Reversal of stage 5 chronic kidney disease by aortic valve replacement in kidney transplant recipient: a case report.

BACKGROUND: Cardiorenal syndrome (CRS) is a group of pathophysiological disorders affecting heart and kidneys. CASE PRESENTATION: We present 44-year-old kidney transplant recipient with acute-on-chronic graft failure in the course of CRS due to acutely decompensated heart failure associated with severe aortic regurgitation successfully treated with aortic valve replacement. Because of graft failure progression and difficult to eradicate infections he was treated with dialysis and radical minimization of immunosuppression. After 74 days of renal replacement therapy the patient regained graft function after successful aortic valve replacement. The dialysis could be stopped and immunosuppressive therapy was reintroduced. Heart and renal function are stable and patient is doing well without dialysis for 3 years. CONCLUSIONS: The return of kidney graft function can occur even after a long period of dialysis therapy due to improved cardiovascular function. Therefore, distinguishing an acute-on-chronic CRS subtype is mandatory to enable specific patient approach.

Lack of Impact of Presence of Positive C4d Staining in Capillaries in Myocardial Biopsies on Long-term Survival of Heart Transplant Patients.

BACKGROUND: The long-term survival of 209 consecutive patients (mean age, 46 ± 15 years) from a single center with ≥1 diagnostic myocardial biopsy after heart transplantation was analyzed. METHODS: Patients were considered as C4d positive if a capillary staining (immunohistochemistry in paraffin samples) was observed in ≥1 myocardial biopsy. Data were analyzed according to pathologic consensus of antibody mediated rejection definition of C4d+ positivity: 2004 definition in group A and the 2013 definition in group B and compared with their respective controls, composed of patients who do not meet those criteria. Age, follow-up time, and number of biopsies were comparable between patients with C4d+ and controls in both groups. Follow-up was 100% complete with mean of observation time 2143 days. RESULTS: During the follow-up period, 62 patients died (group A: C4d+ 32% vs controls 29%; group B: C4d+ 36% vs controls 29% [P = NS]). There were no differences in survival between patients with positive staining and without C4d+ staining when Kaplan-Meier survival curves were compared. CONCLUSIONS: The presence of C4d positive staining in myocardial capillaries of heart biopsies of patients after heart transplantation, as an isolated finding, was not related to worse long-term survival.

Analysis of specificity of anti-human leukocyte antigen antibodies in kidney recipients in reference to clinical outcome.

BACKGROUND: Anti-human leukocyte antigens antibodies (HLA) are not always the main cause of graft injury but can be a marker of immune response to the graft. The aim of this study was to analyze anti-HLA specificities with the use of the most sensitive detection method (Luminex) in reference to clinical condition. METHODS: Sera of 65 kidney recipients (n = 443) were screened with the use of the mixed LABScreen kit, and, for 47 recipients, sera with maximal normalized background ratio (NBG) were subjected to specificity testing. NBG, numbers of specificities, donor-specific antibodies (DSA), and normalized mean fluorescence index (nMFI) of DSA and maximal anti-HLA were analyzed in reference to clinical (acute rejection [AR] diagnosis, immunosuppression), histopathological (C4d staining, chronic allograft nephropathy, AR type), and laboratory parameters (creatinine). RESULTS: We observed 1 to 51 specificities, class I DSA in 26.7%, class II in 10%, and estimated DQ-DSA in 63.3% of tested patients. Patients with AR and humoral AR had significantly higher NBG, number of anti-HLA class I, DQ and DQ-DSA types, and more frequently had anti-HLA and class II DSA-positive sera (P < .052). C4d staining was associated with higher anti-HLA class I (P = .053) and class I DSA (P = .002) type numbers, and maximal anti-HLA nMFI (P = .036) and was more frequent in AR (P = .048) and class II DSA positive patients (P = .046). Patients with chronic allograft nephropathy showed higher DQ-DSA-nMFI (P = .036). DQ-DSA-nMFI and maximal anti-HLA-nMFI correlated with creatinine increase (Spearman range [SR] = 0.64, SR = 0.41). Together with NBG, maximal class I and class II anti-HLA-nMFI correlated with the number of transplantation and maximal panel-reactive antibodies ratio (SR = 0.19-0.40). CONCLUSIONS: Anti-HLA detection allows for humoral AR diagnosis but also for identification of patients with risk of any rejection. However, clear rules of anti-HLA interpretation and studies on their clinical impact are needed.

Graft infection in kidney recipients and its relation to transplanted kidney function.

INTRODUCTION: Following kidney transplantation, septic complications are the leading causes of therapeutic failure including recipient death or graft removal. The serum creatinine level is one of the earliest metrics of kidney metabolic function. We examined the influence of graft infection on serum creatinine levels in kidney recipients. STUDY DESIGN: We analyzed the function of 220 kidneys transplanted in nine centers in Poland. The kidneys were recovered from 146 multiorgan donors. Donor urea and creatinine levels were within the normal range. We investigated the influence of perioperative graft infection incidence on recipient creatinine levels at 1, 2, 3, 7, 14, 30, 90, and 180 days after kidney transplantation. The association of the serum creatinine level with categorical variables was assessed using either Student t test analysis of variance and multivariate techniques. In all analyses P<.05 indicated statistical significance. RESULTS: There were 25 graft infections revealing a significant relationship with increased recipient serum creatinine level after kidney transplantation (P=.003). Multivariate analysis confirmed the impact of infection. CONCLUSION: Perioperative kidney graft infection influenced graft funtion in the early and late periods post-transplantation.

Experience with autosomal dominant polycystic kidney disease in patients before and after renal transplantation: a 7-year observation.

OBJECTIVE: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the presence of multiple cysts in both kidneys. Symptoms of the disease may arise either from the presence of cysts or from increasing loss of kidney function. First symptoms usually appear in the third decade of life: lumbar pain, urinary tract infections, arterial hypertension, or renal colic due to cyst rupture or coexistent nephrolithiasis. An early diagnosis, male gender, large kidneys by sonography, arterial hypertension, hematuria, and urinary tract infections are predictive factors of a faster progression of the disease. Our aim was to establish the indications for nephrectomy among symptomatic ADPKD patients before kidney transplantation and to assess the risks of posttransplantation complications among ADPKD patients without nephrectomy. PATIENTS AND METHODS: The observed group consisted of 183 patients with ADPKD among whom 50 (27.3%) underwent kidney transplantation during a 7-year observation period (2000-2007). Among those subjects were 3 groups: (I) nephrectomy preceding transplantation; (II) nephrectomy during kidney transplantation; and (III) without nephrectomy. RESULTS: Among group I before transplantation we observed: arterial hemorrhage, wound infections, and splenectomy 4 weeks after ADPKD nephrectomy; afterward we observed: urinary tract infections and contralateral cyst infection. Among group II we only observed 1 case of wound infection. Among group III we observed: ascending urinary tract infections, cyst infections, and cyst hemorrhage. Cyst hemorrhage and cyst infections led mainly to ADPKD kidney nephrectomy. During the observation time, 80.95% of grafts were functioning. CONCLUSIONS: Unilateral nephrectomy is a well-founded preliminary surgical treatment before kidney transplantation. Bilateral nephrectomy before or during transplantation eliminates ADPKD complications and does not significantly increase general complications. The greatest numbers of complications and of graft losses were observed among the group without pretransplantation nephrectomy.

Membranous glomerulonephritis may be associated with heavy marijuana abuse.

It is well documented that drug abuse can cause renal diseases. Nephropathy and proteinuria among heroin addicts has been recognized since the early 1970s. The predominant lesions in heroin-associated nephropathy are segmental glomerulosclerosis in African-Americans and membranous glomerulonephritis (MGN) in the Caucasian population. Cocaine may induce kidney damage, predominantly acute renal failure in the course of rhabdomyolysis. However, there are no case reports of nephropathy associated with marijuana smoking. We report a case of a marijuana-addicted 27-year-old Caucasian man after cadaveric kidney transplantation who developed de novo posttransplant MGN. The long period and high level of narcotic intoxication suggested that de novo MGN may have been associated with heavy marijuana abuse.

Purine and cytokine concentrations in the renal vein of the allograft during reperfusion.

The impairment of organ function derived from ischemia-reperfusion injury is still an important problem in solid organ transplantation. Cell alterations induced by ischemia prime the tissue for subsequent damage during the reperfusion phase. The aim of present study was to examine the association between changes in cytokine and purine metabolite concentrations in graft renal vein during reperfusion. The study included 17 recipients of cadaveric renal grafts: 10 men and seven women of overall mean age of 49 +/- 7 years and cold ischemia time 25 +/- 3 hour. The levels of interleukin (IL)-1beta, IL-2, IL-4, IL-6, IL-10, interferon (INF)-gamma, tumor necrosis factor (TNF)-beta, and TNF-alpha in renal graft vein plasma during 5 first minutes of reperfusion were quantified by flow cytometry. Increased concentrations of IL-6, TNF-alpha, and IL-1beta were observed during reperfusion. The IFN-gamma concentrations correlated negatively with xanthine (Xan) concentrations in renal vein blood during reperfusion, whereas there was a positive correlation between IL-2 and Xan concentrations. Moreover, the concentrations of IL-6 and IL-10 correlated negatively with hypoxanthine concentrations, and the concentrations of IL-4 also correlated negatively with Xan concentrations. The results of this study indicated the enhanced release of some cytokines during kidney graft reperfusion. It occurred in association with release of purine metabolites-the markers of energy status of renal tissue. Therefore, the enhanced cytokine production during reperfusion might influence ischemia-reperfusion injury and the early graft function.

Circulating adhesion molecules during kidney allograft reperfusion.

Adhesion molecule expression is an important event during early transplant failure. The aim of the present study was to examine the release of adhesion molecules during the first minutes of kidney allograft reperfusion in relation to delayed graft function and acute graft rejection. We enrolled 49 renal transplant recipients, including 13 cases of delayed graft function (DGF) and 11 cases of acute graft rejection (AR). Plasma concentrations of E-selectin, VCAM-1 and ICAM-1 after 3 min of reperfusion were significantly higher than in the iliac vein before reperfusion. There was no statistically significant difference between patients with and without DGF as regards E-selectin, VCAM-1 and ICAM-1 concentrations in the iliac vein before and in the renal vein after 3 min of reperfusion. Concentrations of adhesion molecules in the iliac vein before reperfusion and in the renal vein after 3 min of reperfusion did not differ significantly between patients with and without AR except for ICAM-1 iliac vein concentration which was significantly increased in AR patients. Plasma levels of E-selectin, ICAM-1 and VCAM-1 were increased after kidney allograft reperfusion. Moreover, elevated serum levels of ICAM-1 before transplantation correlated with subsequent acute kidney allograft rejection. The results suggest that elevated ICAM-1 levels may be implicated in acute graft rejection.

Laparoscopic removal of renal cysts in patients with ADPKD as an alternative method of treatment and patient preparation for kidney transplantation: preliminary results.

BACKGROUND: The most frequent genetic disease of the kidneys occurring in 1 of 1000 inhabitants is autosomal-dominant polycystic kidney disease (ADPKD). Growing renal cysts compress the kidney resulting in damage to parenchyma and functional disorders. Around 10% of these patients are dialyzed due to terminal renal insufficiency. With the advent of laparoscopic techniques, the idea of laparoscopic excision of cysts seemed a tempting alternative to nephrectomy. We assessed the preliminary results of laparoscopic treatment of polycystic kidneys compared with open nephrectomy for patients with ADPKD. MATERIALS AND METHODS: Thirty ADPKD patients were treated between 2000 and 2004. Eleven procedures in five men and six women of mean age 51 years included laparoscopic cyst excisions. In the remaining 19 patients (six men and 13 women) of mean age 54 years, nephrectomy was done. Indications for surgery included pain due to compression by large cysts and cyst contamination. Patients after nephrectomy were prepared for renal transplantation when necessary. RESULTS: Laparoscopic polycyst removal produced better effects than nephrectomy. Mean operative time was significantly shorter (86 minutes for cyst removal vs 108 minutes for nephrectomy; P < .05). Postoperative pain measured with the VAS scale was reduced in patients after laparoscopy. Hospital stay was shorter (5 vs 9 days), as well as time to recovery. Other benefits of laparoscopic cyst removal included maintained urination in the patient and no need for erythropoietin substitution, as well as reduced risk of cyst contamination. When eligible for renal transplantation, patients after laparoscopic polycyst removal have smaller kidneys that do not interfere with the graft and the risk of infection during immunosuppression seems lower. CONCLUSION: Although larger series of patients are required in patients with ADPKD, laparoscopic polycyst removal seemed superior to early nephrectomy.

Hypoxanthine as a graft ischemia marker stimulates catalase activity in the renal vein during reperfusion in humans.

BACKGROUND: The impairment of organ function derived from ischemia-reperfusion injury is still an important problem in solid organ transplantation. Cell alterations induced by ischemia prime the tissue for subsequent damage occurring during the reperfusion phase. Purine nucleotides and oxypurines are products of adenine nucleotide degradation. Reperfusion and reoxygenation are characterized by great production of reactive oxygen species and free radicals. On the contrary, superoxide dismutase, catalase, glutathione, and glutathione peroxidase are involved in protecting against free radicals. The aim of the study was to examine the correlation between concentrations of ischemia markers (hypoxanthine or inosine) and the activity of erythrocyte superoxide dismutase, catalase, or glutathione peroxidase. PATIENTS AND METHODS: The study included 40 renal transplant recipients. Before anastomosis of the kidney vessels with the recipient's iliac vessels, a "0" blood sample was taken from the iliac vein. Then, after anastomosis, the renal vein of the graft was cannulated and blood samples I, II, and III were obtained. The reperfusion of the transplanted kidney was measured with a thermovision camera ThermaCAM SC500. RESULTS: The plasma concentrations of hypoxanthine and inosine increased in statistically significant fashion immediately after total tissue reperfusion (P < .0001). Catalase activity at 4 minutes after total tissue reperfusion correlated positively with hypoxanthine concentrations immediately after total tissue reperfusion (Rs = +0.49), 2 minutes after total tissue reperfusion (Rs = +0.47), and 4 minutes after total tissue reperfusion (Rs = +0.46). There were no statistically significant correlations between hypoxanthine or inosine concentrations or superoxide dismutase or glutathione peroxidase activities. CONCLUSIONS: The results of the present study suggest that catalase activity may correlate with the concentration of hypoxanthine in the graft renal vein and other mediators of oxidative stress.

Activity of CuZn-superoxide dismutase, catalase and glutathione peroxidase in erythrocytes in kidney allografts during reperfusion in patients with and without delayed graft function.

BACKGROUND: Generation of reactive oxygen species (ROS) is the main mechanism involved in the ischemic/reperfusion damage of the transplanted organ. Oxygen burst is a trigger for complex biochemical events leading to generation of oxygenated lipids and changes in microcirculation. Many markers have been researched to prove the presence of ROS in the transplanted tissue. Some of them, like superoxide dismutase (SOD), catalase (CAT), and glutathione peroxidase (GPx) are considered to play a major role in graft protection against oxygen stress during reperfusion. METHODS: The aim of this study was to examine the changes of SOD1, CAT and GPx activity in erythrocytes during the first minutes after total graft reperfusion. Forty patients undergoing kidney transplantation at our center were assigned to two groups: with or without delayed graft function (DGF). Before anastomosing kidney vessels with recipient's iliac vessels, the '0' blood sample was taken from the iliac vein. Next blood samples I, II and III were taken from the graft's renal vein. The reperfusion of the transplanted kidney was evaluated precisely with the thermovision camera. Erythrocyte SOD1, CAT and GPx activity was measured with a spectrophotometric method. RESULTS: We did not observe statistically significant changes in SOD1, CAT and GPx activity in erythrocytes during the early phase of reperfusion in patients with and without DGF. CONCLUSIONS: Erythrocyte-antioxidative system in graft's vein remain stable during the early phase of reperfusion. The results of the study suggest that further studies on extracellular enzymes are required for the assessment of antioxidant system in the conditions of ischemia/reperfusion.

Impact of presence of HBs antigen and anti-hepatitis C virus and anti-cytomegalovirus antibodies on transplanted kidney survival.

INTRODUCTION: Infections are one of the most common complications after organ transplantation. Viral infections such as hepatitis type B (HBV) and C (HCV) or cytomegalovirus (CMV) infections are among the most serious ones. A high frequency of HBV and HCV infections has been recognized in kidney recipients. Viral infections play a special role in graft recipients because of clinical symptoms influencing graft function and recipient survival. Immunosuppressive treatment to decrease immunological reactions after organ transplantation may increase the risk of viral infections. The aim of this study was to evaluate the impact of the presence of HBs antigen and HCV and CMV antibodies on patient and graft survivals. MATERIAL AND METHODS: Two hundred one enrolled kidney transplantation patients (96 women and 105 men) were treated with the same immunosuppressive regimen. Age, sex, and viral state (HBs antigen, anti-HCV and anti-CMV antibodies) were evaluated in every patient. Statistical analysis was performed with the Gompertz model, Kaplan-Meier curves and Cox proportional hazard tests. RESULTS: The presence of HBs antigen was detected in 161 patients (20.4%), HCV antibodies in 61 recipients (30.3%); and CMV antibodies in 12 patients (5.9%). Eighty-seven recipients (43.4%) were seronegative. Average recipient age was 38.5 years. CONCLUSION: Time of graft function was independent of the presence of HBs antigen or HCV or CMV antibodies.

Frequencies of the common promoter polymorphisms in cytokine genes in a Polish population.

Common polymorphisms in cytokine genes, widely distributed in many populations, may affect gene transcription, leading to individual variations in cytokine levels. Some of those polymorphisms were associated with the risk of graft rejection or autoimmune and parasitic diseases development as well as treatment outcome. In the current study the frequencies of promoter polymorphisms in genes encoding interleukin-2 (-330 G/C), interleukin-4 (-590 C/T), interleukin-6 (-174 G/C), interleukin-10 (-1082 G/A, -592 C/A) and TNFalpha (-307 G/A) have been studied in a sample of 205 unrelated healthy Polish subjects of Caucasian origin. The frequencies of the determined cytokine alleles were as follows: interleukin-2 (-330 G/C): T -64.9%, G -35.1%; interleukin-4 (-590 C/T): C -79.8%, T -20.2%; interleukin-6 (-174 G/C): G -53.4%, C -46.6%; interleukin-10 (-1082 G/A, -819 G/T, -592 C/A): GCC -44.1%, ACC -32.0, ATA -23.9%; TNFalpha (-307 G/A): G -85.1%, A -14.9%. We also compared our results with other populations studied to date, describing some significant differences.

Cyclosporine and sirolimus interaction in a kidney transplant patient.

Cyclosporin A and sirolimus are becoming commonly used in immunosuppressive treatment in organ transplant patients. The drugs are both metabolized by cytochrome P450 3A4 and are substrates of P-glycoprotein. Thus, interaction between these drugs is possible. The case reported here illustrated that clinicians should be aware of this important drug-drug interaction.

IL-2 and TNF-alpha promoter polymorphisms in patients with acute kidney graft rejection.

INTRODUCTION: Proinflammatory cytokines have been implicated in the pathogenesis of acute kidney allograft rejection. The aim of the study was to examine the association between interleukin (IL)-2 -330 and tumor necrosis factor (TNF)-alpha -308 promoter polymorphisms and acute kidney allograft rejection. METHODS: The study included 72 patients with long-term stable graft function, and 57 diagnosed with acute kidney allograft rejection. RESULTS: Patients with acute kidney allograft rejection showed a prevalence of subjects with TNF-alpha T2 allele (P < .05). The risk of acute kidney allograft rejection diagnosis was 2.5-fold greater among carriers of the T2 allele than those homozygous for T1T1 (OR 2.53, 95% CI 1.19 to 5.37, P < .05) There was no statistically significant difference in the distribution of IL-2 genotypes between patients with stable graft function and acute kidney allograft rejection. CONCLUSION: The results suggest that TNF-alpha-308 promoter polymorphism is a risk factor for acute kidney allograft rejection.

Experiences in kidney transplantation with duplicated ureters.

INTRODUCTION: The purpose of this study was to evaluate the complications of duplicated ureters in renal transplant recipients. METHODS: Between 1983 and 2004, 12 patients (median age 34 years) received renal transplants from donors with duplicated ureters. In four patients the ureter to bladder anastomoses were performed separately according to the method described by MacKinnon, including two cases transplanted with ureteral catheters because of narrow widths. In the following cases of eight duplicated ureters an anastomosis was performed between the distal part of each ureter to form a common ureteral ostium, which was connected to the urinary bladder. A ureteral catheter was used to the splint ureterovesical anastomosis. RESULTS: No graft loss to ureteral complications was observed. There was no ureteral necrosis in the postoperative period. No clinical symptoms of ureteral junction obstruction were revealed after removing the ureteral catheter. By ultrasound examination four patients showed a slight temporary pyelocaliectasis was observed and four patients developed temporary urinary fistulas. CONCLUSION: Our ureterocystoneostomy procedures with duplicated ureters were safe and useful in kidney transplantation.

The cytokine gene polymorphisms in patients with chronic kidney graft rejection.

Chronic allograft rejection remains an important cause of morbidity after kidney transplantation. The aim of the study was to examine the association between IL-2, IL-6 and TNF-alpha promoter polymorphisms and chronic kidney allograft rejection. The study included 64 patients with long-term stable graft function and 62 with chronic allograft nephropathy. Among patients with chronic allograft nephropathy a statistically significant prevalence of the IL-6 CC genotype associated with low IL-6 expression was observed (p < 0.01, OR 3.18; 95% CI 1.27-8.15). There were no statistically significant differences in distribution of IL-2 and TNF-alpha genotypes between patients with stable graft function and chronic allograft rejection. The results of present study suggest that the genetically determined low IL-6 production may be the risk factor of chronic allograft nephropathy development.

Pretransplant nephrectomy in patients with autosomal dominant polycystic kidney disease.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease and a frequent cause of end-stage renal failure. Transplantation in patients with ADPKD is associated with specific cyst-related problems, especially urinary tract infections (UTI). Although pretransplant nephrectomy has been applied in this group of patients, evidence of the benefits of this strategy is lacking. Therefore, we compared the outcomes and posttransplant complications among patients with or without pretransplant nephrectomy. PATIENTS AND METHODS: ADPKD patients (73) transplanted from cadaveric donors were reviewed retrospectively with regard to posttransplant complications and outcomes. The groups either underwent pretransplant unilateral nephrectomy (n = 30) or were transplanted with native kidneys intact (n = 43). RESULTS: Two patients underwent simultaneous bilateral nephrectomy due to a large size of the polycystic kidneys interfering with the transplant operation. Overall postransplant complications were more frequent in the group without nephrectomy (34% vs 20%); however, the difference was not statistically significant. Most complications were related to cyst infections with 3 deaths (12%) due to lethal septicemia in the group without nephrectomy. No infection-related deaths were noted in the group with pretransplant nephrectomy. CONCLUSIONS: Graft and patient outcomes as well postransplant complications were similar in both groups, independent of previous nephrectomy. It seems that pretransplant unilateral nephrectomy should not be routine and has no advantage over transplantation with both native kidneys intact, although this conclusion is limited by the small number of patients. An Individualized approach should be applied especially when there has been a history of cyst-related infection.

The FcgammaRIIa polymorphism in patients with chronic kidney graft rejection.

The FcgammaRIIa receptors, which provide a crucial link between cellular and humoral components of the immune response, display allelic polymorphism. Individuals are homozygous for either arginine 131 (RR131) or histidine 131 (HH131) or are heterozygous for these two alleles (RH131). The HH131 genotype binds human IgG2 with high RR131 with low, and RH131 with intermediate affinity. The aim of the study was to evaluate the FcgammaRIIa polymorphism in patients with chronic kidney graft rejection. The study included 121 renal transplant recipients: 53 patients with long-term stable graft function and 68 with chronic allograft rejection. The distribution of FcgammaRIIa genotypes in patients with chronic kidney graft rejection did not differ significantly from that in patients with stable graft function. The results suggest that the FcgammaRIIa polymorphism is not an important genetic risk factor for chronic rejection of kidney allografts.

The role of echocardiography in diagnosing double chambered right ventricle in adults.

OBJECTIVE: To evaluate the utility of echocardiography in diagnosing double chambered right ventricle (DCRV) in adults. DESIGN: A retrospective study. SETTING: Department of Congenital Heart Diseases and Department of Cardiac Surgery, Institute of Cardiology, Warsaw, Poland. PATIENTS: 32 patients, mean age 32.5 years, 21 female, and 11 male. INTERVENTION: Transthoracic (TTE) and transoesophageal (TOE) echocardiographic examination in patients with suspected DCRV. MAIN OUTCOME MEASURES: Direct inspection during surgical treatment of 28 patients diagnosed as having DCRV as an isolated lesion or associated with other pathologies. RESULTS: Echocardiography allowed the final diagnosis of DCRV in 26 patients (81%) out of 32 studied. TTE was diagnostic in 5 (15.6%) whereas TOE was diagnostic in 21 of 21 studied by this technique. Of 6 patients with negative TTE, DCRV was identified by cardiac catheterisation in 3 and directly during surgery in the remaining 3. Of 26 patients diagnosed by echocardiography, the anomalous muscle bundle was discrete in 20 (77%) and diffuse in 6 (23%). In 23 patients (88%) right ventricular outflow obstruction was localised low in the right ventricle, and in the remaining 3 (11.5%), the obstruction was localised high in the right ventricle. Abnormal bundles localised high were discrete, bundles localised in the lower part of right ventricle were discrete in 17 (74%) and diffuse in the remaining 6 (26%). DCRV was an isolated lesion in only 2 patients (6.2%). In all subjects surgical inspection confirmed echocardiographic data. CONCLUSIONS: Echocardiography was very useful to diagnose DCRV in adults as well as to identify its anatomic type. Comparing two different approaches, TOE better defines the entire scope of pathology, including estimation of the resultant systolic pressure gradient within the right ventricular cavity.