Severe Systemic Hypertension Research Articles

Cerebral Arteriovenous Malformation in a Pediatric Patient with Severe Systemic Hypertension

AbstractArteriovenous malformations (AVMs) are a tangle of blood vessels that connects arteries and veins in which the feeding arteries are directly connected to the venous channels. Cerebral AVM needs special consideration due to high risk of bleeding and neurological deficit associated with morbidity and mortality. In pediatric population, prevalence rate of cerebral AVM is 0.02%. The systemic hemodynamic changes are seen more frequently with peripheral AVM, whereas its association with cerebral AVM is rare. We report a rare case of cerebral AVM in a 12-year-old child with severe systemic hypertension and electrocardiogram changes of biventricular hypertrophy planned for surgical resection of AVM in view of intracerebral bleed. This case illustrates the importance of detailed cardiac, renal, and endocrine evaluation in children with cerebral AVM with systemic hypertension and left ventricular hypertrophy to rule out other causes of systemic hypertension prior to surgery.

Can Facial Paralysis be a Rare Complication of Hypertension?

Facial paralysis is a disorder that can result from a wide spectrum of etiologies including traumatic, infectious, congenital, neurologic, systemic, neoplastic, and iatrogenic causes. It has significant functional, psychological, and social consequences. The most common cause of fascial nerve paralysis is idiopathic facial nerve palsy (Bell’s palsy). There is a relationship between facial nerve paralysis and severe systemic hypertension. In this report we present a 43-year-old female patient with fascial paralysis who had a history of hypertension.

CARD19: Hemodynamic Capabilities of biventricular HM3 Support

Background: Severe biventricular failure may necessitate the use of biventricular support using two HeartMate 3 (HM3) devices, with the right sided device in an off-label setting. Quantitative analysis of the hemodynamic capabilities has not yet been published. The aim of this study was to extensively investigate the balance between left and right flow, atrial pressures and septal positioning with and without pump speed changes during biventricular HM3 support. Methods: A mock circulation loop was configured to simulate a dual HM3 supported biventricular heart failure condition with left ventricular and right atrial inflows for the left and right pump respectively. Speed adjustments were made to balance systemic and pulmonary flow rates, atrial pressures (left atrial pressure (LAP) 10-15 mmHg, right atrial pressure (RAP) 7-12 mmHg), and septal position (ventricular end diastolic volume (VEDV) difference < 50 mL) to achieve a baseline condition. Systemic hypotension ((SHpo) mean arterial pressure (MAP) < 65 mmHg by decreasing SVR from 1000 to 600 dynes s/cm5), systemic hypertension ((SH) MAP > 106 mmHg by increasing from SVR 1000 to 2000 dynes s/cm5) and pulmonary hypertension ((PH) mean pulmonary arterial pressure (MPAP) > 20 mmHg by increasing PVR from 80 to 300 dynes s/cm5) were independently simulated and results recorded. Left and right pump speed manipulations were completed for each condition in an attempt to restore the flows, atrial pressures and septal position. Results: The largest change in pump speed (LVAD: 6000 to 8200 rpm, RVAD: 2950 to 4900 rpm), was required to restore the simulated condition of SHpo. PH had the biggest impact on the atrial pressures, with LAP down to 4.11 mmHg and RAP up to 13.29 mmHg compared to 10.88 mmHg and 11.11 mmHg at the restored baseline condition respectively. Through HM3 speed manipulation, the LAP was restored to 7.08 mmHg and the RAP to 12 mmHg. PH had the most significant impact on the left and right VEDV difference, with an increase from 28 mL at the restored baseline condition to 228 mL during PH. This imbalance was reduced to 133 mL by adjusting both pump speeds, but could not be restored to the target of 50 mL. Conclusion: Systemic and pulmonary flow and atrial pressures can be restored through pump speed manipulation with biventricular HM3 support during simulations of severe SH. Restoring flows, atrial pressures and septal position was not possible in all conditions when relying solely on pump speed changes.Figure 1. Atrial pressures at different conditions, HF – heart failure; SH – systemic hypertension; SHpo – systemic hypotension; PH – pulmonary hypertension; LAP, RAP – left and right atrial pressures.Figure 2. Ventricular end diastolic volume difference at different conditions, HF – heart failure; SH – systemic hypertension; SHpo – systemic hypotension; PH – pulmonary hypertension; LVEDV, RVEDV – left and right end diastolic volume.

HFpEF animal models display differences in myofibril mechanics.

Heart Failure with preserved Ejection Fraction (HFpEF) is a complex, multi-organ disease historically defined by cardiac aberrations including diastolic dysfunction with normal resting systolic function and depressed systolic reserve, hypertrophy, and impaired relaxation. Though diastolic dysfunction is a hallmark of HFpEF, it is unknown whether organ-level deficiencies in relaxation persist down to the myofibrils, the contractile organelles within cells. Here, we performed mechanics analysis on cardiac myofibrils from three common HFpEF animal models to assess potential differences in myofibrillar force and relaxation kinetics, independent of higher-order deficits associated with the disease. The Göttingen minipig (GM) HFpEF model recapitulates the spectrum of human HFpEF multiorgan pathophysiology, including severe diastolic dysfunction, myocardial hypertrophy, fibrosis, and pulmonary and systemic hypertension. We found that GM-HFpEF myofibrils produced significantly less maximal force versus controls, with no change in either the slow or fast relaxation phases. Therefore, impaired myofibril relaxation likely does not contribute to the diastolic dysfunction in this model. Another model assessed was the Zucker spontaneously hypertensive heart failure F1 (ZSF1) rat, which exhibits elevated E/e′, insulin resistance, elevated end-diastolic pressure, and preserved ejection fraction. Our preliminary data indicate that ZSF1 myofibrils produce normal maximal force, but have a slower initial rate of relaxation compared to controls. These data suggest that impaired myofibril relaxation may contribute to diastolic dysfunction in the ZSF1 model. Moreover, the distinct responses of myofibrils from these models appear to recapitulate cell-level behavior in human cardiomyocytes isolated from patients with different HFpEF subphenotypes. Therefore, certain animal models may mimic unique HFpEF characteristics at the cellular and/or myofibril levels and be better-suited for investigating specific aspects of this extremely complex syndrome, and aid in the design of effective pharmacological or device-based treatments.

Bilateral subclavian artery occlusion masking severe systemic hypertension as cause for heart failure: the role of multi-modality cardiovascular imaging in guiding diagnosis and management.

A 62-year-old female smoker presented with gradually worsening exertional breathlessness over 1 year, despite initiation of treatment for chronic obstructive pulmonary disease. She gave a history of transient ischaemic attack and possible subclavian artery stenosis diagnosed at a different institution. Physical examination revealed blood pressure (BP) readings of 80/50 mmHg in both arms. Electrocardiogram showed sinus rhythm with left bundle branch block (QRS duration 147 ms, Panel A). Echocardiography identified severe left ventricular (LV) systolic dysfunction (ejection fraction 29%) and suspected apical thrombus (Panel B, Supplementary data online, Video S1). She was initiated on ivabradine and considered for cardiac resynchronization therapy due to hypotension which limited the introduction of additional heart failure (HF) pharmacotherapy. Cardiovascular magnetic resonance confirmed LV dysfunction (supplementary data online, Video S2) and mild LV hypertrophy. There was septal fibrosis but no myocardial infarction or LV thrombus (Panel C & D), suggestive of non-ischaemic dilated cardiomyopathy phenotype. Computed tomography coronary angiography showed unobstructed coronary arteries but revealed bilateral subclavian artery occlusion (Panels E–G).

169: SITOSTEROLEMIA PRESENTING WITH SEVERE HYPERTENSION AND CONGESTIVE HEART FAILURE IN A CHILD

Introduction: Sitosterolemia is an autosomal recessive condition caused by pathogenic variants in the ABCG5/ABCG8 genes that prohibit the body from excreting plant sterols, causing build-up in the soft tissues and systemic vasculature. Pediatric vasculopathy secondary to sitosterolemia is poorly described, and there is no clear guidance on the management of complications, including cardiac dysfunction. This case report describes the diagnosis and acute management of a child with severe systemic hypertension, multiple levels of arterial stenosis, and cardiomyopathy presenting in congestive heart failure and ultimately diagnosed with sitosterolemia. Description: An 8-year-old Amish male with no past medical history presented to the emergency department with emesis and associated fatigue and abdominal pain. Vital signs were significant for severe systemic hypertension (BP 220/110), a gallop rhythm, hepatomegaly, cardiomegaly, and mid-aortic narrowing on CT angiogram. Exam was also notable for bilateral knee xanthomas. Laboratory data after admission to the pediatric cardiac ICU showed elevated BNP (peak 4418 pg/mL) and total cholesterol (317 mg/dL) levels. Echocardiogram showed severe mitral and tricuspid valve regurgitation, severely dilated and concentrically hypertrophied and dysfunctional left ventricle, ejection fraction of 30%, and pulmonary hypertension. Consultations included genetics, cardiology, nephrology, and vascular surgery. Initial management included a continuous Milrinone infusion and loop diuretics with improved BP and congestive heart failure symptoms. Cardiac catheterization including coronary and renal angiograms demonstrated severe proximal right coronary artery stenosis and bilateral renal artery stenosis. Right coronary artery and left renal artery angioplasties were performed with good results, and showed significant improvement in parameters of ventricular function and pulmonary arterial hypertension. Genetic testing and positive serum plant sterols confirmed the diagnosis of sitosterolemia. Discussion: Sitosterolemia is a rare genetic disorder and a rare cause of pediatric arteriopathy. Early recognition can prevent the development of life-threatening systemic hypertension, arterial stenosis including coronary artery disease, and cardiomyopathy.

Hypoxemia Destabilizes The Neurovascular Unit During Extreme Apnea In Humans

To what extent voluntary asphyxia imposed by static apnea challenges the functional-structural limits of the neurovascular unit (NVU) in humans characterized by extremes of hypertension, hypoxemia and hypercapnia remains unclear. PURPOSE: To document the transcerebral exchange kinetics of NVU-specific proteins during extreme apnea and independent contributory influence of hypoxemia. METHODS: Ten world class breath-hold divers (6♂, 4♀) performed two maximal dry apneas preceded by normoxic normoventilation (NX: severe hypoxemia and hypercapnia) and hyperoxic hyperventilation (HX: absence of hypoxemia with exacerbating hypercapnia) with measurements obtained before and after apnea. Transcerebral exchange of NVU proteins (ELISA, Single Molecule Array) were calculated as the product of global cerebral blood flow (gCBF, duplex ultrasound) and radial arterial to internal jugular venous concentration gradients. RESULTS: Apnea duration increased from 5 m 6 s in NX to 15 m 59 s in HX (P = < 0.001) resulting in marked elevations in gCBF and venous S100B, glial fibrillary acidic protein, ubiquitin carboxy-terminal hydrolase-L1 and total tau (all P < 0.05 vs. baseline). This culminated in net cerebral output reflecting increased blood-brain barrier permeability and neuro-glio-vascular dysfunction that was more pronounced in NX due to more severe systemic and intracranial hypertension (P < 0.05 vs. HX). CONCLUSION: These findings identify the hemodynamic stress to which the apneic brain is exposed, highlighting the critical contribution of hypoxemia and not just hypercapnia to NVU destabilization.

Endothelins and liver cirrhosis

AbstractEndothelins are a family of 21‐amino acid oligopeptides, called endothelin‐1 (ET‐1), endothelin‐2 (ET‐2), and endothelin 3 (ET‐3). Endothelins act on hepatocytes, liver endothelial cells, and Kupffer cells in a paracrine or autocrine manner through two G protein‐coated receptors, called endothelin A and B receptors, which are mainly located in interlobular veins, interlobular artery endothelial cells and hepatic stellate cells (HSCs). ET B receptor (ETBR)‐1 is responsible for the induction of endothelial nitric oxide synthase, resulting in nitric oxide release and vasodilatation, whereas ETBR‐2 is located on HSCs and is responsible for vasoconstriction. Endothelins are not stored in the organs. Approximately 20% of endothelins are secreted into the circulation system, and are rapidly cleared by the lungs, liver, heart, and kidneys. As a potent vasoconstrictor, endothelins may have a key role for the treatment of hypertensive vascular diseases, inflammation, fibrosis, and metabolic diseases. By clearing ET‐1 from the circulation, endothelin A receptor (ETAR) antagonists can reduce intraportal vascular resistance by dilatating the portal vein, reducing the contraction of HSCs, increasing the diameter of sinusoids, facilitating the regression of liver fibrosis, and restoring liver parenchyma. ET‐1 induces nitric oxide synthase and upregulates cyclooxygenase 2 mRNA levels, making it a key factor during the onset of fibrosis and in the prognosis of patient outcomes. Endothelins can be used to treat porto‐pulmonary hypertension, portal hypertension, angiogenesis, and liver fibrosis and have been approved for the treatment of porto‐pulmonary hypertension. Endothelins are produced on mesangial cells, podocytes, tubular epithelium, and renal collecting tubes in high amounts. Activation of ETAR supports the progression of kidney diseases, whereas activation of ETBR has protective effects on the kidneys. ET‐1 plays an important role in normal cardiovascular homeostasis. Endothelins are closely associated with severe systemic hypertension, congestive heart failure, atherosclerosis, and pulmonary hypertension. Dual ET receptor antagonists reduce blood pressure, but have several side effects, including liver toxicity, acute liver failure, accumulation of salt and water, testicular toxicity, headache, and teratogenicity. Liver enzymes should be checked in all patients who have received ET receptor antagonists, and women of childbearing years should use contraception.

Hypoxemia increases blood-brain barrier permeability during extreme apnea in humans

Voluntary asphyxia imposed by static apnea challenges blood-brain barrier (BBB) integrity in humans through transient extremes of hypertension, hypoxemia and hypercapnia. In the present study, ten ultra-elite breath-hold divers performed two maximal dry apneas preceded by normoxic normoventilation (NX: severe hypoxemia and hypercapnia) and hyperoxic hyperventilation (HX: absence of hypoxemia with exacerbating hypercapnia) with measurements obtained before and immediately after apnea. Transcerebral exchange of NVU proteins (ELISA, Single Molecule Array) were calculated as the product of global cerebral blood flow (gCBF, duplex ultrasound) and radial arterial to internal jugular venous concentration gradients. Apnea duration increased from 5 m 6 s in NX to 15 m 59 s in HX (P = <0.001) resulting in marked elevations in gCBF and venous S100B, glial fibrillary acidic protein, ubiquitin carboxy-terminal hydrolase-L1 and total tau (all P < 0.05 vs. baseline). This culminated in net cerebral output reflecting mildly increased BBB permeability and increased neuronal-gliovascular reactivity that was more pronounced in NX due to more severe systemic and intracranial hypertension (P < 0.05 vs. HX). These findings identify the hemodynamic stress to which the apneic brain is exposed, highlighting the critical contribution of hypoxemia and not just hypercapnia to BBB disruption.

Ischaemic myelopathy in a cat with chronic kidney disease, hyperthyroidism, and hyperaldosteronism

AbstractIschaemic myelopathy (IM) in cats often affects the cervical region. It is generally associated with an underlying predisposing condition with systemic arterial hypertension due to chronic kidney disease and cardiomyopathies the most frequently reported. The present case report describes the successful management of an old cat presented for acute onset of weakness, neck ventroflexion, and ataxia as a result of cervical IM secondary to severe systemic hypertension associated with hyperthyroidism, chronic renal disease, and primary hyperaldosteronism. Feline cervical IM carries a favourable prognosis with resolution of neurological deficits unless the underlying medical condition is not resolved, favouring further ischaemic events.

Clinical Impact of Rare Aortic Arch Variant

This case report describes the clinical implications of peripheral arterial disease in a patient with a rare variant of the aortic arch branches. The patient was a 68-year old-female with an extensive cardiovascular history including multiple severe peripheral arterial stenoses, systemic and pulmonary hypertension, hyperlipidemia, coronary artery disease, multiple myocardial infarctions, atrial fibrillation, and possible aortic valve disease. The focus of the case report will be how the patient’s anatomical variant played a large role in maintaining cerebral circulation in the presence of significant stenoses of the right vertebral artery, left subclavian artery, and left common carotid artery.

A CASE REPORT ON TAKAYASU ARTERITIS

Takayasus arteritis primarily affects young women. Takayasu arteritis (TA) is a type of primary systemic vasculitis mainly affecting the medium and large arteries. The signs and symptoms are due to systemic inflammation or ischemia of an organ or limb and include angiodynia, claudication, peripheral pulselessness, murmurs, ischemic stroke, myocardial infarction and severe systemic arterial hypertension. The disease tends to affect more women than men.The current case of Takayasu arteritis type V focuses on a 21 year female who complained of cold, cough, fever, facial puffiness since 2 months, along with bilateral renal artery stenosis, and resistant hypertension.

A CASE REPORT ON TAKAYASU ARTERITIS

Takayasus arteritis primarily affects young women. Takayasu arteritis (TA) is a type of primary systemic vasculitis mainly affecting the medium and large arteries. The signs and symptoms are due to systemic inflammation or ischemia of an organ or limb and include angiodynia, claudication, peripheral pulselessness, murmurs, ischemic stroke, myocardial infarction and severe systemic arterial hypertension. The disease tends to affect more women than men.The current case of Takayasu arteritis type V focuses on a 21 year female who complained of cold, cough, fever, facial puffiness since 2 months, along with bilateral renal artery stenosis, and resistant hypertension.

Prevalence and risk factors associated with systemic hypertension in dogs with spontaneous hyperadrenocorticism.

BackgroundSystemic hypertension (SH) is common in dogs with hyperadrenocorticism (HAC) however there are not many studies assessing its prevalence and risk factors.ObjectivesTo determine the prevalence and severity of SH in dogs with HAC and its association with clinical and laboratory findings to identify potential risk factors.AnimalsSixty‐six client owned dogs with spontaneous HAC.MethodsRetrospective cross‐sectional study. Medical records of dogs with HAC were reviewed. Systolic blood pressure (SBP) was measured using Doppler ultrasonography. Clinical signs, physical examination findings and clinicopathologic data (CBC, serum biochemistry and electrolytes, urinalysis and urinary culture, and adrenal function tests) were reviewed for analysis.ResultsPrevalence of SH (≥150 mm Hg) was 82% (54/66) and prevalence of severe SH (≥180 mm Hg) was 46% (30/66). All dogs with thrombocytosis had SH (P = .002), and a platelet count ≥438 × 103/μL was 100% specific and 61.1% sensitive to predict SH (AUC = .802, P = .001). Median potassium levels were lower in hypertensive dogs (4.1 mEq/L, range 3.1‐5.4 mEq/L) than in normotensive ones (4.5 mEq/L, range 4.0‐5.0 mEq/L) (P = .007). Dogs with UPC ≥ 0.5 had higher median SBP than those without proteinuria (P = .03). Dogs with concurrent diabetes mellitus seemed to have a reduced risk of SH (OR = .118, 95%CI = .022‐.626, P = .02).Conclusions and Clinical ImportanceSystemic hypertension is common in dogs with HAC and is frequently severe. Blood pressure should be routinely assessed in these dogs, especially if thrombocytosis, proteinuria or low potassium concentrations are present.

Improving the teaching of fundoscopy in veterinary medicine

Learning how to examine the fundus of veterinary patients consistently and well is undeniably difficult, and teaching aids are important to avoid the demotivation of students.1,2 However, the rewards of...

Neonatal hypertension caused by left-to-right shunt flow through a patent ductus arteriosus in a premature infant

AbstractObjectivesThough it is rare and challenging to study, neonatal hypertension (HTN) has been explored and studied by many pediatricians. There were some causes reported in preterm infants such as renal diseases and bronchopulmonary dysplasia.Case presentationWe report a premature female infant who was born at a gestational age of 25 + 6 weeks and developed systemic hypertension on the day of a gestation age of 30 + 3 weeks. This case had a rare cause which involved high-normal serum aldosterone level that could possibly indicate hyperaldosteronism and over-stimulation of renin–angiotensin–aldosterone system (RAAS). We believe elevation of serum aldosterone was caused by a left-to-right shunt flow through a patent ductus arteriosus. Hypertension due to a patent ductus arteriosus sounds like a paradox. However, in our case, the shunt flow was shown to be a possible cause of the systemic hypertension. Surgical closure of the shunt was done and the hypertension resolved. We evaluated serum aldosterone levels and plasma renin activities both, before and after the closure to look for a possible cause.ConclusionsNeonatologist often treats systemic hypertensive infants considering possible causes in order to prevent hypertensive crisis. Our case shows that possibility of hyperaldosteronism and (RAAS) over-stimulation should be considered as causes in case of severe systemic hypertension in preterm infants with large left-to-right shunt flows.

Anesthesia Management for Kidney Transplant Recipient in Patient with Kyphoscoliosis

Kyphoscoliosis is one of the manifestations of renal bone disease due to End stage renal disease (ESRD). Chronic renal failure patients usually present with comorbidities like hypertension, Diabetes, Dyslipedidemias, however, the risk of renal transplant increases multifold when the patient also suffers with respiratory problems. Herein we provide a patient of ESRD with severe kyphoscoliosis having severe pulmonary and systemic hypertension posted for emergency cadaveric donor renal transplantation which was successfully performed under general anesthesia with invasive Monitoring.

Interrupted Aortic Arch in a Patient with Patient-Prosthesis Mismatch after Aortic Valve Replacement.

This case presents a patient who underwent aortic valve replacement and presented 13 years later with high gradients across the prosthesis, mitral insufficiency, and severe systemic hypertension. Her preoperative workup led to the diagnosis of an interrupted aortic arch Type A. Her surgical management included an initial procedure to repair the interruption, and 11 months later after resolution of her hypertension, a second surgery, which included the Ross procedure and mitral valve repair.

Case Report: Takayasu arteritis in a male patient

Takayasu arteritis (TA) is a type of primary systemic vasculitis mainly affecting the medium and large arteries. The signs and symptoms are due to systemic inflammation or ischemia of an organ or limb and include angiodynia, claudication, peripheral pulselessness, murmurs, ischemic stroke, myocardial infarction and severe systemic arterial hypertension. The disease tends to affect more women than men. Here we present a case of TA in a 22-year-old male patient. Our patient presented with complaints of aphasia and right-sided weakness, with on-and-off symptoms of malaise, generalized weakness, unilateral headache, fatigue and shortness of breath lasting two years. Color Doppler ultrasound was sufficient for a diagnosis of TA, after which we started the patient on medical treatment and also consulted the department of vascular surgery. Overall, this case report highlights the importance of screening for TA in male patients so that the diagnosis is not overlooked, and also adds more data to the limited literature on male patients.

Polyarteritis Nodosa in a Cat with Involvement of the Central and Peripheral Nervous Systems

SummaryAn 18-month-old neutered male domestic shorthair cat was referred with a history of pyrexia, polyuria and polydypsia, and transient episodes of bilateral hindlimb paralysis. Cardiac evaluation revealed severe systemic hypertension and severe concentric hypertrophy of the left ventricle. One month later the cat had a new episode of hindlimb paralysis with recurrent seizures, and died in status epilepticus. At necropsy examination, the coronary arteries, arcuate renal arteries and common iliac arteries showed marked thickening with nodules segmentally located along the vessels and consequent narrowing of the lumina. Histologically, acute and chronic inflammatory infiltration of the vascular walls was associated with necrosis of the muscular layer. Lesions were consistent with polyarteritis nodosa and involved the small, medium and large arteries of the heart, kidneys, small and large intestine, mesentery, liver and thyroid. Multifocal meningeal vasculitis associated with focal infarction of the frontal lobe and necrotizing vascular polyneuropathy were detected. Involvement of the central and peripheral nervous systems in polyarteritis nodosa is a novel finding in cats.