Performance of RAPID noncontrast CT stroke platform in large vessel occlusion and intracranial hemorrhage detection.

Background: Noncontrast CT (NCCT) is used to evaluate for intracerebral hemorrhage (ICH) and ischemia in acute ischemic stroke (AIS). Large vessel occlusions (LVOs) are a major cause of AIS, but challenging to detect on NCCT. Aims: The purpose of this study is to evaluate an AI software called RAPID NCCT Stroke (RAPID, iSchemaView, Menlo Park, CA) for ICH and LVO detection compared to expert readers. Methods: In this IRB approved retrospective, multicenter study, stand-alone performance of the software was assessed based on the consensus of 3 neuroradiologists and sensitivity and specificity were determined. The platform's performance was then compared to interpretation by readers comprised of eight general radiologists (GR) and three neuroradiologists (NR) in detecting ICH and hyperdense vessel sign (HVS) indicating LVO. Results: A total of 244 cases were included. Of the 244, 115 were LVOs and 26 were ICHs. One hundred three cases did not have LVO nor ICH. Stand-alone performance of the software demonstrated sensitivities and specificities of 96.2 and 99.5% for ICH and 63.5 and 95.1% for LVO detection. Compared to all 11 readers and eight GR readers only respectively, the software demonstrated superiority, achieving significantly higher sensitivities (63.5% versus 43.6%, p < 0.0001 and 63.5% versus 40.9%, p = 0.001). Conclusion: The RAPID NCCT Stroke platform demonstrates superior performance to radiologists for detecting LVO from a NCCT. Use of this software platform could lead to earlier LVO detection and expedited transfer of these patients to a thrombectomy capable center.

Decreasing false-positive detection of intracranial hemorrhage (ICH) using RAPID ICH 3.

INTRODUCTION: The prompt detection of intracranial hemorrhage (ICH) on a non-contrast head CT (NCCT) is critical for the appropriate triage of patients, particularly in high volume/high acuity settings. Several automated ICH detection tools have been introduced; however, at present, most suffer from suboptimal specificity leading to false-positive notifications. METHODS: NCCT scans from 4 large databases were evaluated for the presence of an ICH (IPH, IVH, SAH or SDH) of >0.4 ml using fully-automated RAPID ICH 3.0 as compared to consensus detection from at least two neuroradiology experts. Scans were excluded for (1) severe CT artifacts, (2) prior neurosurgical procedures, or (3) recent intravenous contrast. ICH detection accuracy, sensitivity, specificity, positive predictive value, negative predictive value, and positive and negative likelihood ratios by were determined. RESULTS: A total of 881 studies were included. The automated software correctly identified 453/463 ICH-positive cases and 416/418 ICH-negative cases, resulting in a sensitivity of 97.84% and specificity 99.52%, positive predictive value 99.56%, and negative predictive value 97.65% for ICH detection. The positive and negative likelihood ratios for ICH detection were similarly favorable at 204.49 and 0.02 respectively. Mean processing time was <40 seconds. CONCLUSIONS: In this large data set of nearly 900 patients, the automated software demonstrated high sensitivity and specificity for ICH detection, with rare false-positives.

Cerebral perfusion imaging predicts final infarct volume after basilar artery thrombectomy.

OBJECTIVES: Cerebral perfusion imaging may be used to identify the ischemic core in acute ischemic stroke (AIS) patients with a large vessel occlusion of the anterior circulation; however, perfusion parameters that predict the ischemic core in AIS patients with a basilar artery occlusion (BAO) are poorly described. We determined which cerebral perfusion parameters best predict the ischemic core after successful endovascular thrombectomy (EVT) in BAO patients. MATERIALS AND METHODS: We performed multicenter retrospective study of BAO patients with perfusion imaging before EVT and a DWI after successful EVT. The ischemic core was defined as regions on CTP, which were co-registered to the final DWI infarct. Various time-to-maximum (Tmax) and cerebral blood flow (CBF) thresholds were compared to final infarct volume to determine the best predictor of the final infarct. RESULTS: 28 patients were included in the analysis for this study. Tmax >8s (r2: 0.56; median absolute error, 16.0 mL) and Tmax >10s (r2: 0.73; median absolute error, 11.3 mL) showed the strongest agreement between the pre-EVT CTP study and the final DWI. CBF <38% (r2: 0.76; median absolute error, 8.2 mL) and CBF <34% (r2: 0.76; median absolute error, 9.1 mL) also correlated well with final infarct volume on DWI. CONCLUSIONS: Pre-EVT CT perfusion imaging is useful to predict the final ischemic infarct volume in BAO patients. Tmax >8s and Tmax >10s were the strongest predictors of the post-EVT final infarct volume.

Collateral status, hyperglycemia, and functional outcome after acute ischemic stroke.

BACKGROUND: Mixed data exist regarding the association between hyperglycemia and functional outcome after acute ischemic stroke when accounting for the impact of leptomeningeal collateral flow. We sought to determine whether collateral status modifies the association between treatment group and functional outcome in a subset of patients with large vessel occlusion enrolled in the Stroke Hyperglycemia Insulin Network Effort (SHINE) trial. METHODS: In this post-hoc analysis, we analyzed patients enrolled into the SHINE trial with anterior circulation large vessel occlusion who underwent imaging with CT angiography prior to glucose control treatment group assignment. The primary analysis assessed the degree to which collateral status modified the effect between treatment group and functional outcome as defined by the 90-day modified Rankin Scale score. Logistic regression was used to model the data, with adjustments made for thrombectomy status, age, post-perfusion thrombolysis in cerebral infarction (TICI) score, tissue plasminogen activator (tPA) use, and baseline National Institutes of Health Stroke Scale (NIHSS) score. Five SHINE trial centers contributed data for this analysis. Statistical significance was defined as a p-value < 0.05. RESULTS: Among the 1151 patients in the SHINE trial, 57 with angiographic data were included in this sub-analysis, of whom 19 had poor collaterals and 38 had good collaterals. While collateral status had no effect (p = 0.855) on the association between glucose control treatment group and functional outcome, patients with good collaterals were more likely to have a favorable functional outcome (p = 0.001, OR 5.02; 95% CI 1.37-16.0). CONCLUSIONS: In a post-hoc analysis using a subset of patients with angiographic data enrolled in the SHINE trial, collateral status did not modify the association between glucose control treatment group and functional outcome. However, consistent with prior studies, there was a significant association between good collateral status and favorable outcome in patients with large vessel occlusion stroke. TRIAL REGISTRATION: ClinicalTrials.gov Identifier is NCT01369069. Registration date is June 8, 2011.

Perfusion Imaging Predicts Favorable Outcomes after Basilar Artery Thrombectomy.

OBJECTIVE: Perfusion imaging identifies anterior circulation stroke patients who respond favorably to endovascular thrombectomy (ET), but its role in basilar artery occlusion (BAO) is unknown. We hypothesized that BAO patients with limited regions of severe hypoperfusion (time to reach maximum concentration in seconds [Tmax] > 10) would have a favorable response to ET compared to patients with more extensive regions involved. METHODS: We performed a multicenter retrospective cohort study of BAO patients with perfusion imaging prior to ET. We prespecified a Critical Area Perfusion Score (CAPS; 0-6 points), which quantified severe hypoperfusion (Tmax > 10) in cerebellum (1 point/hemisphere), pons (2 points), and midbrain and/or thalamus (2 points). Patients were dichotomized into favorable (CAPS ≤ 3) and unfavorable (CAPS > 3) groups. The primary outcome was a favorable functional outcome 90 days after ET (modified Rankin Scale = 0-3). RESULTS: One hundred three patients were included. CAPS ≤ 3 patients (87%) had a lower median National Institutes of Health Stroke Scale score (NIHSS; 12.5, interquartile range [IQR] = 7-22) compared to CAPS > 3 patients (13%; 23, IQR = 19-36; p = 0.01). Reperfusion was achieved in 84% of all patients, with no difference between CAPS groups (p = 0.42). Sixty-four percent of reperfused CAPS ≤ 3 patients had a favorable outcome compared to 8% of nonreperfused CAPS ≤ 3 patients (odds ratio [OR] = 21.0, 95% confidence interval [CI] = 2.6-170; p < 0.001). No CAPS > 3 patients had a favorable outcome, regardless of reperfusion. In a multivariate regression analysis, CAPS ≤ 3 was a robust independent predictor of favorable outcome after adjustment for reperfusion, age, and pre-ET NIHSS (OR = 39.25, 95% CI = 1.34->999, p = 0.04). INTERPRETATION: BAO patients with limited regions of severe hypoperfusion had a favorable response to reperfusion following ET. However, patients with more extensive regions of hypoperfusion in critical brain regions did not benefit from endovascular reperfusion. ANN NEUROL 2022;91:23-32.

Characterizing Blunt Cerebrovascular Injuries and Stroke: A Single Center Retrospective Study.

OBJECTIVE: To highlight the occurrence of ischemic stroke after blunt cerebrovascular injuries and discuss the neurologist's role in preventing and managing ischemic strokes in this trauma population. METHODS: A retrospective chart review was performed and included data from 2016 to 2019 from a Level I trauma center. Demographics, injury mechanism, ischemic stroke occurrence, interventions, and neurology consultations were examined and descriptive statistics were utilized to characterize the nature of ischemic strokes and their management. RESULTS: A total of forty patients (81% male, average age 44) presented with blunt cerebrovascular injury, nine of whom later developed ischemic stroke. Eighteen patients had a carotid artery injury with six developing ischemic stroke. Twenty-seven patients had a vertebral artery injury with three developing ischemic stroke. Six of the nine ischemic strokes occurred on hospital day two, whereas neurology was generally consulted on hospital day four. CONCLUSIONS: A considerable portion of patients may go on to develop ischemic stroke following blunt cerebrovascular injuries. Polytrauma may interfere with prompt diagnosis which may contribute to delayed anti-thrombotic therapy for ischemic stroke prevention. Neurologists have the opportunity to reduce ischemic stroke burden in this trauma population and patients may benefit from earlier neurology consultation.

Concomitant central venous sinus thrombosis and subdural hematoma in acute promyelocytic leukemia: middle meningeal artery embolization enables safe anticoagulation. Illustrative case.

BACKGROUND: Acute promyelocytic leukemia (APL) has long been associated with coagulation disorders. The proposed mechanism is a combination of fibrinolysis, proteolysis, platelet dysfunction, thrombocytopenia, and possibly disseminated intravascular coagulation. Hemorrhagic complications are prominent. OBSERVATIONS: In this case, a 25-year-old female with newly diagnosed APL developed extensive cerebral venous thrombosis (CVT) and was initiated on a protocol with idarubicin and all-trans retinoic acid. The general recommendation for treating CVT is anticoagulation to stabilize the existing thrombus and prevent propagation. The patient was initiated on a heparin drip, but her clinical course was complicated by subdural hemorrhage (SDH) and epidural hemorrhage in the setting of thrombocytopenia. Anticoagulation was held, and her CVT propagated on follow-up imaging. To restart anticoagulation for CVT with a limited risk of SDH, the authors pursued middle meningeal artery (MMA) embolization. The patient was transitioned to apixaban and discharged to home. LESSONS: MMA embolization enables safe anticoagulation in patients with concomitant CVT and SDH. The authors report the complex clinical course and effective management of this rare clinical scenario.

Outcomes of interfacility helicopter transportation in acute stroke care.

OBJECTIVE: To evaluate the long-term functional outcome of interhospital transfer of patients with stroke with suspected large vessel occlusion (LVO) using Helicopter Emergency Medical Services (HEMS). METHODS: Records of consecutive patients evaluated through 2 telestroke networks and transferred to thrombectomy-capable stroke centers between March 2017 and March 2018 were reviewed. Inverse probability of treatment weighting (IPTW) using the propensity score was performed to address confounding factors. Multivariate logistic regression analysis with IPTW was used to determine whether HEMS were associated with good long-term functional outcome (modified Rankin scale score ≤ 2). RESULTS: A total of 199 patients were included; median age was 67 years (interquartile range [IQR] 55-79 years), 90 (45.2%) were female, 120 (60.3%) were white, and 100 (50.3%) were transferred by HEMS. No significant differences between the 2 groups were found in mean age, sex, race, IV tissue plasminogen activator (tPA) receipt, and thrombectomy receipt. The median baseline NIH Stroke Scale score was 14 (IQR 9-18) in the helicopter group vs 11 (IQR 6-18) for patients transferred by ground (p = 0.039). The median transportation time was 60 minutes (IQR 49-70 minutes) by HEMS and 84 minutes (IQR 25-102 minutes) by ground (p < 0.001). After weighting baseline characteristics, the use of HEMS was associated with higher odds of good long-term outcome (OR 4.738, 95% CI 2.15-10.444, p < 0.001) controlling for transportation time, door-in-door-out time, and thrombectomy and tPA receipt. The magnitude of the HEMS effect was larger in thrombectomy patients who had successful recanalization (OR 1.758, 95% CI 1.178-2.512, p = 0.027). CONCLUSIONS: HEMS use was associated with better long-term functional outcome in patients with suspected LVO, independently of transportation time.

Neurothrombectomy for Acute Ischemic Stroke Across Clinical Trial Design and Technique: A Single Center Pooled Analysis.

Introduction: The practice of endovascular therapy has evolved dramatically over the last 10 years with randomized clinical trials investigating the benefit of thrombectomy in select patient populations based on time of presentation, imaging criteria, and procedural technique. We sought to understand the benefit of thrombectomy in patients treated within the context of a clinical trial at a single academic center. Methods: Patient-level data recorded in case forms and core-lab adjudicated data were analyzed from patients enrolled in RCTs investigating the benefit of endovascular thrombectomy over medical management (IMSIII, MR RESCUE, ESCAPE, SWIFT PRIME, and DAWN) between 2007 and 2017 at a single academic referral center. Results: A total of 134 patients (intervention group, n = 81; medical group, n = 53) were identified across five clinical trials (IMSIII, n = 46; MR RESCUE, n = 4; ESCAPE, n = 24; SWIFT PRIME, n = 14; DAWN, n = 46). There were no significant differences between the treatment arm and control arm in terms of age, gender, baseline NIHSS, ASPECTS, and site of occlusion. Rates of good outcome were superior in the intervention group with early neurological recovery (NIHSS of 0-1 or increase NIHSS of 8 points at 24 h) at a higher rate of 49% vs. 17% (p = <0.001) and higher rates of functional independence (90 day mRS 0-2 of 53% vs. 26%, p = 0.002). In multivariate logistic regression analysis, lower NIHSS and younger age were predictors of good outcome. There were comparable rates of good outcome irrespective of clinical trial, imaging selection criteria (CTP vs. MRI), early vs. late time window (0-6 h vs. 6-24 h) and procedural technique (Merci vs. Solitaire/Trevo device). There were no differences in rates of sICH, PH-2 or mortality in the intervention group vs. medical group. Conclusions: At a large academic center, the benefit of endovascular therapy over medical therapy is observed irrespective of clinical trial design, patient selection or procedural technique.

Provocative Testing Prior to Anterior Cerebral Artery Fusiform Aneurysm Embolization.

We report 2 cases of parent artery occlusion (PAO) for anterior cerebral artery (ACA) fusiform aneurysm embolization after superselective provocative testing was performed to confirm distal territory viability. The first case involves a patient in the second decade of life who presented with subarachnoid hemorrhage and underwent PAO after a balloon test occlusion in the distal ACA revealed no neurophysiology changes. The second case involves another patient in the forth decade of life who presented with an enlarging pseudoaneurysm and underwent PAO after a sodium amobarbital infusion in the distal ACA revealed no clinical change. Both patients tolerated PAO without clinical compromise. PAO after provocative testing may be a safe and effective strategy in the management of fusiform aneurysm treatment. KEY MESSAGES: Provocative testing with superselective balloon test occlusion and sodium amobarbital infusion are both viable options for clinical and physiological interrogation of brain tissue prior to parent vessel occlusion. Neurophysiological monitoring may be a useful surrogate for clinical examination after provocative testing, particularly if patients were treated under general anesthesia.

Stent Retriever-Mediated Manual Aspiration Thrombectomy for Acute Ischemic Stroke.

BACKGROUND AND PURPOSE: Stent retriever thrombectomy and manual aspiration thrombectomy (MAT) have each been shown to lead to high rates of recanalization as single-modality endovascular stroke therapy. We sought to describe the safety and efficacy of a multimodal approach combining these two techniques termed 'stent retriever-mediated manual aspiration thrombectomy' (SMAT) and compared them to MAT alone. METHODS: Retrospective review of a prospectively acquired acute endovascular stroke database. RESULTS: 195 consecutive patients with large-vessel occlusion were identified between July 2013 and April 2015. Occlusion distribution was as follows: 52% middle cerebral artery segment 1 (M1), 6% M2, 29% internal carotid artery, and 13% vertebrobasilar. Median onset to treatment time was 278 min. Intravenous rtPA was administered in 33% of cases, whereas 34% of cases had symptom onset beyond 8 h. Effective recanalization (TICI 2b/3) was achieved in 91% of patients and in 49% of patients, only a single pass was necessary. Median groin puncture to recanalization time was 40 min. Symptomatic intracerebral hemorrhage occurred in 5% of patients. Favorable outcomes defined as a modified Rankin Scale score of 0-2 were noted in 42% of patients. Compared with MAT alone, SMAT achieved a similar rate of effective recanalization (91 vs. 88%, p = n.s.) but was associated with faster access to reperfusion times (49 vs. 77 min, p < 0.00001). CONCLUSIONS: SMAT is a safe and efficacious method to achieve rapid revascularization that leads to faster recanalization compared to manual aspiration alone. Future prospective comparisons are necessary to establish the most clinically effective therapy for acute thrombectomy.

Streamlining door to recanalization processes in endovascular stroke therapy.

BACKGROUND: In acute stroke due to large vessel occlusion, faster reperfusion leads to better outcomes. We analyzed the effect of optimization steps aimed to reduce treatment delays at our center. METHODS: Consecutive patients with ischemic stroke treated with endovascular therapy were prospectively analyzed. We divided the patients into pre-optimization (20 April 2012 to 8 October 2013) and post-optimization (9 October 2013 to 29 July 2014) periods. The main interventions included: (1) continuous feedback; (2) standardized immediate emergency department attending to stroke attending communication with interventional team activation for all potential interventions; (3) pre-notification by the emergency medical service; (4) minimizing additional diagnostic testing; (5) direct transport to the CT scanner; (6) transport directly from the CT scanner to the angiography suite. The main metric used to measure improvement was door to groin puncture time (D2P). RESULTS: We included a total of 286 patients (178 pre-optimization, 108 post-optimization). There were no significant differences between major baseline characteristics between the groups with the exception of higher median CT Alberta Stroke Program Early CT Score in the pre-optimization group (p=0.01). Median D2P improved from 105 min pre-optimization to 67 min post-optimization (p=0.0002). Rates of good clinical outcomes (modified Rankin Scale 0-2 at 3 months) were similar in both groups, with a trend toward a better outcome in the post-optimization group in a subgroup analysis of patients with anterior circulation occlusion who received intravenous tissue plasminogen activator. CONCLUSIONS: This pilot study demonstrates that D2P times can be significantly reduced with a standardized multidisciplinary approach. There was no significant difference in the rate of 3-month good outcome, which is most likely due to the small sample size and confounding baseline patient characteristics.

FLAIR Vascular Hyperintensity is a Surrogate of Collateral Flow and Leukoaraiosis in Patients With Acute Stroke Due to Proximal Artery Occlusion.

BACKGROUND: Fluid attenuated inversion recovery (FLAIR) vascular hyperintensity (FVH) is a novel radiographic marker detected in acute ischemic stroke (AIS) patients, which is linked to slow blood flow and potentially salvageable brain tissue. Poor leptomeningeal collateral status in AIS patients with proximal artery occlusion (PAO) is associated with larger final infarct and worse clinical outcomes, which are also affected by severity of white matter hyperintensity (WMH). We sought to evaluate FVH utility as a marker of acute collateral vessel status and its association with WMH burden in AIS patients. METHODS: Consecutive AIS patients with PAO on baseline CT angiography (CTA) were retrospectively selected from a prospectively derived database. FVH was graded by its location, degree, and score on admission MRI obtained immediately after intravenous tissue plasminogen activator administration. Leptomeningeal collateral flow grade was ranked on admission CTA. WMH volume (WMHV) was assessed using a validated volumetric protocol. Relationship between FVH, collateral flow grade, and WMHV were analyzed. RESULTS: Among 39 patients (mean age 70.5 ± 12.7 years; 56% women, mean National Institutes of Health Stroke Scale score 17.2 (± 4.4)), median WMHV was 6.0 cm(3). FVH score and collateral flow grade were significantly correlated (Spearman's ρ = .41, P = .009). In a univariate regression model, FVH degree was inversely associated with WMHV (ß = -.33, P = .04). CONCLUSIONS: FVH score detected on acute MRI can be used as a surrogate of collateral flow grade in AIS patients. FVH degree is inversely associated with WMHV, possibly signifying diffuse disease of cerebral vasculature in patients with severe leukoaraiosis.

Severity of leukoaraiosis, leptomeningeal collaterals, and clinical outcomes after intra-arterial therapy in patients with acute ischemic stroke.

BACKGROUND AND PURPOSE: Leukoaraiosis (LA) is defined as ischemic white matter lesions associated with increased stroke risk and poor post-stroke outcomes. These lesions are likely the result of diffuse angiopathic changes affecting the cerebral small vessels. We investigated whether pre-existing LA burden is associated with outcomes in patients with large cerebral artery occlusion undergoing intra-arterial therapy (IAT) for acute ischemic stroke (AIS). METHODS: We analyzed consecutive AIS subjects undergoing IAT from the institutional Get With The Guidelines-Stroke database enrolled between January 1, 2007 and June 30, 2009, who had National Institutes of Health Stroke Scale scores of ≥ 8, baseline diffusion weighted imaging volume ≤ 100 mL, and evidence of proximal artery occlusion (PAO) on pre-IAT computed tomography angiography (CTA). LA volume (LAv) was assessed on fluid attenuated inversion recovery MRI using a validated semi-automated protocol. We used CTA for collateral grade, post-IAT angiogram for recanalization status (Thrombolysis in Cerebral Infarction score ≥ 2b), and the 24 h head CT for symptomatic intracranial hemorrhage. Logistic regression was used to determine independent predictors of 90 day post-stroke good functional outcome (modified Rankin Scale score ≤ 2) and mortality. RESULTS: Increasing LAv independently reduced the odds of good collateral grade (OR 0.85, 95% CI 0.73 to 0.98). Good functional outcome was independently predicted by intravenous tissue plasminogen activator use (OR 12.86, 95% CI 2.20 to 76.28), and recanalization status (OR 6.94, 95% CI 1.56 to 30.86). Mortality was independently associated with recanalization status (OR 0.08, 95% CI 0.01 to 0.51), age (OR 1.08, 95% CI 1.01 to 1.15), and antecedent use of hypoglycemic agents (OR 6.55, 95% CI 1.58 to 54.01). CONCLUSIONS: Severity of LA is linked to poor collateral grade in AIS patients undergoing IAT for PAO; however, greater LAv appears not to be a contraindication for acute intervention.

Outcomes after endovascular treatment for anterior circulation stroke presenting as wake-up strokes are not different than those with witnessed onset beyond 8†hours.

OBJECTIVE: Previous studies have suggested that patients with wake-up stroke (WUS) may have superior outcomes compared with patients with a witnessed late time of onset after revascularization. We sought to test this hypothesis in patients with anterior circulation large vessel occlusion stroke (ACLVOS) treated with endovascular therapy beyond 8 h from time last seen well (TLSW). METHODS: A single center retrospective review of a prospectively acquired database of consecutive patients was performed to identify patients presenting beyond 8 h of TLSW with radiographic evidence of ACLVOS, small core, and large penumbra who subsequently underwent endovascular treatment. RESULTS: We identified 206 patients. Patients were divided into two groups: (1) patients with WUS (38%, n=78) and (2) patients with witnessed onset beyond 8 h (62%, n=128). The groups were similar in age, baseline National Institutes of Health Stroke Scale score, TLSW to reperfusion, baseline infarct volume, and rate of successful recanalization. Rates of good outcome (modified Rankin Scale score of 0-2 at 90 days, 43% vs. 50%, p=0.3), parenchymal hematoma (9% vs. 5.5%, p=0.3), and final infarct volume (75.2 vs. 61.4 mL, p=0.6) were comparable. Multivariate analysis identified age (OR=0.95, 95% CI 0.91 to 0.99, p<0.042), successful recanalization (OR 6.0, 95% CI 1.5 to 23.5, p=0.009), and final infarct volume (OR 0.98, 95% CI 0.97 to 0.99, p<0.001) but not mode of presentation as predictors of favorable outcomes. CONCLUSIONS: Rates of good outcomes, parenchymal hematoma, and final infarct volumes following endovascular treatment may not be different in patients with WUS compared with patients with witnessed onset of symptoms beyond 8 h.

Persistent growth failure in Prader-Willi syndrome associated with short-chain acyl-CoA dehydrogenase gene variant.

The authors report the rare association of Prader-Willi syndrome and short-chain acyl-CoA dehydrogenase gene variant. Prader-Willi syndrome, associated with paternal chromosome 15q11-q13 silencing, is characterized by neonatal/infantile hypotonia, growth failure, and neurodevelopmental delays in the first 1 to 2 years of life, typically followed by hyperphagia and obesity. Short-chain acyl-CoA dehydrogenase gene variant, with 625 G-to-A and 511 C-to-T changes, impairs C4-C6 fatty acid metabolism and variably causes neonatal/infantile hypotonia with developmental delays. The authors' patient continues to exhibit the classic severe growth failure of early infancy Prader-Willi syndrome at 40 months. Extensive laboratory investigations indicate that the short-chain acyl-CoA dehydrogenase gene variant is likely preventing or delaying the normal expression of the Prader-Willi syndrome phenotype.

Apparent racial disparity in the utilization of invasive testing for risk assessment of cardiac patients undergoing noncardiac surgery.

BACKGROUND: Studies on racial disparity in the use of cardiac diagnostic procedures are limited because they were conducted in the acute clinical setting without control for patient knowledge and emotional state or used models not controlling for racism. Using the setting (model) of elective evaluation of known, stable, cardiac patients undergoing noncardiac surgery, where the surgeon/anesthesiologist's personal interest precludes expression of potential racial bias, we assessed for racial differences in the utilization of diverse cardiac diagnostic procedures for risk assessment and optimization. METHODS: This is a secondary analysis of data from 314 consecutive patients [92 (29%) African-American, 222 (71%) Caucasian] with coronary artery disease (CAD), cardiomyopathy (ejection fraction <45%), or treatment-requiring arrhythmias, who underwent noncardiac surgery. RESULTS: The incidence of angina, prior myocardial infarction, and ischemic cardiomyopathy was higher in Caucasians (75%, 68%, and 164%, P<.0001, respectively), while nonischemic cardiomyopathy was more prevalent among African-Americans (84%, P<.0001). While, multivariately, African race predicted underuse of coronary angiography (odds ratio: 0.10, 95% confidence interval: 0.04-0.26, P<.0001), this predictor was eliminated when presence of CAD plus cardiomyopathy was factored in as a surrogate of severity. The use of noninvasive cardiac procedures and the 30-day mortality and morbidity did not differ by race. CONCLUSION: In a racism-proof model of preoperative evaluation of stable cardiac veterans, the racial disparity in the use of invasive procedures was related to epidemiologic differences. In addition, the parity in mortality and morbidity corroborates no underuse of diagnostic procedures among African-Americans.