RESUMEN
BACKGROUND: Adult congenital heart disease (ACHD) patients pose unique challenges in identifying the time for transplantation and factors influencing outcomes. OBJECTIVE: To identify hemodynamic, functional, and laboratory parameters that correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation. METHODS: A retrospective chart review of long-term outcomes in adult patients with congenital heart disease (CHD) evaluated for heart or heart + additional organ transplant between 2004 and 2014 at our center was performed. A machine learning decision tree model was used to evaluate multiple clinical parameters correlating with 1- and 10-year survival. RESULTS: We identified 58 patients meeting criteria. D-transposition of the great arteries (D-TGA) with atrial switch operation (20.7%), tetralogy of Fallot/pulmonary atresia (15.5%), and tricuspid atresia (13.8%) were the most common diagnosis for transplant. Single ventricle patients were most likely to be listed for transplantation (39.8% of evaluated patients). Among a comprehensive list of clinical factors, invasive hemodynamic parameters (pulmonary capillary wedge pressure (PCWP), systemic vascular pressure (SVP), and end diastolic pressures (EDP) most correlated with 1- and 10-year outcomes. Transplanted patients with SVP < 14 and non- transplanted patients with PCWP < 15 had 100% survival 1-year post-transplantation. CONCLUSION: For the first time, our study identifies that hemodynamic parameters most strongly correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation, using a data-driven machine learning model.
Asunto(s)
Cardiopatías Congénitas , Trasplante de Corazón , Transposición de los Grandes Vasos , Adulto , Humanos , Cardiopatías Congénitas/cirugía , Transposición de los Grandes Vasos/etiología , Estudios Retrospectivos , Trasplante de Corazón/efectos adversosRESUMEN
BACKGROUND: Acute incisional hernia incarceration is associated with high morbidity and mortality yet there is little evidence to guide which patients will benefit most from prophylactic repair. We explored baseline computed tomography (CT) characteristics associated with incarceration. METHODS: A case-control study design was utilized to explore adults (≥18 years) diagnosed with an incisional hernia between 2010 and 2017 at a single institution with a 1-year minimum follow-up. Computed tomography imaging at the time of initial hernia diagnosis was examined. Following propensity score matching for baseline characteristics, multivariable logistic regression was performed to identify independent predictors associated with acute incarceration. RESULTS: A total of 532 patients (27.26% male, mean 61.55 years) were examined, of whom 238 experienced an acute incarceration. Between two well-matched cohorts with and without incarceration, the presence of small bowel in the hernia sac (odds ratio [OR], 7.50; 95% confidence interval [CI], 3.35-16.38), increasing sac height (OR, 1.34; 95% CI, 1.10-1.64), more acute hernia angle (OR, 0.98 per degree; 95% CI, 0.97-0.99), decreased fascial defect width (OR, 0.68; 95% CI, 0.58-0.81), and greater outer abdominal fat (OR, 1.28; 95% CI, 1.02-1.60) were associated with acute incarceration. Using threshold analysis, a hernia angle of <91 degrees and a sac height of >3.25 cm were associated with increased incarceration risk. CONCLUSION: Computed tomography features present at the time of hernia diagnosis provide insight into later acute incarceration risk. Improved understanding of acute incisional hernia incarceration can guide selection for prophylactic repair and thereby may mitigate the excess morbidity associated with incarceration. LEVEL OF EVIDENCE: Prognostic and Epidemiological; Level III.
Asunto(s)
Hernia Ventral , Hernia Incisional , Adulto , Humanos , Masculino , Femenino , Hernia Incisional/diagnóstico por imagen , Hernia Incisional/cirugía , Estudios de Casos y Controles , Hernia , Tomografía Computarizada por Rayos X/métodos , Hernia Ventral/cirugía , HerniorrafiaRESUMEN
BACKGROUND: Among stroke patients, primary intracerebral hemorrhage has the highest mortality rate. Expansion of hematoma plays a prognostic role in these patients. Although fluid levels have been shown to predict subsequent hematoma expansion, there are mimics of fluid levels that may confuse interpretation. We hypothesized that patients with true fluid levels on head computed tomography (CT) have higher hematoma progression rates and worse outcomes compared with patients who have fluid level mimics on CT. METHODS: Adult patients presenting with intracerebral hemorrhage described as a fluid level on initial CT interpretation were included. Medical records were reviewed to extract relevant clinical variables. A CAQ-certified neuroradiologist retrospectively determined whether there was a true fluid level or mimic on CT and then evaluated follow-up CT scans for radiologic progression. We compared radiologic progression, mortality, and anticoagulation status between patients with true fluid levels and fluid level mimics. RESULTS: The study included 12 patients, 8 with true fluid levels and 4 with radiologic mimics. The patients with true fluid levels had a significantly higher likelihood of radiographic progression (P = 0.014). Differences in outcome, use of anticoagulation therapy, and average international normalized ratio were not significant. CONCLUSIONS: A fluid level within intraparenchymal hemorrhage on head CT scan is associated with higher likelihood of intracerebral hemorrhage progression. However, this applies only to true fluid levels, with mimics having a lower likelihood of progression. A careful analysis of potential fluid levels is necessary before assigning prognostic implications.
Asunto(s)
Hemorragia Cerebral , Hematoma , Adulto , Humanos , Estudios Retrospectivos , Hemorragia Cerebral/complicaciones , Hematoma/diagnóstico por imagen , Hematoma/complicaciones , Pronóstico , Tomografía Computarizada por Rayos X , Progresión de la EnfermedadRESUMEN
Cerebrofaciothoracic dysplasia (CFTD) is a developmental disorder characterized by distinctive craniofacial dysmorphism, global developmental delay, and skeletal anomalies. CTFD is the result of biallelic autosomal recessive loss of function mutations in the transmembrane and coiled-coil domains one protein (TMCO1) gene. Based on a population of 27 molecularly confirmed cases, classic brain morphologies associated with CFTD have been described in the literature. Previous studies have demonstrated only mild ventriculomegaly, corpus callosum abnormalities, frontotemporal atrophy, and three cases of associated epilepsy. We present previously undescribed brain MRI findings in two children presenting with seizures due to TMCO1 mutation. MR Imaging demonstrated hippocampal malrotation, olfactory bulb agenesis and olfactory sulcus hypoplasia in both children, pontine hypoplasia, and cochlear nerve agenesis in one child. We demonstrate that TMCO1 may play a more extensive and previously undescribed role in neurodevelopment thereby expanding the phenotype associated with CFTD.