Key result
Five case reports on secondary and malignant hypertension highlight emerging clinical conditions and novel technologies for their management, including renal artery fibrodysplasia and adrenal tumors.
This editorial underscores the necessity of thorough investigation and advanced diagnostic tools in identifying and treating secondary causes of hypertension.
Clinical guidelines for the management of hypertension are regularly updated, and the whole community of hypertension specialists is longing for the promised new guidelines of the ESH/ESC, expected to be released next June 2023. Despite the availability of such important reference points, in daily practice the management of hypertension often requires refined clinical skills. This is particularly true when facing peculiar hypertensive cases, such as resistant hypertension or hypertension of the young, non obese patient. In these cases, the possibility to diagnose a secondary form of hypertension is particularly high, but requires that all the possible causes are explored, even those that are less frequent. This is when the investigation capabilities of the clinician comes at hand, only if supported by a profound knowledge of physiopathology of the disease.In this series of clinical cases in hypertension, five papers were published from January 2022 to January 2023. Again, the relevance of a similar Research Topic is confirmed by it impact around the world (Figure 1). The treated cases focus on specific cases, sometimes neglected by the guidelines for the lack of specific clinical trials. Two cases approach the adrenal gland as causative of hypertension, one regards the renal artery, and two more identify an oncological cause of disease, with very well hidden tumors. Al together, these cases let to point out to emerging clinical conditions in hypertension and the possibility of novel technology for their management.Fibromuscular dysplasia (FMD) is the second-most common etiology of renal artery stenosis (RAS) and renovascular hypertension, especially in young patients [1]. FMD is a systemic arterial disease presenting as arterial stenosis, aneurysm and dissection in virtually any arterial bed. When affecting the renal artery, FMD causes important cardiovascular manifestations including renovascular hypertension. Although several studies have demonstrated benefits with renal artery revascularization, It is difficult to predict the real benefit of this procedure, and evidences are raising that appropriate patient selection is therefore the key to avoid therapeutic failure. [2] In this report, Yuxi Li and colleagues borrow hemodynamic assessment technology from coronary intervention using the combination of pressure guidewire (to calculate Flow Fractional Reserve, FFR) and coherence tomography (OCT)-guided angioplasty treatment in a young hypertensive patient with renal FMD. [3] The Authors walk us through the interpretation of the data received from the combination of both technologies. In particular, OCT helps in confirming the diagnosis in the angiographically ambiguous lesion, properly classifying FMD, and guiding and optimizing the intervention. The FFR before and after the procedure allowed to demonstrated the efficient therapy of the stenosis with a significant reduction and normalization of pressure gradient. The combined use of OCT and rFFR may improve diagnosis, stratification of patient and identify patients who may respond to revascularization, and guide the procedure.2. Superselective adrenal arterial embolization in primary aldosteronism. About 4-5% of patients undergoing abdominal imaging report adrtenal incidentaloma, with an increase in the number of patients in the age group. A similar percentage (6%) is reported in a series of 87,065 autopsies. [4] Interventional radiology of the adrenal glands represents an important interventional radiologic procedure indicated during the primary aldosteronism (PA). Hokotate et c. [5] reported that 33 cases of aldosteronism had been treated with embolization. Results are encouraging: Plasma levels of aldosterone return to normal in 82% of patients. In this issue, the case report of a successful selective embolization of the adrenal adenoma in a 39 year old male with a 5 year history of hypertension. [6] 3. Primary Aldosteronism and Subclinical Cushing Syndrome Over the years, the approach to identifying, diagnosing, and treating primary aldosteronism has remained unchanged and often focuses on detecting and treating the more severe symptoms of the condition. However, recent evidence shows that the prevalence of primary aldosteronism is higher than previously thought, and that the small and abnormal production of aldosterone without renin alteration may cause an increase in cardiovascular risk [7]. The CoSh syndrome (synchronous adrenal aldosterone and excess cortisol ) is a distinct entity in PA, as described by Arlt et al. in 2017 [8]. The disease has been studied for more than 40 years. Recently, this has become one of the most dynamic topics in hormonally active adrenal lesions, due to significant progress in steroid metabolomics, molecular genetics of the immune CYP11B1/B2 gene constellation, and newly developed pathological types in the 2022 WHO classification [8] . Lihua Hu et al. describe the management of a 49 years old women with coexisting primary aldosteronism (PA) and subclinical Cushing's syndrome due a right adrenal adenoma. [9] 4. Pheochromocytoma of the urinary bladder. According to literature, pheochromocytomas account for 0.05-0.1% of patients with hypertension[10]. Although paroxysmal or resistant hypertension is still a major cause for diagnostic workup of pheochromocytoma, there is a steadily increasing proportion of cases found as part of investigation for adrenal incidentalomas or accidentally found extra-adrenal paragangliomas showing few, if any, symptoms.[10] Eighty to eighty-five percent are phaeochromocytomas arising from the adrenal medulla. Fifteen to twenty percent are sympathetic paraganglioma arising from the sympathetic ganglia in thorax, abdomen, and pelvis [11] Liang-Liang Hu1 and colleagues describe the unusual localization of a paragalioma/pheochromocytoma to the baladder of a 52-year-old woman with paroxysmal palpitations. [12] 5. Malignant hypertension Malignant Hypertension is defined as uncontrolled severe hypertension in presence of an acute organ damage, including brain, heart and kidney. This condition is often associated with a thrombotic microangiopathy, which is expression of endothelial acute damage [13]. For this reason it has to be differentiated from thrombotic thrombocytopenic porpora, which would address the management of the case towards plasma exchange. Bosisio et al describe the case of a patient affected by malignant hypertension Prior history of hypertension, high mean arterial pressure, significant renal impairment but relatively modest thrombocytopenia and lack of severe ADAMTS-13 deficiency (activity <10%) at diagnosis are clues to diagnose malignant hypertension-induced thrombotic microangiopathy [14]. This very well described case allow the reader to focus on 3 major issues: 1) Malignant hypertension needs to be included into the flowchart for the diagnosis in presence of acute syndrome, in order to spare time and prevent further damage; 2) fast reduction of blood pressure levels might as well favor the progression of the organ damage, given the reduced perfusion of target organs suche as the heart or the kidney; 3) recidivism occurs in particular to those patients that do not adhere to treatment, therefore strategies for improving adherence (including closer follow-ups) need to be put in place.
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Guido Iaccarino (2023) conducted an editorial in Hypertension. Five case reports on secondary and malignant hypertension highlight emerging clinical conditions and novel technologies for their management, including renal artery fibrodysplasia and adrenal tumors.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: