A clinical case of secondary hypertension in a young woman with coarctation of the aorta

The article presents a case of diagnosis and treatment of coarctation of the aorta in a 20-year-old woman, who previously received follow-up care with the diagnosis of hypertension. This case demonstrates the importance of qualitative examination of young patients with hypertension, including tonometry in the lower extremities and transthoracic echocardiography. The peculiarities of this clinical case encompass a rarer, isolated type of coarctation of the aorta and high physical fitness of the patient, which reduced doctors’ alertness regarding this anomaly. After surgical correction, a significant decrease in the blood pressure was achieved; however, such patients need longterm follow-up in order to detect complications, such as aneurysms, restenosis, or residual stenosis.

Juxtaglomerular cell tumour of the kidney: a rare cause of resistant hypertension

Summary Juxtaglomerular cell tumour (JGCT) is an unusually encountered clinical entity. A 33-year-old man with severe long-standing hypertension and hypokalaemia is described. The patient also suffered from polyuria, polydipsia, nocturia and severe headaches. On admission, laboratory investigation revealed hypokalaemia, kaliuresis, high aldosterone and renin levels, and the abdomen CT identified a mass of 4 cm at the right kidney. Kidney function was normal. Following nephrectomy, the histological investigation revealed the presence of a JGCT. Immunostaining was positive for CD34 as well as for smooth muscle actin and vimentin. Following surgery, a marked control of his hypertension with calcium channel blockers and normalization of the serum potassium, renin or aldosterone levels were reached. According to our findings, JGCT could be included in the differential diagnosis of secondary hypertension as it consists of a curable cause. The association of JGCT with hypertension and hypokalaemia focusing on the clinical presentation, diagnostic evaluation and management is herein discussed and a brief review of the existing literature is provided. Learning points Juxtaglomerular cell tumours (JGCT), despite their rarity, should be included in the differential diagnosis of secondary hypertension as they consist of a curable cause of hypertension. JGCT could be presented with resistant hypertension along with hypokalaemia, kaliuresis and metabolic alkalosis. Early recognition and management can help to prevent cardiovascular complications. Imaging (enhanced CT scans) may be considered as the primary diagnostic tool for the detection of renal or JGCT. For the confirmation of the diagnosis, a histopathologic examination is needed.

Hipertensão secundária: abordagem nos cuidados de saúde primários

Objetivos: Identificar situações sugestivas de hipertensão secundária (HS). Rever a fisiopatologia, manifestações clínicas e/ou laboratoriais, diagnóstico e tratamento das principais formas de HS, bem como estabelecer o papel do médico de família na sua abordagem à luz da evidência mais recente. Fontes de dados: MEDLINE/PubMed, The Cochrane Library, UpToDate, U.S. Preventive Services Task Force e normas de orientação clínica da Direção-Geral da Saúde. Métodos: Foi realizada a pesquisa de artigos científicos em plataformas online de medicina baseada em evidência utilizando as palavras-chave Secondary Hypertension e Primary Health Care. A pesquisa foi realizada em julho de 2019, sendo a seleção dos artigos feita com base no título, conteúdo do resumo e data de publicação. Foram também consultadas normas de orientação clínica e guidelines. Resultados: A HS deve ser suspeita em contexto de hipertensão resistente, início de hipertensão antes dos 30 anos num utente sem fatores de risco, elevação súbita ou instabilidade da pressão arterial basal, hipertensão maligna ou acelerada, início de hipertensão diastólica em idosos, hipertensão associada a distúrbios hidro-eletrolíticos ou hipertensão associada a clínica sugestiva de determinada etiologia específica. Em crianças e adolescentes, as principais causas de HS são as doenças do parênquima renal, ao passo que nos adultos as etiologias mais comuns incluem a síndroma da apneia obstrutiva do sono, doenças do parênquima renal, estenose da artéria renal, hiperaldosteronismo primário e feocromocitoma. O reconhecimento de manifestações clínicas e/ou laboratoriais sugestivas da etiologia da HS deve orientar a realização dos testes de rastreio para posterior tratamento ou referenciação. Conclusão: Avaliar em todos os utentes hipertensos a existência de causas secundárias não é custo-efetivo, pelo que apenas devem ser estudados os utentes com características sugestivas de HS. A realização e interpretação dos testes de rastreio de forma adequada é fundamental para a referenciação e tratamento dos utentes com HS.

Overview on Renovascular Hypertension: Review Article

Renovascular hypertension (RVH) is a prevalent cause of secondary hypertension that frequently develops to resistant hypertension. It is characterised as systemic hypertension that develops as a result of a restricted blood supply to the kidneys. Patients cannot be recognized clinically from those with essential hypertension; therefore, diagnosis requires arteriography, however urography and isotope renography may hint to the diagnosis. Atherosclerotic renal artery stenosis (ARAS) and fibromuscular dysplasia are the two most prevalent causes of RVH. The ultimate objective of controlling RVH, like with other kinds of hypertension, is to minimize the morbidity and mortality associated with high blood pressure The widespread use of effective antihypertensive medication treatment, statins, and other strategies to control vascular disease has resulted in remarkable improvements. In this review we will be looking at etiology, pathogenesis and treatment or RVH.

435 Renal denervation in a patient with uncontrolled arterial hypertension secondary to polycystic kidney disease

Aims Autosomal dominant polycystic kidney disease (ADPKD) represents the most common inherited cause of chronic kidney disease. Typical manifestations of this condition include secondary hypertension, abdominal pain, haematuria and urinary tract infections. Despite the progressive increase in the use of antihypertensive therapy in patients with ADPKD in the last decades, reaching blood pressure target is often difficult in this setting due to the complex physiopathology of arterial hypertension in ADPKD. Catheter-based renal sympathetic denervation (RDN) represents nowadays a therapeutic strategy to approach resistant hypertension. Based on consistent results of several sham-controlled clinical trials, the evidence of safety and efficacy of this procedure is increasing, also in patients with multiple comorbidities including chronic kidney disease. Patients with ADPKD often develop chronic severe kidney-related pain, caused by distension of the renal capsule due to the expansion of the cysts. RDN was proposed to be an effective therapeutic option able to relieve loin pain. Methods A 49-year-old man affected by ADPKD was referred to our centre for resistant uncontrolled arterial hypertension, despite combined therapy with five antihypertensive drugs. He also complained about intense loin pain and 3 years earlier underwent two surgical interventions to remove voluminous renal cysts, that did not relieve chronic pain. His kidney function was moderately decreased at presentation, with progressive decline in the previous years. After a multi-disciplinary discussion with a nephrologist and algologist, the patient was proposed for RDN with the aim of lowering blood pressure and reducing pain. He was treated in July 2018, and after the procedure, was observed a better control of blood pressure but no benefits on pain. Because of the persistence of intractable loin pain, the patient was submitted to a second RDN in December 2018. Also, after this procedure, blood pressure declined remarkably, decreasing his need for antihypertensive medications without a significant worsening of kidney function. Unfortunately, no benefit on chronic pain was observed. Results ADPKD is characterized by the progressive bilateral development of focal renal cysts. Cardiovascular complications, mainly related to hypertension, are a major cause of morbidity and mortality for these patients. RDN could be a valid and safe therapeutic option for the treatment of secondary hypertension in this setting.

Robotic excision of interaortocaval paraganglioma: a case report with the literature review

Background Paragangliomas are rare neuroendocrine tumors of extra adrenal origin. The symptomatic paragangliomas require multimodal treatment approach. Case Presentation We report a case of 34-year-old female who presented with complains of headache and blurring of vision. Examination revealed Hypertensive crisis with grade 3 retinopathy. The patient was diagnosed as a case of extra-adrenal paraganglioma during evaluation for secondary hypertension. Magnetic Resonance Imaging of the abdomen revealed 23*28*28 mm mass lesion in the inter-aortocaval region at the level of L3–L4. The patient was started on medical management for hypertension and was managed safely with robot-assisted surgery, owing to enhanced visualization of feeding vessels and avoidance of excessive manipulation of tumor. Conclusions Our case report emphasizes on the robot assisted surgical technique for the removal of tumor in a vulnerable anatomical position and presents a review of the literature.

Pheochromocytoma due to a novel SDHD variant presenting as unilateral visual loss

Summary A 53-year-old female presented to a tertiary ophthalmology referral centre complaining of unilateral painless loss of vision. Subsequent assessment revealed malignant hypertension causing right-sided cystoid macular oedema. During the course of secondary hypertension workup, she was diagnosed with a 7.8 cm phaeochromocytoma which was resected. Testing for a panel of all predisposing phaeochromocytoma-causing variants using next-generation sequencing resulted in the diagnosis of a novel SDHD variant. Learning points Screening for secondary causes of hypertension is indicated when there is evidence of hypertension-mediated end-organ damage (1). Testing for a predisposing variant should be considered in all patients with phaeochromocytoma or paraganglioma due to the high heritability rate and prevalence of somatic variants (2, 3, 4). Novel variants are commonly uncovered in the Succinate Dehydrogenase (SDH) subunit; proving pathogenicity is a complex, time-consuming process and one challenge of next-generation sequencing (3). SDHB immunohistochemistry as a tool for demonstrating pathogenicity is associated with reduced sensitivity when assessing SDHD variants (5, 6).

Irisin Is Correlated with Blood Pressure in Obstructive Sleep Apnea Patients

Background. Despite approximately 95% primary cases of hypertension, secondary hypertension seems to be common with resistant forms. Notably, obstructive sleep apnea (OSA) is known as a common cause of secondary hypertension and has a major characteristic of obesity. Irisin acts as a link between muscles and adipose tissues in obesity, playing an essential role in human blood pressure (BP) regulation. However, whether irisin is associated with secondary hypertension caused by OSA and how it takes effect essentially have not been elucidated. Purpose. To investigate the changes of irisin and its relationship with BP in OSA. Methods. 72 snoring patients finished Epworth Sleep Scale (ESS) evaluation before polysomnography (PSG). BP was the average of three brachial BP values by mercury sphygmomanometer. Serum irisin level was determined by enzyme-linked immunosorbent assay (ELISA). Results were analyzed by SPSS software. Results. Irisin was higher in the severe and quite severe group than that in control and nonsevere groups ( p < 0.05 ). For BP, significant differences were found between the control group and the other three groups ( p < 0.05 ) and between the quite severe and the other three groups ( p ≤ 0.001 ). Positive correlations were found between irisin and apnea-hypopnea index (AHI), AHI and BP, and irisin level and BP. Negative correlations were between irisin and SpO2 nadir and SpO2 nadir and BP. Positive correlation still existed between AHI and irisin even after adjusting for some obesity-related variables. Conclusions. Irisin may serve as a potential biomarker for severity of OSA independently of obesity and imply the development of hypertension.

Implantation of Covered Stent for Coarctation of the Aorta and Secondary Hypertension in Adolescents—Case Report

Introduction: Coarctation of the aorta represents a narrowing of the thoracic aorta. Hypertensive patients with blood pressure differences ≥20 millimetres of mercury have an indication for surgical or interventional treatment. Implantation of a covered stent became the preferred therapy for the management of this pathology in adolescents/adults. Case report: We report the case of a 14-year-old male sportsman, who presented in the emergency room with headache, dizziness, and tinnitus. The clinical exam revealed blood pressure differences between the upper and lower limbs of up to 50 mmHg. Based on the clinical and paraclinical data, we established the diagnosis of coarctation of the aorta and severe secondary arterial hypertension. The case was discussed by a multidisciplinary team and accepted for covered stent implantation. The 24 h blood pressure Holter monitoring after the procedure indicated the persistence of stage I arterial hypertension. Conclusions: Coarctation of the aorta is a congenital cardiovascular anomaly with high morbidity and mortality rates. Arterial hypertension, heart failure, and aortic dissection are complications of this pathology, some of them being sometimes direct consequences of secondary hypertension. Periodic cardiology follow up after the procedure is mandatory to assess the hemodynamic response, to identify potential complications, and to stratify the cardiovascular risk.