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BACKGROUND: In nonvalvular atrial fibrillation (NVAF), the left atrial appendage (LAA) is the source of thrombus in up to 90% of patients. LAA pseudothrombus (LAAPT), defined as a filling defect on the initial but not the 60-second delayed acquisition on cardiovascular computed tomography scan (CCT), is a recognized phenomenon in NVAF, with unknown clinical relevance. We aimed to determine the relationship between LAAPT and history of stroke in patients with NVAF. METHODS AND RESULTS: The study included 213 consecutive patients with NVAF undergoing CCT who were assessed for LAAPT. LA and LAA dimensions and LAA morphology correlated with clinical demographics including cardiovascular risk factors, history of stroke, thromboembolic stroke, and transient ischemic attack. Mean age (±SD) was 65.1±10.5 years (range 31-89) and 150 of 213 (70.4%) were men. LAAPT was present in 59 of 213 (27.7%) patients. Greater mean LAA ostium area (5.7 versus 4.5, P<0.001), greater mean LAA ostium area:curved length (0.11 versus 0.08, P<0.001), increased LAA volume (14.0 versus 10.2, P<0.001), and lower mean LAA tortuosity index (1.17 versus 1.38, P<0.001) were all associated with the presence of LAAPT. On multivariable analysis, LAAPT on CCT (odds ratio [OR], 3.20 [95% CI, 1.40-7.20]; P<0.006) and higher CHA2DS2-VASc score (OR, 1.65 [95% CI, 1.16-2.35]; P=0.01) were associated with all strokes, with LAAPT remaining a statistically significant risk factor even after adjustment for CHA2DS2-VASc score. CONCLUSIONS: LAAPT on CCT is common in patients with NVAF. It has a strong positive association with stroke prevalence, even after adjustment for CHA2DS2-VASc score. LAAPT on CCT may potentially allow further stratification for stroke risk, additive to the CHA2DS2-VASc score.
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Apéndice Atrial , Fibrilación Atrial , Accidente Cerebrovascular , Humanos , Apéndice Atrial/diagnóstico por imagen , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico por imagen , Fibrilación Atrial/epidemiología , Masculino , Femenino , Anciano , Persona de Mediana Edad , Anciano de 80 o más Años , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/diagnóstico por imagen , Factores de Riesgo , Adulto , Trombosis/diagnóstico por imagen , Trombosis/epidemiología , Trombosis/etiología , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Medición de RiesgoAsunto(s)
Hipertensión Pulmonar , Derrame Pleural , Venas Pulmonares , Sarcoidosis Pulmonar , Humanos , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/diagnóstico , Derrame Pleural/etiología , Derrame Pleural/diagnóstico por imagen , Derrame Pleural/diagnóstico , Venas Pulmonares/diagnóstico por imagen , Sarcoidosis Pulmonar/complicaciones , Tomografía Computarizada por Rayos X , Femenino , Persona de Mediana Edad , MasculinoRESUMEN
Rationale: Clinical trials have shown that use of elexacaftor/tezacaftor/ivacaftor (ETI) is associated with improvements in sweat chloride, pulmonary function, nutrition, and quality of life in people with cystic fibrosis (CF). Little is known about the impact of ETI on ventilation inhomogeneity and lung structure. Objectives: RECOVER is a real-world study designed to measure the impact of ETI in people with CF. The primary endpoints were lung clearance (lung clearance index; LCI2.5) and FEV1. Secondary endpoints included spirometry-controlled chest computed tomography (CT) scores. Methods: The study was conducted in seven sites in Ireland and the United Kingdom. Participants ages 12 years and older who were homozygous for the F508del mutation (F508del/F508del) or heterozygous for F508del and a minimum-function mutation (F508del/MF) were recruited before starting ETI and were followed up over 12 months. LCI2.5 was measured using nitrogen multiple breath washout (MBW) at baseline and at 6 and 12 months. Spirometry was performed as per the criteria of the American Thoracic Society and the European Respiratory Society. Spirometry-controlled chest CT scans were performed at baseline and at 12 months. CT scans were scored using the Perth Rotterdam Annotated Grid Morphometric Analysis (PRAGMA) system. Other outcome measures include weight, height, Cystic Fibrosis Quality of Life Questionnaire-Revised (CFQ-R), and sweat chloride. Measurements and Main Results: One hundred seventeen people with CF ages 12 and older were recruited to the study. Significant improvements were seen in LCI scores (-2.5; 95% confidence interval [CI], -3.0, -2.0) and in the percents predicted for FEV1 (8.9; 95% CI, 7.0, 10.9), FVC (6.6; 95% CI, 4.9, 8.3), and forced expiratory flow between 25% and 75% of expired volume (12.4; 95% CI, 7.8, 17.0). Overall PRAGMA-CF scores reflecting airway disease improved significantly (-3.46; 95% CI, -5.23, -1.69). Scores for trapped air, mucus plugging, and bronchial wall thickening improved significantly, but bronchiectasis scores did not. Sweat chloride levels decreased in both F508del/F508del (-43.1; 95% CI, -47.4, -38.9) and F508del/MF (-42.8; 95% CI, -48.5, -37.2) groups. Scores on the Respiratory Domain of the CFQ-R improved by 14.2 points (95% CI, 11.3, 17.2). At 1 year, sweat chloride levels were significantly lower for the F508del/F508del group compared with scores for the F508del/MF group (33.93 vs. 53.36, P < 0.001). Conclusions: ETI is associated with substantial improvements in LCI2.5, spirometry, and PRAGMA-CF CT scores in people with CF ages 12 years and older. ETI led to improved nutrition and quality of life. People in the F508del/F508del group had significantly lower sweat chloride on ETI treatment compared with the F508del/MF group. Clinical trial registered with www.clinicaltrials.gov (NCT04602468).
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Fibrosis Quística , Humanos , Aminofenoles/uso terapéutico , Benzodioxoles/uso terapéutico , Agonistas de los Canales de Cloruro/uso terapéutico , Cloruros/análisis , Fibrosis Quística/diagnóstico por imagen , Fibrosis Quística/tratamiento farmacológico , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/uso terapéutico , Pulmón , Mutación , Calidad de Vida , Tomografía Computarizada por Rayos XRESUMEN
In situ pulmonary arterial thrombosis in COVID-19 is not visible on CTPA. However, the presence of CT-measured right heart and pulmonary artery dilatation in COVID-19 is likely attributable to this process and may be a possible surrogate for its detection. https://bit.ly/3g7z5TV.
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We present two neonates requiring extracorporeal membrane oxygenation for undiagnosed recalcitrant pulmonary hypertension, highlighting the clinical and ethical dilemmas in management of very rare diseases.
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Oxigenación por Membrana Extracorpórea , Hipertensión Pulmonar , Recién Nacido , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/terapiaRESUMEN
BACKGROUND: The role of dual energy computed tomographic pulmonary angiography (DECTPA) in revealing vasculopathy in coronavirus disease 2019 (COVID-19) has not been fully explored. PURPOSE: To evaluate the relationship between DECTPA and disease duration, right ventricular dysfunction (RVD), lung compliance, D-dimer and obstruction index in COVID-19 pneumonia. MATERIALS AND METHODS: This institutional review board approved this retrospective study, and waived the informed consent requirement. Between March-May 2020, 27 consecutive ventilated patients with severe COVID-19 pneumonia underwent DECTPA to diagnose pulmonary thrombus (PT); 11 underwent surveillance DECTPA 14 ±11.6 days later. Qualitative and quantitative analysis of perfused blood volume (PBV) maps recorded: i) perfusion defect 'pattern' (wedge-shaped, mottled or amorphous), ii) presence of PT and CT obstruction index (CTOI) and iii) PBV relative to pulmonary artery enhancement (PBV/PAenh); PBV/PAenh was also compared with seven healthy volunteers and correlated with D-Dimer and CTOI. RESULTS: Amorphous (n=21), mottled (n=4), and wedge-shaped (n=2) perfusion defects were observed (M=20; mean age=56 ±8.7 years). Mean extent of perfusion defects=36.1%±17.2. Acute PT was present in 11/27(40.7%) patients. Only wedge-shaped defects corresponded with PT (2/27, 7.4%). Mean CTOI was 2.6±5.4 out of 40. PBV/PAenh (18.2 ±4.2%) was lower than in healthy volunteers (27 ±13.9%, p = 0.002). PBV/PAenh correlated with disease duration (ß = 0.13, p = 0.04), and inversely correlated with RVD (ß = -7.2, p = 0.001), persisting after controlling for confounders. There were no linkages between PBV/PAenh and D-dimer or CTOI. CONCLUSION: Perfusion defects and decreased PBV/PAenh are prevalent in severe COVID-19 pneumonia. PBV/PAenh correlates with disease duration and inversely correlates with RVD. PBV/PAenh may be an important marker of vasculopathy in severe COVID-19 pneumonia even in the absence of arterial thrombus.
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Quiste Broncogénico/complicaciones , Quiste Broncogénico/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Estenosis Traqueal/etiología , Estenosis Traqueal/cirugía , Quiste Broncogénico/cirugía , Broncoscopía/métodos , Femenino , Estudios de Seguimiento , Humanos , Lactante , Enfermedades Raras , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos X/métodos , Estenosis Traqueal/diagnóstico por imagen , Resultado del TratamientoRESUMEN
PURPOSE: Primary ciliary dyskinesia (PCD) is a respiratory syndrome in which 'random' organ orientation can occur; with approximately 46% of patients developing situs inversus totalis at organogenesis. The aim of this study was to explore the relationship between organ anatomy and curve convexity by studying the prevalence and convexity of idiopathic scoliosis in PCD patients with and without situs inversus. METHODS: Chest radiographs of PCD patients were systematically screened for existence of significant lateral spinal deviation using the Cobb angle. Positive values represented right-sided convexity. Curve convexity and Cobb angles were compared between PCD patients with situs inversus and normal anatomy. RESULTS: A total of 198 PCD patients were screened. The prevalence of scoliosis (Cobb >10°) and significant spinal asymmetry (Cobb 5-10°) was 8 and 23%, respectively. Curve convexity and Cobb angle were significantly different within both groups between situs inversus patients and patients with normal anatomy (P ≤ 0.009). Moreover, curve convexity correlated significantly with organ orientation (P < 0.001; Ï = 0.882): In 16 PCD patients with scoliosis (8 situs inversus and 8 normal anatomy), except for one case, matching of curve convexity and orientation of organ anatomy was observed: convexity of the curve was opposite to organ orientation. CONCLUSIONS: This study supports our hypothesis on the correlation between organ anatomy and curve convexity in scoliosis: the convexity of the thoracic curve is predominantly to the right in PCD patients that were 'randomized' to normal organ anatomy and to the left in patients with situs inversus totalis.