RESUMO
DESCRIPTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. METHODS: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. RECOMMENDATIONS: The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.
Assuntos
Telangiectasia Hemorrágica Hereditária/diagnóstico , Telangiectasia Hemorrágica Hereditária/terapia , Anemia/etiologia , Anemia/terapia , Malformações Arteriovenosas/etiologia , Malformações Arteriovenosas/terapia , Criança , Epistaxe/etiologia , Epistaxe/terapia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/terapia , Doenças Genéticas Inatas/etiologia , Doenças Genéticas Inatas/terapia , Humanos , Fígado/irrigação sanguínea , Telangiectasia Hemorrágica Hereditária/complicaçõesRESUMO
BACKGROUND AND PURPOSE: Transcranial Doppler (TCD) identifies acute stroke patients with arterial occlusion where treatment may not effectively open the blocked vessel. This study aimed to examine the clinical utility and prognostic value of TCD flow findings in patients enrolled in a multicenter prospective study (CLOTBUST-PRO). METHODS: Patients enrolled with intracranial occlusion on computed tomography angiography (CTA) who underwent urgent TCD evaluation before intravenous thrombolysis was included in this analysis. TCD findings were assessed using the mean flow velocity (MFV) ratio, comparing the reciprocal ratios of the middle cerebral artery (MCA) depths bilaterally (affected MCA-to-contralateral MCA MFV [aMCA/cMCA MFV ratio]). RESULTS: A total of 222 patients with intracranial occlusion on CTA were included in the study (mean age: 64 ± 14 years, 62% men). Eighty-eight patients had M1 MCA occlusions; baseline mean National Institutes of Health Stroke Scale (NIHSS) score was 16, and a 24-hour mean NIHSS score was 10 points. An aMCA/cMCA MFV ratio of <.6 had a sensitivity of 99%, specificity of 16%, positive predictive value (PV) of 60%, and negative PV of 94% for identifying large vessel occlusion (LVO) including M1 MCA, terminal internal carotid artery, or tandem ICA/MCA. Thrombolysis in Brain Ischemia scale, with (grade ≥1) compared to without flow (grade 0), showed a sensitivity of 17.1%, specificity of 86.9%, positive PV of 62%, and negative PV of 46% for identifying LVO. CONCLUSIONS: TCD is a valuable modality for evaluating arterial circulation in acute ischemic stroke patients, demonstrating significant potential as a screening tool for intravenous/intra-arterial lysis protocols.
Assuntos
Sensibilidade e Especificidade , Ultrassonografia Doppler Transcraniana , Humanos , Feminino , Masculino , Ultrassonografia Doppler Transcraniana/métodos , Pessoa de Meia-Idade , Idoso , Estudos Prospectivos , Reprodutibilidade dos Testes , Trombectomia/métodos , Angiografia por Tomografia Computadorizada/métodos , Velocidade do Fluxo Sanguíneo , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/terapia , Resultado do TratamentoRESUMO
BACKGROUND AND PURPOSE: Contrast transthoracic echocardiography (TTCE) is used to screen hereditary hemorrhagic telangiectasia (HHT) patients for right-to-left shunts (RLS) associated with increased stroke risk. We hypothesized that contrast transcranial Doppler (TCDc), shown to be highly sensitive for detecting RLS in patent foramen ovale, will be as comparable to TTCE for screening HHT patients. METHODS: We compared TTCE and TCDc for detecting RLS in 12 patients with HHT who also underwent CT pulmonary studies to determine pulmonary arteriovenous malformation (PAVM) presence. The sensitivity and specificity of TTCE and TCDc in detecting PAVM were determined and the agreement between TTCE and TCDc in detecting RLS was assessed. RESULTS: Both TTCE and TCDc had 100% sensitivity in detecting underlying PAVM; the specificity was 25% and 38%, respectively. The agreement in detecting RLS between TTCE and TCD was high (κ=0.76). TCD was well-tolerated with no immediate adverse or embolic events over the next 3 months. CONCLUSIONS: TCDc offers a simple office-based alternative to TTCE for screening RLS associated with PAVM in HHT patients.
Assuntos
Malformações Arteriovenosas/diagnóstico por imagem , Ecocardiografia/métodos , Telangiectasia Hemorrágica Hereditária/diagnóstico por imagem , Ultrassonografia Doppler Transcraniana/métodos , Adulto , Feminino , Humanos , Masculino , Programas de Rastreamento/métodos , Estudos Retrospectivos , Sensibilidade e EspecificidadeRESUMO
Positional intermittent carotid ischemia has rarely been reported in the literature. We report a case of fluctuating hemiparesis in a 88-year-old woman in whom transcranial Doppler ultrasonography at various head positions proved useful in establishing the mechanism of the hypoperfusion transient ischemic attack. Head rotation to the side of the stenotic internal carotid artery resulted in significant drop in ipsilateral middle cerebral artery mean flow velocity.
Assuntos
Artéria Carótida Interna/fisiopatologia , Estenose das Carótidas/fisiopatologia , Circulação Cerebrovascular , Movimentos da Cabeça , Hemodinâmica , Ataque Isquêmico Transitório/fisiopatologia , Posicionamento do Paciente , Idoso de 80 Anos ou mais , Velocidade do Fluxo Sanguíneo , Artéria Carótida Interna/diagnóstico por imagem , Estenose das Carótidas/complicações , Estenose das Carótidas/diagnóstico por imagem , Angiografia Cerebral , Feminino , Humanos , Ataque Isquêmico Transitório/diagnóstico por imagem , Ataque Isquêmico Transitório/etiologia , Paresia/etiologia , Paresia/fisiopatologia , Valor Preditivo dos Testes , Recidiva , Rotação , Ultrassonografia Doppler TranscranianaRESUMO
BACKGROUND: Arterial flow velocity changes on transcranial Doppler can reflect changes in cerebral flow during position-induced ischemia if obtained during short-term monitoring of positional changes. SUBJECTS AND METHODS: Our multicenter group monitored symptomatic and asymptomatic arteries in patients with recurrent neurological deficits during positional changes and documented intracranial arterial stenosis. Bilateral posterior cerebral and middle cerebral arteries were monitored dependent on clinical symptom localization. The symptomatic artery was monitored distal to the intracranial stenosis, and mean flow velocities (MFV) were recorded at different body positions. The symptomatic artery relative MFV ratio was defined as the ratio of symptomatic artery MFV in the asymptomatic position--MFV in the symptomatic position/MFV in the asymptomatic position. RESULTS: Sixteen patients underwent transcranial Doppler monitoring: mean age 62 +/- 19 years, 11 (69%) men, 6 (40%) with transient ischemic attacks. Ten patients (63%) had posterior and 6 anterior circulation symptoms. Patients developed neurological symptoms while standing up (63%) and/or sitting (44%), walking (13%) or during neck extension (6%). Symptomatic artery MFV dropped by > or =25% from the resting to the symptomatic position in all patients except for one. The mean symptomatic artery MFV relative ratio was higher compared with the mean asymptomatic artery MFV relative ratio: 0.5 +/- 0.28 versus -0.02 +/- 0.1 (p = 0.001, Wilcoxon test). The symptomatic artery relative ratio of >0.25 had a 94% sensitivity and 100% specificity for predicting neurological symptom development during testing (kappa = 0.9, p < 0.001). CONCLUSIONS: A significant reduction in intracranial flow velocity distal to an intracranial stenosis can identify patients whose symptoms can worsen with positional changes. These patients may prove a target for interventional revascularization techniques.
Assuntos
Artérias Cerebrais/fisiopatologia , Transtornos Cerebrovasculares/fisiopatologia , Tontura/fisiopatologia , Arteriosclerose Intracraniana/fisiopatologia , Ataque Isquêmico Transitório/fisiopatologia , Fluxo Sanguíneo Regional/fisiologia , Acidente Vascular Cerebral/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Artérias Cerebrais/diagnóstico por imagem , Transtornos Cerebrovasculares/complicações , Constrição Patológica/complicações , Constrição Patológica/fisiopatologia , Tontura/complicações , Feminino , Humanos , Arteriosclerose Intracraniana/complicações , Ataque Isquêmico Transitório/diagnóstico por imagem , Ataque Isquêmico Transitório/etiologia , Masculino , Pessoa de Meia-Idade , Postura/fisiologia , Estudos Retrospectivos , Sensibilidade e Especificidade , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Síndrome , Ultrassonografia Doppler TranscranianaRESUMO
BACKGROUND AND PURPOSE: Although onset-to-treatment time is associated with early clinical recovery in acute ischemic stroke (AIS) patients treated with intravenous tissue plasminogen activator (tPA), the effect of the timing of tPA-induced recanalization on functional outcomes remains debatable. METHODS: We conducted a multicenter, prospective observational cohort study to determine whether early (within 1-hour from tPA-bolus) complete or partial recanalization assessed during 2-hour real-time transcranial Doppler monitoring is associated with improved outcomes in patients with proximal occlusions. Outcome events included dramatic clinical recovery (DCR) within 2 and 24-hours from tPA-bolus, 3-month mortality, favorable functional outcome (FFO) and functional independence (FI) defined as modified Rankin Scale (mRS) scores of 0-1 and 0-2 respectively. RESULTS: We enrolled 480 AIS patients (mean age 66±15 years, 60% men, baseline National Institutes of Health Stroke Scale score 15). Patients with early recanalization (53%) had significantly (P<0.001) higher rates of DCR at 2-hour (54% vs. 10%) and 24-hour (63% vs. 22%), 3-month FFO (67% vs. 28%) and FI (81% vs. 39%). Three-month mortality rates (6% vs. 17%) and distribution of 3-month mRS scores were significantly lower in the early recanalization group. After adjusting for potential confounders, early recanalization was independently associated with higher odds of 3-month FFO (odds ratio [OR], 6.19; 95% confidence interval [CI], 3.88 to 9.88) and lower likelihood of 3-month mortality (OR, 0.34; 95% CI, 0.17 to 0.67). Onset to treatment time correlated to the elapsed time between tPA-bolus and recanalization (unstandardized linear regression coefficient, 0.13; 95% CI, 0.06 to 0.19). CONCLUSIONS: Earlier tPA treatment after stroke onset is associated with faster tPA-induced recanalization. Earlier onset-to-recanalization time. RESULTS: in improved functional recovery and survival in AIS patients with proximal intracranial occlusions.
RESUMO
In an acute stroke setting, transcranial Doppler (TCD) and transcranial color-coded duplex (TCCD) have an important diagnostic utility in the monitoring of an arterial occlusion and microemboli detection. In addition, TCD has proven to be a very useful tool in the detection and progression of cerebral vasospasm in patients with subarachnoid hemorrhage. TCD/TCCD may have an important role in defining collateral blood flow (CF) in stroke patients. It is a noninvasive technique and can be utilized repeatedly allowing for changes in the blood flow dynamics as treatment is delivered. In this review, we outlined the evolving role of TCD/TCCD in defining CF in patients with an acute ischemic stroke, predicting clinical outcome and monitoring the treatment's efficacy of the CF augmentation.
Assuntos
Isquemia Encefálica/diagnóstico por imagem , Circulação Cerebrovascular/fisiologia , Acidente Vascular Cerebral/diagnóstico por imagem , Ultrassonografia Doppler em Cores/métodos , Ultrassonografia Doppler Transcraniana/métodos , Hemodinâmica , Humanos , Hemorragia Subaracnóidea/diagnóstico por imagem , Vasoespasmo Intracraniano/diagnóstico por imagemRESUMO
BACKGROUND AND PURPOSE: The purpose of this study was to compare Doppler ultrasound (DUS) to other angiographic modalities: computed tomography angiography (CTA), magnetic resonance angiography (MRA), and digital subtraction angiography (DSA). METHODS: All DUS studies performed at Stroke Prevention Clinic (SPC) from 2011 to 2013 and referred for further angiographic modalities were included. Patients were excluded if the corresponding angiographic modality was not performed within 6 months of DUS. Patients were also excluded if they underwent interventions before DUS or between the time of DUS and the corresponding angiographic modality. The degree of stenosis was classified as mild (<50%), moderate (50-69%), severe (70-99%), or occlusion (100%). RESULTS: In total, 245 patients were identified. Nine patients were excluded (3.7%). Overall 472 Doppler studies of single ICAs from 236 patients were included in our analysis. Age was 65 ± 13 years and 136 patients were males (57.6%). There was an excellent agreement between DUS and CTA (kappa = .9 [P < .001], n = 274), good agreement with MRA (kappa = .8 [P < .001], n = 242), and excellent agreement with DSA (kappa = .92 [P < .001], n = 18). There was excellent agreement between CTA and MRA (kappa = .87, n = 46). CONCLUSION: Doppler ultrasound performed in a dedicated SPC by an experienced sonographer and reviewed by a certified stroke neurologist serves as a reliable initial screening tool in determining carotid artery stenosis.
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Angiografia Digital/métodos , Estenose das Carótidas/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Angiografia por Ressonância Magnética/métodos , Ultrassonografia Doppler/métodos , Idoso , Estenose das Carótidas/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Acidente Vascular CerebralRESUMO
BACKGROUND AND PURPOSE: Flow diversion is a relatively new strategy used to treat complex cerebral aneurysms. The optimal method for radiographic follow-up of patients treated with flow diverters has not been established. The rate and clinical implications of in-stent stenosis for these devices is unclear. We evaluate the use of transcranial Doppler ultrasound (TCD) for follow-up of in-stent stenosis. MATERIALS AND METHODS: We analyzed 28 patients treated with the Pipeline embolization device (PED) over the course of 42â months from January 2009 to June 2012. Standard conventional cerebral angiograms were performed in all patients. TCD studies were available in 23 patients. RESULTS: Angiographic and TCD results were compared and found to correlate well. CONCLUSIONS: TCD is a potentially useful adjunct for evaluating in-stent stenosis after flow diversion.
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Embolização Terapêutica/métodos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/terapia , Stents , Ultrassonografia Doppler Transcraniana/métodos , Adulto , Idoso , Angiografia Cerebral/métodos , Embolização Terapêutica/instrumentação , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Stents/efeitos adversos , Resultado do TratamentoRESUMO
Embryonic carotid - basilar anastomosis when persistent in adult life can present with a variety of neurological symptoms. We present a patient with isolated intermittent vertigo attributable to the embryonic anastomosis and describe the different types of persistent trigeminal artery. A 76-year-old Caucasian man presented with isolated intermittent vertigo and symptoms suggestive of anterior and posterior circulation strokes. Impaired vasomotor reactivity was demonstrated on insonation of the anterior and posterior cerebral arteries in this patient with a persistent left trigeminal artery and 75% stenosis of the left internal carotid artery (ICA). The symptom of intermittent vertigo resolved with carotid endarterectomy. Decreased flow across the stenotic segment of the ICA which subserved the posterior circulation resulted in basilar insufficiency. Hypoperfusion to the flocculonodular lobe supplied by the anterior inferior cerebellar artery is a likely cause for the intermittent vertigo.
RESUMO
In patients with critical carotid stenosis and ischemic stroke, it is crucial to determine whether distal hypoperfusion or artery-to-artery embolism is the predominant mechanism. The role of transcranial doppler (TCD) in this setting is vital. We report a patient with fibromuscular dysplasia and recurrent orthostatic transient ischemic attacks where fall in cerebral perfusion was clearly demonstrated by TCD.
Assuntos
Dissecação da Artéria Carótida Interna/fisiopatologia , Circulação Cerebrovascular , Tontura/fisiopatologia , Displasia Fibromuscular/fisiopatologia , Ataque Isquêmico Transitório/fisiopatologia , Ultrassonografia Doppler/métodos , Velocidade do Fluxo Sanguíneo , Dissecação da Artéria Carótida Interna/complicações , Dissecação da Artéria Carótida Interna/diagnóstico , Tontura/diagnóstico , Tontura/etiologia , Displasia Fibromuscular/complicações , Displasia Fibromuscular/diagnóstico por imagem , Humanos , Ataque Isquêmico Transitório/diagnóstico , Ataque Isquêmico Transitório/etiologia , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Transcranial Doppler (TCD) has been subjected to criticism for detecting vasospasm (VSP). Our study's aim is to derive criteria for middle cerebral artery (MCA) vasospasm (MCA-VSP) based on cerebral angiography (CA). METHODS: A prospective data of patients with aneurysmal subarachnoid hemorrhage (aSAH) from January 2004 to August 2009. TCD was performed daily from day 2 to 14 from symptom's onset. Follow-up CA was done at day 7-9. TCD mean flow velocities (MFV) of all vessels at baseline (b), middle (m) and before CA (preangio) were recorded. Several MCA MFV ratios were computed. Moderate to severe VSP on CA was defined as >1/3 luminal narrowing. Univariate and stepwise logistic regression analysis were performed. RESULTS: One hundred sixty-nine patients (338 MCA) with aSAH were included, mean age: 54.8 ± 13, women: 103 (62%). Twenty-nine patients (8.6%) had angiographic MCA-VSP. TCD scoring system of 3 points for MCA-VSP was computed based on (a) bMCA MFV ≥ 120 cm/s (sensitivity: 59.3%, specificity: 85%, PPV: 36.4%, NPV: 93.5%, P < .001) (1 point), Preangio MCA MFV ≥ 150 cm/s (79.3%, 89.9%, 39%, 97.3%, <.001) (1 point), and affected preangio MCA/bMCA MFV ratio ≥ 1.5 (84%, 63%, 25.6%, 96.3%, .001) (1 point). The score of 3 has 96% sensitivity and 96% specificity (OR: 300) whereas the score of 1 has 12% sensitivity and 58% specificity (OR: 4.3) for identifying MCA-VSP. CONCLUSION: TCD stringent criteria for moderate to severe MCA-VSP are feasible and applicable in aSAH population.
Assuntos
Infarto da Artéria Cerebral Média/diagnóstico , Infarto da Artéria Cerebral Média/epidemiologia , Hemorragia Subaracnóidea/diagnóstico , Hemorragia Subaracnóidea/epidemiologia , Ultrassonografia Doppler Transcraniana/estatística & dados numéricos , Vasoespasmo Intracraniano/diagnóstico , Vasoespasmo Intracraniano/epidemiologia , Adulto , Alberta/epidemiologia , Causalidade , Angiografia Cerebral/estatística & dados numéricos , Comorbidade , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Reprodutibilidade dos Testes , Fatores de Risco , Sensibilidade e EspecificidadeRESUMO
BACKGROUND: Collateral flow augmentation using partial aortic occlusion may improve cerebral perfusion in acute stroke. We assessed the effect of partial aortic occlusion on arterial flow velocities of acute stroke patients. METHODS: Patients with neurological deficits following thrombolysis were treated with partial aortic occlusion. Transcranial Doppler ultrasound (TCD) was used to measure arterial flow velocities at baseline, before and during balloon inflation. The augmented mean flow velocity (MFV), peak systolic velocity (PSV), and end diastolic velocity flow percentages (aMFV%, aPSV%, aEDV%) were calculated and compared based on outcome. RESULTS: Of 11 patients, 3 did not have a temporal window and thus were excluded from our analysis. Six of the remaining 8 patients had middle cerebral artery (MCA) occlusions; the final 2 had terminal internal carotid artery (TICA) occlusions. Three of these 8 patients had good outcome at 90 days (mRS < 3). Before intra-aortic balloon inflation (IABI), the mean affected artery MFV was 23 ± 11 cm/s; during the procedure it was 26 ± 12 cm/s (P = .2). Mean affected artery PSV at baseline and during balloon inflation were 37 ± 16 and 46 ± 23, respectively (P = .1). Mean augmented affected artery MFV% in patients with good long-term outcome was 65.4 ± 46, while the result in those with poor outcome was -3.7 ± 21 (P = .03). Three patients developed anterior cross-filling, and of these 2 had good long-term outcome. CONCLUSION: TCD monitoring of patients treated with IABI may help in predicting outcome in this novel device.
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Aorta/diagnóstico por imagem , Oclusão com Balão/métodos , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/terapia , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/terapia , Ultrassonografia Doppler Transcraniana/métodos , Adulto , Idoso , Isquemia Encefálica/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/etiologia , Resultado do Tratamento , Ultrassonografia de Intervenção/métodosRESUMO
Intracranial arterial stenosis (IAS) is thought to be responsible for 8% of all ischemic stroke subtypes. The best medical treatment for this condition is still controversial. Transcranial Doppler (TCD) emboli monitoring may help to guide the treatment by measuring the frequency of microembolic signals (MES). We report a case of IAS where TCD emboli monitoring proved useful in establishing the mechanism of stroke by being artery-to-artery emboli and guiding therapy based on the frequent symptoms and number of MES.