Increased retinal vascular tortuosity appears to be prevalent in adults with cyanotic congenital heart disease and is likely to be in response to hypoxemia and erythrocytosis. This conclusion is consistent with normalization of the retinal vascularity patterns after surgical relief of cyanosis resulting in resolution of hypoxemia and erythrocytosis.

Summary:Purpose: To correlate glucose (and lactate) results obtained from microdialysate to recent studies suggesting that glucose transporter activity may be significantly altered in seizures.Methods: We used a fluorometric technique to quantify glucose and lactate levels in microdialysates collected from two to four depth electrodes implanted per patient in the temporal and frontal lobes of a series of four patients. Hour-by-hour and day-to-day changes in brain glucose and lactate levels at the same site were recorded. Additionally we compared regional variations in lactate/glucose ratios around the predicted epileptogenic region.Results: Lactate/glucose ratios in the range of 1-2:1 were the most commonly seen. When the lactate/glucose ratio was <1:1, we typically observed a relative increase in local glucose concentration (rather than decreased lactate), suggesting increased transport, perhaps without increased glycolysis. In some sites, lactate/glucose ratios of 3:1-15:1 were seen, suggesting that a circumscribed zone of inhibition of tricarboxylic acid cycle activity may have been locally induced. In these dialysates, collected from probes closer to the epileptogenic region, the large increase in lactate/glucose ratios was a result of both increased lactate and reduced glucose levels.Conclusions: We conclude that regional variations in brain extracellular glucose concentrations may be of greater magnitude than previously believed and become even more accentuated in partial seizure patients. Data from concomitant assays of microdialysate lactate and glucose may aid in understanding cerebral metabolism.

Echocardiography is considered as an initial imaging modality of choice in patients with congenital heart disease (CHD), and magnetic resonance (MR) imaging is preferred for detailed functional information. Multi-detector computed tomography (CT) plays an important role in clinical practice in assessing post-operative morphological and functional information of patients with complex CHD when echocardiography and MR imaging are not contributory. Radiologists should understand and become familiar with the complex morphology and physiology of CHD, as well as with various palliative and corrective surgical procedures performed in these patients, to obtain CT angiograms with diagnostic quality and promptly recognise imaging features of normal post-operative anatomy and complications of these complex surgeries.

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Background There is significant interindividual variability in the rate of aortic stenosis (AS) progression that is not accounted for in the current surveillance algorithms. We sought to examine the association between changes in peak aortic jet velocity (Vmax) and mean gradient (MG) among patients with mild or moderate AS and risk of progression to severe disease. Methods Adult subjects referred for echocardiography at a single academic referral center with a diagnosis of mild or moderate AS and ≥2 additional surveillance echocardiograms were included in the study. Changes in Vmax and MG between the first two echocardiograms were indexed to time and tested for association with future progression to severe AS. Results Among three hundred and sixty-four subjects, the median time between first and second echocardiograms was 1.3 years and initial changes in Vmax and MG indexed to time were +0.16 m/s per year and +1.44 mmHg per year, respectively. Fifty-three (15%) and fifty-six (15%) subjects progressed to severe AS defined by Vmax and MG, respectively. In multivariable logistic regression, initial increase in Vmax (OR = 4.19, 95% CI 1.93–9.10, p < 0.001) and initial increase in MG (OR = 1.12, 95% CI 1.06–1.18, p < 0.001) were associated with progression to severe AS. Conclusions Initial changes in Vmax and MG among patients with mild or moderate AS are strongly associated with risk of progression to severe AS and may help guide individualized surveillance strategies.

Takotsubo cardiomyopathy (TC) is associated with significant short-term morbidity and mortality. Several risk factors for poor outcomes have been identified; however, the prognostic implications of pre-existing comorbidity in TC are poorly delineated. We sought to assess the association of aggregate pre-existing comorbidity with short-term outcomes in TC. We performed a retrospective observational study of adult subjects diagnosed with TC at two academic tertiary care hospitals between 2005 and 2018. Overall burden of medical comorbidity was estimated using the Charlson comorbidity index (CCI). Multivariable logistic regression was used to test for independent association of CCI with 30-day mortality and severe shock at index presentation. Multivariable poisson regression was performed to assess the association of CCI with duration of hospitalization. Five-hundred and thirty-eight subjects were diagnosed with TC during the study period. The median CCI score of all subjects was 2 (IQR 1-4). Among subjects with physical triggers of TC, the median CCI score was 2 (IQR 1-4) compared to a median CCI score of 1 (IQR 0-1) in subjects with non-physical triggers of TC (P < 0.001). Seventy-six (14%) subjects died within 30 days of index diagnosis and 185 (34%) subjects experienced severe shock. The median duration of hospitalization was 7 days (IQR 3-14 days). In multivariable logistic regression, CCI was not associated with 30-day mortality or severe shock. In multivariable Poisson regression, CCI (IRR 1.17, 95% CI 1.16-1.18, P < 0.001) was associated with duration of hospitalization. Increased burden of pre-existing medical comorbidity was not independently associated with 30-day mortality or severe shock at index presentation, but was associated with increased duration of hospitalization after diagnosis of TC.

Anti -N-methyl-d-aspartate (NMDA) T he clinical entity of anti-N-methyl-d-aspartate (NMDA) receptor encephalitis was first described in 2007.1 Although more than 600 cases of anti-NMDA receptor encephalitis have been reported since that time, the entity remains so underrecognized that prolonged or repeated hospitalizations are often required before a definitive diagnosis can be reached.2 This malady is a rare, immunotherapyresponsive panencephalitis that typically presents with psychiatric manifestations and movement disorders, which can be fatal. Most patients are young women with ovarian teratomas, although there are reports of affected children and men who have no identifiable tumors. Rarely, ictal asystole occurs among epileptic patients. Case series suggest that anti-NMDA receptor encephalitis patients, in general, are at increased risk of cardiac dysrhythmia. After treatment of the encephalitis, dysrhythmias usually resolve and permanent pacing is not required. Case ReportIn December 2012, a 19-year-old woman with a history of migraine headaches presented at our emergency department (ED) after an apparent seizure. She stated that her last memory before her suspected seizure was of solving an extended problem on a classroom blackboard while preparing for final examinations. Her next memory was of awakening in the ED. While in the ED, she experienced a witnessed, generalized tonic-clonic seizure and was given intravenous lorazepam and phenytoin. An electrocardiogram was notable only for mild QT prolongation and sinus rhythm (Fig. 1). The results of routine laboratory examinations and magnetic resonance angiography of the brain were not helpful. Two weeks before her ED visit, the patient had reported constant headaches, accompanied by a burning sensation in her head that went down her nose and into her chest, lasting an hour at a time. These episodes had recurred a few times per week. She also had reported facial and hand tingling during these same episodes. After her discharge from the ED and her outpatient follow-up with neurology, she reported difficulty thinking, emotional lability (laughing, crying, and thoughts of impending doom), memory problems, and the intermittent hearing of music. Upon leaving the neurology clinic, she was prescribed levetiracetam and was scheduled for outpatient electroencephalography (EEG), in an effort to evaluate her seizure activity.Two days after her outpatient neurology visit, she underwent an EEG that revealed status epilepticus, originating from the right mid-temporal region. She was then admitted to the hospital for evaluation of those seizures. She was afebrile on presentation, and her vital signs fell within normal limits. On neurologic examination, she was alert, oriented, and able to follow commands. She was laughing inappropriately during the Case Reports