Abstract

Fontan procedure. Therefore, the sequence of events is consistent with the hypothesis that the increase in PA pressure may have been caused by obstructive tonsillar hypertrophy and was thereby reversed after tonsillectomy in this patient. Tonsillar hypertrophy is a common cause of OSA in children. Moderate-to-severe OSA is frequently associated with mild pulmonary hypertension, 3 which is characterized by mild pulmonary vascular remodeling and endothelial dysfunction, as shown in human and animal studies. 4,5 Therefore, it is possible that the magnitude of structural and functional alterations in pulmonary vasculature induced by nocturnal desaturation had significant impacts on the Glenn circulation, and that these changes were reversed after tonsillectomy in this case. Furthermore, it is interesting to speculate that such derangement in pulmonary circulation caused by OSA might have similar effects on hemodynamics in patients even after the Fontan procedure. This unique case implies that alleviation of OSA (ie, tonsillectomy) may be an efficacious treatment option for high-risk candidates for the Fontan procedure with high PA pressure after the Glenn procedure.

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