Malignant Hypertension:A Systemic Cardiovascular Disease: JACC Review Topic of the Week.

Malignant hypertension (MHT) is a hypertensive emergency with excessive blood pressure (BP) elevation and accelerated disease progression. MHT is characterized by acute microvascular damage and autoregulation failure affecting the retina, brain, heart, kidney, and vascular tree. BP must be lowered within hours to mitigate patient risk. Both absolute BP levels and the pace of BP rise determine risk of target-organ damage. Nonadherence to the antihypertensive regimen remains the most common cause for MHT, although antiangiogenic and immunosuppressant therapy can also trigger hypertensive emergencies. Depending on the clinical presentation, parenteral or oral therapy can be used to initiate BP lowering. Evidence-based outcome data are spotty or lacking in MHT. With effective treatment, the prognosis for MHT has improved; however, patients remain at high risk of adverse cardiovascular and kidney outcomes. In this review, we summarize current viewpoints on the epidemiology, pathogenesis, and management of MHT; highlight research gaps; and propose strategies to improve outcomes.

Revisiting malignant hypertension: rationale and design of the 'HAMA cohort', on behalf of the ESH working group 'hypertension and the kidney'.

BACKGROUND: Malignant hypertension has not disappeared and is associated with a poor prognosis. Yet, so far, it has received limited attention from the medical community. Guidelines are mainly based on expert consensus and low quality evidences. METHOD: We set up a prospective, multicenter, observational cohort of patients with malignant hypertension. We collect at admission medical history, demographic data, ongoing treatment, clinical parameters, symptoms, care pathways, target organ status and at discharge and during follow up treatment administrated, adverse events, blood pressure level, target organ status. We aim to recruit 500 patients with malignant hypertension in 5 years, with a 5-year follow-up. Our primary objective is to assess the 5 years prognosis of these patients. DISCUSSION: The HAMA (Hypertension Arterielle MAligne, meaning malignant hypertension) registry aims to describe the epidemiology and to assess the prognosis of malignant hypertension in a contemporary multidisciplinary cohort, with emphasis on the diversity of current management and care pathway among the different medical specialties. It may help improving our pathophysiological knowledge, and pave the way to update the definition of this particular form of hypertension. The multidisciplinary network developed in the wake of this project is expected to facilitate the set up therapeutic trials, laying the ground for evidence-based recommendations.

Cost Analysis of Radiofrequency Ablation for Adrenal Adenoma in Patients with Primary Aldosteronism and Hypertension: Results from the ADERADHTA Pilot Study and Comparison with Surgical Adrenalectomy.

PURPOSE: Primary Aldosteronism (PA) is increasingly considered as a common disease affecting up to 10% of the hypertensive population. Standard of care comprises laparoscopic total adrenalectomy but innovative treatment such as RadioFrequency Ablation (RFA) constitutes an emerging promising alternative to surgery. The main aim of this study is to analyse the cost of RFA versus surgery on aldosterone-producing adenoma patient from the French National Health Insurance (FNHI) perspective. METHODS: The ADERADHTA study was a prospective pilot study aiming to evaluate both safety and efficacy of the novel use of adrenal RFA on the patients with PA. This study conducted on two French sites and enrolled adult patients, between 2016 and 2018, presenting hypertension and underwent the RFA procedure. Direct medical (inpatient and outpatient) and non-medical (transportation, daily allowance) costs were calculated over a 6-month follow-up period. Moreover, the procedure costs for the RFA were calculated from the hospital perspective. Descriptive statistics were implemented. RESULTS: Analysis was done on 21 patients in RFA groups and 27 patients in the surgery group. The difference in hospital costs between the RFA and surgery groups was €3774 (RFA: €1923; Surgery: €5697 p < 0.001) in favour of RFA. Inpatient and outpatient costs over the 6-month follow-up period were estimated at €3,48 for patients who underwent RFA. The production cost of implementing the RFA procedure was estimated at €1539 from the hospital perspective. CONCLUSION: Our study was the first to show that RFA is 2 to 3 times less costly than surgery. The trial is registered at ClinicalTrials.gov under the number NCT02756754.

Malignant Hypertension: Current Perspectives and Challenges.

Malignant hypertension is a hypertensive emergency, with rapid disease progression and poor prognosis. Although recognized as a separate entity more than a century ago, significant knowledge gaps remain about its pathogenesis and treatment. This narrative review summarizes current viewpoints, research gaps, and challenges with a view to pooling future efforts at improving treatment and prognosis.

Neurologically asymptomatic patients frequently present cerebral injuries during malignant hypertension: a MRI study.

BACKGROUND: Acute and diffuse microvascular damage characterizes malignant hypertension (MHT), the deadliest form of hypertension (HTN). Although its ophthalmological, renal and cardiological repercussions are well known, brain involvement is considered rare with few descriptions, although it is one of the main causes of death. We hypothesized that brain MRI abnormalities are common in MHT, even in patients without objective neurological signs. METHOD: We analyzed retrospectively the brain MRI of patients admitted for acute MHT between 2008 and 2018 in Bordeaux University Hospital, regardless of their neurological status. A trained operator analyzed every brain MRI, looking for posterior reversible encephalopathy syndrome (PRES), ischemic stroke, intracerebral hematoma (ICH) and microangiopathy markers. We included 58 patients without neurological signs, 66% were men, and mean age was 45.6 ±â€Š11.3 years. RESULTS: Brain MRI were normal in 26% of patients but we found at least one acute abnormality on brain MRI in 29% and an Small Vessel Disease score (SVD score) of two or higher in 52%. In patients with neurological signs, these findings were 9, 53 and 70%, respectively. A PRES was found in 16% of asymptomatic patients and 31% had an ischemic stroke and/or a cerebral hematoma. CONCLUSION: PRES, recent hematoma, ischemic stroke and severe cerebral microangiopathy are common findings in MHT patients without neurological signs on admission. The impact of these findings on patient management, and their cerebrovascular and cognitive prognostic value, should be established. Brain MRI might need to become systematic in patients suffering from MHT episodes.

Radiofrequency ablation for adenoma in patients with primary aldosteronism and hypertension: ADERADHTA, a pilot study.

OBJECTIVE: To evaluate the efficacy and the feasibility of radiofrequency ablation to treat aldosterone-producing adenomas. METHODS: In an open prospective bicentric pilot study, patients with hypertension on ambulatory blood pressure measurement, a primary aldosteronism, an adenoma measuring less than 4 cm, and confirmation of lateralization by adrenal venous sampling were recruited. The primary endpoint, based on ABPM performed at 6 months after the radiofrequency ablation, was a daytime SBP/DBP less than 135/85 mmHg without any antihypertensive drugs or a reduction of at least 20 mmHg for SBP or 10 mmHg for DBP. RESULTS: Thirty patients have been included (mean age = 51 ±â€Š11 years; 50% women). Mean baseline daytime SBP and DBP were 144 ±â€Š19 / 95 ±â€Š15 mmHg and 80% received at least two antihypertensive drugs. At 6 months: 47% (95% CI 28-66) of patients reached the primary endpoint, mean daytime SBP and DBP were 131 ±â€Š14 (101-154)/87 ±â€Š10 (71-107) mmHg; 43% of them did not take any antihypertensive drug and 70% of them did not take potassium supplements. Few complications were recorded: four cases of back pain at day 1 postablation; three limited pneumothoraxes, which resolved spontaneously; one lesion of a polar renal artery. CONCLUSION: Radiofrequency ablation for hypertensive patients with aldosterone-producing adenomas seems to be an emerging promising alternative to surgery. Its efficacy and its feasibility have to be confirmed in a larger sample of patients.

The pharmacological management of malignant hypertension.

: Malignant hypertension (MHT) still remains a severe condition that requires early recognition and treatment. Over the years, the prevention and treatment of MHT have significantly advanced through the introduction of modern antihypertensive agents. However, in the absence of robust clinical trials, there remain no formal guidelines on the treatment of MHT. This review summarizes the historical background and pathophysiological evidence of MHT, which has led to common practices in its pharmacological management but can also introduce challenges. The current consensus for treatment involves early intravenous infusion of antihypertensive agents, but oral blockers of the renin-angiotensin system may improve the management of MHT, and it offers a suitable treatment option in low-income countries where the condition remains relatively prevalent.

Adult onset tubulo-interstitial nephropathy in MT-ND5-related phenotypes.

Kidney is a highly adenosine triphosphate dependent organ in human body. Healthy and functional mitochondria are essential for normal kidney function. Clinical and genetic variability are the hallmarks of mitochondrial disorders. We report here the involvement of two MT-ND5 pathogenic variants encoding for ND5 subunit of respiratory chain complex I, the m.13513G>A and the m.13514A>G, in adult-onset kidney disease in three unrelated patients. The first patient had myopathy encephalopathy lactic acidosis and stroke syndrome, left ventricular hypertrophy with Wolff-Parkinson-White syndrome and tubulo-interstitial kidney disease. The second presented Leber hereditary optic neuropathy associated with tubulo-interstitial kidney disease. The third presented with an isolated chronic tubulo-interstitial kidney disease. These mutations have never been associated with adulthood mitochondrial nephropathy. These case reports highlight the importance to consider mitochondrial dysfunction in tubulo-interstitial kidney disease.

[Malignant hypertension: A bright future]. / L'hypertension artérielle maligne , un futur prometteur.

Malignant hypertension has not disappeared, it has been forgotten. Its incidence is increasing again. It considerably worsens the prognosis of young patients (35 to 55 years old on average). There might be susceptibility factors, several hypotheses are under study. New diagnostic criteria and therapeutic options have been proposed and will have to be validated. Faced with these important challenges for patients, the first prospective multicentric register on this pathology will be set up in France in September 2019.

Malignant hypertension: diagnosis, treatment and prognosis with experience from the Bordeaux cohort.

BACKGROUND: Malignant hypertension, the most severe form of hypertension, is defined by high blood pressure and acute ischemic organ damage. It has a worse prognosis than other forms of hypertension, especially in black patients. New tools to assess organ damage, especially that of the heart and brain, are now available and may contribute to a better evaluation of these patients. This report improves knowledge of the characteristics of involved organs to facilitate diagnosis and to evaluate the effectiveness of our treatment protocol. METHOD: The Bordeaux registry, started in 1995, recruited 168 patients. In addition to evaluations of their eyes and kidneys, these patients had a systematic evaluation of their hearts with ECG and echocardiography and, since 2007, a systematic brain MRI. Blood pressure was lowered with a protocol based on blockers of the renin-angiotensin system started at a very low-dose with forced titration over 48 h. Only an oral route was used for antihypertensive medication. RESULTS: Systematic MRIs found significant brain damage in 93% of patients. Heart involvement was highly prevalent: 82% had left ventricular mass more than 60 g/m, and 56% had systolic dysfunction (estimated by global longitudinal strain). Renal involvement and thrombotic microangiopathy were respectively present in 55 and 15% of patients. Median follow-up was 48 months. Renal survival at 5 years was 90.8%, similar to other studies. CONCLUSION: Malignant hypertension is a systemic disease causing severe damage to the brain, heart, kidneys and eyes, even in absence of symptoms. Renin-angiotensin system blockers seem to be the cornerstone of treatment.

Screening for orthostatic hypotension using home blood pressure measurements.

OBJECTIVE: Orthostatic hypotension is a common condition associated with adverse cardiovascular and cognitive prognosis. Screening for orthostatic hypotension consists of blood pressure measurements in supine (or sitting) and standing position during clinical consultations. As orthostatic hypotension is a poorly reproducible clinical condition, it is likely that the simple measurement carried out during consultations underestimates the true prevalence of the condition. The objective of this study is, therefore, to determine whether screening for orthostatic hypotension with home blood pressure measurements (HBPM) may improve orthostatic hypotension diagnosis without compromising the quality of the blood pressure readings. MATERIALS AND METHODS: We asked all patients with indications for HBPM in the hypertension unit and in a general medical practice to perform a series of home blood pressure measurements, ending each series with a measurement in standing position. RESULTS: We recruited 505 patients of mean age 68 years of which 93% were hypertensive patients. The success rate of HBPM complying with the ESH criteria (12 out of 18 measurements) was 94.5%, which is comparable with previously published series of measurements. Ninety-one percent of patients measured their blood pressure at least once in standing position, and 88% of patients recorded all six standing measurements. Orthostatic hypotension prevalence defined as the presence of one episode of orthostatic hypotension was 37.47%, much higher than orthostatic hypotension prevalence measured in the same cohort in a clinic setting (15%). CONCLUSION: The measurement of blood pressure in standing position during HBPM is feasible without altering the quality of the blood pressure readings in seated position. Our findings show that orthostatic hypotension is significantly more often detected at home by the patient than at the doctor's office, which may allow quicker initiation of preventive and therapeutic strategies.