• Hiroshi Suzuki, Hideo Ohuchi, Yoshimi Hiraumi, Kenji Yasuda, and Shigeyuki Echigo.
• Department of Pediatrics, National Cardiovascular Center, Suita, Osaka, Japan. hsuzukc562@hotmail.com
• Int. J. Cardiol. 2006 Jan 13; 106 (2): 211-7.

AbstractThe aim of this study was to assess whether platypnea and orthodeoxia occur in Fontan patients. We divided 14 Fontan patients into 2 groups: 8 patients who had pulmonary arteriovenous fistulas and/or intra-atrial shunts (group A) and 6 patients who had neither pulmonary arteriovenous fistulas nor intra-atrial shunts (group B). They were compared with 9 controls (group C). Arterial oxygen saturation, minute ventilation per body weight and ventilatory equivalent for carbon dioxide were measured in the supine and then sitting positions. In group A, 1 patient had platypnea and 3 patients had orthodeoxia (changes in the saturation from the supine position to the sitting position were -4% to -7%) accompanied with slight hyperpnea, and all 4 patients had both pulmonary arteriovenous fistulas and intra-atrial shunts. Contrary, patients in group B had neither platypnea nor orthodeoxia. The saturation was significantly lower and the minute ventilation was significantly higher in the sitting position than in the supine position in group A (p < 0.05). The other groups showed no significant difference in the saturation or the minute ventilation between the 2 positions. All groups showed the ventilatory equivalent was significantly higher in the sitting position than in the supine position (p < 0.05 to 0.01). We demonstrated platypnea and orthodeoxia in Fontan patients with pulmonary arteriovenous fistulas and intra-atrial shunts. We believe platypnea and orthodeoxia should be regard as a complication in Fontan patients with pulmonary arteriovenous fistulas and/or intra-atrial shunts.

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