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Introduction Malignant hypertension, the most severe form of hypertension, is characterized by acute and diffuse microvascular damage. The ophthalmological, renal and cardiological consequences are well described. Brain damage is considered rare and poorly known, although it is one of the main causes of death. Method We performed a systematic cerebral MRI on patients of the Bordeaux cohort with malignant hypertension between 2008 and 2018, regardless of their neurological symptoms. A subgroup of patients benefited from a second cerebral MRI. All MRI were analysed by the same trained operator. Results 92 patients were included, 67.5% male, mean age 47.8 ± 12 years. On admission, 41.5% of the patients were totally asymptomatic from a neurological aspect. Cerebral MRI was normal in 18.5% of patients. A posterior reversible encephalopathy syndrom was found in 16.5% of patients, 33.5% had an ischemic or hemorrhagic stroke. Patients had marked microvascular impairment (SVD score 2 or higher) in 59% of cases. In asymptomatic patients or patients with headaches only, MRI found PRES in 15.5% of cases, recent ischemic or hemorrhagic stroke in 29% of cases, and SVD score 2 or higher in 51.5% of cases. Conclusion Most patients with malignant hypertension have brain injuries, regardless of their symptoms. This warrants systematic cerebral MRI, as the results may impact on their management. The consequences of these severe lesions, which are disproportionately severe for young patients, raise questions about the need for cognitive assessment and management.

Introduction Malignant hypertension still remains a severe condition that requires early recognition and treatment. The therapy has evolved from extreme measures in the first 70 years of the last century to intravenous treatment with modern antihypertensive treatment. Today, in the absence of trials and solid evidences, there are no formal guidelines on how to treat MHT. There is a relative consensus on a treatment based on intravenous infusion of antihypertensive treatments in the first days. This review summarizes the historical background and pathophysiological evidences which led to the actual most common pharmacological management of this condition but also can contribute to challenge this management. Oral treatment starting with low dose angiotensin converting enzyme inhibitors appears as a solid option and may improve the management of malignant hypertension in low-income countries where this form of hypertension remains relatively frequent. Future developments are considered according to the latest progress on the topic.

Background Malignant arterial hypertension is a poorly known pathology, with a poor prognosis in absence of adequate emergency care. Since 1995, the excellence center in Bordeaux University Hospital has been implementing a therapeutic protocol based on rapid titration of angiotensin converting enzyme inhibitor for the management of these acute patients. This “Bordeaux protocol” has demonstrated its effectiveness and safety but its widespread use is limited by the fear of complications in patients with renal artery stenosis. Objectives The objective of this study is to compare the protocol safety and efficacy in patients with renovascular and non-renovascular malignant hypertension. Method Our retrospective case-control study compared 2 groups of patients from the Bordeaux cohort of malignant hypertension since 2008. The first group included patients with renovascular malignant hypertension and the second one the rest of the cohort. We excluded patients managed in non-urgent basis. Results We included 10 patients with renovascular malignant hypertension and 74 control patients. No serious adverse events occurred in both groups (acute kidney failure requiring dialysis, threatening hyperkalemia, sudden drop in blood pressure requiring immediate attention). Discontinuation of ACE inhibitors for significant elevation of creatinine occurred once in each group (P = 0.22). Blood pressure drop between admission and discharge was similar in the 2 groups (-73mmHg ± 43 mmHg vs. −75mmHg ± 31.7 in the control group, P = 0.9). One-month prevalence of normotensive patients was also comparable (85% vs. 73% in the control group, P = 0.67). Conclusions Efficacy and safety of the “Bordeaux Protocol” is maintained in patients with renovascular malignant hypertension, thanks to close monitoring. These results need to be replicated to be widespread.

Malignant hypertension (MHT) still exists, leading young people to a poor prognosis. Yet, we lack clear guidelines, mainly because data are scarce. We initiated the first MHT multicentric network to assess these issues. We built a 28 centers network so far, aiming for 40–50 French centers end 2020, and European participation in 2021. We prospectively recruit patients with severe hypertension (> 180/110) and severe hypertensive retinopathy (common definition) or 3-target organ damage among heart, kidney, brain and thrombotic microangiopathy (MOD HTN definition). We hope to recruit 500 patients in 5 years, with a 5 years follow up. We currently collect clinical and examination data, aiming for building a biobank and a target organ damage corelab to entirely reconsider the disease. During the first 6 months, we recruited as planned 58 patients, 41% were male, 49.1 ± 15 years old. Half of them were not known hypertensive, and one third presented a secondary hypertension. Non-observance was reported in 25% of patients as a trigger. Mean blood pressure was 219 ± 29//119 ± 20 mmHg. Patient care pathway was very different according to initial symptoms, target organ damage and centers: hypertension, neurology, nephrology, cardiology, internal medicine, intensive care unit and emergency department. Target organ damage was respectively 70%, 33%, 25% and 25% for kidney, heart, brain and thrombotic microangiopathy, mostly improving during follow up. Most of patients (70%) benefited from intravenous antihypertensive treatment and saline infusion. Length of stay was on average 8 days. Malignant Hypertension clearly didn’t disappear, but was diluted between the different specialities and forgotten. This most severe presentation has to be considered once again to better manage our patients. The unique HAMA network should make it possible to gather high-quality data to improve our knowledge and rediscover the disease.

Resume Des cas familiaux d'anticoagulant circulant de type antiprothrombinase ont ete rarement decrits. Nous rapportons une observation d'anticoagulant circulant de type antiprothrombinase chez deux sœurs, l'une atteinte de purpura thrombopenique immunologique et l'autre de lupus erythemateux dissemine.

Cardiac myxoma is an intracavitary neoplasm. Only 5% are found in the right of left ventricle. Classically, myxoma presents as constitutional, embolic or obstructive symptoms. The authors report a case of ventricular cardiac myxoma revealed by rheumatological signs.

A 26 year-old woman presents a systemic lupus erythematosus. Her sister has an immunologic thrombocytopenic purpura. Both have antiprothrombinase type circulating anticoagulant that seems to be the marker of hereditary immunologic disturbance.

A 69 year old woman presents syncopas related to paroxystic ventricular tachycardia. Endomyocardial and deltoid biopsies show mitochondrial abnormalities suggesting mitochondrial myopathy. No sign of this disease has happened when she was younger.