Pediatric Pulmonary Hypertension Ivy, D. Dunbar, MD; Abman, Steven H., MD; Barst, Robyn J., MD ...
Journal of the American College of Cardiology,
12/2013, Letnik:
62, Številka:
25
Journal Article, Conference Proceeding
Recenzirano
Odprti dostop
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic ...or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.
Objectives To compare registry and electronic health record (EHR) data mining approaches for cohort ascertainment in patients with pediatric pulmonary hypertension (PH) in an effort to overcome some ...of the limitations of registry enrollment alone in identifying patients with particular disease phenotypes. Study design This study was a single-center retrospective analysis of EHR and registry data at Boston Children's Hospital. The local Informatics for Integrating Biology and the Bedside (i2b2) data warehouse was queried for billing codes, prescriptions, and narrative data related to pediatric PH. Computable phenotype algorithms were developed by fitting penalized logistic regression models to a physician-annotated training set. Algorithms were applied to a candidate patient cohort, and performance was evaluated using a separate set of 136 records and 179 registry patients. We compared clinical and demographic characteristics of patients identified by computable phenotype and the registry. Results The computable phenotype had an area under the receiver operating characteristics curve of 90% (95% CI, 85%-95%), a positive predictive value of 85% (95% CI, 77%-93%), and identified 413 patients (an additional 231%) with pediatric PH who were not enrolled in the registry. Patients identified by the computable phenotype were clinically distinct from registry patients, with a greater prevalence of diagnoses related to perinatal distress and left heart disease. Conclusions Mining of EHRs using computable phenotypes identified a large cohort of patients not recruited using a classic registry. Fusion of EHR and registry data can improve cohort ascertainment for the study of rare diseases. Trial registration ClinicalTrials.gov : NCT02249923.
Objective To evaluate in vivo cross-sectional conformational changes of ascending aortic wall excursion in patients undergoing resection for aortic aneurysm with those undergoing elective coronary ...artery bypass grafting (CABG) using epi-aortic echocardiography. Design A prospective observational investigation. Setting A single tertiary care university hospital. Participants Thirty-four patients undergoing elective ascending aorta resection and 23 elective CABG patients. Intervention In an open-chest model and with use of an epi-aortic echocardiographic probe, measurements of aortic wall excursion were made on the ascending aortic aneurysms. Control measurements were made on the transitional neck portions of the aneurysmal aortas (internal control) and CABG aortas (external control). Measurements and Main Results The aortic aneurysm measurements exhibited no difference (2.8%, p < 0.62) between the excursion of the anterior and posterior walls. In contrast, under similar hemodynamic conditions, the anterior wall of the aneurysm neck moved 48.2% ( p < 0.0004) more than the posterior wall. Similarly, in the CABG control group, the anterior wall moved 24% ( p < 0.027) more than the posterior wall. Conclusion This in vivo study documented a lack of asymmetric aortic wall motion in ascending aortic aneurysms. In contrast, both the internal and external control groups (aneurysm neck and CABG) demonstrated asymmetric wall motion. The lack of asymmetric wall motion may be an important aspect of aneurysm pathophysiology and key to the development of management strategies for timing of surgical intervention.
We investigated the effect of pentoxifylline (PTX) on the development of pulmonary edema in a model of adult respiratory distress syndrome in rabbits. Lung injury was induced by repeated saline ...lavages in adult rabbits weighing 2.5 to 3.5 kg. Rabbits pretreated with PTX (20 mg/kg bolus followed by 20 mg/kg/h infusion) developed significantly lower amounts of lung edema 4 h after saline lavage (extravascular lung water to dry weight ratio W/D, 6.9 +/- 0.6 SD versus 8.9 +/- 0.5 in control animals). PTX produced a 25% increase in cardiac output, but there were no differences between treated and untreated groups in calculated pulmonary vascular resistance or microvascular pressure. To determine whether PTX could have lowered pulmonary venous resistance and thus lowered effective microvascular pressure for fluid filtration, we directly measured pulmonary artery and left atrial pressures, and measured by micropuncture the pressure in 20 to 40 microns subpleural venules in four open-chested rabbits 3 to 4 h after lavage. Venous resistance was low (venous pressure drop 0.9 +/- 0.1 mm Hg) and was unchanged by PTX infusion. To determine if PTX decreased lung water by accelerating active alveolar fluid reabsorption, a single 60-ml aliquot of saline was instilled into the lungs of normal rabbits treated with saline or PTX. Both groups had a similar decrease in lung water content 1 and 4 h later. Our data indicate that PTX reduces edema formation in rabbits after saline lavage, not by lowering microvascular pressures for fluid filtration or by acceleration alveolar fluid reabsorption, but possibly by its anti-inflammatory effect on neutrophil function.
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