Left Adrenal Vein Research Articles

Dexmedetomidine for anaesthetic management of phaeochromocytoma in a child with von Hippel-Lindau type 2 syndrome.

Phaeochromocytoma is a rare neoplasm in the paediatric population with fewer than 20% of diagnosed cases.[1] We describe the use of dexmedetomidine infusion for successful management of an 11-year-old male child with von Hippel-Lindau type 2 syndrome (VHL), who underwent bilateral adrenalectomy for phaeochromocytoma.

A review of the anatomy and clinical significance of adrenal veins.

The adrenal veins may present with a multitude of anatomical variants, which surgeons must be aware of when performing adrenalectomies. The adrenal veins originate during the formation of the prerenal inferior vena cava (IVC) and are remnants of the caudal portion of the subcardinal veins, cranial to the subcardinal sinus in the embryo. The many communications between the posterior cardinal, supracardinal, and subcardinal veins of the primordial venous system provide an explanation for the variable anatomy. Most commonly, one central vein drains each adrenal gland. The long left adrenal vein joins the inferior phrenic vein and drains into the left renal vein, while the short right adrenal vein drains immediately into the IVC. Multiple variations exist bilaterally and may pose the risk of surgical complications. Due to the potential for collaterals and accessory adrenal vessels, great caution must be taken during an adrenalectomy. Adrenal venous sampling, the gold standard in diagnosing primary hyperaldosteronism, also requires the clinician to have a thorough knowledge of the adrenal vein anatomy to avoid iatrogenic injury. The adrenal vein acts as an important conduit in portosystemic shunts, thus the nature of the anatomy and hypercoagulable states pose the risk of thrombosis.

V4-08 LAPAROENDOSCOPIC SINGLE-SITE (LESS) ADRENALECTOMY IN MORBIDLY OBESE PATIENT WITH CUSHING SYNDROME

You have accessJournal of UrologyAdrenal/Robotics1 Apr 2014V4-08 LAPAROENDOSCOPIC SINGLE-SITE (LESS) ADRENALECTOMY IN MORBIDLY OBESE PATIENT WITH CUSHING SYNDROME Aly Abdel-Karim, Elsaid Yehia, and Salah Elsalmy Aly Abdel-KarimAly Abdel-Karim More articles by this author , Elsaid YehiaElsaid Yehia More articles by this author , and Salah ElsalmySalah Elsalmy More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2014.02.1541AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail Introduction and Objectives LESS has been described as an alternative minimally invasive treatment option for different urologic pathologies. However, LESS is still has limitations with strict patients selection criteria. We present LESS adrenalectomy for a morbidly obese patient with Cushing syndrome and relatively large left adrenal mass. Methods We present 32 years old female patient who was diagnosed clinically, biochemically and radiologically as Cushing syndrome. Computed tomography (CT) of the abdomen and pelvis showed 6.6 X 5.8 X 4.5 cm left adrenal tumor. BMI of the patient was 42. While the patient in the modified flank position, LESS left adrenalectomy was done using Covedien port that as placed at the umbilicus through 2.5- cm skin incision. During LESS adrenalectomy we used both articulating and straight instruments as well as 5-mm EndoEye camera. Dissection started by medial reflection of the left descending colon. The left adrenal vein was identified and clipped and en-bloc dissection of the adrenal gland and the tumor was done using both blunt and sharp dissection. Finally the gland was retrieved in an Endo-bag through the port site. Results The operative time was 180 minutes. Blood loss was 200 c.c and there was no blood transfusion. There was no conversion to open surgery or conventional laparoscopy. No extra-port was added. There were no intraoperative or postoperative complications. The postoperative umbilical incision after retrieval of the tumor was 3.5 cm. Hospital stay was 3 days. Visual Analogue Pain Scale at discharge was 2. The patient required only non-steroidal anti-inflammatory as postoperative analgesics. Histopathology confirmed the diagnosis of adrenal cortical adenoma. Conclusions LESS adrenalecomy is feasible and safe option for morbidly obese patients with Cushing syndrome. Although technically difficult, the procedure has low morbidity and high patient satisfaction. © 2014FiguresReferencesRelatedDetails Volume 191Issue 4SApril 2014Page: e553 Advertisement Copyright & Permissions© 2014MetricsAuthor Information Aly Abdel-Karim More articles by this author Elsaid Yehia More articles by this author Salah Elsalmy More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Management of Primary Aldosteronism: Should Adrenal Gland Computed Tomography be Replaced by Adrenal Venous Sampling?

Objectives: To evaluate the accuracy of computed tomography (CT) in the management of primary aldosteronism by comparing the concordance between CT and adrenal venous sampling (AVS), and the feasibility of AVS as a standard component in the diagnostic workup. Methods: From January 2009 to December 2012, 44 instances of AVS performed in 39 patients in Kwong Wah Hospital, Hong Kong were reviewed. All these patients had undergone CT of the adrenal glands before AVS. CT findings, and aldosterone and cortisol profiles of the left adrenal vein, right adrenal vein, and inferior vena cava during AVS were compared. Successful cannulation was defined by achieving an adrenal vein to the inferior vena cava cortisol level ratio of higher than 1.1. The positive predictive value of CT and concordance between CT and AVS were calculated. The pathology reports of patients who underwent adrenalectomy were reviewed. Result: AVS was successful in 35 (90%) of the patients on the first attempt, and concordance between CT and AVS was observed in 23 (59%) patients. CT yielded a positive predictive value of 73% and 44% in unilateral disease and bilateral disease, respectively. A total of 27 patients with unilateral disease underwent adrenalectomy. Pathology confirmed cortical adenoma in all specimens. Conclusion: CT is not an accurate tool for the management of primary aldosteronism, especially in bilateral disease. AVS as a standard procedure in the workup is feasible but depends on proper technique and radiologists with adequate experience.

8:00 AM; Abstract No. 165 - Vascular plug-assisted retrograde transvenous obliteration for the treatment of gastric varices and hepatic encephalopathy

Purpose To evaluate the technical and clinical safety and the efficacy of Amplatzer Vascular Plug (AVP)-assisted retrograde transvenous obliteration (PARTO) for the treatment of gastric varices (GV) and hepatic encephalopathy (HE). Materials and Methods From April 2009 to December 2011, we evaluated 20 patients (16 men and four women; mean age, 58 years; range, 47-79 years) who had undergone PARTO, 13 of whom had GV and seven who had HE. After retrograde transvenous placement of an AVP into the left adrenal vein, subsequent gelfoam embolization of both the gastrorenal shunt and the GV was performed. Results Placement of the AVP and subsequent gelfoam embolization were technically successful in all 20 patients. There were no procedure-related complications. Follow-up computed tomography (CT) obtained within one week after PARTO showed complete thrombosis of the GV and gastrorenal shunts in all patients. The clinical symptoms of HE were completely resolved in all seven patients with HE and the mean serum NH3 level, which was 127.4 umol/l ± 58 before PARTO, decreased significantly, to 28.1 umol/l ± 9.8, within one week after PARTO (p = 0.002). The clinical success rate was 100%. Improvement in the Child-Pugh score was observed in 12 of 18 patients (66.7%) at one month after PARTO. The mean follow-up period after PARTO was 422 days (range, 170-1168 days). Eighteen patients who had more than a two-month follow-up period, showed complete obliteration of the GV as well as the gastrorenal shunts on both CT and endoscopy. In these 18 patients, four (22.2%) experienced worsening of their esophageal varices at a mean of 9.4 months (range; 1-25 months) after PARTO. There were no cases of variceal bleeding or HE during a mean follow-up period of 422 days (range, 170-1168 days). Conclusion Our preliminary results suggest that PARTO is technically simple and safe and seems to be clinically effective for the treatment of GV and HE. We therefore believe that PARTO results in complete blockage of the gastrorenal shunt with a very high success rate. PARTO also has the potential to become a standard treatment for GV associated with a gastrorenal shunt either in patients with GV or in patients with HE.

Functional distal splenorenal shunt and splenic artery ligation as portal flow modulation in left lobe living donor transplantation

Functional distal splenorenal shunt and splenic artery ligation as portal flow modulation in left lobe living donor transplantation

Coaxial Guide Wire Placement in the Right Adrenal Vein for Repeated Adrenal Venous Samplings

Many adrenal venous sampling (AVS) protocols require repeated samplings before and after adrenocorticotrophic hormone (ACTH) stimulation. Maintaining catheter selectivity in the adrenal vein over time is essential but can be challenging, especially in the short right adrenal vein, where the catheter is often in an unstable position. The aim of our study was to evaluate guide wire insertion into the right adrenal vein catheter to sustain AVS selectivity (adrenal/peripheral cortisol ratio [Ca/Cp]) over time. This retrospective investigation was approved by our institutional review board, and informed consent was obtained. A 0.014-inch guide wire was inserted in the right adrenal vein 5F catheter to secure its positioning and to facilitate blood sampling. Plasma cortisol levels from the left and right adrenal veins and left iliac vein were assessed in 117 consecutive patients undergoing bilateral, simultaneous sets of AVS at -5 and 0 min (baseline) and 5, 10, and 15 min after intravenous bolus of 250 μg ACTH (stimulated). Ca/Cp ratios of ≥2 for baseline and >10 for stimulated AVS were considered selective. The first sampling, at time -5 min, was nonselective in 41 of 116 (35.3 %) right and 30 of 116 (25.9 %) left AVSs retained for analysis. In patients with a selective first sampling, 74 of 75 (98.7 %) right and 85 of 86 (98.8 %) left AVSs were selective in all post-ACTH samplings. Right and left selectivity rates were not statistically different (p > 0.87). No complications arose from guide wire insertion. Guide wire insertion into the right adrenal vein catheter is safe and effective to maintain AVS selectivity over time.

Detection of adrenal veins on selective retrograde CT adrenal venography in comparison with digital subtraction angiography in subjects with established diagnosis of one-sided adrenal aldosterone-producing tumor confirmed by adrenal vein sampling, histopathology and clinical course

Adrenal vein (AV) sampling (AVS) is the diagnostic gold standard for primary aldosteronism (PA), but right-sided AVS is difficult. We compared detection of AVs by selective retrograde CT adrenal venography (SRCTAV) with digital subtraction angiography (DSA). Data on 29 subjects (11 males, mean age 55 y) with increased serum aldosterone concentrations (SAC) and a diagnosed right or left aldosterone-producing tumor (APT) by AVS who underwent laparoscopic adrenalectomy were retrospectively analyzed. Before AVS, visualizing AVs was attempted by DSA and SRCTAV (Aquilion). If after the adrenocorticotropic hormone loading test serum cortisol concentration (SCC) from either AV was >200 μg/dl, AVS was considered successful. If the SAC/SCC ratio for one side was ≥4 times higher than the other side, we diagnosed a one-sided APT. Left and right AV, respectively, were visualized in 29 (100%) and 22 subjects (76%) by DSA and 29 (100%) and 28 subjects (97%) by SRCTAV, with right-AV detection significantly higher by SRCTAV (p<0.05). Cannulations were regarded successful in 28 subjects having both AVs observed on SRCTAV but not in the remaining subject whose adrenocortical scintigram was positive, however. Adrenalectomy was performed with a diagnosis of adenoma. Among 28 subjects with successful AVS, histopathological diagnoses included adenoma (25), nodular hyperplasia (2) and normal (1). After adrenalectomy, antihypertensive drug usage in 28 patients was reduced or stopped with decreases in SAC (97%). Detection of AV was significantly higher by SRCTAV than DSA, especially on the right side, in 29 subjects diagnosed with one-sided APT.

Comparison of the Central Adrenal Vein and the Common Trunk of the Left Adrenal Vein for Adrenal Venous Sampling

PurposeTo compare left adrenal venous sampling (AVS) in two locations: the central adrenal vein and the common trunk. Materials and MethodsA total of 22 patients (12 men and 10 women; mean age, 50 y; range, 26–65 y) who were suspected of having primary aldosteronism (PA) and underwent successful AVS with cortisol concentration measurement and/or venography between November 2010 and August 2011 were retrospectively analyzed. In regard to the left adrenal vein, collections were done at two locations: at the common trunk below the confluence of the inferior phrenic vein and at the central adrenal vein, which was above the confluence. The effects of the inflow from the inferior phrenic vein on plasma aldosterone and cortisol levels were analyzed. ResultsEight patients had bilateral hypersecreting lesions and 13 had a unilateral lesion. One was diagnosed as having secondary hypertension other than PA. The median cortisol levels below and above the confluence were 129 μg/dL (range, 21–400 μg/dL) and 215 μg/dL (range, 21–690 μg/dL), respectively. The median aldosterone levels were 2,120 pg/mL (range, 164–42,700 pg/mL) and 4,275 pg/mL (range, 119–59,000 pg/mL), respectively. The median aldosterone/cortisol (A/C) ratios were 244 (range, 34–2,401) and 278 (range, 25–2,251), respectively. Cortisol and aldosterone levels were significantly higher above the confluence (P = .0050 and P = .0003, respectively), whereas the A/C ratio showed no significant difference (P = .12). ConclusionsAlthough higher levels of cortisol and aldosterone were obtained upstream, A/C ratio was not significantly different between the central adrenal vein and the common trunk.

Gastric Varices and Hepatic Encephalopathy: Treatment with Vascular Plug and Gelatin Sponge–assisted Retrograde Transvenous Obliteration—A Primary Report

To evaluate technical safety, clinical safety, and effectiveness of vascular plug-assisted retrograde transvenous obliteration (RTO) for treatment of gastric varices (GV) and hepatic encephalopathy (HE). This retrospective study was approved by the institutional review board; written informed consent was waived. From April 2009 to December 2011, 20 patients (13, GV; seven, HE) who had undergone vascular plug-assisted RTO were retrospectively evaluated. After retrograde transvenous placement of a vascular plug in the left adrenal vein or gastrorenal shunt, subsequent gelatin-sponge embolization of both gastrorenal shunt and GV was performed. Follow-up computed tomography (CT) and upper gastrointestinal tract endoscopy were performed; clinical and laboratory data were collected to evaluate primary (technical success, complications, clinical success) and secondary (change of liver function by using the Child-Pugh score, worsening of esophageal varices) end points. Laboratory data before and after vascular plug-assisted RTO were compared (paired-sample t test). Placement of the vascular plug and subsequent gelatin-sponge embolization were technically successful in all 20 patients, with no procedure-related complications. Follow-up CT within 1 week after vascular plug-assisted RTO showed complete thrombosis of GV and gastrorenal shunts in all patients. Clinical symptoms of HE completely resolved in all seven patients with HE; mean serum NH3 level of 127.4 μmol/L ± 58 (standard deviation) before vascular plug-assisted RTO decreased significantly to 28.1 μmol/L ± 9.8 within 1 week after vascular plug-assisted RTO (P = .002). Eighteen patients who underwent follow-up longer than 2 months showed complete obliteration of GV and gastrorenal shunts at CT and endoscopy. There were no cases of variceal bleeding or HE during mean follow-up of 422 days. Improvement in Child-Pugh score was observed in 12 of 18 (67%) patients 1 month after vascular plug-assisted RTO. Worsening of esophageal varices was observed in four (22%) patients at mean follow-up of 9.4 months. Vascular plug-assisted RTO is technically simple and safe and seems to be clinically effective for treatment of GV and HE.

Spleno-adrenal shunt: A novel alternative for portosystemic decompression in children with portal vein cavernous transformation

PurposeChildren with portal vein cavernous transformation (PVCT) can develop life-threatening variceal hemorrhage from progressive portal hypertension. While spleno-renal shunt±splenectomy is the most common portosystemic decompression surgery performed in children, we have adopted a modified spleno-adrenal (SA) shunt for complicated PVCT. We describe our 10year experience focusing on technique evolution and treatment efficacy. MethodsBetween 2001 and 2011, 15 children (9 girls and 6 boys, ages 3–11years, median: 6years) with PVCT, portal hypertension, and hypersplenism were treated with SA shunt with splenectomy in Shanghai Children's Medical Center. All children in the study had endoscopy proven active esophageal variceal bleeding requiring multiple transfusions (mean: 4.2 units) with failed sclerotherapy (mean: 2.6 times). Greater omental vein pressure (GVP) approximating portal venous pressure was measured pre- and post-SA shunt. Pre- and post-operative ammonia levels were obtained. Follow-up ranged from 6months to 10years (mean: 4.2±2years). ResultsIntra-operative adrenal vein diameter and length ranged from 0.7 to 1.8cm and 2 to 3cm, respectively. Intra-operative GVPs pre-and post-SA shunt were (30±11) and (22±7) mmHg, respectively (p<0.01). On follow-up, there have been no recurrences of GI bleeding. Liver function tests remained normal in all children with the exception of elevated post-operative mean blood ammonia levels [Pre (18±7) mmol/L, post (60±17) mmol/L (p<0.05)] in all children. Ammonia levels normalized in all cases on outpatient follow-up. There have been no cases of hepatic encephalopathy, and all have normal age appropriate neurodevelopment (Bayley's assessment). Barium swallow and/or upper endoscopy showed interval resolution of esophageal varices in all children, and vascular ultrasound showed patent shunt anastomosis without stricture in 14 (93%). ConclusionsThe left adrenal vein is a viable conduit for effective selective portosystemic decompression. Similar to the more traditional spleno-renal shunt, SA appears also to have the advantage of preventing hepatic encephalopathy preserving neurodevelopment, although the rise in post-operative ammonia levels was unexpected. Longer follow-up is needed to look for late signs of encephalopathy assessing neurodevelopment long term.

Adrenal venous catecholamine concentrations in patients with adrenal masses other than pheochromocytoma

The aim of this study was to retrospectively examine adrenal venous catecholamine concentrations and related indices in non-pheochromocytoma patients and to estimate from the obtained results whether measurements of adrenal venous catecholamine concentrations by adrenal venous sampling (AVS) are useful for localizing adrenal pheochromocytoma. The study population comprised 15 patients in whom AVS was performed for evaluation of adrenal non-pheochromocytoma masses (primary aldosteronism, n = 8; Cushing syndrome, n = 5; non-hyperfunctioning tumor, n = 2) without hormonal intervention and was successful in bilaterally judging adrenal vein to infra-renal inferior vena cava cortisol ratios as >3.0. Wide ranges of catecholamine concentrations were seen for both right (epinephrine, 35-175,821 pg/ml; norepinephrine, 115-32,102 pg/ml; dopamine, 9-232 pg/ml) and left (epinephrine, 16-27,251 pg/ml; norepinephrine, 155-9,267 pg/ml; dopamine, 5-234 pg/ml) adrenal veins. High- to low-side adrenal vein concentration ratios also showed wide ranges of up to 779 for epinephrine, 22.5 for norepinephrine, and 7.8 for dopamine. Adrenal venous catecholamine concentrations obtained by AVS and simple comparisons between bilateral adrenal veins might not be useful to localize adrenal pheochromocytoma, as wide variations in concentrations and high- to low-side adrenal vein concentration ratios were noted in patients with adrenal non-pheochromocytoma.

Abstract 277: Mutations in the KCNJ5 Gene Imply a Higher Lateralization Index at Adrenal Vein Sampling for Primary Aldosteronism

Background. The mutations that affect the selectivity filter of the KCNJ5 K+ channel can play a role in triggering and/or maintaining aldosterone oversecretion in primary aldosteronism (PA). We therefore hypothesized that these somatic mutations can be associated with an increased aldosterone secretion from the APA, thus translating in raised plasma aldosterone concentrations (PAC) in the ipsilateral adrenal vein. Aim. To investigate if the lateralization index (LI) at adrenal vein sampling (AVS) is higher in the patients with an APA carrying the mutation (KCNJ5mut), as compared to those without the mutation (KCNJ5wt). Methods. Ninety-two consecutive PA patients who underwent AVS and received diagnosis of APA based on the four corners criteria were recruited. Unequivocal information on the presence or absence of the KCNJ5 mutations was available for each patient. The selectivity index (SI) was calculated as ratio between the right or left adrenal vein PCC (PCCside) and the infrarenal IVC PCC and a cutoff of 2.00 was used. The lateralization index (LI) was calculated in the bilaterally selective AVS as the ratio of PAC/PCC at the APA side to PAC/PCC at the contralateral side. We sequenced the KCNJ5 coding region spanning aminoacids 122 to 199, which include the selectivity filer. Results. The overall prevalence rate of KCNJ5 somatic mutations was 34%; G151R, L168R and T158A mutations were found in 19, 10 and 1 APA respectively. The G151E mutation was not found. The KCNJ5mut and KCNJ5wt groups were similar for gender, age, sK+ levels, while PAC and ARR were higher, and PRA lower (all p&lt;0.05) in the KCNJ5 mut group. In the latter group the LI was higher than in the KCNJ5wt group (29.3± 6.7 vs 16.7±3.9, p&lt; 0.02). This was due to a PAC/PCC ratio which was higher in the adrenal vein ipsilateral to the APA side and lower contralaterally in the KCNJ5mut group. Conclusions. These results provide direct in vivo evidence for a higher aldosterone secretion from APA carrying the KCNJ5 mutations, which translates into higher values of the LI, compared to the tumors without such mutations. Hence, the presence of these KCNJ5 mutations can affect the accuracy of the AVS-based diagnosis of the subtype of PA.

Cannabinoid receptor 2 agonist ameliorates mesenteric angiogenesis and portosystemic collaterals in cirrhotic rats

Angiogenesis in liver cirrhosis leads to splanchnic hyperemia, increased portal inflow, and portosystemic collaterals formation, which may induce lethal complications, such as gastroesophageal variceal hemorrhage and hepatic encephalopathy. Cannabinoids (CBs) inhibit angiogenesis, but the relevant influences in cirrhosis are unknown. In this study, Spraque-Dawley rats received common bile duct ligation (BDL) to induce cirrhosis. BDL rats received vehicle, arachidonyl-2-chloroethylamide (cannabinoid receptor type 1 [CB(1) ] agonist), JWH-015 (cannabinoid receptor type 2 [CB(2) ] agonist), and AM630 (CB(2) antagonist) from days 35 to 42 days after BDL. On the 43rd day, hemodynamics, presence of CB receptors, severity of portosystemic shunting, mesenteric vascular density, vascular endothelial growth factor (VEGF), VEGFR-1, VEGFR-2, phospho-VEGFR-2, cyclooxygenase (COX)-1, COX-2, and endothelial nitric oxide synthase (eNOS) expressions as well as plasma VEGF levels were evaluated. Results showed that CB(1) and CB(2) receptors were present in left adrenal veins of sham rats, splenorenal shunts (the most prominent intra-abdominal shunts) of BDL rats, and mesentery of sham and BDL rats. CB(2) receptor was up-regulated in splenorenal shunts of BDL rats. Both acute and chronic JWH-015 treatment reduced portal pressure and superior mesenteric arterial blood flow. Compared with vehicle, JWH-015 significantly alleviated portosystemic shunting and mesenteric vascular density in BDL rats, but not in sham rats. The concomitant use of JWH-015 and AM630 abolished JWH-015 effects. JWH-133, another CB(2) agonist, mimicked the JWH-015 effects. JWH-015 decreased mesenteric COX-1, COX-2 messenger RNA expressions, and COX-1, COX-2, eNOS protein expressions. Furthermore, JWH-015 decreased intrahepatic angiogenesis and fibrosis. CB(2) agonist alleviates portal hypertension (PH), severity of portosystemic collaterals and mesenteric angiogenesis, intrahepatic angiogenesis, and fibrosis in cirrhotic rats. The mechanism is, at least partly, through COX and NOS down-regulation. CBs may be targeted in the control of PH and portosystemic collaterals.

Effect of Adrenocorticotropic Hormone Stimulation During Adrenal Vein Sampling in Primary Aldosteronism

Adrenal vein sampling (AVS) is fundamental for subtype diagnosis in patients with primary aldosteronism. AVS protocols vary between centers, especially for diagnostic indices and for use of adrenocorticotropic hormone (ACTH) stimulation. We investigated the role of both continuous ACTH infusion and bolus on the performance and interpretation of AVS in a sample of 76 patients with confirmed primary aldosteronism. In 36 primary aldosteronism patients, AVS was performed both under basal conditions and after continuous ACTH infusion, and in 40 primary aldosteronism patients, AVS was performed both under basal conditions and after ACTH IV bolus. Both ACTH protocols determined an increase in the rate of successful cannulation of the adrenal veins. Both ACTH infusion and bolus determined a significant increase in selectivity index for the right adrenal vein and ACTH bolus for the left adrenal vein. Lateralization index was not significantly different after continuous ACTH infusion and IV bolus. In 88% and 78% of the patients, the diagnosis obtained was the same before and after ACTH infusion and IV bolus, respectively. However, the reproducibility of the diagnosis was reduced using less stringent criteria for successful cannulation of the adrenal veins. This study shows that ACTH use during AVS may be of help for centers with lower success rates, because a successful adrenal cannulation is more easily obtained with this protocol; moreover, this technique performs at least as well as the unstimulated strategy and in some cases may be even better. Stringent criteria for cannulation should be used to have a high consistency of the diagnosis.

The Roles of Angiotensin II Receptors in the Portosystemic Collaterals of Portal Hypertensive and Cirrhotic Rats

Background/Aims:In liver cirrhosis/portal hypertension, collaterals as varices may bleed and are influenced by vasoresponsiveness. An angiotensin blockade ameliorates portal hypertension but the influence on collaterals is unknown. Methods:Portal hypertension and cirrhosis were induced by portal vein (PVL) and common bile duct ligation (BDL). Hemodynamics, real-time PCR of angiotensin II receptors (AT₁R, AT₂R) in the left adrenal vein (LAV, sham) and splenorenal shunt derived from LAV (PVL, BDL) were performed. With an in situcollateral perfusion model, angiotensin II vasoresponsiveness with different preincubations was evaluated: (1) vehicle; (2) AT₁R blocker losartan; (3) losartan plus nonselective nitric oxide synthase (NOS) inhibitor (N^ω-nitro-L-arginine); (4) AT₂R blocker PD123319; (5) PD123319 plus N^ω-nitro-L-arginine; (6) N^ω-nitro-L-arginine, and (7) losartan plus inducible NOS inhibitor aminoguanidine. Results: LAV AT₁R and AT₂R expression decreased in PVL and BDL rats. Losartan attenuated angiotensin II-elicited vasoconstriction but PD123319 had no effect. N^ω-nitro-L-arginine but not aminoguanidine reversed the losartan effect. Conclusions:Angiotensin receptors are downregulated in the collateral vessel of portal hypertensive and cirrhotic rats. The AT₁R blockade attenuates the angiotensin II vasoconstrictive effect, suggesting AT₁R mediates collateral vasoconstriction and the influence of AT₂R is negligible. The lack of aminoguanidine influence indicates that endothelial NOS participates in the losartan effect.

Aliskiren reduces portal pressure in portal hypertensive rats

Aliskiren is a direct renin inhibitor used in the treatment for arterial hypertension. It can also augment nitric oxide (NO) production, which plays a crucial role in the pathogenesis of portal hypertension and modulation of porto-systemic collaterals. This study investigated the effects of aliskiren on portal pressure and porto-systemic collaterals of portal vein-ligated (PVL) rats. Sham-operated and PVL rats received aliskiren (50 mg/kg per day) or distilled water (control) treatment for 10 days. The mean arterial pressure and portal pressure were measured by catheterization of the right femoral artery and mesenteric vein, while the superior mesenteric arterial blood flow was measured by Doppler technique. The left adrenal vein and superior mesentery artery were dissected for mRNA study. The PVL rats also underwent preincubation with (i) Krebs solution (control); (ii) 10(-4) M aliskiren; or (iii) 10(-4) M aliskiren plus nonselective NO inhibitor N(ω)-nitro-L-arginine (10(-4) M), followed by the addition of arginine vasopressin (AVP) to evaluate the collateral vascular responsiveness. Aliskiren had systemic arterial pressure- and portal pressure-lowering effects in PVL rats. Superior mesentery arterial resistance also decreased. The constitutive NO synthase was enhanced in the left adrenal vein and superior mesentery artery after aliskiren treatment. Aliskiren attenuated the collateral vasoconstrictive effects of AVP, but the vasodilatory effects were abolished after nonselective NO synthase inhibition. Chronic aliskiren use reduces portal pressure in portal hypertensive rats partly due to the modulation of splanchnic and collateral NO synthase.

Is there still a place for adrenal venous sampling in the diagnostic localization of pheochromocytoma?

Our objective is to outline the utility of adrenal venous sampling (AVS) with measurements of metanephrine to normetanephrine ratios for diagnostic localization of phaeochromocytoma in a patient with normal plasma levels of catecholamines. A 53-year-old-woman was referred for evaluation of recurrent pheochromocytoma following a right adrenalectomy 14 years earlier. Diagnosis of recurrent disease was established from elevations in plasma metanephrines with normal levels of catecholamines. Magnetic resonance imaging indicated two 1-2 cm masses in the right surgical bed and another 1-1.5 cm mass in the left adrenal. These masses were negative on (123)I-metaiodobenzylguanidine scintigraphy. There was no evidence of a hereditary syndrome. We, therefore, carried out two investigational approaches to identify the tumor masses. Hybrid positron emission tomography/computed tomography with (68)Ga-DOTATOC ((68)Ga-DOTATOC-PET/CT) confirmed the presence of recurrent disease in the right surgical bed and also suggested additional left adrenal involvement. Normal plasma catecholamines precluded the use of adrenal venous sampling (AVS) with catecholamine measurements. Hence, we performed AVS with measurements of plasma metanephrines, which were 4- to 7-fold higher in the left adrenal vein than in central venous plasma. We observed a reversal of the normally high metanephrine to normetanephrine ratio (mean value ± SD 5.28 ± 1.86; range 3.36-8.84, n = 13) to 0.73, establishing the presence of a left adrenal pheochromocytoma. Surgical pathology confirmed bilateral disease. This case highlights a scenario where a combination of (68)Ga-DOTATOC-PET/CT and AVS with measurement of the metanephrine to normetanephrine ratio was crucial for the preoperative assessment of a patient with bilateral pheochromocytoma.

A Rare Case of ACTH-independent Macronodular Adrenal Hyperplasia Associated with Aldosterone-producing Adenoma

A 52-year-old man was evaluated for incidentally discovered bilateral adrenal masses. He had drug-resistant hypertension but lacked Cushingoid features. Endocrinological tests revealed autonomous secretion of cortisol and aldosterone with suppressed plasma ACTH and renin activity. A selective adrenal venous sampling demonstrated aldosterone hypersecretion from the left adrenal vein. The clinical diagnosis of subclinical Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia (AIMAH) associated with primary aldosteronism was made, and he underwent left adrenalectomy; the resected adrenal lesion was consistent with the pathological diagnosis of AIMAH coexistent with aldosterone-producing adenoma (APA). This is a very rare case of AIMAH with concomitant unilateral APA, whose hypertension improved after surgery.

Bilaterale selektive Nebennierenvenen-Blutentnahme: Einzelkatheter-Technik mittels eines 4F-Kobra-Katheters in Standard- und Inversionstechnik

In Conn's syndrome, adrenal vein sampling (AVS) is used to secure the site of hormonal overproduction. The left adrenal vein is usually easy to explore, the right is difficult. Examination requires several differently configured catheters. Here, we introduce a new technique for AVS using a single cobra catheter, which was tested in n = 13 patients. In an inverted configuration, the cobra catheter can be used to explore the left side, while the regular configuration can be used for the right side. The technical details are described in the manuscript. In all patients, left AVS could be performed successfully using the inverted catheter configuration. In n = 11 patients, right AVS was carried out successfully with a cobra catheter in regular configuration. In two cases, a Sidewinder-I catheter was required due to a steep angle of the adrenal vein.