Stargardt's pigmentosa: A novel combination of two inherited retinal dystrophies.

60-year-old woman referring visual disability. She presented bone spicule pigmentation and retinal atrophy in all peripheral retina, as well as macular retinal flecks. Multimodal imaging showed typical findings of both inherited retinal dystrophies (IRD). Electroretinogram confirmed rod dysfunction. Biallelic mutations were found in ABCA4 and CNGA1 genes. Although not common, different IRDs may be present in a same patient at the same time. This is the first reported case of the combination of RP with late-onset Stargardt's disease. We propose the name 'Stargardt's pigmentosa' for this novel clinical entity.

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment.

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as "the scimitar sign", is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.

Riding with the ants.

Isolates of Teratosphaeriaceae have frequently been found in the integument of attine ants, proving to be common and diverse in this microenvironment. The LSU phylogeny of the ant-isolated strains studied revealed that they cluster in two main lineages. The first was associated with the genus Xenopenidiella whereas the other represented two ant-isolated lineages sister to the taxa Penidiella aggregata and P. drakensbergensis, which are allocated to the new genus Penidiellomyces. The genus Penidiella is limited to the lineage containing P. columbiana, which is not congeneric with Penidiellomyces or Penidiellopsis, nor with Simplicidiella, a novel genus introduced here to accommodate a strain isolated from ants. For species level analysis, the final 26 aligned sequences of the ITS (498 characters), cmdA (389 characters), tef1 (342 characters) and tub2 (446 characters) gene regions lead to the introduction of six new species in Xenopenidiella, and one in respectively Penidiellopsis and Simplicidiella. The species described in this study were distinguished by the combination of morphological and phylogenetic data. Novelties on the integument of leaf-cutting ants from Brazil include: Penidiellopsis ramosus, Xenopenidiella clavata, X. formica, X. inflata, X. laevigata, X. nigrescens, X. tarda spp. nov., and Simplicidiella nigra gen. & sp. nov. Beta-tubulin is recommended as primary barcode for the distinction of species in Penidiellopsis, whereas ITS was sufficient to distinguish species of Xenopenidiella.

[Complicated transcatheter aortic-valve endocarditis with abscess and pseudoaneurysm: Value of the ECG-gated multidetector computed tomography angiography]. / Endocardite infectieuse sur TAVI compliqué d'abcès et pseudo-anévrysme : apport du scanner cardiaque synchronisé à l'ECG.

A 80-year-old man was admitted to catheterization room for an acute infero-lateral ST-elevation myocardial infarction (STEMI). Coronary angiography showed a thrombotic occlusion of the second left marginal branch, and normal other coronary arteries. The thrombo-embolic mechanism of the STEMI, and the infectious context in this patient who had had a transcatheter aortic valve implantation (TAVI) two months earlier, led us to suspect a bioprosthesis endocarditis. It was confirmed by transthoracic and transoesophageal echocardiography, which showed an aortic-mitral curtain abscess and aortic bioprosthesis vegetations, associated to Enterococcus faecalis bacteriemia. In order to specify the diagnosis, an ECG-gated multidetector CT angiography (MDCTA) had been performed. Additionally to echocardiographic findings, MDCTA showed a pseudo-aneurysm, sized 20 to 22mm, beginning from the outflow tract of the left ventricle to end on the antero-lateral face of the aorta. The patient was referred for emergency aortic bioprosthesis removal and replacement. Through this case, MDCTA showed its importance for the diagnosis and the prognostic evaluation of cardiac prosthesis endocarditis. MDCTA provided additional informations that echocardiography could not detect, because of artifacts caused by the prosthetic material and calcifications, frequent in elderly patients with comorbidities.

Assessment of global longitudinal strain at low-dose anthracycline-based chemotherapy for the prediction of subsequent cardiotoxicity.

OBJECTIVES: The aim of this study was to assess whether global longitudinal strain (GLS) measured early during treatment with anthracycline (at a cumulative dose of 150mg/m2) can predict subsequent alterations in left ventricular ejection fraction (LVEF). METHODS AND RESULTS: Eighty-six patients suffering from Hodgkin's disease, non-Hodgkin's lymphoma or acute leukemia and receiving anthracyclines were prospectively included. They underwent complete echocardiography on four separate occasions: baseline (V1); after reaching a cumulative dose of 150mg/m2 (V2); end of treatment (V3); one year follow-up (V4). Six patients developed cardiotoxicity defined by a decrease in LVEF by more than 10 percentage points to a value of at least less than 53% at V4. Both GLS measured at V1 and at V2 were significantly lower in the cardiotoxicity group compared with the control group (P=0.042 and P=0.01, respectively). Compared to GLS at V1, GLS obtained at V2 provided implemental predictive information and appeared to be the strongest predictor of cardiotoxicity (area under the receiver operating characteristic curve, 0.823). At a threshold of -17.45% for GLS measured at V2, the sensitivity and specificity of detecting cardiotoxicity were 67% (95%CI: [33-100%]) and 97% (95%CI: [94-100%]) respectively. CONCLUSION: GLS>-17.45%, obtained after 150mg/m2 of anthracycline therapy, is a significant predictor of future anthracycline-induced cardiotoxicity. This study should encourage physicians to perform echocardiography earlier during treatment with anthracyclines.

[Evolution of the interventional reperfusion strategy and reperfusion times in acute ST-segment elevation myocardial infarction]. / Évolution de la prise en charge interventionnelle et des délais de reperfusion à la phase aiguë de l'infarctus du myocarde avec sus-décalage du segment ST.

BACKGROUND: In patients with acute ST-segment elevation myocardial infarction (STEMI) treated with primary percutaneous coronary intervention (pPCI), the recommended times (first medical contact-to-balloon (M2B) <120 or <90min, and door-to-balloon (D2B) <45min) are reached in less than 50% of patients. PURPOSE: To compare the interventional reperfusion strategy and reperfusion times between two series of consecutive STEMI patients referred for pPCI within 12hours of symptom onset, in 2007 and 2012. METHODS: Retrospective study of 182 patients, 87 admitted from January 2007 to March 2008 (period 1), and 95 admitted from January to December 2012 (period 2). The procedural characteristics and the different times between onset of pain and mechanical reperfusion were gathered and compared by non-parametric tests. RESULTS: Radial access, thromboaspiration, and drug eluting stents were more frequent, and cardiogenic shock was less common during period 2, compared with the period 1. The median time from first medical contact to balloon (M2B) decreased by 26% (135min, [quartiles: 113-183] in 2007 versus 100 [76-137] in 2012, P<0.001), in relation to the reduction in both prehospital times and time in the catheterization laboratory (D2B: 51 [44-65] and 44min [37-55], respectively, P<0.01). CONCLUSIONS: The D2B and M2B times significantly decreased in our centre between 2007 and 2012, and reached the recommended values in >60% of the cases. This may be explained by better coordination between emergency medical units and interventional cardiologists, and by the presence of two paramedics in the catheterization laboratory for 24/24 7/7 pPCI since 2010 in France, in accordance with recent national regulation.

[Coronary stent evaluation with cardiac CT: Literature review]. / Évaluation des stents coronaires en scanner cardiaque: revue de la littérature.

Since the introduction of the 64-generation scanners, the accuracy and robustness of the diagnosis of coronary artery disease has progressed. The main advantage of cardiac CT is the exclusion of coronary artery disease by its excellent negative predictive value. Currently, cardiac CT applications extend thanks to innovations both in terms of technological development systems scanner or stents implanted. This is a literature review of stent evaluation with cardiac CT.

Establishment of diagnostic reference levels in cardiac CT in France: a need for patient dose optimisation.

The objective of this study was to propose diagnostic reference levels (DRLs) for coronary computed tomography angiography (CCTA), in the context of a large variability in patient radiation dose, and the lack of European recommendations. Volume Computed Tomography Dose Index (CTDIvol) and dose-length product (DLP) were collected from 460 CCTAs performed over a 3-month period at eight French hospitals. CCTAs (∼50 per centre) were performed using the routine protocols of the centres, and 64- to 320-detector CT scanners. ECG gating was prospective (n = 199) or retrospective (n = 261). The large gap in dose between these two modes required to propose specific DRLs: 26 and 44 mGy for CTDIvol, and 370 and 970 mGy cm for DLP, respectively. This study confirms the large variability in patient doses during CCTA and underlines the need for the optimisation of cardiac acquisition protocols. Availability of national DRLs should be mandatory in this setting.

[Usefulness of transesophageal echocardiography to optimize treatment after ischemic stroke]. / Intérêt de l'échocardiographie trans-œsophagienne dans l'optimisation de la stratégie thérapeutique après un accident vasculaire cérébral ischémique.

AIM OF THE STUDY: In the setting of ischemic stroke, the place of transesophageal echocardiography (TEE) is still matter of debate. The aim of the study is to evaluate the therapeutic impact provided by TEE and to characterize patients in whom TEE is warranted. PATIENTS AND METHOD: Three hundred and fifty-nine consecutive patients were included in the study. "Decisive TEE" (DTEE) was defined by echographic findings resulting in a change of treatment, whereas "informative TEE" (ITEE) was defined by TEE revealing a potential cardiac or aortic source of embolism. RESULTS: Three hundred and forty-one patients underwent TEE. Twenty-eight patients (8.2%) had DTEE and 184 (53.9%) had ITEE. DTEE were as follows: thrombus in the left atrial appendage in 6 patients, complex aortic plaques in 10 patients, patent foramen ovale (PFO) associated with atrial septal aneurism (ASA) and an important right to left shunt (3 patients), FOP associated with ASA and lower limb phlebitis (1 patient), 4 cases of endocarditis and 4 patients with intense spontaneous echo contrast in the left atrium. In most cases of DTEE (67.8%), the patient was given anticoagulation drugs. Left atrial dilatation (P=0.005) and multivessel territory stroke (P=0.018) were statistically predictive of DTEE. CONCLUSIONS: In the setting of ischemic stroke, TEE provides important additional informations, but modifies therapeutic strategy in less than 10% of cases. Multivessel territory stroke, and left atrial dilatation were predictive of DTEE.

Assessment of coronary bypass graft patency by first-line multi-detector computed tomography.

The purpose of the study was to assess whether a strategy based on a MDCT performed routinely before CA can reduce the radiation dose during the CA, without increased global exposure in patients who need imaging of CABG. A total of 147 consecutive patients were included. The radiation dose during CA (KAP 12.1 vs 22.0 Gy/cm(2), P<.01) and the volume of iodinated contrast (155 vs 200 mL, P<.02) were reduced when preceded by a MDCT. Patients' cumulative exposures were not different in the 2 strategies (5.0 vs 5.1 mSv, P=.76). MDCT performed in first line is a valuable strategy for the assessment of CABG.

[Cardiac CT: new applications]. / Scanner cardiaque : nouvelles applications.

Since the introduction of the 64-generation scanners, the accuracy and robustness of the diagnosis of coronary artery disease has progressed. The main advantage of cardiac CT is the exclusion of coronary artery disease by its excellent negative predictive value. Currently, cardiac CT applications extend thanks to innovations both in terms of technological development systems scanner or stents implanted, that the evolution of surgical procedures such as TAVI.

Leaf-cutting ants: an unexpected microenvironment holding human opportunistic black fungi.

Fungus-growing ants of the genus Atta are known for their leaf-cutting habit, a lifestyle they have maintained since their 50-million-year-old co-evolution with a mutualistic fungus, cultivated as food. Recent studies have highlighted that, in addition to the mutualistic fungus, nests of ants harbor a great diversity of microbial communities. Such microorganisms include the dematiaceous fungi, which are characterized by their melanized cell walls. In order to contribute to the knowledge of fungal ecology, as well as opportunistic strains that may be dispersed by these social insects, we isolated and identified fungi carried by gynes of Atta capiguara and Atta laevigata, collected from colonies located in Fazenda Santana, Botucatu (São Paulo, Brazil). The isolation was carried out using the oil flotation technique, which is suitable for the growth of black fungi. Inoculated plates were incubated at 25 and 35 °C until black cultures were visible (20-45 days). Isolates were identified based on microscopic and molecular characteristics. Some isolated genera were: Cladophialophora, Cladosporium, Exophiala, Ochroconis, Phaeococcomyces, Phialophora and Penidiella. Hyaline species were also found. The results obtained from this work showed that leaf-cutting gynes may contribute to the dispersal of opportunistic dematiaceous fungi. It is suggested that more attention should be paid to this still unexplored subject.

[Undifferentiated sarcoma: usefulness of multimodality cardiac imaging in characterizing a rare intracardiac mass]. / Sarcome cardiaque indifférencié : intérêt de l'imagerie multimodale dans la caractérisation d'une masse intracardiaque.

We report the case of a man presenting with a Pierre Marie-Bamberger syndrome. This paraneoplastic syndrome revealed an undifferentiated intracardiac sarcoma. This case emphasizes the need for multimodality imaging to characterize intracardiac tumor.

[Cardiac magnetic resonance with a MRI compatible pacemaker]. / IRM cardiaque avec un stimulateur IRM compatible.

Patients with cardiac implantable electronic devices are usually excluded from MRI examinations due to contraindication for MRI. The MRI-conditional pacemaker system may allow the benefits of MRI (system 1.5T) to be more accessible to pacemaker patients. A 62-year-old man was admitted with acute coronary syndrome and atrial fibrillation. A conventional angiography showed normal coronaries. A cardiac cardioversion revealed a significant sinus node dysfunction and a magnetic resonance imaging (MRI) compatible dual chamber system was implanted. At 6-week follow-up, a cardiac MRI revealed a typical anterior myocardial infarction with diagnostic quality images despite pacemaker. This is one of the first reports of cardiovascular MRI in a patient with MRI-conditional pacing system.

[Cardiac MRI: technology, clinical applications, and future directions]. / IRM cardiaque : technologie actuelle, applications cliniques et perspectives futures.

The field of cardiovascular MRI has evolved rapidly over the past decade, feeding new applications across a broad spectrum of clinical and research areas. Advances in magnet hardware technology, and key developments such as segmented k-space acquisitions, advanced motion encoding techniques, ultra-rapid perfusion imaging and delayed myocardial enhancement imaging have all contributed to a revolution in how patients with ischemic and non-ischemic heart disease are diagnosed and treated. Actually, cardiac MRI is a widely accepted method as the "gold standard" for detection and characterization of many forms of cardiac diseases. The aim of this review is to present an overview of cardiac MRI technology, advances in clinical applications, and future directions.

Comparison of various methods for quantitative evaluation of myocardial infarct volume from magnetic resonance delayed enhancement data.

BACKGROUND: Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) enables the estimation of myocardial infarct (MI) extent. Nevertheless, manual quantification is time consuming and subjective. We sought to assess MI volume with different quantitative methods in both acute (AMI) and chronic MI (CMI). METHODS: CMR was performed 50 ± 21 h after MI in 52 patients and was repeated 100 ± 21 days later in a subgroup of 34 patients. Then, necrosis volumes were quantified using: 1) manual delineation, 2) automated fuzzy c-means method, and 3) +2 to 6 SD thresholding approaches. Results were compared against peak values of serum Troponin I (TnI), creatine kinase (CK) and left ventricular (LV) functional parameters: LV ejection fraction (LVEF), indexed end-diastolic (EDVi), end-systolic volumes (ESVi) and the number of hypokinetic segments (NbHk). RESULTS: For CMI, quantitative evaluation of infarct size using manual, +2SD, +3 SD and fuzzy c-means provided equivalent results in terms of correlation coefficients for comparisons of MI volumes against LV function parameters (LVEF: r>0.79, p<0.0001; ESVi: r>0.82, p<0.0001, EDVi: r>0.67, p<0.0001, NbHk: r>0.54, p<0.0009). For AMI, +2SD and fuzzy c-means approaches provided higher correlations for comparisons of AMI volumes against biochemical markers (CK: r>0.79, p<0.0001,TnI: r>0.77, p<0.0001) and chronic LV function parameters (LVEF: r>0.82, p<0.0001, NbHk: r>0.59, p<0.0002). CONCLUSIONS: The fuzzy c-means and 2SD methods provided highest correlations with biochemical MI quantification as well as LV function parameters. The fuzzy c-means approach which does not require an arbitrary identification of the remote myocardium is fast and reproducible. It may be clinically useful in the evaluation of patients with MI.

[High-altitude retinopathy]. / Retinopatía de gran altura.

CASE REPORT: This case report presents a 36 year-old male with a sudden loss of vision while taking part in an expedition in the Daulaghiri (8,167 metres high peak located in the Himalayan Mountain Range). DISCUSSION: High altitude retinal haemorrhage is a common condition in those mountaineers who reach altitudes over 5,500m. Depending on its location it may not present any symptoms and the condition improves with no further complications. However, in case of macular involvement the vision decreases dramatically and the consequences are uncertain.