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.

Optical coherence tomography angiography findings in malignant hypertensive retinopathy / Achados da angiografia por tomografia de coerência óptica na retinopatia hipertensiva maligna

ABSTRACT A 33-year-old male presented to our clinic with low vision in both eyes that started during the previous week. Visual acuity was 20/63 in the right eye and 20/50 in the left eye. Fundus examination revealed signs of hypertensive retinopathy; thus, a multidisciplinary approach was adopted for the diagnosis and treatment of this patient. We consulted the nephrology and cardiology departments on this case. Upon diagnosing malignant hypertension and renal failure, the patient was put on hemodialysis. His visual acuity was 20/20 at 6 months, whereas foveal assessment on optical coherence tomography angiography revealed neither marked superficial and deep capillary density loss and foveal avascular zone enlargement nor a decrease in disc flow and radial peripapillary capillary density. Early diagnosis and treatment of malignant hypertension are critical in preventing progression of end-organ damage including the eyes. Optical coherence tomography angiography may be useful in cases when fundus fluorescein angiography is relatively contraindicated (e.g., renal failure).

RESUMO Um homem de 33 anos apresentou-se à nossa clínica com baixa visão em ambos os olhos que começou uma semana antes. A acuidade visual foi de 20/63 no olho direito e 20/50 no olho esquerdo. O exame de fundo de olho revelou sinais de retinopatia hipertensiva; então, adotou-se uma abordagem multidisciplinar para o diagnóstico e tratamento desse paciente. Consultamos os departamentos de nefrologia e cardiologia neste caso. Ao diagnosticar hipertensão maligna e insuficiência renal, o paciente foi colocado em hemodiálise. Sua acuidade visual era 20/20 aos 6 meses, enquanto a avaliação foveal com angiotomografia de coerência óptica não revelou perda de densidade capilar superficial e profunda acentuada e aumento da zona avascular foveal nem uma diminuição no fluxo de disco e na densidade capilar peripapilar radial. O diagnóstico precoce e o tratamento da hipertensão maligna são fundamentais na preveção da progressão de danos nos órgãos-alvo, incluindo os olhos. A Angiografia por tomografia de coerência óptica pode ser útil nos casos em que a angiografia com fluoresceína do fundo de olho é relativamente contraindicada (por exemplo, insuficiência renal).

Optical coherence tomography angiography findings in malignant hypertensive retinopathy.

A 33-year-old male presented to our clinic with low vision in both eyes that started during the previous week. Visual acuity was 20/63 in the right eye and 20/50 in the left eye. Fundus examination revealed signs of hypertensive retinopathy; thus, a multidisciplinary approach was adopted for the diagnosis and treatment of this patient. We consulted the nephrology and cardiology departments on this case. Upon diagnosing malignant hypertension and renal failure, the patient was put on hemodialysis. His visual acuity was 20/20 at 6 months, whereas foveal assessment on optical coherence tomography angiography revealed neither marked superficial and deep capillary density loss and foveal avascular zone enlargement nor a decrease in disc flow and radial peripapillary capillary density. Early diagnosis and treatment of malignant hypertension are critical in preventing progression of end-organ damage including the eyes. Optical coherence tomography angiography may be useful in cases when fundus fluorescein angiography is relatively contraindicated (e.g., renal failure).

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.

Takayasu arteritis presenting with malignant hypertension; a rare manifestation of a rare disease: a case report and review of the literature.

Takayasu arteritis (TA) is a chronic inflammatory and obliterative disease of large vessels, which mainly affects the aorta and its major branches. TA can lead to renal failure and renovascular hypertension in 60% of patients; it is rare in children aged <10 years and, more rarely, it presents with malignant hypertension in the paediatric age group. Here we present a case of 9-year-old boy with TA who presented with malignant hypertension and required surgical intervention to control the blood pressure. Subsequently, his medications were titrated using 24 h ambulatory blood pressure monitoring (ABPM) and is doing well on follow-up.

When the chimney is blocked: malignant renovascular hypertension after endovascular repair of abdominal aortic aneurysm.

BACKGROUND: The Chimney graft (CG) procedure is one of the novel modification techniques of the endovascular aneurysm repair (EVAR) surgery to treat suprarenal and juxtarenal abdominal aortic aneurysms. Other indications for the use of CG placement include thoracic and thoracoabdominal aneurysms with supraortic branches orifice involvement and cases of common iliac artery aneurysms with or without internal iliac artery involvement. The technique is used in patients who due to aortic-neck morphology and lack of adequate fixation and/or sealing zones are not eligible for standard EVAR. In this procedure, a parallel stent-graft is placed adjacent to the main body of the aortic endograft to maintain blood supply to renovisceral or supraortic branches, once the body of the aortic stent-graft is deployed. Symptomatic occlusions of the CG with novel renovascular hypertension were not described until now. CASE PRESENTATION: A-64-year-old male patient, presented with new-onset malignant hypertension, 13 months after an EVAR operation with CG placement to the left renal artery. The patient was on preventive clopidrogel therapy, which was withheld temporarily for several days, one month before presentation. Imaging studies revealed a novel form of iatrogenic renovascular hypertension, caused by occlusion of the CG. Any attempt to recanalize the covered stent or revascularize the left kidney was rejected and conservative treatment was chosen. Seven months after presentation, blood pressure was within normal ranges with little need for antihypertensive therapy. CONCLUSIONS: Physicians should be aware that the novel emerging techniques of EVAR to overcome the limitations of the aortic-neck anatomy may still adversely influence the renal outcome with potential development of new-onset hypertension.

A comprehensive assessment of cardiac structure and function in patients with treated malignant phase hypertension: the West Birmingham Malignant Hypertension project.

BACKGROUND: Previous studies have confirmed that cardiac structural and functional abnormalities exist in patients with malignant hypertension (MHT). The effect of long-term blood pressure control in MHT patients on cardiac structure and function is still unknown. METHODS: We performed detailed left ventricle (LV) assessment using two-dimensional (2DE) and three-dimensional (3DE) echocardiography, and tissue Doppler imaging (TDI) in patients with previous MHT (but now in stable phase) who were compared with patients with treated 'high risk' hypertension (HHT, but non-MHT) and healthy controls (HC). Vasodilator stress myocardial contrast echocardiography (in addition to wall motion analysis) was used to exclude significant coronary artery disease, as part of our comprehensive echocardiographic assessment. Septal and posterior wall thickness, LV mass index, LV volumes and ejection fraction, mitral valve inflow indices (E, A) mitral annular velocity (S, E') and left atrial volume index (LAVI), were calculated using 2DE, 3DE, and TDI. MHT patients had good blood pressure control for an average of 144months. RESULTS: A total of 95 subjects (MHT=15; HHT and HC=40 each) were studied. Both posterior and septal wall thickness were significantly higher in the MHT and hypertensive groups compared to normal controls with no difference between MHT and HHT. No significant difference in LV ejection fraction was found between the 3 groups. Increased LAVI (p<0.05 MHT vs. HC and HHT vs. HC), reduced 'S' velocity on TDI (p=0.05 MHT vs. HC and vs.HHT, p<0.001 HHT vs. HC) and higher E/E' (p=0.029 HHT vs. HC) and lower E/A ratio (p=0.001 MHT vs. HC, p<0.001 HHT vs. HC) values were detected in the two hypertensive groups. CONCLUSION: Despite long-term good blood pressure control, MHT patients have persistent structural and functional changes in LV function on echocardiography, comparable to that seen in HHT.

Vascular ventricular coupling in patients with malignant phase hypertension: the West Birmingham malignant hypertension project.

Patients with malignant hypertension (MHT) have persistent vascular dysfunction and a much worse clinical prognosis than non-MHT hypertensive patients, despite good long-term blood pressure (BP) control. We hypothesized that abnormal arterial (arterial elastance (Ea); arterial elastance index (EaI)) and ventricular (End-systolic elastance (Ees) and End-diastolic elastance (Eed)) elastances are present in treated MHT patients, compared with non-MHT hypertensive controls. Echocardiographic parameters of cardiac and vascular stiffness (EaI, Ees and Eed) were quantified in patients with stable MHT and treated 'high-risk' hypertension patients (HHT, but non-MHT). All patients had well-controlled BP, with a median follow-up time for MHT of 144 months. Ea was calculated from stroke volume and systolic BP and adjusted by body area (EaI). Ees was calculated using systolic and diastolic BP, stroke volume, ejection fraction, time intervals and estimated normalized ventricular elastance at arterial end diastole. Eed was calculated from Doppler parameters and the diastolic filling volume. Both study groups had preserved left ventricular contractility, with no significant differences on 3D-echocardiography (P=0.10) There were no significant differences in EaI (P=0.83), Ees (P=0.32), Eed (P=0.23) and arterial-ventricular interaction (Ees/Ea, P=0.69). In the MHT group, Eed positively correlated with age (r=0.56, P=0.38) and systolic BP (r=0.68, P=0.008). On multivariable regression analysis, MHT status was not predictive of the ventricular and Ea. Despite documented vascular dysfunction in patients with previously diagnosed stable MHT, the arterial and systolic elastances were similar to HHT patients, suggesting that adequate BP control in MHT patients allows preservation or restoration of normal arterial-ventricular coupling.

Persistent macrovascular and microvascular dysfunction in patients with malignant hypertension.

Endothelial dysfunction is characteristic of patients with essential hypertension, but only limited data are available on different aspects of endothelial function in patients with malignant-phase hypertension. We investigated myocardial perfusion using real-time quantitative myocardial contrast echocardiography with concurrent assessment of macrovascular and microvascular endothelial damage/dysfunction in patients with previous malignant hypertension (but now in stable phase), who were compared with patients with treated "high-risk" hypertension (hypertension) and healthy controls. We measured flow (hyperemia)-mediated dilation and response to glyceryl trinitrate of brachial artery (ultrasound), microvascular (forearm) response to acetylcholine and sodium nitroprusside (laser Doppler), pulse wave velocity, circulating endothelial and endothelial progenitor cells in 15 patients with malignant hypertension, 40 matched patients with hypertension, and 40 healthy controls. Patients with malignant hypertension had impaired endothelial-dependant response to acetylcholine (P<0.001, but not to sodium nitroprusside) compared with hypertension and impaired reaction to both stimuli compared with healthy subjects (P<0.001). Patients with malignant hypertension had increased circulating endothelial cells (P=0.001), endothelial progenitors (P=0.008), and stiffness (P=0.003). Both hypertensive groups had impaired response to hyperemia and glyceryl trinitrate when compared with healthy controls (P<0.05). Both hypertensive groups had similar myocardial perfusion, which was significantly lower than in healthy controls. There were no significant differences in hyperemia and endothelium-independent stimuli between the 2 hypertensive groups. In conclusion, despite fairly well-controlled blood pressure, malignant hypertension patients had more pronounced abnormalities of macrovascular and microvascular function (which seem to be both endothelium dependent and endothelium independent) compared with patients with hypertension and healthy controls.

Echocardiographic changes in patients with malignant phase hypertension: the West Birmingham Malignant Hypertension Register.

In order to study the echocardiographic abnormalities in consecutive patients with malignant phase hypertension (MHT), we reviewed echocardiograms of 31 patients (23 male; mean age 52+/-14 years) with MHT who were admitted to our unit. Trans-thoracic echocardiography was carried out in all patients, and echocardiographic measurements were compared with those of 39 patients (30 male; mean age 54+/-10 years) with controlled nonmalignant essential hypertension, and 32 (19 male; mean age 51+/-10 years) healthy normotensive volunteers. Patients with MHT had a significantly higher mean systolic and diastolic blood pressure (P<0.001) compared to the other two groups. MHT patients had significantly greater mean left atrial dimensions (P=0.002), as well as aortic root dimensions (P=0.01) and left ventricular (LV) dimensions (with the exception of the diastolic internal diameter) (P<0.001). MHT patients also had a mean larger LV mass and LV mass index (both P<0.001) when compared to the other two groups. The mean ejection fraction was also lower in the MHT group (P<0.001). In conclusion, patients with MHT have significant cardiac hypertrophy, in association with systolic dysfunction and dilated left atria, irrespective of the duration of known hypertension. These abnormalities may predispose MHT patients to cardiovascular complications including heart failure and cardiac arrhythmias, such as atrial fibrillation.

Control of blood pressure and prevention of end-organ damage in patients with accelerated hypertension by combination with arotinolol and extended release nifedipine.

In patients with accelerated (malignant) hypertension, end-organ damage is the determinant factor for prognosis. Although recent advances in antihypertensive therapy have improved the outcome of patients with accelerated hypertension, the effectiveness of antihypertensive therapy still remains less convinced. In this study, we followed 13 patients clinically diagnosed with accelerated hypertension (defined as diastolic blood pressure > 130 mmHg, retinopathy with K-W IV and accelerated renal impairment) for 3 yr. One patient died due to acute myocardial infarction arising from poor compliance with antihypertensive therapy. One patient was maintained on hemodialysis for 3 yr. One patient was introduced for continuous ambulatory peritoneal dialysis (CAPD) for a year and then lived without dialysis therapy. The remaining 10 patients were followed for 3 yr. All patients were initially treated with intravenous administration of calcium antagonist for reduction of blood pressure, followed by hemodialysis therapy if needed. After stabilization of blood pressure, combination therapy with extended release nifedipine (40 to 80 mg daily) and arotinolol (20 mg daily) was started. The targets for blood pressure control were a systolic pressure of 135 mmHg and a diastolic pressure of 80 mmHg. If blood pressure control was unsatisfactory, guanabenz (2 to 4 mg before bedtime), a central acting drug, was added. At presentation, the mean diastolic blood pressure (mDBP) among the 10 remaining patients was 134 +/- 2 mmHg, the mean serum creatinine (mScr) was 4.5 +/- 0.7 mg/dl and the left ventricular mass index (LVMi) as measured by echocardiography was 150 +/- 9 g/m2. At 1 yr, the mDBP was reduced to 90 +/- 3 mmHg, the mScr to 2.9 +/- 0.9 mg/dl and the LVMi to 140 +/- 9 g/m2. At 3 yr, the mDBP was stabilized at 79 +/- 3 mmHg, the mScr maintained at 2.2 +/- 0.4 mg/dl, and the LVMi reduced to 128 +/- 9 g/m2. These results indicate that appropriate blood pressure control is important for improvement of renal impairment and cardiac damage in patients with accelerated hypertension. Moreover, combination therapy with arotinolol and extended release nifedipine may be beneficial for this purpose.

[Acute renal vein thrombosis in children. Early detection with duplex and color-coded Doppler ultrasound]. / Akute Nierenvenenthrombose bei Kindern. Früher Nachweis mit Duplex- und farbkodierter Dopplersonographie.

Two children with clinically suspected renal vein thrombosis were evaluated by duplex Doppler sonography and colour flow imaging. Both cases presented unspecific findings with an enlarged kidney and loss of cortico-medullary delineation on gray-scale ultrasound, but an unusual flow pattern with retrograde plateau-like frequency shifts during diastole. No venous signal could be obtained. By colour flow imaging only the main renal artery and its proximal branches could be visualised with a reverberating oscillation of blood flow. In addition, partial thrombosis of inferior vena cava in one patient and iliac vein thrombosis in the other could be demonstrated. Clinical improvement during fibrinolytic therapy in one case and nephrectomy in the other case confirmed the diagnosis.

[Cerebral, central and intracardiac hemodynamics in patients with progressive arterial hypertension following the surgical suppression of adrenal hyperfunction]. / Sostoianie tserebral'noi, tsentral'noi i vnutriserdechnoi gemodinamiki u bol'nykh progressiruiushchei arterial'noi gipertenziei posle khirurgicheskogo podavleniia giperfunktsii nadpochechnikov.

Forty-eight patients with progressive (malignant) arterial hypertension resistant to conservative therapy were subjected to surgical portalization of the adrenal blood stream in order to metabolize excessively produced aldosterone in the liver of the patients. Cerebral hemodynamics was studied before by tetra- and bipolar rheoencephalography, central hemodynamics was studied by tetrapolar transthoracic rheography, and intracardiac hemodynamics by echocardiography before and after surgery; blood plasma aldosterone and hydrocortisone concentrations and plasma renin activity were measured. A significant reduction of arterial pressure, elimination or alleviation of subjective and objective manifestations of chronic hypertensive encephalopathy were seen in the majority of patients after surgery. Cerebral blood flow improved, blood plasma aldosterone and renin activity reduced, myocardial hypertrophy decreased, and a trend to normalization of intracardiac hemodynamics was observed.

Abnormal captopril renogram in a patient without renovascular hypertension.

Captopril renal scintigraphy is a well-known noninvasive tool in the diagnosis of renovascular hypertension. The authors present a case of a patient with malignant hypertension whose captopril renal scintigraphy suggested bilateral and equal renovascular hypertension. His renal angiogram, however, demonstrated no renal artery stenosis or abdominal aortic atherosclerotic disease. False-positive captopril renal scintigraphy has been reported but can usually be attributed to the patient's hypotensive episode after captopril administration, volume or salt depletion, or chronic glomerulonephropathy. This patient demonstrated mild blood pressure changes after captopril administration and was not volume or salt depleted. His creatinine was 1.6 mg/dl on admission but demonstrated appropriate renal function on subsequent noncaptopril renal scintigraphy.

Ventricular function by radionuclide ventriculography in malignant hypertension.

Malignant hypertension is a unique and natural model for the study of abnormalities of left ventricular function due to arterial hypertension, because the development and regression of these abnormalities can be observed in a short period. Studies of ventricular function by radionuclide ventriculography, either before or after therapy, have not been previously reported in malignant hypertensive patients. We used this methodology to study left ventricular function in 17 malignant/accelerated hypertensive patients at the time of admission to the hospital and 3, 6, and 9 months after discharge. Seventy percent of patients (12 of 17) had symptoms of congestive heart failure at admission. We compared these data with those obtained in 12 normotensive subjects and 13 mild-to-moderate untreated hypertensive patients. Blood pressure of malignant hypertensive patients was 213 +/- 26/140 +/- 17 mm Hg at admission and 165 +/- 23/101 +/- 15 after 9 months of therapy. Radionuclide ventriculography at admission showed that peak filling rates of malignant hypertensive patients (2.13 +/- 0.21 end-diastolic volume [counts] [EDV]/sec) were significantly lower than those in normotensive subjects (2.40 +/- 0.41) and in mild-to-moderate hypertensive patients (2.46 +/- 0.21). In contrast, peak ejection rates were significantly higher in malignant hypertensive patients (3.44 +/- 0.38 EDV/sec) than in the two control groups (3.01 +/- 0.32 and 3.10 +/- 0.43, respectively). Ejection fractions were similar in the three groups of patients. After 9 months of therapy, peak filling rates of malignant hypertensive patients increased to 2.38 +/- 0.35 EDV/sec, whereas peak ejection rates decreased to 2.89 +/- 0.43 EDV/sec, both not significantly different from data in controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Progressive vascular lesions in Williams-Beuren syndrome.

We report two patients with Williams-Beuren syndrome. The first patient showed no evidence of coarctation of the aorta at the first examination. Seven years later, she developed coarctation of the aorta. In the second patient, we found the progression of renal artery stenosis by serial angiography. We report that vascular lesions may be progressive in Williams-Beuren syndrome.

Computed tomographic changes of hypertensive encephalopathy.

Computed tomographic (CT) scans were evaluated in 11 patients with acute hypertensive encephalopathy. Hypertensive encephalopathy is characterized by an acute, severe rise in blood pressure associated with headache, nausea, vomiting, altered mental status, and focal neurologic deficits, and rapid improvement after control of blood pressure. The systolic blood pressure range is 200-280 mm Hg; diastolic is 130-170 mm Hg. The most common CT finding was white-matter edema, diffuse or focal, affecting the supratentorial compartment in all cases and the infratentorial compartment in eight. These changes resolved after the blood pressure was lowered in all six patients studied by follow-up CT. Permanent areas of infarction were demonstrated in three patients. These abnormalities are correlated with the neuropathologic findings in hypertensive encephalopathy.