected normal range. We conclude that gastro-intestinal protein loss is not a significant factor in the production of cardiac cachexia.