Alpha1-antitrypsin deficiency: An updated review.

Alpha1-antitrypsin deficiency (AATD) is a rare autosomal recessive disease associated with the homozygous Z variant of the SERPINA1 gene. Clinical expression of AATD, reported 60 years ago associate a severe deficiency, pulmonary emphysema and/or liver fibrosis. Pulmonary emphysema is due to the severe alpha1-antitrypsin deficiency of the ZZ homozygous status and is favored by smoking. Liver fibrosis is due to the ZZ homozygous status and is favored by obesity and excessive chronic alcohol intake, with a risk of liver cancer. Diagnosis is based on serum level and either isoelectric focusing determination of the biochemical phenotype or PCR detection of some variants. SERPINA1 gene sequencing is necessary in case of discrepancies between the results of these tests. No treatment is available for the liver disease in AATD. Although no specific trial has been performed, COPD in AATD should be treated as per COPD recommendations. Based on a randomized clinical trial, augmentation therapy is indicated in non-smoking adults less than 70 years of age with emphysema at chest CT, confirmed homozygous AATD, and FEV1 between 35% and 70% of predicted. In contrast Z heterozygosis (MZ or SZ) brings a risk of lung or liver disease only in association with further risk factors. Early detection, in all patients with COPD and chronic liver disease, is critical for the correct information of Z variant carriers. News ways of correcting the liver production of alpha1-antitrypsin will modify the care of AATD patients.

Effect of contrast material injection protocol on first-pass myocardial perfusion assessed by dual-energy dual-layer computed tomography.

Background: Dual-energy dual-layer computed tomography (CT) scanners can provide useful tools, such as iodine maps and virtual monochromatic images (VMI), for the evaluation of myocardial perfusion defects. Data about the influence of acquisition protocols and normal values are still lacking. Methods: Clinically indicated coronary CT-angiographies performed between January-October 2018 in a single university hospital with dual-energy dual-layer CT (DE-DLCT) and different injection protocols were retrospectively evaluated. The two protocols were: 35 mL in patients <80 kg and 0.5 mL/kg in patients >80 kg at 2.5 mL/s (group A) or double contrast dose at 5 mL/s (group B). Patients with coronary stenosis >50% were excluded. Regions of interest were manually drawn on 16 myocardial segments and iodine concentration was measured in mg/mL. Signal-to-noise, contrast-to-noise ratios (CNR) and image noise were measured on conventional images and VMI. Results: A total of 30 patients were included for each protocol. With iodine concentrations of 1.38±0.41 mg/mL for protocol A and 2.07±0.73 mg/mL for protocol B, the two groups were significantly different (P<0.001). No significant iodine concentration differences were found between the 16 segments (P=0.47 and P=0.09 for group A and B respectively), between basal, mid and apical segments for group A and B (P=0.28 and P=0.12 for group A and B respectively) and between wall regions for group A (P=0.06 on normalised data). In group B, iodine concentration was significantly different between three wall regions [highest values for the lateral wall, median =2.03 (1.06) mg/mL]. Post-hoc analysis showed highest contrast-to-noise and signal-to-noise in VMI at 40 eV (P<0.05). Conclusions: Iodine concentration in left ventricular myocardium of patients without significant coronary artery stenosis varied depending on the injection protocol and appeared more heterogeneous in different wall regions at faster injection rate and greater iodine load. Signal-to-noise and contrast-to-noise gradually improved when decreasing VMI energy, although at the expenses of higher noise, demonstrating the potential of DE-DLCT to enhance objective image quality.

Embolization of Recurrent Pulmonary Arteriovenous Malformations by Ethylene Vinyl Alcohol Copolymer (Onyx®) in Hereditary Hemorrhagic Telangiectasia: Safety and Efficacy.

OBJECTIVES: To evaluate short- and long-term safety and efficacy of embolization with Onyx® for recurrent pulmonary arteriovenous malformations (PAVMs) in hereditary hemorrhagic telangiectasia (HHT). METHODS: In total, 45 consecutive patients (51% women, mean (SD) age 53 (18) years) with HHT referred to a reference center for treatment of recurrent PAVM were retrospectively included from April 2014 to July 2021. Inclusion criteria included evidence of PAVM recurrence on CT or angiography, embolization using Onyx® and a minimal 1-year-follow-up CT or angiography. Success was defined based on the standard of reference criteria on unenhanced CT or pulmonary angiography if a recurrence was suspected. PAVMs were analyzed in consensus by two radiologists. The absence of safety distance, as defined by a too-short distance for coil/plug deployment, i.e., between 0.5 and 1 cm, between the proximal extremity of the primary embolic material used and a healthy upstream artery branch, was reported. RESULTS: In total, 70 PAVM were analyzed. Mean (SD) follow-up was 3 (1.3) years. Safety distance criteria were missing in 33 (47%) PAVMs. All procedures were technically successful, with a short-term occlusion rate of 100% using a mean (SD) of 0.6 (0.5) mL of Onyx®. The long-term occlusion rate was 60%. No immediate complication directly related to embolization was reported, nor was any severe long-term complication such as strokes or cerebral abscesses. CONCLUSIONS: In HHT, treatment of recurrent PAVM with Onyx® showed satisfactory safety and efficacy, with an immediate occlusion rate of 100% and a long-term rate of 60%.

Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study).

In patients with chronic fibrosing interstitial lung disease (ILD), a progressive fibrosing phenotype (PF-ILD) may develop, but information on the frequency and characteristics of this population outside clinical trials is lacking.We assessed the characteristics and outcomes of patients with PF-ILD other than idiopathic pulmonary fibrosis (IPF) in a real-world, single-centre clinical cohort. The files of all consecutive adult patients with fibrosing ILD (2010-2017) were examined retrospectively for pre-defined criteria of ≥10% fibrosis on high-resolution computed tomography and progressive disease during overlapping windows of 2 years. Baseline was defined as the date disease progression was identified. Patients receiving nintedanib or pirfenidone were censored from survival and progression analyses.In total, 1395 patients were screened; 617 had ILD other than IPF or combined pulmonary fibrosis and emphysema, and 168 had progressive fibrosing phenotypes. In 165 evaluable patients, median age was 61 years; 57% were female. Baseline mean forced vital capacity (FVC) was 74±22% predicted. Median duration of follow-up was 46.2 months. Annualised FVC decline during the first year was estimated at 136±328 mL using a linear mixed model. Overall survival was 83% at 3 years and 72% at 5 years. Using multivariate Cox regression analysis, mortality was significantly associated with relative FVC decline ≥10% in the previous 24 months (p<0.05), age ≥50 years (p<0.01) and diagnosis subgroup (p<0.01).In this cohort of patients with PF-ILD not receiving antifibrotic therapy, the disease followed a course characterised by continued decline in lung function, which predicted mortality.

Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications.

OBJECTIVES: Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke. METHODS: This retrospective study included patients with HHT-related PAVMs. CT results, i.e. PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess. All CTs were reviewed in consensus by two radiologists. RESULTS: Of 170 patients, 73 patients had unique (42.9 %), 49 multiple (28.8 %), 36 disseminated (21.2 %) and 12 diffuse (7.1 %) PAVMs. Fifteen patients presented with brain abscess; 26 patients presented with ischaemic stroke. The number of PAVMs was significantly correlated with brain abscess (11.5 vs. 6.2, respectively; p=0.025). The mean diameter of the largest feeding artery was significantly correlated with ischaemic stroke frequency (4.9 vs. 3.2 mm, respectively; p=0.0098). CONCLUSIONS: The number of PAVMs correlated significantly with risk of brain abscess, and a larger feeding artery significantly with more ischaemic strokes. These findings can lead to a better recognition and management of the PAVMs at risk of cerebral complications. KEY POINTS: • Chest CT helps clinicians to facilitate appropriate PAVM management strategies. • Pulmonary arteriovenous malformation CT findings are correlated with risk of cerebral complications. • Risk of brain abscess is significantly correlated with number of PAVMs. • Risk of ischaemic stroke is significantly correlated with large feeding artery PAVMs. • Prevalence of observed of brain abscess and ischaemic stroke is 26 %.

Comparative assessment of image quality for coronary CT angiography with iobitridol and two contrast agents with higher iodine concentrations: iopromide and iomeprol. A multicentre randomized double-blind trial.

OBJECTIVES: To demonstrate non-inferiority of iobitridol 350 for coronary CT angiography (CTA) compared to higher iodine content contrast media regarding rate of patients evaluable for the presence of coronary artery stenoses. METHODS: In this multicentre trial, 452 patients were randomized to receive iobitridol 350, iopromide 370 or iomeprol 400 and underwent coronary CTA using CT systems with 64-detector rows or more. Two core lab readers assessed 18 coronary segments per patient regarding image quality (score 0 = non diagnostic to 4 = excellent quality), vascular attenuation, signal and contrast to noise ratio (SNR, CNR). Patients were considered evaluable if no segment had a score of 0. RESULTS: Per-patient, the rate of fully evaluable CT scans was 92.1, 95.4 and 94.6 % for iobitridol, iopromide and iomeprol, respectively. Non-inferiority of iobitridol over the best comparator was demonstrated with a 95 % CI of the difference of [-8.8 to 2.1], with a pre-specified non-inferiority margin of -10 %. Although average attenuation increased with higher iodine concentrations, average SNR and CNR did not differ between groups. CONCLUSIONS: With current CT technology, iobitridol 350 mg iodine/ml is not inferior to contrast media with higher iodine concentrations in terms of image quality for coronary stenosis assessment. KEY POINTS: • Iodine concentration is an important parameter for image quality in coronary CTA. • Contrast enhancement must be balanced against the amount of iodine injected. • Iobitridol 350 is non-inferior compared to CM with higher iodine concentrations. • Higher attenuation with higher iodine concentrations, but no SNR or CNR differences.

Vein Diameter on Unenhanced Multidetector CT Predicts Reperfusion of Pulmonary Arteriovenous Malformation after Embolotherapy.

OBJECTIVE: To evaluate the value of the diameter of the draining vein of pulmonary arteriovenous malformation (PAVM) on unenhanced chest MDCT in diagnosing reperfusion after percutaneous vaso-occlusion therapy. METHODS: We retrospectively reviewed our long-term experience of patients with hereditary haemorrhagic telangiectasia and selected cases on the following criteria: an initial pulmonary angiogram with embolotherapy of at least one PAVM, a follow-up MDCT examination in the following year followed by a second pulmonary angiogram with embolotherapy if needed. Follow-up unenhanced chest MDCT examinations were analyzed blindly from results of pulmonary artery angiogram and clinical data, the diameter of the efferent vein close to the PAVM sac was measured, then compared to those of pulmonary artery angiogram as a gold standard. RESULTS: Eighty-eight of 100 patients met inclusion criteria, in whom 62 of 176 PAVMs were reperfused at angiogram. The mean diameter of the efferent vein on MDCT was 4.3 ± 2.1 mm in patent PAVMs and 1.8 ± 0.9 mm in non-patent PAVMs (p < 0.0001). The optimal cutoff diameter based on ROC analysis was 2.5 mm (sensitivity = 98.4 %; specificity = 87.7 %). CONCLUSION: A diameter of the draining vein of PAVM of 2.5 mm or greater on unenhanced MDCT is a strong predictor of reperfusion. KEY POINTS: • Diameter of draining vein of 2.5 mm or greater is associated with reperfusion. • Unenhanced chest MDCT predicts reperfusion of PAVMs with good sensitivity and specificity. • Unenhanced MDCT can guide a decision of repeat pulmonary angiogram and embolotherapy. • The mean vein diameter change of PAVMs occluded at follow-up is 3.8 mm. • Overall success rate after a median of 6 months embolotherapy was 64.7 %.

Pre-PCI angiographic TIMI flow in the culprit coronary artery influences infarct size and microvascular obstruction in STEMI patients.

OBJECTIVE: The influence of initial-thrombolysis in myocardial infarction (i-TIMI) coronary flow in the culprit coronary artery on myocardial infarct and microvascular obstruction (MVO) size is unclear. We assessed the impact on infarct size of i-TIMI flow in the culprit coronary artery, as well as on MVO incidence and size, by contrast-enhanced cardiac magnetic resonance (ce-CMR). METHODS: In a prospective, multicenter study, pre-percutaneous coronary intervention (PCI) coronary occlusion was defined by an i-TIMI flow ≤1, and patency was defined by an i-TIMI flow ≥2. Infarct size, as well as MVO presence and size, were measured on ce-CMR 72h after admission. RESULTS: A total of 140 patients presenting with ST-elevated myocardial infarction referred for primary PCI were included. There was no significant difference in final post-PCI TIMI flow between the groups (2.95±0.02 vs. 2.97±0.02, respectively; p=0.44). In the i-TIMI flow ≤1 group, infarct size was significantly larger (32±17g vs. 21±17g, respectively; p=0.002), MVO was significantly more frequent (74% vs. 53%, respectively; p=0.012), and MVO size was significantly larger [1.3 IQR (0; 7.1) vs. 0 IQR (0; 1.6)], compared to in the i-TIMI ≥2 patient group. CONCLUSION: Initial angiographic TIMI flow in the culprit coronary artery prior to any PCI predicted final infarct size and MVO size: the better was the i-TIMI flow, the smaller were the infarct and MVO size.

A novel contribution towards coherent and reproducible intravalvular measurement of the aortic annulus by multidetector computed tomography ahead of transcatheter aortic valve implantation.

BACKGROUND: As current multidetector computed tomography (MDCT) measurements underestimate the size of the aortic annulus ahead of transcatheter aortic valve implantation (TAVI), a strategy of approximate annulus area oversizing has been adopted recently. AIMS: To measure the aortic annulus using a novel complementary intravalvular MDCT slice. METHODS: Fifty-five patients with severe aortic stenosis were selected for MDCT ahead of and 1 month after CoreValve(®) TAVI. Two MDCT slices were analysed and compared: the current standard virtual basal ring (VBR) at the nadir of the aortic cusps; and a novel slice, defined as the basal (lowest) complete commissural coaptation (BCCC) plane. RESULTS: BCCC is an intravalvular plane lying 5.2±0.8 mm above the VBR. The BCCC annulus is almost circular, unlike the VBR (mean eccentricity index 0.09±0.04 vs 0.3±0.1, respectively). The mean BCCC annulus diameter was 26.6±2.3 mm, 16% larger than that of the VBR (23.9±2.2 mm; P<0.001). The BCCC annulus area proved coherent with the orifice area measured after TAVI on the projection of the same slice (i.e. systematically equal to or greater than the latter [mean difference, +2.3±1.4 mm]), in contrast to the wider scatter found for the VBR (-1.3±2.0 mm). Once the sclerotic calcified valves have been pushed back by the implant, the aortic orifice after TAVI will inevitably be equal to or less than the diameter of the virtually unvalved annulus before TAVI. CONCLUSION: Based on the present results, we recommend including a BCCC slice to complete aortic annulus sizing, in order to optimize implant calibration.

Postconditioning attenuates no-reflow in STEMI patients.

After acute myocardial infarction, the presence of no-reflow (or microvascular obstruction: MVO) has been associated with adverse left ventricular (LV) remodeling and worse clinical outcome. This study examined the effects of mechanical ischemic postconditioning on early and late MVO size in acute ST-elevation myocardial infarction (STEMI) patients. Fifty patients undergoing primary coronary angioplasty for a first STEMI with TIMI grade flow 0-1 and no collaterals were randomized to ischemic postconditioning (PC) (n = 25) or control (n = 25) groups. Ischemic PC consisted in the application of four consecutive cycles of a 1-min balloon occlusion, each followed by a 1-min deflation at the onset of reperfusion. Early (3 min post-contrast) and late (10 min post-contrast) MVO size were assessed by contrast-enhanced cardiac-MRI within 96 h after reperfusion. PC was associated with smaller early and late MVO size (3.9 ± 4.8 in PC versus 7.8 ± 6.6% of LV in controls for early MVO, P = 0.02; and 1.8 ± 3.1 in PC versus 4.1 ± 3.9% of LV in controls for late MVO; P = 0.01). This significant reduction was persistent after adjustment for thrombus aspiration, which neither had any significant effect on infarct size, nor on early or late MVO (P = NS for all). Attenuation of MVO was associated to infarct size reduction. Mechanical postconditioning significantly reduces MVO in patients with acute STEMI treated with primary angioplasty.

Acute myocardial infarction: early CT aspects of myocardial microcirculation obstruction after percutaneous coronary intervention.

OBJECTIVE: To evaluate the capabilities of delayed enhanced multidetector CT (DE-MDCT), performed immediately after percutaneous coronary intervention (PCI), in predicting myocardial microvascular obstruction (MVO) formation assessed by delayed enhanced MRI (DE-MRI). METHODS: Thirty-two patients presenting with a primary acute myocardial infarction, successfully recanalised by PCI, underwent a DE-MDCT immediately after PCI and a DE-MRI within 1 week. The left ventricle was split into 64 subsegments, rated as "healthy", "infarcted" or "MVO" on DE-MRI. Their mean density was measured on DE-MDCT and calculated relative to the patient's mean healthy myocardium density. Hypoenhanced DE-MDCT subsegments, termed "CT early MVO", were also recorded. Sensitivity and specificity of DE-MDCT for MRI-assessed "MVO" subsegments detection was calculated for mean CT relative density (threshold determined from a ROC analysis), "CT early MVO" and both. RESULTS: Mean CT relative density was higher in MRI-assessed "MVO" than in "infarcted" and "healthy" subsegments (1.82 ± 0.46, 1.43 ± 0.36 and 1.0 ± 0.13 respectively; P < 0.001) leading to a sensitivity and specificity of 94.3 % and 89.2 % for a cutoff of 1.36. Sensitivity and specificity were respectively 16.9 % and 99.8 % for "CT early MVO" and 95.3 % and 89.3 % when considering the two patterns. CONCLUSION: DE-MDCT, performed immediately after PCI, allows for an accurate prediction of MVO formation. KEY POINTS: • Myocardial microvascular obstruction (MVO) is an important prognostic sequel following myocardial infarction. • MVO can be accurately predicted by multidector CT (MDCT). • Both hypo- and hyperenhanced myocardial areas can be analysed by MDCT. • MDCT may become a useful prognostic tool for acute MI outcome.

Hypereosinophilic obliterative bronchiolitis: a distinct, unrecognised syndrome.

Biopsy-proven cases of eosinophilic bronchiolitis have only been reported in isolation, and all come from Japan. We present six patients with hypereosinophilic obliterative bronchiolitis (HOB), defined by the following criteria: 1) blood eosinophil cell count >1 G·L(-1) and/or bronchoalveolar lavage eosinophil count >25%; 2) persistent airflow obstruction despite high-dose inhaled bronchodilators and corticosteroids; and 3) eosinophilic bronchiolitis at lung biopsy (n=1) and/or direct signs of bronchiolitis (centrilobular nodules and branching opacities) on computed tomography (n=6). Chronic dyspnoea and cough which was often severe, without the characteristic features of asthma, were the main clinical manifestations. Atopy and asthma were present in the history of three and two patients, respectively. One patient met biological criteria of the lymphoid variant of idiopathic hypereosinophilic syndrome. Mean blood eosinophil cell count was 2.7 G·L(-1) and mean eosinophil differential percentage at bronchoalveolar lavage was 63%. Mean initial forced expiratory volume in 1 s/forced vital capacity ratio was 50%, normalising with oral corticosteroid therapy in all patients. HOB manifestations recurred when oral prednisone was decreased to 10-20 mg·day(-1), but higher doses controlled the disease. HOB is a characteristic entity deserving to be individualised among the eosinophilic respiratory disorders. Thorough analysis is needed to determine whether unrecognised and/or smouldering HOB may further be a cause of irreversible airflow obstruction in chronic eosinophilic respiratory diseases.

Post-conditioning reduces infarct size and edema in patients with ST-segment elevation myocardial infarction.

OBJECTIVES: This study aimed to determine whether post-conditioning at the time of percutaneous coronary intervention could reduce reperfusion-induced myocardial edema in patients with acute ST-segment elevation myocardial infarction (STEMI). BACKGROUND: Myocardial edema is a reperfusion injury with potentially severe consequences. Post-conditioning is a cardioprotective therapy that reduces infarct size after reperfusion, but no previous studies have analyzed the impact of this strategy on reperfusion-induced myocardial edema in humans. METHODS: Fifty patients with STEMI were randomly assigned to either a control or post-conditioned group. Cardiac magnetic resonance imaging was performed within 48 to 72 h after admission. Myocardial edema was measured by T2-weighted sequences, and infarct size was determined by late gadolinium enhancement sequences and creatine kinase release. RESULTS: The post-conditioned and control groups were similar with respect to ischemia time, the size of the area at risk, and the ejection fraction before percutaneous coronary intervention. As expected, post-conditioning was associated with smaller infarct size (13 ± 7 g/m(2) vs. 21 ± 14 g/m(2); p = 0.01) and creatine kinase peak serum level (median [interquartile range]: 1,695 [1,118 to 3,692] IU/l vs. 3,505 [2,307 to 4,929] IU/l; p = 0.003). At reperfusion, the extent of myocardial edema was significantly reduced in the post-conditioned group as compared with the control group (23 ± 16 g/m(2) vs. 34 ± 18 g/m(2); p = 0.03); the relative increase in T2W signal intensity was also significantly lower (p = 0.02). This protective effect was confirmed after adjustment for the size of the area at risk. CONCLUSIONS: This randomized study demonstrated that post-conditioning reduced infarct size and edema in patients with reperfused STEMI.

T2-weighted cardiac MR assessment of the myocardial area-at-risk and salvage area in acute reperfused myocardial infarction: comparison of state-of-the-art dark blood and bright blood T2-weighted sequences.

PURPOSE: To compare different state-of-the-art T2-weighted (T2w) imaging sequences combined with late gadolinium enhancement (LGE) for myocardial salvage area (MSA) assessment by cardiac magnetic resonance (CMR). T2w imaging has been used to assess the myocardial area at risk (AAR) in acute myocardial infarction (AMI) patients, but its clinical application is challenging due to technical and physical limitations. MATERIALS AND METHODS: Thirty patients with reperfused AMI underwent complete CMR imaging 2-5 days after hospital admission. Myocardial AAR and MSA were quantified on four different T2w sequences: (a) free-breathing T2-prepared single-shot balanced steady-state free precession (T2p_ssbSSFP); (b) breathhold T2-weighted acquisition for cardiac unified T2 edema (ACUTE); (c) breathhold T2w dark-blood inversion recovery turbo-spin echo (IR-TSE) (short-term inversion recovery: STIR); and (d) free-breathing high-resolution T2 dark-blood navigated BLADE. The diagnostic performance of each technique was also assessed. RESULTS: Quantitative analysis showed significant differences in myocardial AAR extent as quantified by the four T2w sequences (P < 0.05). There were also significant differences in sensitivity, specificity and overall diagnostic performance. CONCLUSION: Detection and quantification of AAR, and thus of MSA, by T2wCMR in reperfused AMI patients varied significantly between different T2w sequences in the same clinical setting.

Determination of the myocardial area at risk with pre- versus post-reperfusion imaging techniques in the pig model.

The purpose of this study was to compare the accuracy of post-reperfusion cardiac magnetic resonance (CMR) and pre-reperfusion multidetector computed tomography (MDCT) imaging to measure the size of the area at risk (AAR), using pathology as a reference technique in a porcine acute myocardial infarction model. Fifteen pigs underwent balloon-induced coronary artery occlusion for 40 min followed by reperfusion. The AAR was assessed with arterial enhanced MDCT performed during occlusion, while two different T2 weighted (T2W) CMR imaging sequences and the contrast-enhanced (ce-) CMR endocardial surface length (ESL) were performed after 90 min of reperfusion. Animals were euthanized and the AAR was assessed by pathology. Additional measurements of the myocardial water content in the AAR, remote and the AAR border zones were performed. AAR by pathology best correlated with measurements made by MDCT (R(2) = 0.88; p < 0.001) with little bias on Bland-Altman plots (bias 2.5%, SD 6.1% LV area). AAR measurements obtained by T2W STIR, T2W ACUTE sequences or the ESL on ce-CMR showed a fair correlation with pathology (R(2) = 0.72, R(2) = 0.65 and R(2) = 0.69, respectively; all p ≤ 0.001), but significantly overestimated the size of the AAR with important bias (17.4 ± 10.8% LV area; 11.7 ± 11.0% LV area; 13.0 ± 10.3% LV area, respectively). The myocardial water content in the AAR border zones was significantly higher than the remote (82.8 vs. 78.8%; p < 0.001). Our data suggest that post-reperfusion imaging methods overestimated the AAR likely due to the presence of edema outside of the boundaries of the AAR. Pre-reperfusion arterial enhanced MDCT showed the greatest accuracy for the assessment of the AAR.

Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease.

OBJECTIVE: Connective tissue diseases (CTDs) are associated with several interstitial lung diseases. The aim of this study was to describe the recently individualized syndrome of combined pulmonary fibrosis and emphysema (CPFE) in a population of patients with CTD. METHODS: In this multicenter study, we retrospectively investigated data from patients with CTD who also have CPFE. The demographic characteristics of the patients, the results of pulmonary function testing, high-resolution computed tomography, lung biopsy, and treatment, and the outcomes of the patients were analyzed. RESULTS: Data from 34 patients with CTD who were followed up for a mean±SD duration of 8.3±7.0 years were analyzed. Eighteen of the patients had rheumatoid arthritis (RA), 10 had systemic sclerosis (SSc), 4 had mixed or overlap CTD, and 2 had other CTDs. The mean±SD age of the patients was 57±11 years, 23 were men, and 30 were current or former smokers. High-resolution computed tomography revealed emphysema of the upper lung zones and pulmonary fibrosis of the lower zones in all patients, and all patients exhibited dyspnea during exercise. Moderately impaired pulmonary function test results and markedly reduced carbon monoxide transfer capacity were observed. Five patients with SSc exhibited pulmonary hypertension. Four patients died during followup. Patients with CTD and CPFE were significantly younger than an historical control group of patients with idiopathic CPFE and more frequently were female. In addition, patients with CTD and CPFE had higher lung volumes, lower diffusion capacity, higher pulmonary pressures, and more frequently were male than those with CTD and lung fibrosis without emphysema. CONCLUSION: CPFE warrants inclusion as a novel, distinct pulmonary manifestation within the spectrum of CTD-associated lung diseases in smokers or former smokers, especially in patients with RA or SSc.