A single-centre experience of the implementation of adrenal vein sampling procedure: the impact on the diagnostic work-up in primary aldosteronism.

BACKGROUND: Primary aldosteronism is one of the most common causes of secondary hypertension. Adrenal vein sampling (AVS) remains a "gold standard" procedure in differentiation between unilateral (adenoma) and bilateral (hyperplasia) disease. AIM: The aim of this study was to present our single-centre experience in establishing and implementating the AVS procedure. METHODS: All patients had primary aldosteronism confirmed in a salt-infusion test. AVS was performed sequentially during a continuous intravenous infusion of cosyntropin and was preceded by multislice contrast-enhanced computed tomography (CT) examination of adrenal glands performed a few weeks before AVS in the majority of patients. AVS was regarded as successful if the ratio of each adrenal vein cortisol to inferior vena cava cortisol levels (selectivity index [SI]) was higher than 3.0. In the case of failure, a second attempt was considered in a few weeks. Patients were divided into four groups according to the order of the procedure by quartiles. RESULTS: Between 31 May, 2012 and 5 May, 2016, AVS was performed in 124 patients (69% males, aged 55.3 ± 10.3 years) and was successful in 120 (96.8%) patients. All failed cases were due to the failure of cannulation of the right adrenal vein. The first-attempt success rate was 94.3% (117 of 124 patients) and increased from 83.9% in the first 31 patients to 100% in the last 31 patients. Similarly, the overall success rate increased from 93.5% to 100%. The right SI was significantly higher than the left one (26.4 vs. 11.0, p < 0.0001). Both indices did not differ across quartiles of patients. No complications occurred during the procedure. CONCLUSIONS: The AVS procedure, preceded by adrenal CT, may be implemented into daily diagnostic practice safely with an excellent success rate.

Stent sizing strategies in renal artery stenting: the comparison of conventional invasive renal angiography with renal computed tomographic angiography.

INTRODUCTION: Randomized trials comparing invasive treatment of renal artery stenosis with standard pharmacotherapy did not show substantial benefit from revascularization. One of the potential reasons for that may be suboptimal procedure technique. AIM: To compare renal stent sizing using two modalities: three-dimensional renal computed tomography angiography (CTA) versus conventional angiography. MATERIAL AND METHODS: Forty patients (41 renal arteries), aged 65.1 ±8.5 years, who underwent renal artery stenting with preprocedural CTA performed within 6 months, were retrospectively analyzed. In CTA analysis, reference diameter (CTA-D) and lesion length (CTA_LL) were measured and proposed stent diameter and length were recorded. Similarly, angiographic reference diameter (ANGIO_D) and lesion length (ANGIO_LL) as well as proposed stent dimensions were obtained by visual estimation. RESULTS: The median CTA_D was 0.5 mm larger than the median ANGIO_D (p < 0.001). Also, the proposed stent diameter in CTA evaluation was 0.5 mm larger than that in angiography (p < 0.0001). The median CTA_LL was 1 mm longer than the ANGIO_LL (p = NS), with significant correlation of these variables (r = 0.66, p < 0.0001). The median proposed stent length with CTA was equal to that proposed with angiography. The median diameter of the implanted stent was 0.5 mm smaller than that proposed in CTA (p < 0.0005) and identical to that proposed in angiography. The median length of the actual stent was longer than that proposed in angiography (p = 0.0001). CONCLUSIONS: Renal CTA has potential advantages as a tool adjunctive to angiography in appropriate stent sizing. Careful evaluation of the available CTA scans may be beneficial and should be considered prior to the planned procedure.

Renal resistive index in patients with true resistant hypertension: results from the RESIST-POL study.

BACKGROUND: Increased ultrasound Doppler renal resistive index (RRI) is a marker of atherosclerotic and hypertensive organ damage both at renal and systemic level. AIM: To evaluate RRI in patients with true resistant hypertension (TRHT) in the RESIST-POL study. METHODS: From 204 patients diagnosed with TRHT in the RESIST-POL study, 151 patients (90 male, 61 female, mean age: 47.7 ± 10.4, range: 19-65 years) without secondary hypertension were included into the analysis. All patients were characterised by estimated glomerular filtration rate > 60 mL/min/1.73 m2 and no history of diabetes prior to the study. As a control group we included 50 age- and gender-matched patients (35 male, 15 female, mean age: 46.8 ± 10.4, range: 19-65 years) with primary well-controlled hypertension. The groups also did not differ in respect to the number of years of known history of hypertension. The RRIs were evaluated on the basis of the Doppler ultrasound examination. Increased RRI was defined as ≥ 0.7. RESULTS: Both groups did not differ in terms of renal function. Patients with TRHT were characterised by higher RRI as compared with the group with well-controlled hypertension (0.62 ± 0.05 vs. 0.60 ± 0.05, p < 0.05). In the TRHT group RRI correlated significantly with age, clinic and ambulatory blood pressure measurement, diastolic blood pressure (DBP) levels, as well as with clinic pulse pressure (PP) (r = 0.297; p = 0.001), with daytime (r = 0.355; p < 0.001) and nighttime (r = 0.313; p < 0.001) PP, and with fasting glucose concentration (r = 0.215; p = 0.008) and E/E' ratio (r = 0.289; p = 0.001) on echocardiography. RRI values were significantly higher in TRHT patients with newly diagnosed diabetes as compared with TRHT patients without diabetes (0.65 ± 0.05 vs. 0.62 ± 0.05, p = 0.022). Age, daytime DBP, daytime PP, and E/E' ratio but not fasting glucose concentration correlated independently with RRI in the model. Among patients with TRHT, patients with increased RRI were characterised by older age (52.2 ± 4.9 vs. 47.3 ± 10.6 years, p = 0.012), higher body mass index (32.8 ± 6.0 vs. 29.7 ± 4.5 kg/m2, p = 0.034), as well as lower daytime and nighttime DBP values and lower daytime and nighttime heart rate, as compared to patients with RRI < 0.7. The TRHT patients with increased RRI as compared to patients with RRI < 0.7 were characterised also by higher daytime and nighttime PP. Both groups did not differ in respect of renal function. CONCLUSIONS: Our study showed that the patients with TRHT were characterised by significantly higher RRI values as compared to the subjects with well-controlled hypertension. It may also be suggested that in the subjects with TRHT renal vascular resistance is related to blood pressure values, selected echocardiographic abnormalities, and some surrogate markers for metabolic and cardiovascular events, including fasting glucose plasma concentration and PP, respectively.

Usefulness of Somatostatin Receptor Scintigraphy (Tc-[HYNIC, Tyr3]-Octreotide) and 123I-Metaiodobenzylguanidine Scintigraphy in Patients with SDHx Gene-Related Pheochromocytomas and Paragangliomas Detected by Computed Tomography.

AIMS: The aim of this study was to assess the usefulness of somatostatin receptor scintigraphy (SRS) using (99m)Tc-[HYNIC, Tyr3]-octreotide (TOC) and 123I-metaiodobenzylguanidine (mIBG) in patients with SDHx-related syndromes in which paragangliomas were detected by computed tomography and to establish an optimal imaging diagnostic algorithm in SDHx mutation carriers. METHODS: All carriers with clinical and radiological findings suggesting paragangliomas were screened by SRS and 123I-mIBG. Lesions were classified by body regions, i.e. head and neck, chest, abdomen with pelvis and adrenal gland as well as metastasis. RESULTS: We evaluated 46 SDHx gene mutation carriers (32 index cases and 14 relatives; 28 SDHD, 16 SDHB and 2 SDHC). In this group, 102 benign tumors were found in 39 studied patients, and malignant disease was diagnosed in 7 patients. In benign tumors, the sensitivity of SRS was estimated at 77% and of 123I-mIBG at 22.0%. The SRS and mIBG sensitivity was found to be clearly region dependent (p < 0.001). The highest SRS sensitivity was found in head and neck paragangliomas (HNP; 91.4%) and the lowest was found in abdominal paragangliomas and pheochromocytomas (40 and 42.9%, respectively). The highest 123I-mIBG sensitivity was found in pheochromocytomas (sensitivity of 100%) and the lowest in HNP (sensitivity of 3.7%). In metastatic disease, SRS was superior to mIBG (sensitivity of 95.2 vs. 23.8%, respectively). CONCLUSION: SRS and 123I-mIBG single photon emission computed tomography (SPECT) sensitivity in SDHx patients is highly body region dependent. In malignant tumors, SRS is superior to 123I-mIBG SPECT.

Relationship between primary aldosteronism and obstructive sleep apnoea, metabolic abnormalities and cardiac structure in patients with resistant hypertension.

INTRODUCTION: The aim of this study was to evaluate in patients with resistant hypertension (RHTN) enrolled in the RESIST-POL study the relationship between primary aldosteronism (PA) and obstructive sleep apnoea (OSA) and their effect on metabolic abnormalities and cardiac structure. MATERIAL AND METHODS: We included 204 patients (123 M, 81 F, mean age 48.4 yrs) with true RHTN, eGFR > 60 mL/min/1,73 m(2) and no known diabetes. OSA was defined as an apnoea/hypopnoea index of 15/h or more. Metabolic syndrome components were assessed. On echocardiography, left ventricular hypertrophy (LVH), concentric remodelling (RWT > 0.45), E' velocity, E/E' index and global strain (GLS) were evaluated. RESULTS: PA was diagnosed in 32 patients (15.7%). OSA occurred more frequently in patients with PA (59.4 v. 42.4%; p = 0.058). Patients were divided into four groups: PA+ OSA+ , PA+ OSA-, PA-OSA+ and PA-OSA-. Newly diagnosed diabetes, impaired glucose tolerance and increased fasting glucose were most frequent in the PA+ OSA+ group compared to other groups. The presence of OSA was associated with concentric remodelling, and the presence of PA was associated with higher left ventricular mass and higher frequency of left ventricular hypertrophy. In the PA+ OSA+ and PA+ OSA- groups, the most frequent geometry patterns were concentric hypertrophy (68.4%) and eccentric hypertrophy (54.5%) respectively. E' velocity was lowest and E/E' was highest in PA+ OSA+ compared to other groups. GLS was lower in patients with OSA compared to those without OSA. CONCLUSIONS: Both metabolic abnormalities and target organ damage are more pronounced in patients with RHTN, PA and OSA. OSA and PA influence differently left ventricular geometry.

[Primary pulmonary artery sarcoma in 36-year-old women: 3-years follow-up after partial resection and radiotherapy]. / Pierwotny miesak tetnicy plucnej u 36-letniej pacjentki: obserwacja 3-letnia po czesciowej resekcji i radioterapii.

Intimal sarcoma of the heart and pulmonary artery is a very rare, malignant, primary tumour. The prognosis in patients with primary sarcoma of the pulmonary artery, including intimal sarcoma, is poor. We present the case and 3-years follow-up of 36-year-old woman who was successfully treated with surgical, partial resection of the tumour followed by radiotherapy.

Testing new susceptibility genes in the cohort of apparently sporadic phaeochromocytoma/paraganglioma patients with clinical characteristics of hereditary syndromes.

BACKGROUND: Phaeochromocytoma (PCC) and paraganglioma (PGL) can occur sporadically or as a part of familial cancer syndromes. Red flags of hereditary syndromes are young age and multifocal tumours. We hypothesized that such patients are candidates for further molecular diagnosis in case of normal results in 'classical' genes. MATERIAL AND METHODS: We selected patients with PCC/PGL under the age of 40 and/or with multiple tumours. First, we tested the genes RET, VHL, NF1, SDHB, SDHC and SDHD. Patients without mutations in these genes were tested for mutations in MAX, TMEM127 and SDHAF2. RESULTS: In 153 patients included, mutations were detected in the classical genes in 72 patients (47%) [RET-22 (14%), VHL-13 (9%), NF1-3 (2%), SDHB-13 (9%), SDHC-3 (2%), SDHD-16 (11%), SDHB large deletions- 2 (1%)]. One patient with MAXc.223C>T (p.R75X) mutation was detected. It was a male with bilateral, metachronous phaeochromocytomas diagnosed in 36 and 40 years of age. Remarkably, he showed in the period before the MAX gene was detected, a RET p. Y791F variant. During 10-year follow-up, we did not find any thyroid abnormalities. LOH examination of tumour tissue showed somatic loss of the wild-type allele of MAX. CONCLUSION: Analysis of the MAX gene should be performed in selected patients, especially those with bilateral adrenal phaeochromocytoma in whom mutations of the classical genes are absent. Our study provides with further support that Y791F RET is a polymorphism.

[Mid-aortic syndrome in 18 year-old woman with arterial hypertension and bilateral renal artery stenosis]. / Zespól aorty brzusznej u 18-letniej chorej z nadcisnieniem tetniczym i obustronnym zwezeniem tetnic nerkowych.

Mid-aortic syndrome (MAS) is characterised by narrowing of the abdominal aorta, often with involvement of the renal and splanchnic arterial branches. Although uncommon, MAS is an important cause of renovascular hypertension in children and adolescents and should be considered in the differential diagnosis of hypertension . Hypertension is typically severe and often difficult to manage. The management of MAS should always be individualised and may include percutaneous transluminal renal angioplasty, stent implantation or surgical revascularisation. We present a 18 year-old woman with hypertension and MAS coexisting with bilateral renal artery stenosis who underwent left renal artery angioplasty and than was followed-up for one year.

The coexistence of acute aortic dissection with autosomal dominant polycystic kidney disease--description of two hypertensive patients.

BACKGROUND: Acute aortic dissection is a rare but potentially lethal disease characterized by high early mortality when left untreated. However, survival may be significantly improved by the timely institution of appropriate medical and/or surgical therapy. Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary disorders, accounting for 8-10% of the cases of end-stage renal disease. Hypertension occurs frequently and is an early manifestation of ADPKD in approximately 50-70% of non-azotemic patients. ADPKD, often caused by mutations in the PKD1 gene, is associated with life-threatening vascular abnormalities that are commonly attributed to the frequent occurrence of hypertension. METHODS: We present two cases of hypertensive patients with acute aortic dissection (type A Stanford) coexisting with ADPKD. RESULTS: A 54-year-old male hypertensive patient and a 47-year-old male hypertensive patient, both with known ADPKD were diagnosed and operated with acute dissection of ascending aorta. Both patients were characterized by severe hypertension; therefore, in both cases, degree of hypertension is likely to be causative for this vascular complication. However, since ADPKD is associated with the vascular complications mentioned above, abnormality of the structure of the arterial wall of the thoracic aorta cannot be ruled out. CONCLUSION: Aortic dissection is a rare but potentially disastrous complication, and clinicians should always consider this when dealing with patients with ADPKD who present with chest pain or collapse.

The role of biochemical tests and clinical symptoms in differential diagnosis of primary aldosteronism.

BACKGROUND: Primary aldosteronism (PA) is a secondary form of hypertension resulting from the autonomous hypersecretion of aldosterone. The recognition of PA has an important impact on clinical management, since the choice of therapy is different - surgical for adenoma and medical for hyperplasia. AIM: To evaluate patients with PA in regard to clinical and biochemical factors differentiating between adenoma of adrenal cortex (APA) and idiopathic adrenal hyperplasia (IHA). METHODS: We retrospectively analysed 62 patients with PA (33 females, 29 males, mean age 49.3+/-12.5 years, range 26-78) diagnosed in the Department of Hypertension between 1990-2001. In 37 patients (mean age 47.4+/-12.1 years, 22 females, 15 males) APA was diagnosed whereas in the remaining 26 patients (mean age 52.2+/-12.6 years, 14 males, 11 females) IHA was detected. Clinical manifestation, biochemical, serum aldosterone (SA), plasma renin activity (PRA) as well as echocardiographic parameters and blood pressure (BP) levels were evaluated. Diagnostic accuracy of computed tomography (CT) and scintigraphy was also assessed. RESULTS: Mean systolic BP was significantly higher in the patients with APA. Both groups had similar mean diastolic BP. Severe hypertension, resistant to three or more medications, was found in 63.3% of all patients. Muscle weakness was reported by 39.7% of patients, polyuria - by 19%, and polydypsia - by 10.3% of patients. Patients with muscle weakness had higher mean systolic BP level and lower plasma potassium level than patients without this complaint. Symptoms suggesting cardiac arrhythmia were reported by 45% of patients. A normal potassium level was found in 25.8% of all patients. The hypokalemic patients were younger, had shorter known duration of hypertension, higher mean systolic BP level and higher SA concentration than the normokalemic patients. Supine SA levels were significantly higher in the APA group than in the IHA group (50.3+/-29.0 ng% vs 30.5+/-14.7 ng%; p<0.001). The SA/PRA ratio higher than 30:1 was found in all patients. Response to postural test with a rise in SA concentration higher than 30% was observed in 40% patients with APA and in 87.5% patients with IHA (p<0.0001). There was a strong correlation between supine and upright SA level, and systolic and diastolic BP level as well as plasma potassium level. Left ventricular hypertrophy was present in 60% of patients. The differentiation between APA and IHA was possible using CT, scintigraphy or both methods in 75%, 89.2% and 100% of patients, respectively. CONCLUSIONS: One quarter of patients with PA were normokalemic. PA should be suspected especially in patients with severe hypertension, resistant to three or more antihypertensive drugs. Changes in SA concentration during the postural tests such as CT and scintigraphy are useful for differentiation between APA and IHA.