Pediatric cardiology
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Pediatric cardiology · Sep 2005
Modified Fontan operation in patients with anomalies of systemic and pulmonary venous connection.
The presence of anomalies of the systemic and pulmonary venous connection associated with single ventricle anomalies has been considered a contraindication for the Fontan operation (FO). The aim of this study is to outline the technical considerations associated with the presence of anomalies of systemic and/or pulmonary venous connections and to identify the risk factors for mortality in this group of patients undergoing the modified FO. Between 1989 and 2004, 63 patients (median age, 3.2 years) with anomalous systemic or pulmonary venous connection underwent a Fontan procedure at our institution. ⋯ At a mean follow-up of 4.6 +/- 3.4 years, there have been 5 late deaths (8%) and 45 patients (71%) have undergone Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5 and 10 years-not significantly different from the overall survival of the Fontan patients. We conclude that the modified FO can be successfully performed in patients with anomalous systemic or pulmonary venous connections, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients with normal connections.
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Pediatric cardiology · Sep 2005
Case ReportsUse of dexmedetomidine in a pediatric heart transplant patient.
Dexmedetomidine is a new sedative and analgesic agent that has a unique property of lack of respiratory depression. We describe a pediatric case in which the use of dexmedetomidine enabled us to successfully extubate a 12-year-old heart transplant patient with acute pneumonia after several failed attempts with the use of more conventional sedation.
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Pediatric cardiology · Sep 2005
Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response.
Inhaled nitric oxide (NO) has been used in the preoperative evaluation of patients with congenital heart disease and pulmonary hypertension. The purpose of this study was to characterize responses in pulmonary vascular resistance (PVR) to oxygen and increasing doses of NO during cardiac catheterization and to determine if any related factors affect the response of the pulmonary vascular bed to NO. A prospective analysis of 42 patients (median age, 3.0 years) with congenital heart disease and pulmonary hypertension who underwent NO testing was performed. ⋯ There is no difference in determining PVR response with doses of NO beyond 20 ppm during cardiac catheterization. Age, gender, and baseline PVR/SVR ratio are not associated with responsiveness to NO. Patients with trisomy 21 may be less responsive to NO.