Masakazu Otsuka, Yoshihiko Kodama, Ayako Kuraoka, Yuichi Ishikawa, Makoto Nakamura, Toshihide Nakano, Hideaki Kado, Shintaro Umemoto, Ayako Ishikita, Ichiro Sakamoto, Tomomi Ide, Hiroyuki Tsutsui, Koichi Sagawa
Fukuoka Children’s Hospital. Kyushu University Hospital.
Japan
Pediatric Cardiology
Pediatr Cardiol 2022; 43: 360-365
DOI: 10.1007/s00246-021-02727-6
Abstract
Patients with Down’s syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children’s Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient significantly increased, and arterial oxygen saturation significantly decreased over time in DS patients after the Fontan procedure compared with those without DS. CVP in DS patients after the Fontan procedure increased over time compared with non-DS patients. Better management including the efficacy of Pulmonary arterial hypertension-specific therapy should be clarified in further studies.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Genetic Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No