The Role of Dual Antiplatelet Therapy (DAPT) vs Surgery in a Case of Moyamoya Disease: A Case Report and Review of the Literature.

Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by non-atherosclerotic and non-inflammatory progressive narrowing of the intracranial part of the carotid artery and its proximal branches. The disease process is commonly associated with the development of weak, dilated collateral blood vessels at the base of the brain. This gives it a classic smoky appearance on cerebral angiograms and hence the name "Moyamoya" which means "puff of smoke" in Japanese. When a patient has similar vasculopathy in the setting of another disease then it is known as Moyamoya syndrome (MMS). The associated diseases are sickle cell anemia, neurofibromatosis, long-standing diabetes, uncontrolled hypertension, or chemotherapy. Despite being known as a disease of the East Asian population, the disease is no longer exclusive to Asians, as evidenced by the rising incidence among non-Asian groups such as Caucasians, Hispanics, and African Americans. Patients can remain asymptomatic or present with ischemic or hemorrhagic stroke, headache, seizures, or recurrent transient ischemic attacks. Conventional cerebral angiography is considered the gold standard for diagnosing MMD. Treatment may be supportive, medical, or surgical. We present the case of a 42-year-old African American woman with several comorbidities who presented with sudden onset of ischemic stroke and upon further workup was found to have MMD. Equally important is to identify the most effective therapeutic approaches based on individual patients to achieve better clinical outcomes. Our case report highlights the importance of surgery in symptomatic MMD with a lack of supporting evidence indicating the benefits of dual antiplatelet therapy (DAPT).

Endovascular Revascularization of Multi Segment Chronically Occluded ICA.

This case report describes a novel endovascular method for treating chronically occluded internal carotid artery (COICA). The patient is a 55-year-old male with vascular risk factors who presented to an outside institution with right-sided weakness and dysarthria, was diagnosed as having a stroke, and discharged with medical management. The patient's symptoms failed to improve throughout the week prompting him to visit another outside institution, where computed tomography (CT) angiography showed bilateral occlusion of the ICAs at their origins extending intracranially. The patient was then transferred to our hospital, where head CT revealed bilateral acute infarcts predominantly in the left centrum ovale/corona radiata and left temporoparietal region. CT perfusion showed a large area of hypoperfusion in the entire left hemisphere as well as part of the right hemisphere (mismatch volume of 438-526 mL). The patient had significant neurological deficits despite sustained high perfusion pressure, so the following morning, the patient was taken for angiography showing complete occlusion of the left ICA with support mostly from the left external carotid artery (ECA)/ophthalmic collateralization. The microcatheter was able to be advanced to the level of the ophthalmic segment of the left ICA, so the decision was made to proceed with stenting from the left ophthalmic ICA to the cervical ICA. Seven consecutive coronary-carotid stents were placed to essentially reconstruct the left ICA. Post-stenting, the patient was treated with an Integrilin drip and transitioned to Aspirin and Brilinta the following morning. The patient's symptoms markedly improved after the procedure. CT perfusion, as well as diffusion magnetic resonance imaging (MRI), revealed recovery of the patient's penumbra and stability of the existing infarcts despite the delayed nature of revascularization respectively. This is a rarely reported study in literature describing the successful deployment of multiple stents in recreating the ICA from its extracranial to intracranial portion.

Etiologic Subtypes of Ischemic Stroke in SARS-CoV-2 Patients in a Cohort of New York City Hospitals.

Objective: To describe the ischemic stroke subtypes related to coronavirus disease 2019 (COVID-19) in a cohort of New York City hospitals and explore their etiopathogenesis. Background: Most neurological manifestations are non-focal, but few have reported the characteristics of ischemic strokes or investigated its pathophysiology. Methods: Data were collected prospectively April 1-April 15, 2020 from two centers in New York City to review possible ischemic stroke types seen in COVID-19-positive patients. Patient presentation, demographics, related vascular risk factors, associated laboratory markers, as well as imaging and outcomes were collected. Results: The age of patients ranged between 27 and 82 years. Approximately 81% of patients had known vascular risk factors, the commonest being hypertension (75%) followed by diabetes (50%) coronary disease or atrial fibrillation. Eight patients presented with large vessel occlusion (LVO) with median age 55 years (27-82) and all were male. Eight patients presented with non-LVO syndromes, with median age 65.5 years (59-82) and most were female (62.5%). Both groups were 50% African Americans and 37.5% South Asian. Both groups had similar D-dimer levels although other acute phase reactants/disease severity markers (Ferritin, CRP, procalcitonin) were higher in the LVO group. The LVO group also had a significantly higher mortality compared to the non-LVO group. The most common etiology was cryptogenic (6 patients) followed by small vessel occlusion (3 patients) and undetermined-unclassified (3 patients). For the remaining 4 patients, 2 were identified as cardioembolic and 2 with large artery atherosclerosis. Conclusion: COVID-19-related ischemic events can present as small vessel occlusions, branch emboli or large vessel occlusions. The most common etiology is cryptogenic. Patients with LVO syndromes tend to be younger, male and may have elevated acute inflammatory markers.

Emergency Medicine Physicians Accurately Select Acute Stroke Patients for Tissue-Type Plasminogen Activator Treatment Using a Checklist.

Background and Purpose- There is uncertainty among many emergency medicine physicians about the decision to give intravenous tPA (tissue-type plasminogen activator), which limits its use. A checklist approach has been suggested as a solution. We compared agreement on tPA treatment in suspected acute ischemic stroke patients between emergency medicine residents (EMRs) using a checklist and vascular neurology fellows (VNFs). Methods- Every suspected acute stroke patient brought to our comprehensive stroke center emergency room within 4.5 hours from symptom onset was prospectively evaluated simultaneously and independently by VNFs and EMRs. The latter used a tPA screening checklist, which included guideline exclusion criteria to help with their treatment decision. Agreement was determined using kappa (k) statistics. Results- Over 6 months, 60 patients were enrolled; 10% large vessel atherosclerosis, 18% cardioembolism, 12% small vessel, 12% cryptogenic, and 47% mimic. Forty-two percent were deemed tPA eligible by the EMR, 30% by the VNF, and 37% by the vascular neurology faculty. There were no complications in any tPA-treated patients. Agreement was substantial between EMR and VNF (κ=0.68 [95% CI, 0.49-0.87]) and between EMR and vascular neurology faculty (κ=0.69 [95% CI, 0.50-0.87]). Stroke mimics were the main cause of disagreement between EMR and VNF (κ=0.24 [95% CI, -0.15 to 0.63]) and between EMR and vascular neurology faculty (κ=0.35 [95% CI, -0.08 to 0.78]). Conclusions- Our data suggest that with the aid of a checklist, EMRs can accurately treat stroke patients with tPA. Areas for improvement include recognition of stroke mimics. Further studies are warranted to evaluate checklist-enhanced tPA treatment to allay emergency medicine physician uncertainty and expand the use of tPA.

Safety and Feasibility of Argatroban, Recombinant Tissue Plasminogen Activator, and Intra-Arterial Therapy in Stroke (ARTSS-IA Study).

BACKGROUND: A randomized trial of concurrent recombinant tissue-type plasminogen activator (r-tPA) + thrombin-inhibition with Argatroban in stroke patients recently demonstrated safety and signal of efficacy compared to r-tPA alone, but patients having endovascular therapy (EVT) were excluded. The current study intended to study feasibility and safety of concurrent r-tPA and Argatroban in patients undergoing EVT. METHODS: We conducted a single-arm, feasibility, and safety study of patients that received standard-dose r-tPA, had intracranial large vessel occlusions, and underwent EVT within 6 hours of stroke onset. During r-tPA, a 100 µg/kg Argatroban bolus, followed by 12-hour infusion, targeted an activated Partial Thromboplastin Time (aPTT) 2.25 timesbaseline. Feasibility was defined as ability to combine treatments without EVT time-metric delays, compared to cotemporaneous r-tPA + EVT treatments. Safety was incidence of symptomatic intracerebral hemorrhage (sICH), systemic hemorrhage, or EVT complications. RESULTS: All preplanned 10 patients were enrolled. Arterial occlusions were middle cerebral artery (n = 8), internal carotid artery (n = 1), and posterior cerebral artery (n = 1). All received Argatroban before EVT and completed infusions. There were no delays in time-metrics compared to nonstudy patients during the same period. Nine patients achieved excellent angiographic reperfusion (Thrombolysis In Cerebral Ischemia [TICI] ≥2b); with 7 complete (TICI = 3). There were no sICH, systemic hemorrhage, or EVT complications. At 90 days, 6 (60%) patients had a modified Rankin Scale of 0-2 and none died. CONCLUSIONS: In patients treated with r-tPA and EVT, concomitant Argatroban is feasible, does not delay EVT provision, produces high rates of recanalization, is probably safe, and warrants further study.

Correlation of Prehypertension with Left Ventricular Mass Assessed by Cardiac Magnetic Resonance Imaging.

Introduction. The purpose of this observational cross-sectional study was to assess left ventricular mass (LVM) in prehypertensive individuals in comparison to normotensives and to determine if central blood pressure (BP) correlates better with LVM index (LVMI) than brachial BP. Methods and Result. Brachial and central BP measurements were completed at first visit and at 4 weeks in 65 healthy volunteers who were at least 40 years old and not on medication. Subjects were divided into two groups of normotensives and prehypertensives based on JNC-7 criteria and LVM was obtained using cardiac magnetic resonance imaging. Prehypertensives had significantly higher LVMI compared to normotensives (P < 0.01). Brachial and central BP also both positively correlate with LVMI (r = 0.460, P < 0.01; r = 0.318, P = 0.012, resp.) in both groups and neither method was superior to the other. After multivariate regression analysis and adjusting for cardiovascular risk factors, prehypertension remained an independent determinant of LVM. Conclusion. Prehypertension is associated with cardiovascular target organ damage, and central BP was not superior to brachial BP or vice versa for association with LVMI.

Blood conservation techniques in spinal deformity surgery: a retrospective review of patients refusing blood transfusion.

STUDY DESIGN: A retrospective review. OBJECTIVE: To review the effectiveness of blood conservation techniques in the spinal fusion of patients that refuse blood transfusion; specifically the Jehovah's witnesses population. SUMMARY OF BACKGROUND: Spinal surgery can be challenging in patients refusing blood transfusion. There is paucity in the literature examining blood conservation techniques in spinal surgery. METHODS: The radiographic and medical records of 19 Jehovah's witnesses patients who underwent spinal deformity surgery at a single institution between 2000 and 2003 were reviewed. Patients were assessed for excessive blood loss (EBL), deformity correction, operative time, perioperative complications, and hospital stay. At latest follow-up (mean, 40 months; range, 8-76) the patients were examined for radiographic fusion, progression and complications. RESULTS: Spinal fusion was attempted in 19 patients, with a mean age of 17 years (range, 10-36 years). All 19 patients were identified through the "Bloodless Surgery Program." Hypotensive anesthesia, hemodilution, and cell saver was employed for all 19 cases. Erythropoietin with supplemental iron was used in 15 patients. Aprotinin was used in 3 patients. EBL and blood returned by cell saver averaged 855 and 341 mL, respectively. Operative times average 315 minutes. The average drop in hemoglobin from after surgery was 3.1 g/dL. There were 2 intraoperative complications: (i) transient loss of somatosensory evoked potential/motor evoked potential signals; and (ii) one surgery abandoned due to EBL. The average spinal deformity correction was 58%. There were 3 postoperative complications, none related to their refusal of a transfusion. 17 patients were available for radiographic and clinic follow-up of at least 24 months. All displayed radiographic fusion without progression. CONCLUSION: These blood conservation techniques allow satisfactory completion of deformity surgery on those patients not willing to be transfused and without major anesthetic or medical complications.

Efficacy of aprotinin as a blood conservation technique for adult deformity spinal surgery: a retrospective study.

STUDY DESIGN: A retrospective study that involved a total of 82 adult deformity patients who underwent spinal fusion surgery with instrumentation of 6 or more levels. OBJECTIVE: The purpose of this study is to assess the efficacy and safety of aprotinin in reducing blood loss in adult spinal deformity patients. SUMMARY OF BACKGROUND DATA: Aprotinin is an antifibrinolytic agent, which has been proven to be effective as a blood-conserving agent in cardiac surgery. This study focused specifically on adult spine deformity patients. METHODS: This retrospective study analyzed a total of 82 adult deformity patients (patients with diagnosis of adult scoliosis and kyphosis) who underwent spinal fusion surgery with instrumentation of 6 or more levels. Forty-one patients who received aprotinin were compared with a control group of 41 patients who did not receive aprotinin. Specific surgical outcome parameters and postoperative complications were assessed. RESULTS: The average age of the patients was 47 years and 41 years for the aprotinin group and the control group respectively. Thirty-nine percent of cases and 44% of controls had staged anterior/posterior fusion surgery, 39% of cases and 41% of controls underwent single stage posterior fusion surgery and the remaining 27% of cases and 15% of controls had same day anterior and posterior fusion surgery. The average number of levels fused and instrumented was 11.2 for the aprotinin group and 10.7 for the control group. The differences in mean blood loss and blood returned by cell-saver were statistically significantly lower in the aprotinin group (1324 mL vs. 2113 mL; P = 0.005 and 390 mL vs. 657 mL; P = 0.003). The amount of drainage after surgery was similar (1421 mL vs. 1388 mL; P = 0.982). The aprotinin group received an average of 2.73 units of blood. This was significantly lower than the average 5.02 units that the controls received (P = 0.003). The rate of complications was comparable and no allergic reaction to aprotinin was noted. One case exhibited a transitory elevation of blood urea nitrogen/creatinine which resolved before discharge. CONCLUSION: The results of our study suggest that the administration of aprotinin is a safe and effective method to reduce blood loss and to decrease the need for transfusions without increasing risk of complication in the younger patients in relatively good general health undergoing spinal deformity surgery requiring 6 or more levels of fusion.

Selective serotonin reuptake inhibitor use and outcomes in pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary vascular resistance which leads to right ventricular failure. Serotonin and the serotonin transporter play an important role in animal and human studies of PAH. We therefore hypothesized that PAH patients treated with high-affinity selective serotonin reuptake inhibitors (SSRIs) would have a reduced risk of death compared to PAH patients not treated with SSRIs. METHODS: We performed a retrospective cohort study of 84 consecutive adult PAH patients who underwent initial evaluation from January 1994 to June 2002 at the Pulmonary Hypertension Center of the New York Presbyterian Hospital. Patient-time while receiving high-affinity SSRIs (K(d)<1nmol) (paroxetine, sertraline, or fluoxetine) was considered "exposed". Patient-time while receiving tricyclic, atypical, or no antidepressants was considered "unexposed". RESULTS: Thirteen of the 84 patients (15%) used high-affinity SSRIs during the study period. Five patients were taking high-affinity SSRIs at baseline and 8 initiated high-affinity SSRIs during the follow-up period. The median time from baseline evaluation until initiation of high-affinity SSRIs was 125 (0-1227) days. The median duration of high-affinity SSRI use was 482 (110-1624) days and the total at-risk time on high-affinity SSRIs was 18.1 person-years. Seventy-nine (94%) patients were treated with warfarin; 38 (45%) received continuous intravenous epoprostenol; 12 (14%) received continuous subcutaneous treprostinil, and 23 (27%) were treated with oral bosentan. The median follow-up was 764 days. Twenty-four patients died and one underwent lung transplantation during the study period. There were no differences in age, gender, diagnosis, hemodynamics, or incidence of acute vasoreactivity between SSRI users and non-users. The risk of death for high-affinity SSRI users was lower but not statistically significantly different from that of non-users (hazard ratio=0.53, 95% CI 0.07 to 3.9, p=0.53). Adjustment for demographics, diagnosis, hemodynamics, or other therapies did not significantly change this result. CONCLUSIONS: SSRI use was associated with a 50% reduction in the risk of death in a cohort of PAH patients which was not statistically significant. Larger cohort studies may better define this relationship; an adequately powered trial of high-affinity SSRIs in PAH patients may be warranted.

von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension.

OBJECTIVES: We hypothesized that higher plasma levels of von Willebrand factor (vWF) and reduced ristocetin cofactor activity (RCA)/vWF ratios at baseline and follow-up would be associated with an increased risk of death in patients with pulmonary arterial hypertension (PAH). BACKGROUND: Endothelial injury and dysfunction cause increased vWF levels in patients with cardiovascular disease. Increased vWF is associated with a higher risk of death in patients with coronary artery disease, congestive heart failure, and ARDS. In PAH, vWF is elevated and functions abnormally, resulting in reduced platelet binding. The ability of vWF to bind platelets is reflected by RCA. METHODS: We performed a retrospective cohort study of 66 PAH patients who underwent initial evaluation at our center from January 1994 to June 2002. The primary outcome was death or lung transplantation. RESULTS: vWF level was directly associated with Factor VIII activity, RCA, fibrinogen, and prothrombin time (p < or = 0.01). vWF levels at baseline and follow-up were associated with the risk of death (hazard ratio [HR] per 50% increase, 1.4; 95% confidence interval [CI], 1.1 to 1.8 [p = 0.02]; and HR per 50% increase, 1.5; 95% CI, 1.1 to 2.0 [p = 0.011]; n = 48, respectively). RCA/vWF ratios were not associated with outcome. These results were unaffected by adjustment for demographics, baseline hemodynamics, laboratory results, or PAH therapy in multivariate analyses. CONCLUSIONS: Increased vWF levels at baseline and follow-up are associated with worse survival in patients with PAH; however, RCA/vWF ratios are not. The hemostatic effects of vWF do not account for these findings. This suggests that endothelial injury and dysfunction may persist despite treatment and may impact on disease course. Therapies that target endothelial injury may therefore improve outcomes for patients with PAH.

New predictors of outcome in idiopathic pulmonary arterial hypertension.

Idiopathic pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. New therapies have improved the outcome of this condition; accordingly, the factors that determine outcome may have changed. We aimed to identify determinants of survival in a cohort of consecutive patients with PAH: which was idiopathic, familial, or associated with anorexigen use. We performed a retrospective cohort study of 84 consecutive patients with PAH who underwent initial evaluation at our center from January 1994 to June 2002. The primary outcome was death or lung transplantation. Survival at 1, 3, and 5 [corrected] years was 87%, 75%, and 61%, respectively. Multivariate analysis showed that being of African-American or Asian descent was associated with an increased risk of death. Warfarin use was associated with a reduced risk of death. Higher serum albumin and cardiac index and acute vasoreactivity were independently associated with improved survival. These data suggest that the determinants of outcome have changed. Race is identified as a new risk factor, which may be attributable to biologic or socioeconomic differences. Cardiac function and acute reactivity of the pulmonary vascular bed remain strong independent predictors of outcome.