IntroductionIdiopathic intracranial hypertension (IIH) is a headache disorder that can result in irreversible blindness. Currently there are no published guidelines on the management of IIH. The aim ...of this audit is to determine whether patients presenting to Frimley Park Hospital (FPH) with IIH are adequately assessed and treated, based upon evidence from published clinical trials.MethodsPatients presenting to FPH with a new diagnosis or relapse of chronic IIH between 01/01/2013 and 31/12/2015 were identified using coding. Data was collected retrospectively from healthcare records.Results20 patients were audited. 14 were new diagnoses. Diagnosis was confirmed in all patients by fundoscopy, MRI brain and lumbar puncture (LP). 86% had visual acuity recorded at presentation. 72% patients reported partial symptom improvement with weight loss, 37% reported benefit with acetazolamide. 79% underwent more than one LP but response reduced with successive procedures. No patient requiring >3 LP’s achieved complete remission. 3 patients underwent CVS stenting.ConclusionEarly follow up may help identify patients who are unlikely to achieve complete remission with medical therapy alone. The role of early aggressive repeat LP needs prospective evaluation given that remission seems to be achieved. Guidelines are needed to develop future standards of management.
Pre-capillary pulmonary arterial hypertension (PAH) is hemodynamically characterized by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, pulmonary capillary wedge pressure (PAWP) ≤15 mmHg and ...pulmonary vascular resistance (PVR) > 2. PAH is classified in six clinical subgroups, including idiopathic PAH (IPAH) and PAH associated to connective tissue diseases (CTD-PAH), that will be the main object of this review. The aim is to compare these two PAH subgroups in terms of epidemiology, histological and pathogenic findings in an attempt to define disease-specific features, including autoimmunity, that may explain the heterogeneity of response to therapy between IPAH and CTD-PAH.
ObjectiveThis study assessed the long-term effects of triple therapy with prostanoids on patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), as there is ...limited information on the safety and efficacy of this treatment approach.MethodsA retrospective cohort study was conducted on patients with PAH-CHD who were actively followed up at our centre. All patients were already receiving dual combination therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walking test distance (6MWTD) and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, were documented before initiating triple therapy and annually for a 2-year follow-up period.ResultsA total of 60 patients were included in the study, with a median age of 41 years and 68% being women. Of these, 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH and 1 had a significant left-to-right shunt. After 1 year of triple combination initiation, a significant improvement in 6MWTD was observed (406 vs 450; p=0.0027), which was maintained at the 2-year follow-up. FC improved in 79% of patients at 1 year and remained stable in 76% at 2 years. NT-proBNP levels decreased significantly by 2 years, with an average reduction of 199 ng/L. Side effects were experienced by 33.3% of patients but were mostly mild and manageable. Subgroup analysis showed greater benefits in patients without Eisenmenger syndrome and those with pre-tricuspid defects.ConclusionsTriple therapy with prostanoids is safe and effective for patients with PAH-CHD, improving FC, 6MWTD and NT-proBNP levels over 2 years. The treatment is particularly beneficial for patients with pre-tricuspid defects and non-Eisenmenger PAH-CHD.
Abstract
BACKGROUND
Hypertensive disorders in pregnancy and other adverse pregnancy outcomes (APOs) increase the risk of developing chronic hypertension and cardiovascular disease. Perceptions of ...stress and neighborhood context also influence blood pressure (BP) fluctuations. We examined if APOs, higher perceived stress, and neighborhood deprivation were associated with hypertension phenotypes a decade after pregnancy in untreated individuals.
METHODS
Participants were 360 individuals who gave birth between 2008 and 2009 and participated in a research study 8–10 years following pregnancy. Standardized office and home BP readings were obtained, and we applied the AHA/ACC 2017 guidelines to identify sustained, white coat, and masked hypertension phenotypes. We measured personal stress with the perceived stress scale and neighborhood deprivation with the CDC Social Vulnerability Index.
RESULTS
Of the 38.3% (138/360) with any hypertension, 26.1% (36/138) reported a diagnosis of hypertension but were currently untreated. Sustained hypertension was the most common (17.8%), followed by masked and white coat hypertension, both 10.3%. Hypertensive disorders in pregnancy were associated with sustained (odds ratio OR 5.54 95% confidence interval, CI 2.46, 12.46 and white coat phenotypes (OR 4.20 1.66, 10.60, but not masked hypertension (OR 1.74 0.62, 4.90). Giving birth to a small for gestational age infant was also associated with sustained hypertension. In covariate adjusted models, perceived stress, but not neighborhood deprivation, was significantly associated with masked hypertension.
CONCLUSIONS
A decade after delivery, APOs were associated with sustained and white coat hypertension, but not masked hypertension. Exploration of the mechanisms underlying, and clinical implications of, these associations is warranted.
Graphical Abstract
Graphical Abstract
Updated Clinical Classification of Pulmonary Hypertension Simonneau, Gerald, MD; Gatzoulis, Michael A., MD, PhD; Adatia, Ian, MD ...
Journal of the American College of Cardiology,
12/2013, Letnik:
62, Številka:
25
Journal Article, Conference Proceeding
Recenzirano
Odprti dostop
In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic ...approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension PAH) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1. Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4.
Background: Hypertension from smoking is a chronic medical condition that impacts human health. It has been proved that it could be a significant risk factor for different disorders like brain ...damage, the lung disease and has been related to the growth of oral diseases. However, there are few and conflicting research on its effect on salivary electrolytes concentrations.
Aim of the study: To assess the effect of hypertension with and without smoking on electrolyte concentrations in saliva and to identify its effect on normal oral balance.
Methods: A total number of 90 subjects included, which is comprised of (49) (54.4 %) males subjects and (41) (45.6 %) females subjects in the gender. They were divided into three groups, G1, G2 and G3 groups. Group 1 comprised 30 subjects of hypertension with smoking, Group 2 comprised 30 subjects of hypertension without smoking and Group 3 comprised 30 subjects of healthy non-smoking controls.
Results: Findings displayed a highly significant difference in concentration of calcium in saliva and there was no significant difference in potassium and sodium for the three groups. There was a significant difference of calcium in saliva concentration for all study group with respect to P-Value (0.000).
Conclusion: The salivary calcium concentration in the saliva can be affected by hypertension with smoking and the increase in salivary calcium levels might be a risk factor for development of periodontal diseases.
IntroductionVenous sinus stenting (VSS) is a safe, effective, and increasingly popular treatment option for selected patients with idiopathic intracranial hypertension (IIH). Serious complications ...associated with VSS are rarely reported.MethodsSerious complications after VSS were identified retrospectively from multicenter databases. The cases are presented and management strategies are discussed.CasesSix major acute and chronic complications after VSS were selected from a total of 811 VSS procedures and 1466 venograms for IIH. These included an acute subdural hematoma from venous extravasation, cases of both intraprocedural and delayed stent thrombosis, an ultimately fatal cerebellar hemorrhage resulting in acute obstructive hydrocephalus, venous microcatheter perforation during venography and manometry, and a patient who developed SAH and SDH after cerebellar cortical vein perforation. The 6 cases are reviewed and learning points regarding complication avoidance and management are presented.ConclusionWe report on 6 rare, major complications after VSS for IIH. Familiarity with these potential complications and appropriate timely management may allow for good clinical outcomes.Disclosures R. Townsend: None. A. Jost: None. M. Amans: None. F. Hui: None. M. Bender: None. S. Satti: None. R. Maurer: None. K. Liu: None. W. Brinjikji: None. K. Fargen: None.
BMP9 (bone morphogenetic protein 9) is a circulating endothelial quiescence factor with protective effects in pulmonary arterial hypertension (PAH). Loss-of-function mutations in BMP9, its receptors, ...and downstream effectors have been reported in heritable PAH.
To determine how an acquired deficiency of BMP9 signaling might contribute to PAH.
Plasma levels of BMP9 and antagonist soluble endoglin were measured in group 1 PAH, group 2 and 3 pulmonary hypertension (PH), and in patients with severe liver disease without PAH.
BMP9 levels were markedly lower in portopulmonary hypertension (PoPH) versus healthy control subjects, or other etiologies of PAH or PH; distinguished PoPH from patients with liver disease without PAH; and were an independent predictor of transplant-free survival. BMP9 levels were decreased in mice with PH associated with CCl
-induced portal hypertension and liver cirrhosis, but were normal in other rodent models of PH. Administration of ALK1-Fc, a BMP9 ligand trap consisting of the activin receptor-like kinase-1 extracellular domain, exacerbated PH and pulmonary vascular remodeling in mice treated with hypoxia versus hypoxia alone.
BMP9 is a sensitive and specific biomarker of PoPH, predicting transplant-free survival and the presence of PAH in liver disease. In rodent models, acquired deficiency of BMP9 signaling can predispose to or exacerbate PH, providing a possible mechanistic link between PoPH and heritable PAH. These findings describe a novel experimental model of severe PH that provides insight into the synergy between pulmonary vascular injury and diminished BMP9 signaling in the pathogenesis of PAH.
Background: Hypertension from smoking is a chronic medical condition that impacts human health. It has been proved that it could be a significant risk factor for different disorders like brain ...damage, the lung disease and has been related to the growth of oral diseases. However, there are few and conflicting research on its effect on salivary electrolytes concentrations. Aim of the study: To assess the effect of hypertension with and without smoking on electrolyte concentrations in saliva and to identify its effect on normal oral balance. Methods: A total number of 90 subjects included, which is comprised of (49) (54.4 %) males subjects and (41) (45.6 %) females subjects in the gender. They were divided into three groups, G1, G2 and G3 groups. Group 1 comprised 30 subjects of hypertension with smoking, Group 2 comprised 30 subjects of hypertension without smoking and Group 3 comprised 30 subjects of healthy non-smoking controls. Results: Findings displayed a highly significant difference in concentration of calcium in saliva and there was no significant difference in potassium and sodium for the three groups. There was a significant difference of calcium in saliva concentration for all study group with respect to P-Value (0.000). Conclusion: The salivary calcium concentration in the saliva can be affected by hypertension with smoking and the increase in salivary calcium levels might be a risk factor for development of periodontal diseases.
Abstract only
Introduction
Venous sinus stenosis (VSS) stenting in medically refractory Idiopathic Intracranial Hypertension (IIH) patients has emerged as an effective treatment with low rates of ...failure and recurrence. However, the best treatment strategy following recurrence of symptoms in previously stented patients with initial response is unclear and frequently leads to shunt placement. We investigated the role of re‐stenting in patients with recurrence after prior successful stenting.
Methods
This is a retrospective review of a prospectively maintained IIH registry. Inclusion criteria included patients with confirmed IIH and angiographically demonstrable VSS who underwent interventions from 2012–2022. Patients were divided into those who underwent a single stenting procedure (Group S) and those who underwent placement of an additional stent due to recurrence of clinical symptoms and angiographic stenosis (Group R). All re‐stenting was performed in adjacent or remote regions of stenosis. Bivariate analyses were utilized to compare clinical outcomes.
Results
86 patients were included with a median age of 33 IQR 26‐39 and 94% were females. Group S (79/86) and Group R (7/86) had similar baseline characteristics. There was similar improvement in post‐stenting opening pressure (22.4±6.7 vs. 25.2±4.6 cm H2O, p = 0.35, Group S vs. Group R), change in opening pressure (11.6±10.4 vs. 5.3±12.2 cm H2O, p = 0.20, Group S vs. Group R) and resolution of tinnitus (91.8 vs. 80%, p = 0.28, Group S vs. Group R). Headache improvement was higher in Group S at 6 weeks, but similar at 6 months (92.8 vs. 60%, p = 0.02 and 88.2 vs. 80%, p = 0.59, Group S vs. Group R). Group S also had higher improvement in visual disturbances at 6 months (93.8 vs. 60%, p = 0.01, Group S vs. Group R), but similar improvement in papilledema (92.2 vs 100%, p = 0.68, Group S vs. Group R). None of the re‐stented patients required VPS rescue.
Conclusions
VSS re‐stenting in IIH patients with recurrence after initial stenting is feasible with similar efficacy in reducing intracranial pressure and symptoms.
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