Cardiac Magnetic Resonance as an Etiological Diagnosis Tool in Recovered Sudden Cardiac Death or Unstable Ventricular Arrhythmia Patients. / Ressonância Magnética Cardíaca como Ferramenta Diagnóstica Etiológica em Pacientes Recuperados de Morte Súbita Cardíaca ou Arritmias Ventriculares Instáveis.

BACKGROUND: Cardiac magnetic resonance (CMR) has an increasing diagnostic relevance in survivors of sudden cardiac death (SCD) or unstable ventricular arrhythmia (UVA) in developed countries. OBJECTIVE: To evaluate retrospectively the additional role of CMR in a developing country where few resources are available, and should be used more effectively. METHODS: The study included SCD or UVA survivors admitted between 2009 and 2019 at a tertiary academic institution referred to CMR. Demographic, clinical, and laboratory data were collected from the medical records. CMR images and reports were reviewed and their impact on the final etiological diagnosis was determined. A descriptive analysis was performed and p<0.05 established as significant. RESULTS: Sixty-four patients, 54.9±15.4 years old, and 42 (71.9%) males. Most events (81.3%) were out of the hospital and ventricular tachycardia was the most common rhythm. Cardiovascular medications were previously used by 55 patients, and beta-blockers were the most used medications (37.5%). Electrocardiogram had electrical inactive areas in 21.9% and all of them had fibrosis at CMR. Mean left ventricular ejection fraction (LVEF) was 44±14%, with 60.9% ≤50% and only 29.7% ≤35%. Late gadolinium enhancement was identified in 71.9%, with a transmural pattern in 43.8%. Chagas cardiomyopathy was the most common etiology (28.1%), followed by ischemic cardiomyopathy (17.2%). Among 26 without a previously identified etiology, CMR could define it (15 patients - 57%). CONCLUSION: In accordance with previous studies in developed countries, CMR was capable of increasing etiological diagnosis and identifying the arrhythmogenic substrate, allowing better care in half of the underdiagnosed patients.

FUNDAMENTO: A ressonância magnética cardíaca (RMC) tem relevância diagnóstica crescente em sobreviventes de morte súbita cardíaca (MSC) ou arritmia ventricular instável (AVI) em países desenvolvidos. OBJETIVO: Procuramos avaliar retrospectivamente o papel adicional da RMC em um país em desenvolvimento com poucos recursos disponíveis e que pode direcionar um uso mais eficaz desses recursos. MÉTODOS: Foram incluídos sobreviventes de MSC ou AVI admitidos entre 2009 e 2019 em uma instituição acadêmica terciária após a realização de RMC. Dados demográficos, clínicos e laboratoriais foram coletados dos prontuários. Imagens e laudos de RMC foram analisados e o impacto disso no diagnóstico etiológico final foi afirmado. Realizou-se análise descritiva e definiu-se p<0,05 como significativo. RESULTADOS: Sessenta e quatro pacientes, 54,9±15,4 anos, sendo 42 (71,9%) do sexo masculino. A maioria dos eventos (81,3%) foi extra-hospitalar e a taquicardia ventricular foi o ritmo mais comum. Medicamentos cardiovasculares foram utilizados anteriormente por 55 pacientes, sendo os betabloqueadores os medicamentos mais utilizados (37,5%). O eletrocardiograma apresentava áreas elétricas inativas em 21,9% e todos apresentavam fibrose na RMC. A média da fração de ejeção do ventrículo esquerdo (FEVE) foi de 44±14%, com 60,9% ≤50% e apenas 29,7% ≤35%. Identificou-se realce tardio com gadolínio em 71,9%, com padrão transmural em 43,8%. A miocardiopatia chagásica foi a etiologia mais comum (28,1%), seguida da miocardiopatia isquêmica (17,2%). Entre 26 sem etiologia previamente identificada, foi possível definir com RMC (15 pacientes - 57%). CONCLUSÃO: De acordo com estudos anteriores em países desenvolvidos, a RMC foi capaz de aumentar o diagnóstico etiológico e identificar o substrato arritmogênico, permitindo melhor atendimento em metade dos pacientes subdiagnosticados.

Ressonância Magnética Cardíaca como Ferramenta Diagnóstica Etiológica em Pacientes Recuperados de Morte Súbita Cardíaca ou Arritmias Ventriculares Instáveis / Cardiac Magnetic Resonance as an Etiological Diagnosis Tool in Recovered Sudden Cardiac Death or Unstable Ventricular Arrhythmia Patients

Resumo Fundamento A ressonância magnética cardíaca (RMC) tem relevância diagnóstica crescente em sobreviventes de morte súbita cardíaca (MSC) ou arritmia ventricular instável (AVI) em países desenvolvidos. Objetivo Procuramos avaliar retrospectivamente o papel adicional da RMC em um país em desenvolvimento com poucos recursos disponíveis e que pode direcionar um uso mais eficaz desses recursos. Métodos Foram incluídos sobreviventes de MSC ou AVI admitidos entre 2009 e 2019 em uma instituição acadêmica terciária após a realização de RMC. Dados demográficos, clínicos e laboratoriais foram coletados dos prontuários. Imagens e laudos de RMC foram analisados e o impacto disso no diagnóstico etiológico final foi afirmado. Realizou-se análise descritiva e definiu-se p<0,05 como significativo. Resultados Sessenta e quatro pacientes, 54,9±15,4 anos, sendo 42 (71,9%) do sexo masculino. A maioria dos eventos (81,3%) foi extra-hospitalar e a taquicardia ventricular foi o ritmo mais comum. Medicamentos cardiovasculares foram utilizados anteriormente por 55 pacientes, sendo os betabloqueadores os medicamentos mais utilizados (37,5%). O eletrocardiograma apresentava áreas elétricas inativas em 21,9% e todos apresentavam fibrose na RMC. A média da fração de ejeção do ventrículo esquerdo (FEVE) foi de 44±14%, com 60,9% ≤50% e apenas 29,7% ≤35%. Identificou-se realce tardio com gadolínio em 71,9%, com padrão transmural em 43,8%. A miocardiopatia chagásica foi a etiologia mais comum (28,1%), seguida da miocardiopatia isquêmica (17,2%). Entre 26 sem etiologia previamente identificada, foi possível definir com RMC (15 pacientes - 57%). Conclusão De acordo com estudos anteriores em países desenvolvidos, a RMC foi capaz de aumentar o diagnóstico etiológico e identificar o substrato arritmogênico, permitindo melhor atendimento em metade dos pacientes subdiagnosticados.

Abstract Background Cardiac magnetic resonance (CMR) has an increasing diagnostic relevance in survivors of sudden cardiac death (SCD) or unstable ventricular arrhythmia (UVA) in developed countries. Objective To evaluate retrospectively the additional role of CMR in a developing country where few resources are available, and should be used more effectively. Methods The study included SCD or UVA survivors admitted between 2009 and 2019 at a tertiary academic institution referred to CMR. Demographic, clinical, and laboratory data were collected from the medical records. CMR images and reports were reviewed and their impact on the final etiological diagnosis was determined. A descriptive analysis was performed and p<0.05 established as significant. Results Sixty-four patients, 54.9±15.4 years old, and 42 (71.9%) males. Most events (81.3%) were out of the hospital and ventricular tachycardia was the most common rhythm. Cardiovascular medications were previously used by 55 patients, and beta-blockers were the most used medications (37.5%). Electrocardiogram had electrical inactive areas in 21.9% and all of them had fibrosis at CMR. Mean left ventricular ejection fraction (LVEF) was 44±14%, with 60.9% ≤50% and only 29.7% ≤35%. Late gadolinium enhancement was identified in 71.9%, with a transmural pattern in 43.8%. Chagas cardiomyopathy was the most common etiology (28.1%), followed by ischemic cardiomyopathy (17.2%). Among 26 without a previously identified etiology, CMR could define it (15 patients - 57%). Conclusion In accordance with previous studies in developed countries, CMR was capable of increasing etiological diagnosis and identifying the arrhythmogenic substrate, allowing better care in half of the underdiagnosed patients.

Induced sputum abnormalities in gas station attendants.

PURPOSE: To investigate inflammatory changes in the induced sputum (IS) of gas station attendants (GSAs) at risk of exposure to fuel vapors through inhalation and susceptible to respiratory complaints and impaired lung function. METHODS: Hypertonic saline-IS was collected from 52 GSAs who had never smoked (42 men, age = 35.9 ± 8.9 years) and had no known comorbidities. A group of 22 non-smokers (11 men, age = 30.5 ± 5.1 years) selected from the University staff served as control. The GSAs answered a questionnaire and underwent spirometry and chest tomography. A total of 15 inflammatory biomarkers associated with inflammation, including cytokines, chemokines, and mediators of immunological response, were also measured. RESULTS: The most common symptoms of GSAs were coughing (42.3%) and dyspnea (59.6%) based on the New York Heart Association (NYHA; Class II) classification. Significant elevations (p < 0.05) in forced vital capacity and forced expiratory volume within the first second were observed in the GSAs relative to those in the controls (GSA vs. control: 99 ± 12% vs. 90 ± 9% and 94 ± 11% vs. 87 ± 10%, respectively). The GSAs had a lower percentage of IS lymphocytes than that in the control group (4.5 ± 5.7% vs. 7.7 ± 9.8%). The GSAs also had significantly lower concentrations of IL-4, IL-5, IL-10, IL-12P70, IFN-γ, and MIP-1α, but IL-3 levels were higher. No differences were observed in the airway thickness and the amount of emphysema between the GSAs and the controls. CONCLUSION: Despite normal lung function and absence of abnormalities on HRCT, GSAs have a higher frequency of respiratory complaints, with evidence of impairment of lymphocytic activity in the airways.

Circulating Plasma miRNA and Clinical/Hemodynamic Characteristics Provide Additional Predictive Information About Acute Pulmonary Thromboembolism, Chronic Thromboembolic Pulmonary Hypertension and Idiopathic Pulmonary Hypertension.

Idiopathic pulmonary artery hypertension (IPAH), chronic thromboembolic pulmonary hypertension (CTEPH), and acute pulmonary embolism (APTE) are life-threatening cardiopulmonary diseases without specific surgical or medical treatment. Although APTE, CTEPH and IPAH are different pulmonary vascular diseases in terms of clinical presentation, prevalence, pathophysiology and prognosis, the identification of their circulating microRNA (miRNAs) might help in recognizing differences in their outcome evolution and clinical forms. The aim of this study was to describe the APTE, CTEPH, and IPAH-associated miRNAs and to predict their target genes. The target genes of the key differentially expressed miRNAs were analyzed, and functional enrichment analyses were carried out. The miRNAs were detected using RT-PCR. Finally, we incorporated plasma circulating miRNAs in baseline and clinical characteristics of the patients to detect differences between APTE and CTEPH in time of evolution, and differences between CTEPH and IPAH in diseases form. We found five top circulating plasma miRNAs in common with APTE, CTEPH and IPAH assembled in one conglomerate. Among them, miR-let-7i-5p expression was upregulated in APTE and IPAH, while miRNA-320a was upregulated in CTEP and IPAH. The network construction for target genes showed 11 genes regulated by let-7i-5p and 20 genes regulated by miR-320a, all of them regulators of pulmonary arterial adventitial fibroblasts, pulmonary artery endothelial cell, and pulmonary artery smooth muscle cells. AR (androgen receptor), a target gene of hsa-let-7i-5p and has-miR-320a, was enriched in pathways in cancer, whereas PRKCA (Protein Kinase C Alpha), also a target gene of hsa-let-7i-5p and has-miR-320a, was enriched in KEGG pathways, such as pathways in cancer, glioma, and PI3K-Akt signaling pathway. We inferred that CTEPH might be the consequence of abnormal remodeling in APTE, while unbalance between the hyperproliferative and apoptosis-resistant phenotype of pulmonary arterial adventitial fibroblasts, pulmonary artery endothelial cell and pulmonary artery smooth muscle cells in pulmonary artery confer differences in IPAH and CTEPH diseases form. We concluded that the incorporation of plasma circulating let-7i-5p and miRNA-320a in baseline and clinical characteristics of the patients reinforces differences between APTE and CTEPH in outcome evolution, as well as differences between CTEPH and IPAH in diseases form.

Pulmonary Vascular Volume Estimated by Automated Software is a Mortality Predictor after Acute Pulmonary Embolism. / Volume Vascular Pulmonar Estimado por Software Automatizado é um Preditor de Mortalidade após Embolia Pulmonar Aguda.

BACKGROUND: Acute pulmonary embolism (APE) has a variable clinical outcome. Computed tomography pulmonary angiography (CTPA) is the gold standard for this diagnosis. OBJECTIVE: To evaluate if the pulmonary vascular volume (PVV) quantified by automated software is a mortality predictor after APE. METHODS: Retrospective cohort study where the CTPA imaging of 61 patients with APE was reanalyzed. Pulmonary vascular volume (PVV) and pulmonary volume (PV) were automatically estimated using the Yacta software. We calculated the adjusted PVV by the ratio: PVV(cm3)/PV(liters). Classical prognostic CTPA parameters (clot load index, right ventricle/left ventricle diameter ratio, pulmonary artery/aorta diameter ratio, ventricular septal bowing, pulmonary infarction and reflux of contrast into the hepatic vein) were assessed. The outcome assessed was one-month mortality. We considered a p-value <0.05 as statistically significant. RESULTS: Seven deaths (11%) occurred at one month among these 61 patients. PVV<23cm3/L was an independent predictor of one-month mortality in the univariate [odds ratio (OR): 26; 95% confidence interval (CI): 3-244; p=0.004] and multivariate analyses [adjusted OR: 19; 95%CI: 1.3-270; p=0.03]. The classical CTPA parameters were not associated with one-month mortality in this sample. The PVV<23cm3/L showed a sensitivity of 86%, a specificity of 82%, a negative predictive value of 94% and a positive predictive value of 64% to identify the patients who died. CONCLUSION: PVV<23cm3/L was an independent predictor of one-month mortality after APE. This parameter showed better prognostic performance than other classical CTPA findings. (Arq Bras Cardiol. 2020; 115(5):809-818).

FUNDAMENTO: A embolia pulmonar aguda (EPA) tem desfecho clínico variável. A angiotomografia computadorizada (angio-CT) é considerada o padrão-ouro para o diagnóstico. OBJETIVO: Avaliar se o volume vascular pulmonar (VVP) quantificado por software automatizado é um preditor de mortalidade após EPA. MÉTODOS: Estudo de coorte retrospectivo no qual a imagem da angio-CT de 61 pacientes com EPA foi reanalisada. O VVP e o volume pulmonar (VP) foram estimados automaticamente pelo software Yacta. Calculamos o VVP ajustado pela razão: VVP(cm3)/VP(litros). Parâmetros prognósticos clássicos da angio-CT (carga embólica; razão do diâmetro do ventrículo direito/ventrículo esquerdo; razão do diâmetro da artéria pulmonar/aorta; desvio do septo interventricular; infarto pulmonar e refluxo de contraste na veia hepática) foram avaliados. A mortalidade em 1 mês foi o desfecho analisado. Consideramos um valor de p <0,05 como estatisticamente significativo. RESULTADOS: Sete mortes (11%) ocorreram entre os 61 pacientes durante 1 mês de seguimento. O VVP ajustado <23cm3/L foi um preditor independente de mortalidade na análise univariada (odds ratio [OR]: 26; intervalo de confiança de 95% [IC95%]: 3-244; p=0,004) e na análise multivariada (OR ajustado: 19 [IC95%: 1,3-270]; p=0,03). Os parâmetros clássicos da angio-CT não foram associados à mortalidade em 1 mês nesta amostra. O VVP ajustado <23cm3/L apresentou sensibilidade de 86%, especificidade de 82%, valor preditivo negativo de 94% e valor preditivo positivo de 64% para identificação dos pacientes que morreram. CONCLUSÃO: VVP ajustado <23cm3/L foi um preditor independente de mortalidade após EPA. Esse parâmetro mostrou melhor desempenho prognóstico do que os outros achados clássicos da angio-CT. (Arq Bras Cardiol. 2020; 115(5):809-818).

Volume Vascular Pulmonar Estimado por Software Automatizado é um Preditor de Mortalidade após Embolia Pulmonar Aguda / Pulmonary Vascular Volume Estimated by Automated Software is a Mortality Predictor after Acute Pulmonary Embolism

Resumo Fundamento: A embolia pulmonar aguda (EPA) tem desfecho clínico variável. A angiotomografia computadorizada (angio-CT) é considerada o padrão-ouro para o diagnóstico. Objetivo: Avaliar se o volume vascular pulmonar (VVP) quantificado por software automatizado é um preditor de mortalidade após EPA. Métodos: Estudo de coorte retrospectivo no qual a imagem da angio-CT de 61 pacientes com EPA foi reanalisada. O VVP e o volume pulmonar (VP) foram estimados automaticamente pelo software Yacta. Calculamos o VVP ajustado pela razão: VVP(cm3)/VP(litros). Parâmetros prognósticos clássicos da angio-CT (carga embólica; razão do diâmetro do ventrículo direito/ventrículo esquerdo; razão do diâmetro da artéria pulmonar/aorta; desvio do septo interventricular; infarto pulmonar e refluxo de contraste na veia hepática) foram avaliados. A mortalidade em 1 mês foi o desfecho analisado. Consideramos um valor de p <0,05 como estatisticamente significativo. Resultados: Sete mortes (11%) ocorreram entre os 61 pacientes durante 1 mês de seguimento. O VVP ajustado <23cm3/L foi um preditor independente de mortalidade na análise univariada (odds ratio [OR]: 26; intervalo de confiança de 95% [IC95%]: 3-244; p=0,004) e na análise multivariada (OR ajustado: 19 [IC95%: 1,3-270]; p=0,03). Os parâmetros clássicos da angio-CT não foram associados à mortalidade em 1 mês nesta amostra. O VVP ajustado <23cm3/L apresentou sensibilidade de 86%, especificidade de 82%, valor preditivo negativo de 94% e valor preditivo positivo de 64% para identificação dos pacientes que morreram. Conclusão: VVP ajustado <23cm3/L foi um preditor independente de mortalidade após EPA. Esse parâmetro mostrou melhor desempenho prognóstico do que os outros achados clássicos da angio-CT. (Arq Bras Cardiol. 2020; 115(5):809-818)

Abstract Background: Acute pulmonary embolism (APE) has a variable clinical outcome. Computed tomography pulmonary angiography (CTPA) is the gold standard for this diagnosis. Objective: To evaluate if the pulmonary vascular volume (PVV) quantified by automated software is a mortality predictor after APE. Methods: Retrospective cohort study where the CTPA imaging of 61 patients with APE was reanalyzed. Pulmonary vascular volume (PVV) and pulmonary volume (PV) were automatically estimated using the Yacta software. We calculated the adjusted PVV by the ratio: PVV(cm3)/PV(liters). Classical prognostic CTPA parameters (clot load index, right ventricle/left ventricle diameter ratio, pulmonary artery/aorta diameter ratio, ventricular septal bowing, pulmonary infarction and reflux of contrast into the hepatic vein) were assessed. The outcome assessed was one-month mortality. We considered a p-value <0.05 as statistically significant. Results: Seven deaths (11%) occurred at one month among these 61 patients. PVV<23cm3/L was an independent predictor of one-month mortality in the univariate [odds ratio (OR): 26; 95% confidence interval (CI): 3-244; p=0.004] and multivariate analyses [adjusted OR: 19; 95%CI: 1.3-270; p=0.03]. The classical CTPA parameters were not associated with one-month mortality in this sample. The PVV<23cm3/L showed a sensitivity of 86%, a specificity of 82%, a negative predictive value of 94% and a positive predictive value of 64% to identify the patients who died. Conclusion: PVV<23cm3/L was an independent predictor of one-month mortality after APE. This parameter showed better prognostic performance than other classical CTPA findings. (Arq Bras Cardiol. 2020; 115(5):809-818)

Familial pulmonary fibrosis: a heterogeneous spectrum of presentations.

OBJECTIVE: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. METHODS: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. RESULTS: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. CONCLUSIONS: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.

Acute Effects of Oscillatory PEP and Thoracic Compression on Secretion Removal and Impedance of the Respiratory System in Non-Cystic Fibrosis Bronchiectasis.

BACKGROUND: Bronchiectasis is characterized by abnormal and permanent dilatation of the bronchi, caused mainly by the progression of inflammatory processes and loss of the ability to remove mucus. Techniques to clear the airways are essential for the treatment of these patients. In this study, we aimed to evaluate the acute effects of oscillatory PEP and thoracic compression on both the clearance of secretions and impedance of airways in subjects with bronchiectasis. METHODS: This was a randomized crossover single-blinded study that involved both subjects with bronchiectasis and healthy subjects evaluated by using an impulse oscillometry system, which assessed resistance at 5 Hz and resistance 20 Hz, reactance at 5 Hz, reactance area, and resonant frequency, before, after, and 30 min after oscillatory PEP, chest compression, or control sessions. Dry and total weights, adhesiveness, purulence of the expectorated secretions, the dyspnea scale score, the acceptability and tolerance scale score, pulse oximetry, and difficulty in expectoration were also assessed. RESULTS: The dry and total weights of secretions were higher after the use of the oscillatory PEP technique than those in a control session (P = .005 and P = .039, respectively). In the bronchiectasis group, there was a decrease after oscillatory PEP in total airway resistance (P = .04), peripheral resistance (P = .005), and reactance area (P = .001). After compression, there was a decrease in peripheral resistance Hz (P = .001) and reactance area (P = .001). In the healthy group, there was an increase in resistance at 5 Hz (P = .02) after oscillatory PEP. There were no differences in acceptability and tolerance, dyspnea, and oxygen saturation. CONCLUSIONS: The oscillatory PEP technique was effective for the removal of secretions and in decreasing total and peripheral respiratory system resistance; thoracic compression had comparable positive effects on the peripheral resistance. Both techniques were safe and well tolerated by the subjects with bronchiectasis. ClinicalTrials.gov registration NCT02509637.).

Artificial intelligence, machine learning, computer-aided diagnosis, and radiomics: advances in imaging towards to precision medicine.

The discipline of radiology and diagnostic imaging has evolved greatly in recent years. We have observed an exponential increase in the number of exams performed, subspecialization of medical fields, and increases in accuracy of the various imaging methods, making it a challenge for the radiologist to "know everything about all exams and regions". In addition, imaging exams are no longer only qualitative and diagnostic, providing now quantitative information on disease severity, as well as identifying biomarkers of prognosis and treatment response. In view of this, computer-aided diagnosis systems have been developed with the objective of complementing diagnostic imaging and helping the therapeutic decision-making process. With the advent of artificial intelligence, "big data", and machine learning, we are moving toward the rapid expansion of the use of these tools in daily life of physicians, making each patient unique, as well as leading radiology toward the concept of multidisciplinary approach and precision medicine. In this article, we will present the main aspects of the computational tools currently available for analysis of images and the principles of such analysis, together with the main terms and concepts involved, as well as examining the impact that the development of artificial intelligence has had on radiology and diagnostic imaging.

Familial pulmonary fibrosis: a heterogeneous spectrum of presentations / Fibrose pulmonar familiar: um espectro heterogêneo de apresentações

ABSTRACT Objective: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. Methods: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. Results: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. Conclusions: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.

RESUMO Objetivo: Descrever as características clínicas, funcionais e radiológicas de um grupo de casos índice diagnosticados com fibrose pulmonar familiar (FPF) no Brasil. Métodos: Trinta e cinco pacientes com FPF (18 mulheres; 51,4%), com mediana de idade de 66,0 anos (variação: 35,5-89,3 anos), responderam a um questionário padronizado e foram submetidos a testes de função pulmonar e TCAR de tórax. Tecido pulmonar foi obtido para revisão em 6 casos: a partir de biópsias cirúrgicas em 5 e de autópsia em 1. Resultados: Antecedentes de tabagismo e de exposição a aves ou mofo foram referidos por 45,7% e 80,0% dos casos, respectivamente. Tosse e dispneia significante foram referidas por 62,8% e 48,6% dos pacientes, respectivamente. Estertores finos foram detectados em 91,4% dos indivíduos. Em 4 pacientes, os achados levantaram suspeitas de doença dos telômeros. As medianas da CVF e da DLCO foram, respectivamente, de 64,9% (variação: 48,8-105,7%) e 38,9% (variação: 16,0-60,0%) em porcentagem dos valores previstos. Apesar de espirometria normal, 9 pacientes exibiram DLCO reduzida. Em relação às TCAR, padrões típicos de pneumonia intersticial usual foram encontrados em 6 pacientes (17,1%). Em 25 casos (71,5%) os achados tomográficos foram mais consistentes com um diagnóstico de não relacionado a fibrose pulmonar idiopática. Em 11 pacientes (31,4%) o padrão radiológico foi incaracterístico para doença pulmonar intersticial. Das seis amostras de tecido pulmonar analisadas, quatro mostraram pneumonias intersticiais com acentuação bronquiolocêntrica e, em função de outros dados clínicos e radiológicos, pneumonite de hipersensibilidade foi diagnosticada. Conclusões: Pacientes com FPF podem apresentar características clínicas diversas. A maioria das TCAR desses pacientes exibe padrões não típicos de pneumonia intersticial usual. A pesquisa da história clínica de outros casos de pneumopatias fibrosantes na família deve ser feita em todos os pacientes em investigação, independentemente da idade.

Advances in Imaging and Automated Quantification of Malignant Pulmonary Diseases: A State-of-the-Art Review.

Quantitative imaging in lung cancer is a rapidly evolving modality in radiology that is changing clinical practice from a qualitative analysis of imaging features to a more dynamic, spatial, and phenotypical characterization of suspected lesions. Some quantitative parameters, such as the use of 18F-FDG PET/CT-derived standard uptake values (SUV), have already been incorporated into current practice as it provides important information for diagnosis, staging, and treatment response of patients with lung cancer. A growing body of evidence is emerging to support the use of quantitative parameters from other modalities. CT-derived volumetric assessment, CT and MRI lung perfusion scans, and diffusion-weighted MRI are some of the examples. Software-assisted technologies are the future of quantitative analyses in order to decrease intra- and inter-observer variability. In the era of "big data", widespread incorporation of radiomics (extracting quantitative information from medical images by converting them into minable high-dimensional data) will allow medical imaging to surpass its current status quo and provide more accurate histological correlations and prognostic value in lung cancer. This is a comprehensive review of some of the quantitative image methods and computer-aided systems to the diagnosis and follow-up of patients with lung cancer.

Use of computed tomography and automated software for quantitative analysis of the vasculature of patients with pulmonary hypertension / Análise quantitativa por tomografia computadorizada da vasculatura pulmonar em pacientes com hipertensão pulmonar utilizando programa automático

Abstract Objective: To perform a quantitative analysis of the lung parenchyma and pulmonary vasculature of patients with pulmonary hypertension (PH) on computed tomography angiography (CTA) images, using automated software. Materials and Methods: We retrospectively analyzed the CTA findings and clinical records of 45 patients with PH (17 males and 28 females), in comparison with a control group of 20 healthy individuals (7 males and 13 females); the mean age differed significantly between the two groups (53 ± 14.7 vs. 35 ± 9.6 years; p = 0.0001). Results: The automated analysis showed that, in comparison with the controls, the patients with PH showed lower 10th percentile values for lung density, higher vascular volumes in the right upper lung lobe, and higher vascular volume ratios between the upper and lower lobes. In our quantitative analysis, we found no differences among the various PH subgroups. We inferred that a difference in the 10th percentile values indicates areas of hypovolemia in patients with PH and that a difference in pulmonary vascular volumes indicates redistribution of the pulmonary vasculature and an increase in pulmonary vasculature resistance. Conclusion: Automated analysis of pulmonary vessels on CTA images revealed alterations and could represent an objective diagnostic tool for the evaluation of patients with PH.

Resumo Objetivo: Avaliar, quantitativamente, as alterações do parênquima e da vasculatura pulmonar em exames de angiotomografia computadorizada (angio-TC) de pacientes com hipertensão pulmonar (HP), utilizando programa de análise totalmente automática. Materiais e Métodos: Avaliamos, retrospectivamente, prontuários e exames de angio-TC de 45 pacientes com HP (28 mulheres e 17 homens; idade de 53 ± 14,7 anos), em comparação com um grupo controle mais jovem sem comorbidades detectadas (13 mulheres e 7 homens; idade de 35 ± 9,6 anos). Os grupos diferiram estatisticamente quanto à idade (p = 0,0001). Resultados: Análise automática mostrou valores menores do percentil 10 da densidade dos pulmões, maior volume vascular do lobo superior direito e volume vascular relativo entre lobos superiores e inferiores nos pacientes com HP, quando comparados com o grupo controle. Não encontramos diferenças da análise quantitativa por angio-TC entre grupos de pacientes com HP. Inferimos que a diferença nos valores do percentil 10 indique a presença das áreas de oligoemia nos pacientes com HP, enquanto as diferenças nos volumes vasculares indiquem redistribuição da trama e aumento da resistência vascular pulmonar. Conclusão: A análise quantitativa automática da vasculatura pulmonar em imagens de angio-TC identificou alterações e pode representar ferramenta diagnóstica objetiva na avaliação da HP.

Whole-body MRI in pediatric patients with cancer.

Cancer is the leading cause of natural death in the pediatric populations of developed countries, yet cure rates are greater than 70% when a cancer is diagnosed in its early stages. Recent advances in magnetic resonance imaging methods have markedly improved diagnostic and therapeutic approaches, while avoiding the risks of ionizing radiation that are associated with most conventional radiological methods, such as computed tomography and positron emission tomography/computed tomography. The advent of whole-body magnetic resonance imaging in association with the development of metabolic- and function-based techniques has led to the use of whole-body magnetic resonance imaging for the screening, diagnosis, staging, response assessment, and post-therapeutic follow-up of children with solid sporadic tumours or those with related genetic syndromes. Here, the advantages, techniques, indications, and limitations of whole-body magnetic resonance imaging in the management of pediatric oncology patients are presented.

Use of computed tomography and automated software for quantitative analysis of the vasculature of patients with pulmonary hypertension.

OBJECTIVE: To perform a quantitative analysis of the lung parenchyma and pulmonary vasculature of patients with pulmonary hypertension (PH) on computed tomography angiography (CTA) images, using automated software. MATERIALS AND METHODS: We retrospectively analyzed the CTA findings and clinical records of 45 patients with PH (17 males and 28 females), in comparison with a control group of 20 healthy individuals (7 males and 13 females); the mean age differed significantly between the two groups (53 ± 14.7 vs. 35 ± 9.6 years; p = 0.0001). RESULTS: The automated analysis showed that, in comparison with the controls, the patients with PH showed lower 10th percentile values for lung density, higher vascular volumes in the right upper lung lobe, and higher vascular volume ratios between the upper and lower lobes. In our quantitative analysis, we found no differences among the various PH subgroups. We inferred that a difference in the 10th percentile values indicates areas of hypovolemia in patients with PH and that a difference in pulmonary vascular volumes indicates redistribution of the pulmonary vasculature and an increase in pulmonary vasculature resistance. CONCLUSION: Automated analysis of pulmonary vessels on CTA images revealed alterations and could represent an objective diagnostic tool for the evaluation of patients with PH.

Resumo. OBJETIVO: Avaliar, quantitativamente, as alterações do parênquima e da vasculatura pulmonar em exames de angiotomografia computadorizada (angio-TC) de pacientes com hipertensão pulmonar (HP), utilizando programa de análise totalmente automática. MATERIAIS E MÉTODOS: Avaliamos, retrospectivamente, prontuários e exames de angio-TC de 45 pacientes com HP (28 mulheres e 17 homens; idade de 53 ± 14,7 anos), em comparação com um grupo controle mais jovem sem comorbidades detectadas (13 mulheres e 7 homens; idade de 35 ± 9,6 anos). Os grupos diferiram estatisticamente quanto à idade (p = 0,0001). RESULTADOS: Análise automática mostrou valores menores do percentil 10 da densidade dos pulmões, maior volume vascular do lobo superior direito e volume vascular relativo entre lobos superiores e inferiores nos pacientes com HP, quando comparados com o grupo controle. Não encontramos diferenças da análise quantitativa por angio-TC entre grupos de pacientes com HP. Inferimos que a diferença nos valores do percentil 10 indique a presença das áreas de oligoemia nos pacientes com HP, enquanto as diferenças nos volumes vasculares indiquem redistribuição da trama e aumento da resistência vascular pulmonar. CONCLUSÃO: A análise quantitativa automática da vasculatura pulmonar em imagens de angio-TC identificou alterações e pode representar ferramenta diagnóstica objetiva na avaliação da HP.

Quantitative computed tomography analysis of the airways in patients with cystic fibrosis using automated software: correlation with spirometry in the evaluation of severity / Análise quantitativa por tomografia computadorizada das vias aéreas, usando programa automático, em pacientes com fibrose cística: avaliação da gravidade em correlação com a espirometria

Abstract Objective: To perform a quantitative analysis of the airways using automated software, in computed tomography images of patients with cystic fibrosis, correlating the results with spirometric findings. Materials and Methods: Thirty-four patients with cystic fibrosis were studied-20 males and 14 females; mean age 18 ± 9 years-divided into two groups according to the spirometry findings: group I (n = 21), without severe airflow obstruction (forced expiratory volume in first second [FEV1] > 50% predicted), and group II (n = 13), with severe obstruction (FEV1 ≤ 50% predicted). The following tracheobronchial tree parameters were obtained automatically: bronchial diameter, area, thickness, and wall attenuation. Results: On average, 52 bronchi per patient were studied. The number of bronchi analyzed was higher in group II. The correlation with spirometry findings, especially between the relative wall thickness of third to eighth bronchial generation and predicted FEV1, was better in group I. Conclusion: Quantitative analysis of the airways by computed tomography can be useful for assessing disease severity in cystic fibrosis patients. In patients with severe airflow obstruction, the number of bronchi studied by the method is higher, indicating more bronchiectasis. In patients without severe obstruction, the relative bronchial wall thickness showed a good correlation with the predicted FEV1.

Resumo Objetivo: Realizar a análise quantitativa das vias aéreas utilizando programa automático, em imagens de tomografia computadorizada de pacientes com fibrose cística, correlacionando com a espirometria. Materiais e Métodos: Foram estudados 34 pacientes com fibrose cística - 20 masculinos e 14 femininos; idade de 18 ± 9 anos -, divididos em dois grupos segundo a espirometria: grupo I (n = 21) - sem obstrução grave ao fluxo aéreo (volume expiratório forçado no primeiro segundo [VEF1] > 50% previsto); grupo II (n = 13) - com obstrução grave (VEF1 ≤ 50% previsto). Foram automaticamente obtidos: diâmetro, área, espessura e atenuação da parede da árvore traqueobrônquica. Resultados: Na média, foram estudados 52 brônquios por paciente. O número de brônquios analisados foi maior no grupo II. A correlação com a espirometria foi melhor no grupo I, principalmente entre a espessura relativa da parede da terceira a oitava geração brônquica e o VEF1 previsto. Conclusão: A análise quantitativa das vias aéreas em imagens de tomografia computadorizada pode ser útil na avaliação da gravidade da doença na fibrose cística. Nos pacientes com obstrução grave ao fluxo aéreo, o número de brônquios estudados pelo método é maior, indicando mais bronquiectasias. Nos pacientes sem obstrução grave, a espessura relativa da parede dos brônquios tem boa correlação com o VEF1 previsto.

A Challenging Differential Diagnosis: Distinguishing between Endomyocardial Fibrosis and Apical Hypertrophic Cardiomyopathy.

Endomyocardial fibrosis, which is a cause of restrictive cardiomyopathy, is characterized by the deposition of fibrous tissue in the apical region of 1 or both ventricles. The condition not only affects the diastolic dynamics of the ventricles, but also the function of the atrioventricular valves. The disease occurs predominantly in tropical regions worldwide and in sub-Saharan Africa. This condition is not well understood, with varied manifestations, from subclinical presentations to chronic and progressive edematous syndromes. Here, we present the challenging case of a patient with an indeterminate echocardiographic image, suggesting apical hypertrophy, plus severe aortic stenosis and fibrosis of the left ventricular outflow tract. An electrocardiogram revealed symmetrical T-wave inversion, which is a characteristic manifestation of apical hypertrophy. The importance of cardiac imaging examinations such as echocardiography and cardiac magnetic resonance for differentiating between endomyocardial fibrosis and apical hypertrophy is highlighted in this patient's case.