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With the surgical improvement of congenital heart disease, Fontan operation has been applied to many complicated patients in recent years. This is the first report of a child with panhypopituitarism who underwent Fontan operation. A 5-year-old boy was scheduled for Fontan operation. He previously underwent Blalock-Taussig shunt and bidirectional Glenn operations for univentricular heart with double-outlet right ventricle and pulmonary atresia. He was receiving hydrocortisone and 1-desamino-8-D-arginine vasopressin (DDAVP) for panhypopituitarism secondary to removal of craniopharyngioma performed at the age of three years. Although urine output and serum sodium concentration were adequately controlled by adjustment of vasopressin infusion rate during surgery, massive pleural effusions and ascites developed postoperatively, which required several days for control by adjusting the dose of oral DDAVP and normalize the serum sodium level. Intraoperative management of Fontan operation for a patient with panhypopituitarism was controllable by appropriate hormone replacement. However, postoperative fluid management was complicated by the clinical features of panhypopituitarism and Fontan physiology., Author(s): Kohei Sunoki [sup.1], Yoji Otsuka [sup.1], Hidetaka Iwai [sup.1], Tatsuya Nagano [sup.1], Naoyuki Taga [sup.1], Masaaki Kawada [sup.2], Mamoru Takeuchi [sup.1] [sup.3] Author Affiliations: (1) grid.410804.9, 0000000123090000, Department of [...]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2009.04.021 Byline: Hideo Ohuchi (a), Yoshihiro Miyamoto (b), Masaki Yamamoto (a), Haruko Ishihara (a), Hidemi Takata (a), Aya Miyazaki (a), Osamu Yamada (a), Toshikatsu Yagihara (c) Abstract: Abnormal glucose metabolism (AGM) adversely impacts morbidity and mortality in patients with chronic heart failure. No data on AGM in adult patients with congenital heart disease (ACHD) are available. Author Affiliation: (a) Department of Pediatrics, National Cardiovascular Center, Osaka, Japan (b) Department of Atherosclerosis and Diabetes, National Cardiovascular Center, Osaka, Japan (c) Department of Thoracic Surgery, National Cardiovascular Center, Osaka, Japan Article History: Received 18 December 2008; Accepted 2 April 2009

Objectives: The aim of this study was to relate changes in energy expenditure and growth in infants with congenital heart disease (CHD), to the timing of corrective cardiac surgery. Methods: Prospective cohort study of infants less than 1 year with CHID admitted for cardiac surgery to Royal Children's Hospital, between January to September 2005. Infants were assessed using anthropometry and indirect calorimetry and compared to healthy age-matched controls. Results: Infants (38) underwent corrective (n=25) or palliative (n=13) cardiac surgery either at [less than or equal to] 10 days or at >10 days. Infants undergoing corrective surgery after 10 days had deficits in z-scores for weight compared with infants undergoing early surgery (-1.15 [+ or -] 1.02 vs -0.24 [+ or -] 0.98; CI 95%: -1.736 to -0.085; P Conclusion: Increased REE observed in infants with CHID normalizes within 1 week following corrective cardiac surgery. Deficits in weight and growth were greater in infants undergoing corrective cardiac surgery>10 days of age compared with infants undergoing surgery in the first 10 days of life. doi:10.1038/sj.ejcn.1602956; published online 14 November 2007 Keywords: congenital heart disease; energy expenditure; cardiac surgery; indirect calorimetry; resting energy expenditure, Introduction Disturbance in energy balance is a major limiting factor for growth, and cognitive and motor development (Wells, 1998). During episodes of acute illness or in chronic diseases, even previously [...]