What You See Is Not What You Get: Frontal Sinus Metastasis From the Renal Cell Carcinoma.

Metastatic neoplasms occurring in the nasal and paranasal sinuses are infrequent occurrences. In this study, we present one such case of a 61-year-old male patient with a known clear cell renal carcinoma presenting to us with signs and symptoms of acute sinusitis. The patient subsequently underwent CT and MRI examinations, which revealed a neoplastic mass in the right frontal sinus, which was surgically resected and was later confirmed histologically as a metastatic deposit from clear cell renal carcinoma. The patient is currently being treated with chemotherapy and radiotherapy and is doing well.

Effect of Obstructive Sleep Apnea and Positive Airway Pressure Therapy on Cardiac Remodeling as Assessed by Cardiac Biomarker and Magnetic Resonance Imaging in Nonobese and Obese Adults.

It is unknown whether obesity modifies the effect of obstructive sleep apnea (OSA) and positive airway pressure (PAP) therapy on cardiac remodeling and NT-proBNP (N-terminal pro-B-type natriuretic peptide) levels. We compared NT-proBNP and cardiac magnetic resonance imaging in adults without OSA (n=56) and nonobese (n=73; body mass index <30 kg/m2) and obese (n=136; body mass index ≥30 kg/m2) adults with OSA. We also investigated these traits in nonobese (n=45) and obese (n=78) participants with OSA adherent to 4 months of PAP treatment. At baseline, left ventricular mass to end-diastolic volume ratio, a measure of left ventricular concentricity, was greater in both nonobese and obese participants with OSA compared with those without OSA. Participants with OSA and obesity exhibited reduced phasic right atrial function. No significant differences in baseline NT-proBNP were observed across groups. The effect of PAP treatment on NT-proBNP and left atrial volume index was significantly modified by obesity. In nonobese participants, PAP therapy was associated with a decrease in NT-proBNP (P<0.0001) without a change in left atrial volume index, whereas in obese participants, PAP was associated with an increase in left atrial volume index (P=0.006) without a change in NT-proBNP. OSA was associated with left ventricular concentric remodeling independent of obesity and right atrial dysfunction in participants who were obese. PAP treatment was associated with reduced NT-proBNP in nonobese participants with OSA, but left atrial enlargement in obese participants with OSA, suggesting that PAP-induced reduction in BNP release (which is known to occur during obstructive apnea episodes) may lead to volume retention in obese participants with OSA. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT01578031.

Improved brain perfusion after electrical cardioversion of atrial fibrillation.

AIMS: Atrial fibrillation (AF) has been associated with reduced brain volume, cognitive impairment, and reduced cerebral blood flow. The causes of reduced cerebral blood flow in AF are unknown, but no reduction was seen in individuals without the arrhythmia in a previous study. The aim of this study was to test the hypothesis that brain perfusion, measured with magnetic resonance imaging (MRI), improves after cardioversion of AF to sinus rhythm (SR). METHODS AND RESULTS: All patients undergoing elective cardioversion at our institution were invited to participate. A total of 44 individuals were included. Magnetic resonance imaging studies were done before and after cardioversion with both brain perfusion and cerebral blood flow measurements. However, 17 did not complete the second MRI as they had a recurrence of AF during the observation period (recurrent AF group), leaving 17 in the SR group and 10 in the AF group to complete both measurements. Brain perfusion increased after cardioversion to SR by 4.9 mL/100 g/min in the whole brain (P < 0.001) and by 5.6 mL/100 g/min in grey matter (P < 0.001). Cerebral blood flow increased by 58.6 mL/min (P < 0.05). Both brain perfusion and cerebral blood flow remained unchanged when cardioversion was unsuccessful. CONCLUSION: In this study of individuals undergoing elective cardioversion for AF, restoration, and maintenance of SR for at least 10 weeks after was associated with an improvement of brain perfusion and cerebral blood flow measured by both arterial spin labelling and phase contrast MRI. In those individuals where cardioversion was unsuccessful, there was no change in perfusion or blood flow.

[MINOCA in Iceland. Acute coronary syndrome in patients with normal or nonobstructive coronary arteries].

INTRODUCTION: The classical pathophysiological process underlying acute coronary syndromes has been considered to be plaque rup-ture followed by platelet activation and aggregation and subsequent thrombus formation leading to myocardial ischemia and infarction. A substantial number of patients with acute coronary syndromes appear to have normal or near normal (<50% stenosis) coronary arteries on angiography. Recently, this clinical entity has been coined MINOCA (Myocardial Infarction with Non-Obstructive Coronary Arteries). The purpose of this paper is to describe the proportion of MINOCA among ACS patients in Iceland. MATERIAL AND METHODS: We performed a retrospective analysis of all admissions for acute coronary syndromes at Landspitali University Hospital, the single coronary catheterization facility in Iceland, during a five year period between 2012 and 2016. All patients admitted for STEMI or NSTEMI that turned out to have normal or near normal coronary arteries were consecutively included in the study. For each patient the diagnosis was re-evaluated according to further assessments using a diagnostic algorithm specially constructed for this study. RESULTS: During the five year study period 1708 patients were studied with coronary angiography during first hospitalization for STEMI or NSTEMI. Among these, 225 (13.2%) had normal or non-obstructive coronary arteries with less than 50% luminal narrowing. The final diagnosis of these patients were plaque erosion / rupture in 72 indi-viduals (32%), myocarditis in 33 (14.7%), takotsubo cardiomyopathy in 28 (12.4%), type II myocardial infarction in 30 (13.3%), vasospastic angina in 31 (13.8%) and other or undetermined cause in 31 (13.8%) patients. CONCLUSION: The proportion of MINOCA in Iceland is 13.2% of patients admitted for acute coronary syndromes. Plaque erosion / rupture was considered a likely cause in one third of patients with other causes beeing evenly distributed with approximately half that frequency. Identification of the underlying cause of MINOCA would become more accurate with a consistent use of cardiac magnetic resonance imaging in these patients as it provided a definitive diagnosis in all of those -studied.

Atrial fibrillation is associated with decreased total cerebral blood flow and brain perfusion.

Aims: Atrial fibrillation (AF) has been associated with cognitive impairment. Additionally, brain volume may be reduced in individuals with AF. Potential causes may include cerebral micro-embolism or reduced stroke volume due to the beat-to-beat variation in AF. The aims of this study were to measure cerebral blood flow and estimate whole brain perfusion in elderly individuals with and without AF. Methods and results: Blood flow in the cervical arteries was measured with phase contrast MRI and brain perfusion estimated in a large cohort from the AGES-Reykjavik Study. Individuals were divided into three groups at the time of the MRI: persistent AF, paroxysmal AF, and no history of AF. Of 2291 participants (mean age 79.5 years), 117 had persistent AF and 78 had paroxysmal AF but were in sinus rhythm at the time of imaging AF. Those with persistent AF had lower cholesterol and used more anti-hypertensive medication and warfarin. The three groups were similar with regard to other cardiovascular risk factors. Those in the persistent AF group had significantly lower total cerebral blood flow on average, 472.1 mL/min, both when compared with the paroxysmal AF group, 512.3 mL/min (P < 0.05) and the no AF group, 541.0 mL/min (P < 0.001). Brain perfusion was lowest in the persistent AF group, 46.4 mL/100 g/min compared with the paroxysmal AF group, 50.9 mL/100 g/min in (P < 0.05) and those with no AF, 52.8 mL/100 g/min (P < 0.001). Conclusion: Persistent AF decreases blood flow to the brain as well as perfusion of brain tissue compared with sinus rhythm.

Fabry Disease in Families With Hypertrophic Cardiomyopathy: Clinical Manifestations in the Classic and Later-Onset Phenotypes.

BACKGROUND: The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA(α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B). METHODS AND RESULTS: Familial screening of at-risk relatives identified mutations in 16 family A members (8 men and 8 heterozygotes) and 25 family B members (10 men and 15 heterozygotes). Clinical assessments, α-galactosidase A (α-GalA) activities, glycosphingolipid substrate levels, and in vitro mutation expression were used to categorize p.D322E as a classic FD mutation and p.I232T as a later-onset FD mutation. In vitro expression revealed that p.D322E and p.I232T had α-GalA activities of 1.4% and 14.9% of the mean wild-type activity, respectively. Family A men had markedly decreased α-GalA activity and childhood-onset classic manifestations, except for angiokeratoma and cornea verticillata. Family B men had residual α-GalA activity and developed FD manifestations in adulthood. Despite these differences, all family A and family B men >30 years of age had left ventricular hypertrophy, which was mainly asymmetrical, and had similar late gadolinium enhancement patterns. Ischemic stroke and severe white matter lesions were more frequent among family A men, but neither family A nor family B men had overt renal disease. Family A and family B heterozygotes had less severe or no clinical manifestations. CONCLUSIONS: Men with classic or later-onset FD caused by GLA missense mutations developed prominent and similar cardiovascular disease at similar ages, despite markedly different α-GalA activities.

Secondary sclerosing cholangitis in patients with drug-induced liver injury.

BACKGROUND: Secondary sclerosing cholangitis has clinical features similar to primary sclerosing cholangitis but originates from a known pathological entity. Secondary sclerosing cholangitis has not been investigated in patients with drug-induced liver injury. METHODS: Overall 102 patients diagnosed with drug-induced liver injury were identified and magnetic resonance cholangiopancreatography images of 25 patients were reviewed. RESULTS: Ten patients (all females) out of 102 had confirmed features of secondary sclerosing cholangitis on biliary imaging. Overall 70% of patients with sclerosing cholangitis had jaundice vs. 25% without sclerosing cholangitis (p<0.01). All sclerosing cholangitis patients had cholestatic/mixed type of liver injury and compared with patients with cholestatic/mixed liver injury without confirmed abnormal MRCP (n=52), they also had more frequently jaundice, 70% vs. 23% (p=0.0065), higher peak alkaline phosphatase 551 (352-716) vs. 329 (202-543) (p=0.055) and longer time to resolution of liver injury 152 days (123-353) vs. 62 days (36-91) than patients without confirmed sclerosing cholangitis (p<0.0009). CONCLUSIONS: Our results indicate that drugs can lead to bile duct injury visualized on imaging. This should be a part of the differential diagnoses of secondary sclerosing cholangitis. These patients were more likely to present with jaundice and longer recovery of liver injury than other patients with cholestatic/mixed type of drug-induced liver injury.

Cardiac myxoma in Iceland: a case series with an estimation of population incidence.

Cardiac myxoma (CM) is the most common primary benign tumor of the heart, but the true age-standardized incidence rate (ASR) has remained unknown. We therefore used nationwide registries in Iceland to study CM and establish its incidence rate. This was a retrospective study involving all patients diagnosed with CM in Iceland between 1986 and 2010. Cases were identified through three different registries, and hospital charts and histology results reviewed. An ASR was estimated based on a world standard population (w). Nine cases of CM (six women) were identified with a mean age of 62.8 years (range: 37-85), giving an ASR of 0.11 (95% CI: 0.05-0.22) per 100,000. The mean tumor size was 4.4 cm (range: 1.5-8.0) with all the tumors located in the left atrium. Dyspnea (n = 6) and ischemic stroke (n = 2) were the most common symptoms. All patients underwent complete resection of the tumor and there were no postoperative deaths or CM-related deaths at follow-up (mean 85 months). The ASR of CM in Iceland was 0.11 per 100,000. To our knowledge, this is the first study to determine the incidence of CM in an entire population. In Iceland, the presenting symptoms and mode of detection of CM are similar to those in other series.

Dynamic contrast-enhanced MR imaging for differentiation of rounded atelectasis from neoplasm.

PURPOSE: To characterize rounded atelectasis (RA) with dynamic contrast-enhanced MRI in the differential diagnosis of solitary peripheral pulmonary neoplasm. MATERIALS AND METHODS: Twenty-four patients with diagnostically equivocal peripheral pulmonary nodules were examined with dynamic contrast-enhanced MRI. 13 patients had a total of 16 rounded atelectases and 11 had a neoplasm. The final diagnosis was made either by histology (n = 14) or follow-up examinations of at least 24 months (n = 10). The peripheral nodules were evaluated concerning their morphology and contrast-enhancement dynamics. Curves for signal intensity (SI) versus time were produced and the relative increase in SI, slope of SI during wash-in, and slope of SI during wash-out calculated. Additionally, SI time curves were evaluated using a two compartment model where the ratio for the SI of the fast and the slow component were calculated. Mean values from different tissues of interest were compared by an unpaired two-sided t-test. RESULTS: Analysis of the SI-time curves of the RAs revealed a curve shape similar to the pulmonary artery, but a magnitude in SI between artery and normal lung tissue. Linear curve fit showed a significantly steeper slope during wash-in and wash-out, and higher relative signal increase in atelectases as compared to neoplasms. Results from the two compartment model showed increased flow and a high ratio of the slow to the fast components with a long mean transit time in neoplasms. CONCLUSION: Three parameters, slope of SI during wash-in and wash-out, and the slow/fast ratio can be used as diagnostic tools for discrimination of RA and neoplasm.

[Apical ballooning syndrome, case series]. / Broddthensluheilkenni--sjúkratilfelli og yfirlit.

Apical ballooning syndrome is a cardiac syndrome typically characterized by transient focal dyskinesia or akinesia of the mid and apical regions of the left ventricle and hyperkinesia of the basal region. The symptoms and signs of the patient mimic myocardial infarction, with chest pain, electrocardiographic changes and elevation of cardiac enzymes but without significant coronary artery disease. The syndrome is frequently preceded by physical or emotional stress. We describe three cases of apical ballooning syndrome diagnosed during 10 days in December 2007 at Landspítali University Hospital Reykjavík.