Wide-volume versus helical acquisition in unenhanced chest CT: prospective intra-patient comparison of diagnostic accuracy and radiation dose in an ultra-low-dose setting.

OBJECTIVES: Diagnostic performance and potential radiation dose reduction of wide-area detector CT sequential acquisition ("wide-volume" acquisition (WV)) in unenhanced chest examination are unknown. This study aims to assess the image quality, the diagnostic performance, and the radiation dose reduction of WV mode compared with the classical helical acquisition for lung parenchyma analysis in an ultra-low-dose (ULD) protocol. METHODS: After Institutional Review Board Approval and written informed consent, 64 patients (72% men; 67.6 ± 9.7 years old; BMI 26.1 ± 5.3 kg/m2) referred for a clinically indicated unenhanced chest CT were prospectively included. All patients underwent, in addition to a standard helical acquisition (120 kV, automatic tube current modulation), two ULD acquisitions (135 kV, fixed tube current at 10 mA): one in helical mode and one in WV mode. Image noise, subjective image quality (5-level Likert scale), and diagnostic performance for the detection of 9 predetermined parenchymal abnormalities were assessed by two radiologists and compared using the chi-square or Fisher non-parametric tests. RESULTS: Subjective image quality (4.2 ± 0.7 versus 4.2 ± 0.8, p = 0.56), image noise (41.7 ± 8 versus 40.9 ± 8.7, p = 0.3), and diagnostic performance were equivalent between ULD WV and ULD helical. Radiation dose was significantly lower for the ULD WV acquisition (mean dose-length product 14.1 ± 1.3 mGy cm versus 15.8 ± 1.3, p < 0.0001). CONCLUSION: An additional 11% dose reduction is achieved with the WV mode in ULD chest CT with fixed tube current, with equivalent image quality and diagnostic performance when compared with the helical acquisition. KEY POINTS: • Image quality and diagnostic performance of ultra-low-dose unenhanced chest CT are identical between wide-volume mode and the reference helical acquisition. • Wide-volume mode allows an additional radiation dose reduction of 11% (mean dose-length product 14.1 ± 1.3 mGy cm versus 15.8 ± 1.3, p < 0.0001).

Brain abscesses caused by Cladophialophora bantiana in a lung transplant patient: A case report and review of the literature.

Cladophialophora bantiana brain abscesses are rare, but are frequently and quickly lethal in transplanted patients. We report the case of a 63-year-old man who had undergone lung transplantation for chronic obstructive pulmonary disease and presented with headaches and a neurological deficit. Magnetic resonance imaging revealed multiple brain abscesses. C. bantiana was identified by DNA sequencing performed directly on cerebral tissue obtained by surgical biopsy. After 6 months of antifungal treatment, the brain abscesses were replaced by ischemic sequelae. The patient died suddenly 2 months later from a pulmonary bacterial infection. This is the second reported case of C. bantiana brain abscesses in a lung transplant recipient, to our knowledge, who experienced a long survival period with medical antifungal treatment alone. We review the literature and discuss our treatment.

Late gadolinium enhancement cardiac imaging on a 3T scanner with parallel RF transmission technique: prospective comparison of 3D-PSIR and 3D-IR.

OBJECTIVE: To qualitatively and quantitatively compare different late gadolinium enhancement (LGE) sequences acquired at 3T with a parallel RF transmission technique. METHODS: One hundred and sixty participants prospectively enrolled underwent a 3T cardiac MRI with 3 different LGE sequences: 3D Phase-Sensitive Inversion-Recovery (3D-PSIR) acquired 5 minutes after injection, 3D Inversion-Recovery (3D-IR) at 9 minutes and 3D-PSIR at 13 minutes. All LGE-positive patients were qualitatively evaluated both independently and blindly by two radiologists using a 4-level scale, and quantitatively assessed with measurement of contrast-to-noise ratio and LGE maximal surface. Statistical analyses were calculated under a Bayesian paradigm using MCMC methods. RESULTS: Fifty patients (70 % men, 56yo ± 19) exhibited LGE (62 % were post-ischemic, 30 % related to cardiomyopathy and 8 % post-myocarditis). Early and late 3D-PSIR were superior to 3D-IR sequences (global quality, estimated coefficient IR > early-PSIR : -2.37 CI = [-3.46 ; -1.38], prob(coef > 0) = 0 % and late-PSIR > IR : 3.12 CI = [0.62 ; 4.41], prob(coef > 0) = 100 %), LGE surface estimated coefficient IR > early-PSIR: -0.09 CI = [-1.11; -0.74], prob(coef > 0) = 0 % and late-PSIR > IR : 0.96 CI = [0.77; 1.15], prob(coef > 0) = 100 %). Probabilities for late PSIR being superior to early PSIR concerning global quality and CNR were over 90 %, regardless of the aetiological subgroup. CONCLUSIONS: In 3T cardiac MRI acquired with parallel RF transmission technique, 3D-PSIR is qualitatively and quantitatively superior to 3D-IR. KEY POINTS: • Late gadolinium enhancement is an essential part of a cardiac MRI examination • PSIR and IR sequences are the two possible options for LGE imaging • At 3T with parallel RF transmission, PSIR sequences are significantly better • One LGE sequence is sufficient, allowing an optimization of the acquisition time.

Multimodality Imaging Diagnostic Approach of Systemic-to-Pulmonary Vein Fistulae.

A 26-year-old man with a history of bilateral lung transplantation for pulmonary cystic fibrosis 6 months before was admitted in our institution for acute heart failure. Cardiac magnetic resonance imaging (CMR) showed an increased aortic output, as aortic flow assessed by velocity mapping was twofold the pulmonary flow, an occluded superior vena cava (SVC), and enlarged azygos vein. A systemic-to-pulmonary vein fistula (SAPVF) was suspected. The selective angiography showed numerous fistulae between intercostals, thyro-cervical, internal mammary arteries and pulmonary veins. The thoracic CT performed before the CMR, which was initially considered as normal, showed well these arteriovenous fistulae after 3D MIP reconstruction. This particular observation highlights the great value of multimodality imaging for the diagnosis of this rare pathology. The MR velocity mapping is a noninvasive imaging technique of great interest to guide the diagnosis of arteriovenous fistulae, and further indicating more invasive complementary imaging modalities like selective arterial angiography.

Quantification of left ventricular dyssynchrony in patients with systolic dysfunction: a comparison of circumferential strain MR-tagging metrics.

PURPOSE: To define which circumferential strain MR-tagging metrics of left intraventricular dyssynchrony better identifies patients with systolic dysfunction against control subjects. MATERIALS AND METHODS: One hundred fifty subjects were studied: (i) controls with ejection fraction (EF) > 55% (n = 84), (ii) patients with EF ≤ 55% not eligible for cardiac resynchronization therapy (CRT) (n = 52), and (iii) patients eligible for CRT according to the ESC guidelines (n = 14). Tagging cine MR-based circumferential filtered strain curves were extracted. Six dyssynchrony indices were studied: standard deviation (SD) of peak strain (SD_Ecc_ES), SD of time-to-peak (SD_TTP), strain delay index (LIM), regional variance vector (RVV), circumferential uniformity ratio estimate (CURE), and uniformity of strain TTP (US_TTP). RESULTS: All metrics show significant differences between the three groups (ANOVA, P < 10(-4) ) and are correlated with EF. Significantly higher AUC values of ROC curves between patients with normal vs. decreased EF were obtained with SD_TTP (0.998) and CURE (0.995). Agreement among different methods was fair to good (kappa 0.32 to 0.89). Interobserver variability was best for CURE (1.2%) and US_TTP (0.8%) while more than 3-times larger for other metrics. CONCLUSION: SD_TTP and CURE are the most discriminant dyssynchrony metrics for systolic dysfunction. However, taking into account the method's variability argues in favor of indices of uniformity of the strain, ie, CURE and US_TTP.

Cerebral staging of lung cancer: is one single contrast-enhanced T1-weighted three-dimensional gradient-echo sequence sufficient?

INTRODUCTION: Gadolinium-enhanced magnetic resonance imaging (MRI) is the gold standard for cerebral staging in thoracic oncology. We hypothesize that a minimalist examination, consisting of a single contrast-enhanced T1-weighted three-dimensional gradient-echo sequence (CE 3D-GRE), would be sufficient for the cerebral staging of nonsymptomatic lung cancer patients. METHODS: Seventy nonsymptomatic patients (50 % men; 62 years ± 10.2) referred for cerebral staging of a lung cancer were retrospectively included. All underwent a standard 3 T MRI examination with T1, FLAIR, T2* GRE, diffusion, and CE 3D-GRE sequences, for a total examination time of 20 min. The sole CE 3D-GRE (acquisition time: 6 min) was extracted and blindly interpreted by two radiologists in search of brain metastases. Hemorrhagic features of potential lesions and relevant incidental findings were also noted. Discrepant cases were reviewed by a third reader. The full MRI examination and follow-up studies were used as a reference to calculate sensitivity and specificity of the sole CE 3D-GRE. RESULTS: Thirty-eight point six percent (27 out of 70) of the patients had brain metastases. Performances and reader's agreement with the sole CE 3D-GRE sequence were excellent for the diagnosis of brain metastases (sensitivity=96.3 %, specificity=100 %, κ=0.91) and incidental findings (sensitivity=85.7 %, specificity=100 %, κ=0.62) but insufficient for the identification of hemorrhages within the metastases (sensitivity=33.3 %, specificity=85.7 %, κ=0.47). CONCLUSIONS: In the specific case of lung cancer, cerebral staging in nonsymptomatic patients can be efficiently achieved with a minimalistic protocol consisting of a single CE 3D-GRE sequence, completed if positive with a T2* sequence for hemorrhagic assessment, thus halving appointment delays.

Impact of 3D conformal radiotherapy on lung function of patients with lung cancer: a prospective study.

BACKGROUND: The development of three-dimensional conformal radiotherapy (3D-RT) has enabled the restriction of the dose to normal lung, limiting radiation-induced lung injury. OBJECTIVES: This study was designed to describe the time course of lung function until 7.5 months after 3D-RT in patients with lung cancer, and assess the relationship between lung function changes and dose-volume histogram (DVH) analysis or computed tomography scan changes. Radiation doses were optimized according to recent guidelines. METHODS: Sixty-five lung cancer patients treated with 3D-RT agreed to participate in this prospective, hospital-based study. Lung volumes, forced expiratory volume in 1 s (FEV1) and diffusing capacity of the lung for carbon monoxide (DLCO) were measured before radiotherapy (RT), 10 weeks, 4 and 7.5 months after the beginning of 3D-RT. RESULTS: Eleven lung cancer patients (17%) developed grade 2-3 respiratory symptoms after RT. At 7.5 months, vital capacity (VC) was 96 ± 2%, total lung capacity (TLC) 95 ± 2%, FEV1 93 ± 2% and DLCO 90 ± 2% of the initial value. Only 15% of patients showed pulmonary function reduction > 20%. Patients with FEV1 or DLCO < 60% before RT did not show significant changes after RT. There were weak correlations between reduction of VC, TLC, FEV1 or DLCO and radiation dosimetric parameters and between reduction of VC or FEV1 and radiation-induced pneumonitis images. CONCLUSIONS: In lung cancer, the reduction of lung function within 7.5 months after 3D-RT was small and correlated, albeit weakly, with DVH parameters. Patients with initially impaired lung function showed tiny changes in spirometry and DLCO values.

Disseminated Trichosporon mycotoxinivorans, Aspergillus fumigatus, and Scedosporium apiospermum coinfection after lung and liver transplantation in a cystic fibrosis patient.

Trichosporon mycotoxinivorans is a novel pathogen recently found in cystic fibrosis patients. We report the first case of a disseminated fatal infection with T. mycotoxinivorans associated with invasive Aspergillus fumigatus and Scedosporium apiospermum infection after lung and liver transplantation in a cystic fibrosis patient.

Myocardial tagging with MR imaging: overview of normal and pathologic findings.

Magnetic resonance tagging is used to evaluate the dynamic deformation of lines or grids superimposed on the myocardium during the cardiac cycle. From these data, a specific postprocessing procedure provides two kinds of metrics: (a) three orthogonal components of myocardial motion (longitudinal, circumferential, and radial), and (b) rotation and torsion. Strain expresses the local myocardial deformation and is prone to important physiologic heterogeneities. Peak systolic strain is in the range of -15% to -20% for the longitudinal and circumferential components (fiber shortening) and 30%-40% for the radial component (wall thickening). The helical arrangement of myofibers that run in opposite directions at the epicardium and endocardium explains systolic twist (~15°). This torsion may be enhanced during the early stage of several diseases (eg, hypertrophic cardiomyopathy) or in heart failure with a normal left ventricular ejection fraction. Strain is generally impaired in ischemic heart disease and cardiomyopathy, but the most diagnostically significant finding is the early identification of contractile dysfunction on the basis of longitudinal and circumferential strain reduction in patients with apparently preserved systolic function. Thus, strain impairment appears to be a sensitive and promising marker of subclinical disease, with the potential for improving patient management.

Impact of Morphotype on Image Quality and Diagnostic Performance of Ultra-Low-Dose Chest CT.

OBJECTIVES: The image quality of an Ultra-Low-Dose (ULD) chest CT depends on the patient's morphotype. We hypothesize that there is a threshold beyond which the diagnostic performance of a ULD chest CT is too degraded. This work assesses the influence of morphotype (Body Mass Index BMI, Maximum Transverse Chest Diameter MTCD and gender) on image quality and the diagnostic performance of a ULD chest CT. METHODS: A total of 170 patients from three prior prospective monocentric studies were retrospectively included. Renewal of consent was waived by our IRB. All the patients underwent two consecutive unenhanced chest CT acquisitions with a full dose (120 kV, automated tube current modulation) and a ULD (135 kV, fixed tube current at 10 mA). Image noise, subjective image quality and diagnostic performance for nine predefined lung parenchyma lesions were assessed by two independent readers, and correlations with the patient's morphotype were sought. RESULTS: The mean BMI was 26.6 ± 5.3; 20.6% of patients had a BMI > 30. There was a statistically significant negative correlation of the BMI with the image quality (ρ = -0.32; IC95% = (-0.468; -0.18)). The per-patient diagnostic performance of ULD was sensitivity, 77%; specificity, 99%; PPV, 94% and NPV, 65%. There was no statistically significant influence of the BMI, the MTCD nor the gender on the per-patient and per-lesion diagnostic performance of a ULD chest CT, apart from a significant negative correlation for the detection of emphysema. CONCLUSIONS: Despite a negative correlation between the BMI and the image quality of a ULD chest CT, we did not find a correlation between the BMI and the diagnostic performance of the examination, suggesting a possible use of the ULD protocol in obese patients.

Tacrolimus-associated posterior reversible encephalopathy syndrome after solid organ transplantation.

Tacrolimus (TAC) is an immunosuppressant drug discovered in 1984 by Fujisawa Pharmaceutical Co., Ltd. This drug belongs to the group of calcineurin inhibitors, which has been proven highly effective in preventing acute rejection after transplantation of solid organs. However, neurotoxicity and nephrotoxicity are its major adverse effects. Posterior reversible encephalopathy syndrome (PRES) is the most severe and dramatic consequence of calcineurin inhibitor neurotoxicity. It was initially described by Hinchey et al. in 1996 [N Engl J Med 1996;334:494-450]. Patients typically present with altered mental status, headache, focal neurological deficits, visual disturbances, and seizures. Magnetic resonance imaging is the most sensitive imaging test to detect this. With the more deep-going studies done recently, we have learnt more about this entity. It was noted that this syndrome is frequently reversible, rarely limited to the posterior regions of the brain, and often located in gray matter and cortex as well as in white matter. Therefore, in this review, the focus is on the current understanding of clinical recognition, pathogenesis, neuroimaging and management of TAC-associated PRES after solid organ transplantation.

Thoracic manifestations of primary humoral immunodeficiency: a comprehensive review.

Humoral immunodeficiencies, which are characterized by defective production of antibodies, are the most common types of primary immunodeficiency. Pulmonary changes are present in as many as 60% of patients with humoral immunodeficiency. Chronic changes and recurrent infections in the respiratory airways are the main causes of morbidity and mortality in those affected by a humoral immunodeficiency. Medical imaging, especially computed tomography (CT), plays a crucial role in the initial detection and characterization of changes and in monitoring the response to therapy. The spectrum of abnormalities seen at thoracic imaging includes noninfectious airway disorders, infections, chronic lung diseases, chronic inflammatory conditions (granulomatosis, interstitial pneumonias), and benign and malignant neoplasms. Recognition of characteristic CT and radiographic features, and correlation of those features with clinical and laboratory findings, are necessary to differentiate between the many possible causes of parenchymal and mediastinal disease seen in patients with primary humoral immunodeficiencies.

320-row CT transcatheter aortic valve replacement planning with a single reduced contrast media bolus injection.

OBJECTIVE: To reduce the iodine load required for CT Transcatheter Aortic Valve Replacement (TAVR) planning on a 320-row scanner by acquiring the two CT TAVR steps (ECG-gated aortic root CTA and non-gated aorto-ilio-femoral CTA) within a single contrast media bolus injection. METHODS: 50 consecutive patients (82.6±6.9 years; 56% female) were prospectively enrolled and underwent a TAVR planning using a 320-row CT, with ECG-gated aortic root CTA immediately followed by a non-gated aorto-iliac acquisition, all within a single bolus of 40-70mL of Iohexol 350mgI/mL. The Iodine load, image quality, SNR, CNR and radiation dose were compared using a Mann-Whitney test to that of 24 consecutive patients (84.3±4.8 years, 58% female) previously imaged on a 64-row scanner with a conventional two-step protocol. RESULTS: Iodine load was reduced by 44%. All examinations were of diagnostic quality, with improvement of the aortic root CTA image quality (4.9±0.3 versus 4.6±0.5, p<0.01) and a non-significant decrease of the aorto-iliac CTA image quality (4.7±0.6 versus 4.9±0.3, p = 0.07). SNR and CNR were significantly improved in the aortic root CTA (14.0±5.3 and 10.4±4.5 versus 10.3±4.2 and 6.8±3.3, p<0.01 for both) and non-significantly higher in the aorto-iliac CTA (16.5±8.0 and 14.1±7.9 versus 14.7±5.5 and 12.5±5.0, p = 0.42 and p = 0.66). Total radiation dose was reduced by 32%. CONCLUSION: 320-row CT scanner enables a 44% reduction of iodine load in TAVR planning, while maintaining excellent aorto-ilio-femoral arterial enhancement and lowering radiation dose.

Correlation between high-resolution computed tomography findings and lung function in pulmonary Langerhans cell histiocytosis.

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon interstitial lung disease which can lead to serious respiratory failure. The correlation between high-resolution computed tomography (HRCT) findings and lung function have not been studied in depth. OBJECTIVES: To assess the relationship between HRCT findings and lung function in PLCH. METHODS: Since HRCT abnormalities in PLCH consist mainly of nodular opacities and cystic abnormalities, we determined semiquantitative scores of nodular profusion and cystic extent. We therefore assessed the relationship between HRCT abnormalities on one hand and lung function and gas exchange parameters on the other, in patients with PLCH. RESULTS: In our series of 26 consecutive patients, we found no significant correlation between the score of nodular profusion and lung function or gas exchange parameters. The score of cystic extent showed a quite strong and significant correlation with FEV(1)/FVC (r = -0.62; p = 0.01), but also with PaO(2) (r = -0.69; p = 0.001) and carbon monoxide diffusing capacity (r = -0.60; p < 0.01). Furthermore, the patients with a predominant cystic pattern (n = 7) had the highest grade of dyspnea on exertion (p = 0.004), the lowest FEV(1)/FVC ratio (p = 0.02) and the lowest PaO(2) (p = 0.02) compared to patients with a predominant nodular (n = 12) or a mixed pattern (n = 7). CONCLUSIONS: We conclude that in PLCH, the cystic extent on HRCT, but not the nodular profusion, correlates significantly with lung function abnormalities and impairment of gas exchange.

Diagnostic Performance of Ultra-Low-Dose Computed Tomography for Detecting Asbestos-Related Pleuropulmonary Diseases: Prospective Study in a Screening Setting.

OBJECTIVE: To evaluate the diagnostic performance of Ultra-Low-Dose Chest CT (ULD CT) for the detection of any asbestos-related lesions (primary endpoint) and specific asbestos-related abnormalities, i.e. non-calcified and calcified pleural plaques, diffuse pleural thickening, asbestosis and significant lung nodules (secondary endpoints). MATERIAL AND METHODS: 55 male patients (55.7±8.1 years old) with occupational asbestos exposure for at least 15 years and where CT screening was indicated were prospectively included. They all underwent a standard unenhanced chest CT (120kV, automated tube current modulation), considered as the reference, and an ULD CT (135kV, 10mA), both with iterative reconstruction. Two chest radiologists independently and blindly read the examinations, following a detailed protocol. Sensitivity, specificity, positive predictive value, negative predictive value, accuracy and error rate of ULD CT were calculated using the exact method of Pearson with a confidence interval of 95%. RESULTS: Radiation dose was 17.9±1.2mGy.cm (0.25mSv) for the ULD-CT versus 288.8 ±151mGy.cm (4mSv); p <2.2e-16. Prevalence of abnormalities was 20%. The ULD CT's diagnostic performance in joint reading was high for the primary endpoint (sensitivity = 90.9%, specificity = 100%, positive predictive value = 100%, negative predictive value = 97.8%), high for lung nodules, diffuse pleural thickening and calcified pleural plaques (sensitivity, specificity, PPV and NPV = 100%) and fair for asbestosis (sensitivity = 75%, specificity = 100%, PPV = 00%, NPV = 98.1%). Intra-reader accuracy between the ULD CT and the reference CT for the primary endpoint was 98% for the senior and 100% for the junior radiologist. Inter-reader agreement for the primary endpoint was almost perfect (Cohen's Kappa of 0.81). CONCLUSION: ULD CT in the screening of asbestos exposure related diseases has 90.9% sensitivity and 100% specificity, and could therefore be proposed as a first line examination.

[Ultra-low dose chest CT: The end of chest radiograph?]. / Scanner thoracique ultra-basse dose : la mort de la radiographie thoracique ?

Ultra-low dose chest CT (ULD-CT) is acquired at a radiation dose lowered to that of a PA and lateral chest X-ray. Its image quality is degraded, yet remains diagnostic in many clinical indications. Technological improvements, with iterative reconstruction at the foreground, allowed a strong increase in the image quality obtained with this examination, which is achievable on most recent (<5 years) scanner. Established clinical indications of ULD-CT are increasing, and its non-inferiority compared to the reference "full dose" chest CT are currently demonstrated for the detection of solid nodules, for asbestos-related pleural diseases screening and for the monitoring of infectious pneumonia. Its current limitations are the obese patients (BMI>35) and the interstitial pneumonia, situations in which their performances are insufficient.

Left Ventricular Function Evaluation on a 3T MR Scanner with Parallel RF Transmission Technique: Prospective Comparison of Cine Sequences Acquired before and after Gadolinium Injection.

OBJECTIVES: To compare cine MR b-TFE sequences acquired before and after gadolinium injection, on a 3T scanner with a parallel RF transmission technique in order to potentially improve scanning time efficiency when evaluating LV function. METHODS: 25 consecutive patients scheduled for a cardiac MRI were prospectively included and had their b-TFE cine sequences acquired before and right after gadobutrol injection. Images were assessed qualitatively (overall image quality, LV edge sharpness, artifacts and LV wall motion) and quantitatively with measurement of LVEF, LV mass, and telediastolic volume and contrast-to-noise ratio (CNR) between the myocardium and the cardiac chamber. Statistical analysis was conducted using a Bayesian paradigm. RESULTS: No difference was found before or after injection for the LVEF, LV mass and telediastolic volume evaluations. Overall image quality and CNR were significantly lower after injection (estimated coefficient cine after > cine before gadolinium: -1.75 CI = [-3.78;-0.0305], prob(coef>0) = 0% and -0.23 CI = [-0.49;0.04], prob(coef>0) = 4%) respectively), but this decrease did not affect the visual assessment of LV wall motion (cine after > cine before gadolinium: -1.46 CI = [-4.72;1.13], prob(coef>0) = 15%). CONCLUSIONS: In 3T cardiac MRI acquired with parallel RF transmission technique, qualitative and quantitative assessment of LV function can reliably be performed with cine sequences acquired after gadolinium injection, despite a significant decrease in the CNR and the overall image quality.

Native T1 mapping of the heart - a pictorial review.

T1 mapping is now a clinically feasible method, providing pixel-wise quantification of the cardiac structure's T1 values. Beyond focal lesions, well depicted by late gadolinium enhancement sequences, it has become possible to discriminate diffuse myocardial alterations, previously not assessable by noninvasive means. The strength of this method includes the high reproducibility and immediate clinical applicability, even without the use of contrast media injection (native or pre-contrast T1). The two most important determinants of native T1 augmentation are (1) edema related to tissue water increase (recent infarction or inflammation) and (2) interstitial space increase related to fibrosis (infarction scar, cardiomyopathy) or to amyloidosis. Conversely, lipid (Anderson-Fabry) or iron overload diseases are responsible for T1 reduction. In this pictorial review, the main features provided by native T1 mapping are discussed and illustrated, with a special focus on the awaited clinical purpose of this unique, promising new method.

Left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy - a cardiac magnetic resonance imaging study.

BACKGROUND: Few studies evaluated left ventricular (LV) involvement in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). The aim of this study is to determine the frequency, clinical presentation, and pattern of LV involvement in ARVD/C (LV-ARVD/C). METHODS: We retrospectively evaluated the cardiac magnetic resonance (CMR) in 202 patients referred between 2008 and 2012 to our institution, and we determined the presence or the absence of CMR criteria in the revised task force criteria 2010 for the diagnosis of ARVD/C. A total of 21 patients were diagnosed with ARVD/C according to the revised task force criteria 2010. All included patients had no previous history of myocarditis, acute coronary syndrome, or any other cardiac disease that could interfere with the interpretations of structural abnormalities. The LV involvement in ARVD/C was defined by the presence of one or more of the following criteria: LV end-diastolic volume (LVEDV; >95 mL/m(2)), LV ejection fraction (LVEF; <55%), LV late enhancement of gadolinium (LVLE) in a non-ischemic pattern, and LV wall motion abnormalities (WMAs). In the follow-up for the occurrence of cardiac death, ventricular tachycardia (VT) was obtained at a mean of 31 ± 20.6 months. RESULTS: A total of 21 patients had ARVD/C. The median age was 48 (33-63) years. In all, 11 patients (52.4%) had LV-ARVD/C. The demographic characteristics of patients with or without LV were similar. There was a higher frequency of left bundle-branch block (LBBB) VT morphology in ARVD/C (P = 0.04). In CMR, regional WMAs of right ventricle (RV) and RV ejection fraction (RVEF; <45%) were strongly correlated with LV-WMAs (r = 0.72, P = 0.02, r = 0.75, P = 0.02, respectively). RV late enhancement of gadolinium (RVLE) was associated with LV-WMs and LVLE (r = 0.7, P = 0.03; r = 0.8, P = 0.006). LVLE was associated with LV-WMAs, LVEF, and LVEDV (r = 0.9, P = 0.001; r = 0.8, P = 0.001; r = 0.8, P = 0.01). CONCLUSION: LV involvement in ARVD/C is common and frequently associated with moderate to severe right ventricular (RV) abnormalities. The impact of LV involvement in ARVD/C on the prognosis needs further investigations.

Lung transplantation for advanced cystic lung disease due to nonamyloid kappa light chain deposits.

RATIONALE: Cystic lung light chain deposition disease (LCDD) is a severe and rare form of nonamyloid kappa light chain deposits localized in the lung, potentially leading to end-stage respiratory insufficiency. OBJECTIVES: To assess the outcome after lung transplantation (LT) in this setting with particular attention to disease recurrence. METHODS: We conducted a retrospective multicenter study of seven patients who underwent LT for cystic lung LCDD in France between September 1992 and June 2012 in five centers. MEASUREMENTS AND MAIN RESULTS: In total, five females and two males (mean age, 39.1 ± 5.3 yr) underwent one single LT or seven double LT (one retransplantation). Before LT, the patients showed a constant obstructive ventilatory pattern with low carbon monoxide diffusing capacity and resting hypoxemia. Lung computed tomography revealed widespread cysts with occasional micronodulations. No extrapulmonary disease or plasma cell neoplasm was detected. The serum-free kappa/lambda light chain ratio was increased in three cases. The median follow-up after LT was 56 months (range, 1-110 mo). Kaplan-Meier survival was 85.7, 85.7, and 64.3% at 1, 3, and 5 years, respectively. Three patients died from multiorgan failure (n = 1), chronic rejection (n = 1), and breast cancer (n = 1) at 23 days, 56 months, and 96 months, respectively. At the end of follow-up, no patients showed recurrence on imaging or histopathology. CONCLUSIONS: This small case series confirms that cystic lung LCDD is a severe disease limited to the lung, affecting mostly young females. LT appears to be a good therapeutic option allowing for satisfactory long-term survival. We found no evidence of recurrence of the disease after LT.