Congenital heart disease
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Congenital heart disease · Mar 2011
Multicenter StudyVariation in postoperative care following stage I palliation for single-ventricle patients: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.
The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. ⋯ Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.
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Congenital heart disease · Mar 2011
Effects of pulmonary vasodilator therapy on ventilatory efficiency during exercise in adults with Eisenmenger syndrome.
Eisenmenger syndrome, characterized by systemic-level pulmonary arterial resistance with resultant right-to-left shunt, is associated with low exercise capacity and hyperpnea at rest and exercise. Because ventilatory requirements are augmented by right-to-left shunting, we hypothesized that if pulmonary vasodilator treatment improved pulmonary perfusion in this condition, this would also improve ventilatory efficiency during exercise. ⋯ These findings are consistent with reduction in right-to-left shunt due to improved pulmonary blood flow, though attenuation of ventilatory drive is not excluded. Treatment of adult Eisenmenger patients with pulmonary the pulmonary vasodilators bosentan or sildenafil leads to improvement in parameters of ventilatory efficiency during exercise.