Risk Factors for Macroscopic Haemoglobinuria After Sclerotherapy Using Ethanolamine Oleate for Venous Malformations.

OBJECTIVES: Sclerotherapy is an essential component of the treatment for venous malformations, and ethanolamine oleate (EO) is known as a useful sclerosing agent. However, macroscopic haemoglobinuria (MH) and subsequent renal impairment are severe complications after sclerotherapy using EO. The present study aimed to clarify the MH risk factors for better peri-operative management of venous malformations. METHODS: Data collected during 130 procedures involving 94 patients who were undergoing sclerotherapy using EO for venous malformation were retrospectively analysed. Pre-operative and operative variables, including sex, age, pre-operative body mass index, location, depth, type of lesion, size, number of procedures, type of drainage vein, ratio of sclerosant to air, and injected total dose of 5% EO per body weight (BW), were examined. Univariable analysis and multivariable logistic regression were performed to determine the possible risk factors for MH. RESULTS: Following sclerotherapy, MH occurred in 27.7% of patients, but no patient developed post-operative renal impairment because of aggressive hydration and haptoglobin administration. On univariable analysis, diffuse lesion, lesion size ≥50 cm2, and total injected dose of 5% EO ≥ 0.18 mL/kg were found to be the MH risk factors. Multivariable logistic regression analysis identified a total injected dose of 5% EO ≥ 0.18 mL/kg as the significant independent factor contributing to MH risk. CONCLUSIONS: Macroscopic haemoglobinuria is a reversible complication if immediate and appropriate interventions with aggressive hydration and haptoglobin administration are performed; therefore, it should be closely monitored following sclerotherapy, especially when using 5% EO ≥ 0.18 mL/kg.

[Hemoglobinuria in children hospitalized in Ouagadougou: short term inpatient care and prognosis]. / Hémoglobinurie chez l'enfant à Ouagadougou: prise en charge hospitalière et pronostic à court terme.

INTRODUCTION: The purpose of this study was to analyze the epidemiological, diagnostic, therapeutic and evolutionary features of hemoglobinuria in children hospitalized in the Pediatric University Hospital Charles de Gaulle, Ouagadougou. METHODS: We conducted a cross-sectional descriptive study over the period 01st July-31st December 2014. All children aged 0-15 years hospitalized in the Department of Medical Pediatrics of the Pediatric University Hospital Charles de Gaulle and diagnosed with macroscopic hemoglobinuria during the study period were enrolled. RESULTS: Thirty-eight patients were included in the study. Hospitalization rate for hemoglobinuria was 1.9%. The average age of patients was 80.8 ± 44.1 months (ranging from 21 to 168). The study involved 23 boys (60.5%) and 15 girls (39.5%). The major clinical signs were: fever (86.8%), dark urines like « coca cola ¼ (86.8%), pallor (63.2%), hepatomegaly (50%). Glomerular filtration flow was less than 80 mL/min/1.73m2 in 23 patients (69.7%); 21 patients had Glucose-6-phosphate dehydrogenase (G6PD) deficiency. The main suspected causes of hemoglobinuria were: severe malaria, bacterial and viral infections, G6PD deficiency, biliary haemoglobinuric fever. Treatments included: artemisinin derivatives, antibiotics and antipyretics. One patient underwent dialysis. CONCLUSION: Hemoglobinuria is a symptom mainly causing diagnostic problems in our context. It is a severe disorder which can result in acute renal failure (ARF).

Co-inheritance of glucose-6-phosphate dehydrogenase deficiency mutations and hemoglobin E in a Kachin population in a malaria-endemic region of Southeast Asia.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency and hemoglobin E (HbE, ß26 Glu-Lys) are two common red cell disorders in Southeast Asia. G6PD deficiency produces hemolytic anemia, which can be triggered by certain drugs or infections. HbE is asymptomatic or is manifested as microcytic, minimally hemolytic anemia. The association between G6PD deficiency and HbE is little understood. This study aimed to investigate G6PD deficiency and HbE in a Kachin ethnic group in the China-Myanmar border area. G6PD enzyme activity was measured using a quantitative G6PD assay, G6PD variants genotyped by the SNaPshot assay, and an HbE gene mutation identified by an amplification refractory mutation system and subsequently confirmed by using a reverse dot blot hybridization assay from 100 unrelated individuals in the study area. G6PD enzyme activity ranged from 0.4 to 24.7 U/g Hb, and six males had severe G6PD deficiency (<0.12-1.2 U/g Hb), while six males and 12 females had mild G6PD deficiency (>1.2-4.5 U/g Hb). Among the 24 G6PD-deficient subjects, 22 (92%) had the Mahidol 487G>A mutation (12 male hemizygotes, one female homozygote, and nine female heterozygotes), while the G6PD genotypes in two female subjects were unknown. HbE was identified in 39 subjects (20 males and 19 females), including 15 HbEE (seven males and eight females) and 24 HbAE (13 males and 11 females). Twenty-three subjects co-inherited both G6PD deficiency and HbE (22 with HbAE and one with HbEE). Whereas mean Hb levels were not significantly different between the HbA and HbE groups, G6PD-deficient males had significantly lower Hb levels than G6PD-normal males (P < 0.05, t-test). However, it is noteworthy that two G6PD-deficient hemizygous males with HbAE were severely anemic with Hb levels below 50 g/L. This study revealed high prevalence of co-inheritance of G6PD deficiency with HbAE in the Kachin ethnicity, and a potential interaction of the G6PD Mahidol 487G>A and HbAE in males leading to severe anemia. The presence of 6% males with severe G6PD deficiency raised a major concern in the use of primaquine for radical cure of vivax malaria.

Clinico-Haematological Features of Paroxysmal Nocturnal Haemoglobinuria.

The aim of this study was to determine the frequency of various clinico-haematological features in patients suffering from paroxysmal nocturnal haemoglobinuria (PNH). It was an observational study carried out from October 2008 - January 2016. All the patients of PNH, diagnosed on the basis of clinical and laboratory findings and confirmed by CD55 and CD59 deficiency on red cells by means of flow cytometry, were included in the study. A total of 22 patients were diagnosed which included 18 (81.8%) males and 4 (18.1%) females. Median age was 27 years. Pallor, fever, fatigability and haemoglobinuria were the most common clinical features. Pancytopenia was seen in 13 (59.09%) and hypocellular marrow was found in 14 (63.6%) patients. One patient presented with Budd Chiari syndrome.

Correlation of BACH1 and Hemoglobin E/Beta-Thalassemia Globin Expression.

OBJECTIVE: The diverse clinical phenotype of hemoglobin E (HbE)/ß-thalassemia has not only confounded clinicians in matters of patient management but has also led scientists to investigate the complex mechanisms involved in maintaining the delicate red cell environment where, even with apparent similarities of α- and ß-globin genotypes, the phenotype tells a different story. The BTB and CNC homology 1 (BACH1) protein is known to regulate α- and ß-globin gene transcriptions during the terminal differentiation of erythroid cells. With the mutations involved in HbE/ß-thalassemia disorder, we studied the role of BACH1 in compensating for the globin chain imbalance, albeit for fine-tuning purposes. MATERIALS AND METHODS: A total of 47 HbE/ß-thalassemia samples were analyzed using real-time quantitative polymerase chain reaction and correlated with age, sex, red blood cell parameters, globin gene expressions, and some clinical data. RESULTS: The BACH1 expression among the ß-thalassemia intermedia patients varied by up to 2-log differences and was positively correlated to age; α-, ß-, and γ-globin gene expression level; and heme oxygenase 1 protein. BACH1 was also negatively correlated to reticulocyte level and had a significant correlation with splenectomy. CONCLUSION: This study indicates that the expression of BACH1 could be elevated as a compensatory mechanism to decrease the globin chain imbalance as well as to reduce the oxidative stress found in HbE/ß-thalassemia.

C1q nephropathy and isolated CD59 deficiency manifesting as necrotizing crescentic glomerulonephritis: A rare association of two diseases.

C1q nephropathy is a recently described clinico-pathologic entity with a variable clinical presentation and pathology. Crescentic glomerulonephritis (GN) has been reported in only two patients in the available literature. CD59 deficiency, along with lack of CD55, is responsible for paroxysmal nocturnal hemoglobinuria (PNH). Few cases of isolated CD59 deficiency have been described with PNH-like features. A middle-aged adult male presented with rapidly progressive renal failure. Serological investigations were negative. A renal biopsy revealed necrotizing crescentic GN with rupture of Bowman's capsule. Immunofluorescence on the frozen sections showed dominant mesangial deposits of C1q along with IgM. Hematological work-up of the patient revealed isolated CD59 deficiency. Hence, a final diagnosis of C1q nephropathy and CD59 deficiency manifesting as crescentic GN and hemolytic anemia was made. The co-existence of two rare disorders, C1q nephropathy and CD59 deficiency, in a patient with necrotizing crescentic GN is described for the first time to the best of our knowledge. The pathogenetic link of these two entities with the clinical manifestation requires further study.

Global migration and the changing distribution of sickle haemoglobin: a quantitative study of temporal trends between 1960 and 2000.

BACKGROUND: Changes in the geographical distribution of genetic disorders are often thought to happen slowly, especially when compared with infectious diseases. Whereas mutations, genetic drift, and natural selection take place over many generations, epidemics can spread through large populations within a few days or weeks. Nevertheless, population movements can interfere with these processes, and few studies have been done of their eff ect on genetic disorders. We aimed to investigate the eff ect of global migration on the distribution of the sickle-cell gene-the most common and clinically significant haemoglobin structural variant. METHODS: For each country, we extracted data from the World Bank's Global Bilateral Migration Database about international human migrations between 1960 and 2000. We combined this information with evidence-based estimates of national HbS allele frequencies, generated within a Bayesian geostatistical framework, to analyse temporal changes in the net numbers of migrants, and classified countries with an index summarising these temporal trends. FINDINGS: The number of international migrants increased from 92.6 million in 1960, to 165.2 million in 2000. The estimated global number of migrants with HbS increased from about 1.6 million in 1960, to 3.6 million in 2000. This increase was largely due to an increase in the number of migrants from countries with HbS allele frequencies higher than 10%, from 3.1 million in 1960, to 14.2 million in 2000. Additionally, the mean number of countries of origin for each destination country increased from 70 (SE 46) in 1960, to 98 (48) in 2000, showing an increasing diversity in the network of international migrations between countries. Our index of change map shows a patchy distribution of the magnitude of temporal changes, with the highest positive and negative values scattered across all continents. INTERPRETATION: Global human population movements have had a substantial eff ect on the distribution of the HbS gene. Population movements can create a long-term burden on health-care systems. Our findings, which emphasise countries in which migration fluxes are changing the most, should increase awareness about the global burden of haemoglobinopathies and encourage policy makers to implement specific public health interventions, such as screening programmes and genetic counselling. FUNDING: Wellcome Trust, European Research Council, Bill & Melinda Gates Foundation, National Institute of Allergy and Infectious Diseases-National Institutes of Health, the Research and Policy for Infectious Disease Dynamics program, Fogarty International Center.

[Tropical malaria in children in the Republic of Tajikistan].

The complications of tropical malaria were noted in 25 (9.4%) of 196 pediatric patients followed up. These included cerebral malaria in 8 (3%), severe hemolytic anemia in 15 (5.7%), hemoglobinuric fever in 1 (0.37%), and malarial hepatitis in 1 (0.37%). The occurrence of complications was associated with the late referral of patients to a health care facility and untimely treatment, as well as with preliminary misdiagnoses (acute respiratory viral infection, typhoid-parathyphoid fever, meningitis, acute enteric infection, viral hepatitis, sepsis). The main reasons for late diagnosis were the absence of malarial paroxysm at the onset of disease in infants and the wrong type of a temperature curve.

Haemoglobinuria among children with severe malaria attending tertiary care in Ibadan, Nigeria.

BACKGROUND: Haemoglobinuria is one of the manifestations of severe malaria and results from severe intravascular haemolysis. Glucose-6-phosphate dehydrogenase (G6PD) deficiency has been implicated in its aetiology. Haemoglobinuria may be associated with severe anaemia and, less frequently, acute renal failure. METHODS: A prospective case-control study was carried out to determine the incidence of haemoglobinuria as confirmed by dipstick urinalysis, microscopy and spectrophotometric measurement, among children with severe malaria. A total of 251 children presenting at the Children's Emergency Ward with severe malaria were recruited over a period of 21 months. The G6PD status and the outcomes of severe malaria in children with and without haemoglobinuria was studied with respect to renal failure, the recurrence of haemoglobinuria and blood pressure changes over a three-month follow-up period. RESULTS: It was found that the incidence of haemoglobinuria among children with severe malaria is 19.1%. Children <5 years constituted 76.8% of all the study patients. Patients with haemoglobinuria had median age of 52.5 months, which was significantly higher than 35 months in patients without haemoglobinuria (p=0.001). Although, haemaglobinuria was commoner among boys (54.2%) than girls (45.8%), the difference was not statistically significant. There were no significant differences between children with and without haemoglobinuria regarding their nutritional status or parasite densities. Among the clinical features of the study patients, only jaundice was significantly associated with haemoglobinuria (p=0.0001). Renal failure occurred in three out of 48 children with haemoglobinuria and in none of the 203 without. There was not recurrence of haemoglobinuria in the follow-up period. At discharge, blood pressure was elevated in six children (one previously haemoglobinuric), but all returned to normal within the follow-up period. CONCLUSIONS: Haemoglobinuria was a prominent feature of severe malaria and it was significantly associated with jaundice at presentation. Haemoglobinuria was commoner in older children than younger children but not related to sex. G6PD deficiency was not an independent predictor of the occurrence or outcome of haemoglobinuria. Blood pressure was not affected by haemoglobinuria on admission nor during follow-up.

Renal disease in adult Nigerians with sickle cell anemia: a report of prevalence, clinical features and risk factors.

Renal abnormalities in adult Nigerians with sickle cell anemia (SCA) have not been extensively studied. To determine the prevalence, pattern and the associated risk factors of renal disease, 72 subjects with SCA from two centers in the southwestern Nigeria were investigated. Socio-demographic data, body mass index and clinical findings were documented. The urine analysis, serum bio-chemistry, hemogram and renal factors attributable to SCA were determined. Presence of albuminuria of at least 1+ or microalbuminuria in those negative with dipstick; and the estimated glomerular filtration rate (eGFR) using the Cockcroft-Gault formula categorized subjects to various stages of chronic kidney disease (CKD). Subjects with and without albuminuria were compared to determine the relative risk associated with renal disease. Four (5.6%) subjects had macro-albuminuria, while 32 (44.4%) had micro-albuminuria and 30 (41.7%) had hemoglobinuria. In the subjects with albuminuria, age, hematocrit, systolic blood pressure, serum creatinine, urea and creatinine clearance were numerically higher while the eGFR was numerically lower. There was no significant difference in the clinical parameters studied in the two groups of subjects. The diastolic blood pressure was significantly higher in the albuminuric group. Based on eGFR, 22 (30.6%) subjects had hyperfiltration (GFR > 140 mL/min/1.73 m2), of whom 36.4% had albuminuria, 18 (25.0%) had stage 1 CKD, 30 (41.7%) had stage 2 CKD and two (2.7%) subjects had stage 3 CKD with albuminuria. None had stage 4 and 5 CKD. We conclude that renal abnormalities, importantly albuminuria, is common in adult Nigerians with SCA and the pattern and incidence are similar to those reported from other parts of the world. Regular blood pressure monitoring, early diagnosis and active intervention are advocated to delay progression to end-stage kidney disease in view of poor outcomes of renal replacement therapy in SCA patients with nephropathy.

Renal impairment, hemoglobinuria, and hemoglobinemia among patients with idiopathic thrombocytopenic purpura.

Renal impairment (RI) and events potentially leading to RI were reported in idiopathic thrombocytopenic purpura (ITP) patients with specific medications. This study was conducted to estimate the incidence rate (IR) of RI, hemoglobinuria and hemoglobinemia (HE) and characterize baseline risk factors in ITP and ITP-free patients. Incident ITP and matched non-ITP patients were identified from an electronic medical record database from 1990 to 2002. ITP patients were classified by the treatment first received (initiators) or ever received (users). All cohorts were followed for study outcomes. IRs were calculated and standardized by age and gender. A total of 881 ITP and 4,496 ITP-free patients yielded 3,044 and 16,006 person-years, respectively. The ITP cohort had a slightly higher prevalence of autoimmune diseases and infections than the ITP-free cohort. The IR (/10,000 person-years) for RI, hemoglobinuria and HE was 14.2, 35.7, and 7.1 in the ITP cohort; 10.0, 48.8, and 0 in the ITP-free cohort; and 18.3, 37.1, and 6.1 in untreated ITP patients, respectively. The risk of RI, HE or hemoglobinuria was not found to differ substantially between ITP and non-ITP patients or across ITP treatments.

Glucose-6-phosphate dehydrogenase (G6PD) mutations and haemoglobinuria syndrome in the Vietnamese population.

BACKGROUND: In Vietnam the blackwater fever syndrome (BWF) has been associated with malaria infection, quinine ingestion and G6PD deficiency. The G6PD variants within the Vietnamese Kinh contributing to the disease risk in this population, and more generally to haemoglobinuria, are currently unknown. METHOD: Eighty-two haemoglobinuria patients and 524 healthy controls were screened for G6PD deficiency using either the methylene blue reduction test, the G-6-PDH kit or the micro-methaemoglobin reduction test. The G6PD gene variants were screened using SSCP combined with DNA sequencing in 82 patients with haemoglobinuria, and in 59 healthy controls found to be G6PD deficient. RESULTS: This study confirmed that G6PD deficiency is strongly associated with haemoglobinuria (OR = 15, 95% CI [7.7 to 28.9], P < 0.0001). Six G6PD variants were identified in the Vietnamese population, of which two are novel (Vietnam1 [Glu3Lys] and Vietnam2 [Phe66Cys]). G6PD Viangchan [Val291Met], common throughout south-east Asia, accounted for 77% of the variants detected and was significantly associated with haemoglobinuria within G6PD-deficient ethnic Kinh Vietnamese (OR = 5.8 95% CI [114-55.4], P = 0.022). CONCLUSION: The primary frequency of several G6PD mutations, including novel mutations, in the Vietnamese Kinh population are reported and the contribution of G6PD mutations to the development of haemoglobinuria are investigated.

Renal abnormalities in patients with sickle cell disease: a single center report from Saudi Arabia.

Patients with sickle cell disease (SCD) are at increased risk of serious morbidity and mortality. Renal abnormalities in SCD are well known but renal involvement in Saudi patients with SCD has not been studied. We sought to identify renal abnormalities in adolescent and adult Saudi patients with SCD. We prospectively studied 73 patients with SCD followed up at King Khalid University Hospital, Riyadh, Saudi Arabia from July 2005 to November 2006,. All patients underwent evaluation of kidney function and urine examination to detect proteinuria and other urinary abnormalities. In addition, 53 patients from the cohort had 24-hour urine collection to measure creatinine clearance and to quantitate proteinuria. The patient population consisted of 34 males (46.5%) and 39 females (53.5%) with a median age of 23 years (range 14-40). Proteinuria was present in 30 patients (41%). Creatinine clearance was low in 12 patients (22.5%) and seven of these patients had low or low-normal serum creatinine despite reduced creatinine clearance. Low serum creatinine was common and present in 28 patients (38%). Two patients had chronic renal failure and one of them is on regular dialysis. Other abnormalities detected include hematuria in seven patients (8.5%) and hemoglobinuria in 12 patients (14.5%). In conclusion, renal abnormalities are present in a significant number of Saudi patients with SCD and proteinuria is the most common abnormality. Serum creatinine may remain low or within low-normal range in SCD patients despite reduced creatinine clearance. As proteinuria is a risk factor for developing renal failure in future, routine screening of SCD patients is recommended for timely intervention in order to prevent or delay renal damage.

Hemoglobin E disease in North Indian population: a report of 11 cases.

Hemoglobin E (beta26Glu --> Lys) is the most common hemoglobin (Hb) variant in Southeast Asia and the second most prevalent worldwide. However in India, it is prevalent in Bengal and the north-eastern region, but relatively rare in the rest of the country. Identification of this Hb variant is important, because the doubly heterozygous state for HbE and beta-thalassemia is characterized clinically by thalassemia major, a situation different from other compound heterozygous states for structural beta-chain variants and beta-thalassemia. Thus, the affected individual may be symptomatic and transfusion dependent at an early age. This paper reports four cases with Hb E trait, three cases with hemoglobin E disease and another four cases with Ebeta-thalassemia. Laboratory investigations are based on RBC indices and high performance liquid chromatography (HPLC). A negative correlation has been found to exist between levels of HbA(2) and RBC indices including the MCV and MCH. A similar correlation has been seen between levels of HbF with Hb, RBC count, and MCV. The main aim is to increase the awareness of this relatively rare disorder, so that it can be included in the differential diagnosis of patients presenting clinically like thalassemia intermedia or thalassemia major. This awareness may also help in prenatal diagnosis, genetic counseling and clinical management. The clinical, hematological and laboratory features of this disorder are also discussed.

[Evolution of severe pediatric malaria in Togo between 2000 and 2002]. / Evolution du paludisme grave de l'enfant au Togo de 2000 à 2002.

The aim of this study was to analyse the clinical and evolutive aspects of severe malaria in hospitalised children in 2000, 2001, and 2002 in Togo. The study included 361 children in the pediatrics department of Lomé-Tokoin University hospital. All them received a 10% dextrose infusion, then an infusion of quinine or intramuscular artemether. Malaria accounted for 4.37% of all hospitalizations. Children aged 1 to 5 years were more affected (69.53%). The most frequent clinical forms were anaemia (55.7%) followed by cerebral manifestations. The frequency of hemoglobinuria increased (17.2%) as well as renal failure (3%) compared to previous years. Thirty-five children died (9.7%). Most of them presented with anaemia, neurological manifestations, or respiratory distress. Neurological sequels were present in 2.2% of patients.

Urinary schistosomiasis on Zanzibar: application of two novel assays for the detection of excreted albumin and haemoglobin in urine.

As part of a urinary schistosomiasis control programme on Zanzibar, an aged cross-sectional survey of 305 children from three schools on Unguja was conducted to investigate the relationships between levels of excreted albumin and haemoglobin in urine and Schistosoma haematobium infection status. Diagnosis was determined by standard parasitological methods, dipstick reagents for microhaematuria, visual inspection for macrohaematuria as well as collection of case-history questionnaire data for self-diagnosis. Prevalence of infection as determined by parasitology was 53.9% and approximately, one quarter of the children examined were anaemic (<11 g dl(-1)). A statistically significant negative association of blood haemoglobin levels of boys and S. haematobium infection intensity status was observed (rs=-0.23, P=0.005). Through sensitivity analysis of urine-albumin values it was determined that a concentration of above >40 mg l(-1), as measured with the HemoCue urine-albumin photometer, had sensitivity, specificity, positive and negative predictive values of 0.90, 0.83, 0.86 and 0.89 respectively against 'gold-standard' parasitology. There was a clear association of reported pain upon micturition for children with elevated urine-albumin levels, with an odds ratio of 20 to 1. Levels of excreted blood in urine were quantified with the HemoCue Plasma/Low Hb photometer. However, dipsticks remain the method of choice for urine-haemoglobin of 0.1 g l(-1) and below. Urine parameters over a 24-h period were assessed in a small sub-sample. Reductions in both albumin and haemoglobin excretion were observed in 11 children 54 days after praziquantel treatment. It was concluded that these rapid, high-through-put, portable HemoCue assays could play a role in better describing and monitoring the occurrence, severity and evolution of urinary schistosomiasis disease. The urine-albumin assay has particular promise as a biochemical marker of S. haematobium induced kidney- and upper urinary tract-morbidity.

Long-term results of heart valve replacement with the Edwards Duromedics bileaflet prosthesis: a prospective ten-year clinical follow-up.

OBJECTIVE: The Edwards Duromedics valve (Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.) was designed with a self-irrigating hinge mechanism to reduce thromboembolic complications. After good initial clinical results, distribution was suspended in 1988 after reports of valve fracture after 20,000 valves had been implanted. The manufacturer conducted extensive studies to improve the Edwards Duromedics and reintroduced a modified version, which is available as Edwards Tekna. The purpose of the study was the evaluation of long-term results of the original Edwards Duromedics that might be important for the current version, the Edwards Tekna valve. METHODS: A prospective clinical 10-year follow-up was performed of 508 patients who underwent valve replacement with the Edwards Duromedics valve in the aortic (n = 268), mitral (n = 183), and aortic and mitral (n = 56) position. RESULTS: The perioperative mortality rate was 6.9%; follow-up was 98% complete, comprising 3648 patient-years for a mean follow-up of 86 months (range: 33 to 144 months). The actuarial freedom from complications at the 10-year follow-up and the incidence rate (percent per patient-year) were as follows: late mortality rate, 69.2% +/- 2.4% (3.5% per patient-year); thromboembolism, 90.7% +/- 1.6% (0.96% per patient-year); anticoagulation-related hemorrhage, 87.7% +/- 1.7% (1.34% per patient-year); prosthetic valve endocarditis, 96.7% +/- 0.09% (0.38% per patient-year); valve-related mortality rate, 89.3% +/- 1.6% (1.21% per patient-year); valve failure, 86.2% +/- 1.85% (1.54% per patient-year); and valve-related morbidity and mortality rate, 71.1% +/- 2.3% (3.2% per patient-year). Three leaflet escapes were observed (one lethal, two successful reoperations; 99.1% +/- 0.05% freedom, 0.08% per patient-year). All patients functionally improved (86% in New York Heart Association classes I and II), and incidence of anemia was insignificant. CONCLUSIONS: These results confirm that the Edwards Duromedics valve shows excellent performance concerning thromboembolism, hemolysis, and functional improvement and will serve as a reference for the last version, the Edwards Tekna valve, where comparable long-term data are currently not available.