Utility of Carbon Dioxide Venography and Intraprocedural CT for Adrenal Venous Sampling in Patients with an Allergy to Iodinated Contrast Media.

PURPOSE: To assess the diagnostic performance of carbon dioxide (CO2) and intraprocedural unenhanced computed tomography (CT) for adrenal venous sampling (AVS) (CO2-intraprocedural unenhanced CT-AVS) in patients with primary aldosteronism (PA) and a history of iodine contrast medium allergy. MATERIALS AND METHODS: CO2-intraprocedural unenhanced CT-AVS was performed in 18 patients with iodine contrast media allergies at the authors' hospital between December 2015 and January 2021. CT and noncontrast magnetic resonance angiography were used to evaluate the preoperative adrenal vein anatomy. CO2 venography was performed to confirm adrenal vein catheterization. Additionally, intraprocedural unenhanced CT was also performed to confirm catheter position in the right adrenal gland. RESULTS: In all cases in which CO2-intraprocedural unenhanced CT-AVS was performed, the right and left adrenal veins were catheterized appropriately, leading to a localized diagnosis. Catheterization of the left adrenal vein was confirmed using CO2 venography in all cases. In 7 of the 18 cases, CO2 venography demonstrated selection of the right adrenal vein. In 15 of 18 cases, intraprocedural unenhanced CT demonstrated selection of the right adrenal vein. CONCLUSIONS: CO2-intraprocedural unenhanced CT-AVS demonstrated the same diagnostic ability for PA localization as conventional AVS with iodine contrast media. The proposed method is clinically feasible for AVS, in which iodine contrast media use is restricted.

Bipolar Radiofrequency Ablation of the Adrenal Gland: In Vivo and Ex Vivo Experiments.

Laparoscopic adrenalectomy is currently the standard treatment modality for unilateral aldosterone-producing adenoma (APA); however, a less-invasive treatment is needed for its treatment. A new bipolar ablation system that poses a lower risk of complications has been recently developed. This study aimed to evaluate the safety and performance of a novel bipolar radiofrequency ablation (RFA) system for the treatment of APAs. Ablations were performed in an ex vivo study using bovine adrenal glands [group A: n = 6, single-probe; group B: n = 6, two probes, interprobe distance (ID) = 12 mm; group C: n = 6, two probes, ID = 20 mm]. The in vivo study was conducted in groups A and B (n = 2 each) using porcine adrenal glands. For the ex vivo study, the mean vertical diameter (Dv) of the coagulative necrosis area and the mean transverse diameter (Dt) values were 11.99 mm and 10.96 mm for group A, 12.66 mm and 10.0 mm for group B, and 23.37 mm and 22.10 mm for group C, respectively. For the in vivo study, the mean Dv and Dt values were 12.23 mm and 9.03 mm for group A, and 16.38 mm and 9.52 mm for group B, respectively. No heat-induced damage to the adjacent organs was observed. To our best knowledge, this is the first study to evaluate the performance of the bipolar system in RFA of the adrenal gland. RFA using the new bipolar ablation system is safe and produces a sufficient coagulation area to treat APAs.

Safety and feasibility of radiofrequency ablation using bipolar electrodes for aldosterone-producing adenoma: a multicentric prospective clinical study.

Evaluation of feasibility and safety of percutaneous radiofrequency ablation using bipolar radiofrequency devices in a prospective multicenter cohort of patients with benign aldosterone-producing adenoma. A total of five institutions participated. CT-guided percutaneous RFA was performed for patients diagnosed as APA. The safety of the procedure was evaluated using the Common Terminology Criteria for Adverse Events. During the 84-day follow-up period, serial changes in plasma aldosterone concentration and plasma renin activity were measured. The percentage of patients with normalized hormonal activity after the procedure, was calculated with 95% confidence intervals. Forty patients were enrolled, and two patients were excluded for cerebral hemorrhage and no safe puncture root. In another patients, RFA was tried, but an intraprocedural intercostal arterial injury occurred. Consequently, RFA was completed in thirty-seven patients (20 men, 17 women; mean age, 50.4 ± 10.0 year). The tumor size was 14.8 ± 3.8 mm. The treatment success rate of the ablation was 94.6% (35/37), and a 2nd session was performed in 2.7% (1/37) patients. Grade 4 adverse events were observed in 4 out of 38 sessions (10.5%). The normalization of plasma aldosterone concentration or aldosterone-renin ratio was 86.5% (72.0-94.1: 95% confidence interval) on day 84. Percutaneous CT-guided RFA for APA using a bipolar radiofrequency system was safe and feasible with clinical success rate of 86.5% on day 84.

Transvenous Radiofrequency Ablation of Adrenal Gland: Experimental Study.

PURPOSE: The aim was to evaluate a flexible device for transvenous adrenal gland radiofrequency ablation in vitro and in an in vivo animal model. MATERIALS AND METHODS: A flexible radiofrequency-tip catheter with an inner-cooling mechanism and a guidewire lumen was made. Then, using a polyvinyl alcohol gel model, the ablation diameter was evaluated and how much energy to deliver in vivo was determined. Finally, transvenous radiofrequency ablation of the left adrenal glands of two pigs was performed, delivering 5000 or 7000 J in a single dose to each. The ablation effects were also assessed by histological examination of hematoxylin-eosin-stained sections. RESULTS: The mean ablation diameters in the gel model were 20.2 and 21.9 mm in the short axis and 15 and 20 mm in the long axis for 5000 or 7000 J, respectively. The device was inserted into porcine left adrenal vein with no complications. The mean ablation diameters were 10 mm in the shorter axis (whole thickness of porcine left adrenal gland) in the porcine model for 7000 J. Transient increases in blood pressure and heart rate occurred during ablation. Histologically, the adrenal gland showed severe necrosis at ablated area. There was venous congestion upstream in a non-ablated area, and thermal damage to surrounding organs was not observed. CONCLUSIONS: A flexible radiofrequency-tip catheter could be inserted successfully into the left adrenal vein. The left adrenal gland was entirely ablated without any thermal damage to surrounding organs. We suggest transvenous adrenal ablation has potential as a therapeutic option for primary aldosteronism.

Fibromuscular dysplasia with recurrence after "long-term" following percutaneous transcatheter renal angioplasty: two case reports with a review of 26 patients.

BACKGROUND: Fibromuscular dysplasia (FMD) often causes renal artery stenosis with renovascular hypertension. Recent clinical outcomes encourage percutaneous transluminal renal angioplasty (PTRA) to treat FMD; however, the necessary follow-up period remains unclear. Moreover, previous studies have not revealed the difference in the period until recurrence between two major types of FMD-multifocal and focal. CASE PRESENTATION: We describe two patients with multifocal FMD who developed hypertension during their teenage years and had recurrence of FMD > 10 years after PTRA. We further examined the types of FMD and age of onset in 26 patients who underwent PTRA. The period until recurrence of multifocal FMD was longer than that of focal FMD. Moreover, patients with early-onset multifocal FMD are likely to have a delayed recurrence after PTRA compared to other types. CONCLUSIONS: Our report suggests that patients with multifocal FMD, especially those with onset at an early age, may need long-term follow-up for at least ≥ 10 years.

The Potential of Computed Tomography Volumetry for the Surgical Treatment in Bilateral Macronodular Adrenal Hyperplasia: A Case Report.

Although adrenal resection is a major option to control hypercortisolemia in patients with bilateral macronodular adrenal hyperplasia, a predictive method for postoperative cortisol production has not been established. A 53-year-old man with ulcerative colitis was referred to our hospital for bilateral multiple adrenal nodules and hypertension. Physical and endocrinological examination revealed inappropriate cortisol production and suppressed secretion of adrenocorticotropic hormone with no typical signs of Cushing's syndrome. Imaging analysis revealed bilateral adrenal nodular enlargement, the nodules of which had the radiological features of adrenocortical adenomas without inter-nodular heterogeneity. In addition, computed tomography volumetry demonstrated that the left adrenal gland (70 mL) accounts for three quarters of the total adrenal volume (93 mL). The patient was diagnosed as subclinical Cushing's syndrome due to bilateral macronodular adrenal hyperplasia, and subsequently underwent a left laparoscopic adrenalectomy with the estimation of 75% decrease in the cortisol level based on the adrenal volume. The surgical treatment ultimately resulted in control of the cortisol level within the normal range, which was compatible to our preoperative prediction. However, regardless of the sufficient cortisol level, ulcerative colitis was exacerbated after the surgery, which needed a systemic therapy for remission. This case indicates successful surgical control of hypercortisolemia based on computed tomography volumetry in bilateral macronodular adrenal hyperplasia, as well as the perioperative exacerbation risk for inflammatory diseases in Cushing's syndrome. We report the potential utility of computed tomography volumetry as a quantitative method with retrospective evaluation of our historical cases.

Treatment of Refractory Hypertension with Timely Angioplasty in Total Renal Artery Occlusion with Atrophic Kidney.

Angioplasty for cases of chronic total occlusion of renal artery with/without atrophic kidney is generally not recommended. We herein report a 57-year-old man who presented with renin-mediated refractory hypertension caused by occlusion of a unilateral renal artery leading to kidney atrophy (length: 69 mm). Angioplasty favorably achieved blood pressure control with normalized renin secretion and enlargement of the atrophic kidney to 85 mm. Timely angioplasty can be beneficial in select patients, even with an atrophic kidney and total occlusion, especially in cases with deterioration of hypertension within six months and the presence of collateral perfusion to the affected kidney.

Aldosterone-induced cardiac damage in primary aldosteronism depends on its subtypes.

OBJECTIVES: This study compared cardiac function, morphology, and tissue characteristics between two common subtypes of primary aldosteronism (PA) using a 3T MR scanner. DESIGN: A retrospective, single-center, observational study. METHODS: We retrospectively reviewed 143 consecutive patients with PA, who underwent both adrenal venous sampling and cardiac magnetic resonance. We acquired cine, late gadolinium enhancement, and pre- and postcontrast myocardial T1-mapping images. RESULTS: PA was diagnosed as unilateral aldosterone-producing adenoma (APA) in 70 patients and bilateral hyperaldosteronism (BHA) in 73. The APA group showed significantly higher plasma aldosterone concentration (PAC) and aldosterone to renin rate (ARR) than the BHA group. After controlling for age, sex, antihypertensive drugs, systolic and diastolic blood pressure, and disease duration, the parameters independently associated with APA were: left ventricular end-diastolic volume index (EDVI: adjusted odds ratio (aOR) = 1.06 (95% CI: 1.030-1.096), P < 0.01), end-systolic volume index (ESVI: 1.06 (1.017-1.113), P < 0.01), stroke index (SI: 1.07 (1.020-1.121), P < 0.01), cardiac index (CI: 1.001 (1.000-1.001), P < 0.01), and native T1 (1.01 (1.000-1.019), P = 0.038). Weak positive correlations were found between PAC and EDVI (R = 0.28, P < 0.01), ESVI (0.26, P < 0.01), and SI (0.18, P = 0.03); and between ARR and EDVI (0.25, P < 0.01), ESVI (0.24, P < 0.01), and native T1 (0.17, P = 0.047). CONCLUSIONS: APA is associated with greater LV volumetric parameters and higher native T1 values, suggesting a higher risk of volume overload and myocardial damage.

Embolization of a paraumbilical shunt by the transparaumbilical venous approach and one-sheath inverse method: A case report.

We describe treatment of a 53-year-old man with chronic hepatic encephalopathy. Contrast-enhanced computed tomography demonstrated a recanalized paraumbilical vein as a portosystemic shunt connecting the left branch of the portal vein and bilateral iliac veins. Percutaneous embolization was performed. The paraumbilical vein was punctured under ultrasonographic guidance; a 7-Fr sheath was inserted in the cranial direction. The hepatic side of the shunt was embolized with a vascular plug. The sheath direction was inverted to the caudal side; the pelvic side of the shunt was then embolized with another vascular plug. This report demonstrates that the percutaneous transparaumbilical venous approach is useful and safe for portosystemic shunt intervention. Moreover, the one-sheath inverse method was useful for embolization of upstream and downstream sides of the puncture site.

Kidney enlargement effect of angioplasty for nonatherosclerotic renovascular disease: reversibility of ischemic kidney.

Renal artery stenosis causes kidney ischemia, reducing the size of the affected kidney, which eventually results in atrophy. Although renal atrophy is considered irreversible, resolution of the ischemia occasionally restores kidney size when the cause is renal artery stenosis. Angioplasty is effective in patients with nonatherosclerotic renovascular diseases (non-ARVDs). Nevertheless, renal enlargement after angioplasty has not been fully examined. We conducted a retrospective study to examine this phenomenon in non-ARVD patients. Ten patients with a <100-mm pole-to-pole length of the poststenotic kidney were treated with angioplasty. Data were collected up to 12 months after angioplasty. The mean age was 28 years; the estimated glomerular filtration rate was 92 ± 7 mL/min/1.73 m2 (mean ± SEM); blood pressure was 150/99 mmHg; 80% were women; and fibromuscular dysplasia was present in 90% of the patients. All patients had hypertension. The lengths of the poststenotic and contralateral kidney before angioplasty were 91 ± 1 and 111 ± 3 mm, respectively. After angioplasty, the length of the poststenotic kidney gradually increased during the 3 months after treatment (+5.4 mm) and that of the contralateral kidney decreased over the same time course (-3.7 mm). Enlargement was also found in the moderate atrophy subgroup (length < 92 mm), and it was greater in the <30 years old group. In a noteworthy case, renal size in the poststenotic kidney recovered from 87 to 102 mm after angioplasty. Our findings demonstrated that reduced renal size can be reversed after optimal angioplasty in non-ARVD patients, especially young patients, suggesting reversibility of the ischemic kidney.

Unique Sex Steroid Profiles in Estrogen-Producing Adrenocortical Adenoma Associated With Bilateral Hyperaldosteronism.

Because of its rarity, our understanding of steroidogenesis in estrogen-producing adrenocortical adenoma, including the response to adrenocorticotropic hormone (ACTH) stimulation, remains limited. A 65-year-old man was referred to us because of primary aldosteronism and a right adrenal tumor. Endocrinological evaluations revealed secondary hypogonadism due to hyperestrogenemia. Adrenal venous sampling (AVS) and subsequent liquid chromatography-tandem mass spectrometry (LC-MS/MS) indicated bilateral hyperaldosteronism and a right estrogen-producing adrenocortical tumor. He subsequently underwent right unilateral adrenalectomy, which resulted in clinical remission of hypogonadism. Subsequent histopathological analysis identified a right estrogen-producing adrenocortical adenoma and multiple, concomitant adrenocortical micronodules. Sequential evaluation of steroid profiles using LC-MS/MS revealed unique hormone production, including adrenal androgens, and less responsiveness to ACTH in the right estrogen-producing adrenocortical adenoma as compared to the nonneoplastic adrenal cortex. This case study revealed unique profiles of steroid production in estrogen-producing adrenocortical adenoma associated with concomitant primary aldosteronism. Sequential steroid profiling analysis using LC-MS/MS in combination with AVS can contribute to the diagnosis of various adrenal disorders.

Apparent Diffusion Coefficient in the Resolution of Renal Ischemia after Angioplasty on Diffusion-weighted Imaging: Renal Artery Stenosis Caused by Progressive Thrombosis in Residual Chronic Aortic Dissection.

We report a case in which diffusion-weighted magnetic resonance imaging (DWI) demonstrated renal artery stenosis-related renal ischemia and the therapeutic efficacy of revascularization. The patient was a 73-year-old man, who underwent descending thoracic aortic replacement due to DeBakey IIIb chronic aortic dissection, and who showed progressive renal dysfunction due to right renal artery stenosis caused by false lumen thrombosis. DWI demonstrated a decreased apparent diffusion coefficient (ADC) in the right kidney, indicating renal ischemia. Angioplasty with stenting restored renal perfusion and improved the renal function, resulting in the normalization of the decreased ADC in the treated kidney. Thus, DWI can be used to monitor renal ischemia in cases involving advanced renal artery stenosis.

18-Oxocortisol Synthesis in Aldosterone-Producing Adrenocortical Adenoma and Significance of KCNJ5 Mutation Status.

Peripheral 18-oxocortisol (18oxoF) level could contribute to the detection of aldosterone-producing adenoma (APA) in patients with primary aldosteronism. However, peripheral 18oxoF varies among such patients, which is a big drawback concerning its clinical application. We studied 48 cases of APA, 35 harboring KCNJ5 mutation, to clarify the significance of clinical and pathological parameters about peripheral 18oxoF. Peripheral 18oxoF concentration ranged widely from 0.50 to 183.13 ng/dL and correlated positively with intratumoral areas stained positively for steroidogenic enzymes ( P<0.0001). The peripheral 18oxoF level also correlated significantly with that of circulating aldosterone ( P<0.0001) but not with that of cortisol, a precursor of 18oxoF. However, a significant correlation was detected between peripheral 18oxoF and intratumoral glucocorticoids ( P<0.05). In addition, peripheral 18oxoF correlated positively with the number of hybrid cells double positive for 11ß-hydroxylase and aldosterone synthase ( P<0.0001). Comparing between the cases with and those without KCNJ5 mutation, the KCNJ5-mutated group demonstrated a significantly higher concentration of peripheral 18oxoF (28.4±5.6 versus 3.0±0.9 ng/dL, P<0.0001) and a larger intratumoral environment including the hybrid cells ( P<0.001), possibly representing a deviation from normal aldosterone biosynthesis. After multivariate analysis, KCNJ5 mutation status turned out to be the most associated factor involved in 18oxoF synthesis in APA ( P<0.0001). Results of our present study first revealed that enhanced 18oxoF synthesis in APA could come from a functional deviation of aldosterone biosynthesis from the normal zona glomerulosa and the utility of peripheral 18oxoF measurement could be influenced by the prevalence of KCNJ5 mutation in an APA.

Effect of a notch at the distal end of a microcatheter on vein deformation in segmental adrenal venous sampling: a preliminary study using computational fluid dynamics.

This study aimed to evaluate the effect of a notch at the distal end of a microcatheter on vein deformation in segmental adrenal venous sampling. A three-dimensional fluid-structure interaction simulation was performed using commercial finite element software. A computational model of a vein with a catheter inserted into it was constructed. The outer and inner diameters of the vein were 0.9 mm and 0.6 mm, respectively, whereas those of the catheter were 0.6 mm and 0.5 mm, respectively. The velocity of the blood flow at the outlet was 85 mm/s. The pressure at the inlet was 0 Pa. The mesh consisted of approximately 660,000 elements. The effect of the number (0-4) and shape (no notch, 1/4 circular, 1/3 circular, semicircular, 2/3 circlecircular, and 3/4 circular) of the notches at the distal end of the microcatheter on the vein deformation when a suction pressure was applied was evaluated. The venous wall displacement was the smallest with the one-notch catheter, followed by the four-notch catheter, and was the smallest with the catheter having 1/4-circular notches, followed by the one with 1/3-circular notches. In conclusion, microcatheters having one notch and 1/4-circular notches reduce vein deformation and lead to successful segmental adrenal venous sampling. Graphical abstract Comparing catheters having different notch shapes.

Renovascular hypertension associated with JAK2 V617F positive myeloproliferative neoplasms treated with angioplasty: 2 cases and literature review.

Myeloproliferative neoplasms (MPNs) with Janus kinase 2 (JAK2) mutation are associated with a high risk for occlusive vascular diseases. We report 2 cases of renovascular hypertension associated with JAK2 V617F mutation-positive MPNs and provide a literature review. In Case 1, a 63-year-old woman had resistant hypertension, massive proteinuria, and erythrocytosis. Evaluations revealed right renal artery stenosis causing renovascular hypertension and polycythemia vera with JAK2 V617F mutation. Renin-angiotensin system inhibitors and subsequent angioplasty controlled the blood pressure and the proteinuria resolved. In Case 2, a 74-year-old woman had resistant hypertension and thrombocytosis. Evaluations confirmed left renal artery stenosis and essential thrombocythemia with JAK2 V617F. Angioplasty cured the hypertension. A literature review of 18 cases revealed the following as the most common characteristics of MPN-associated renovascular hypertension: manifests primarily in women; is associated with untreated polycythemia vera and essential thrombocythemia, concomitant leukocytosis, and JAK2 mutation positivity; and is responsive to angioplasty. This report demonstrates that JAK2 mutation-positive MPNs are a less common but important underlying cause of adult renovascular hypertension.

Image quality and radiation dose of low-tube-voltage CT with reduced contrast media for right adrenal vein imaging.

OBJECTIVES: To compare image quality and radiation dose of right adrenal vein (RAV) imaging computed tomography (CT) among conventional, low kV, and low kV with reduced contrast medium protocols. METHODS: One-hundred-and-twenty patients undergoing adrenal CT were randomly assigned to one of three protocols: contrast dose of 600mgI/kg at 120-kV tube voltage setting (600-120 group), 600mgI/kg at 80kV (600-80 group), and 360mgI/kg at 80kV (360-80 group). Iterative reconstruction was used for 80-kV groups. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) of the RAV and size-specific dose estimates (SSDE) were measured. Three radiologists evaluated 4-point visualisation scores of RAV by consensus reading. RESULTS: The RAV detectability was 95%, 97.2%, and 97.3% for 600-120, 600-80, and 360-80 groups, respectively (p=1.000). Visualisation scores were not significantly different among the groups (p=0.152). There were no significant differences in CNR or SNR between the 600-120 and 360-80 groups. SSDE of the 360-80 group was significantly lower than that of the 600-120 group (5.86mGy±1.44 vs. 7.27mGy±1.81, p<0.001). CONCLUSIONS: 80-kV scans with 360 mgI/kg contrast media showed comparable detectability of RAV to conventional scans, while reducing 19% of SSDE.

Selective embolization therapy for intrarenal artery stenosis causing renovascular hypertension: Efficacy and follow-up renal imaging.

We report the case of a young woman treated with selective renal embolization for renovascular hypertension caused by intrarenal artery stenosis and show follow-up imaging of the treated kidney. An 18-year-old woman had renin-dependent hypertension with intrarenal artery stenosis caused by fibromuscular dysplasia. A middle branch artery was nearly occluded, resulting in segmental renal ischemia with excessive renin secretion. Because our angioplasty attempt for revascularization failed as a result of technical difficulty, we performed selective embolization of the diseased vessel by anhydrous ethanol. The embolization promptly ameliorated hyperreninemia and resistant hypertension without deterioration of renal function. Findings from magnetic resonance imaging showed disappearance of the blood flow in the embolized area corresponding to the ischemic lesion that had been revealed by diffusion-weighted imaging. Thus, selective embolization can be effective in treating renovascular hypertension by intrarenal stenosis for which angioplasty is not feasible. Additionally, renal magnetic resonance imaging is useful for evaluating the causative ischemic lesion and embolized area.

Recommendation of Repeated Ammonia Tests for Intrahepatic Portal-Systemic Shunt Without Cirrhosis in Elderly Patients With Psychiatric Symptoms.

We report an elderly male patient with hyperammonemia induced by intrahepatic portal-systemic shunt without cirrhosis (IPSSwoC). The occasional emergence of his erratic behaviors was misdiagnosed as a psychiatric disorder. Regardless of his uneven symptoms, IPSSwoC was suspected due to his hyperammonemia. The contrast computed tomography of the abdomen revealed a congenital type of IPSSwoC. As blood ammonia levels are inconstant, repeated blood tests are recommended when this disease is suspected in elderly patients with psychiatric symptoms.

Anatomical Variations of the Right Adrenal Vein: Concordance Between Multidetector Computed Tomography and Catheter Venography.

Adrenal venous sampling is the most reliable diagnostic procedure to determine surgical indications in primary aldosteronism. Because guidelines recommend multidetector computed tomography (CT) to evaluate the adrenal gland, some past reports used multidetector CT as a guide for adrenal venous sampling. However, the detailed anatomy of the right adrenal vein and its relationship with an accessory hepatic vein remains uncertain. The purpose of this study was to describe detailed anatomical variations of the right adrenal vein and to determine the concordance between CT and catheter venography in patients with primary aldosteronism. In total, 440 consecutive patients who underwent adrenal venous sampling were included. Four-phase dynamic CT was performed. Anatomical locations and variations of the right adrenal vein and its relationship with the accessory hepatic vein were compared with catheter venographic findings. Successful catheterization was achieved in 437 patients (99%). The right adrenal vein was visualized in the late arterial phase with CT in 420 patients (95%). The right adrenal vein formed a common trunk with the accessory hepatic vein in 87 patients (20%). CT identified the correct craniocaudal level of the orifice in 354 patients (84%). Anatomical variations, location, and angle of inflow of the right adrenal vein based on CT demonstrated high concordance with catheter venography. CT may provide useful information for preparation before adrenal venous sampling.