Original articlePediatric cardiacImproving Outcomes of the Surgical Management of Right Atrial Isomerism
Section snippets
Study Design
We conducted a single-center retrospective review of the medical records of 60 patients (32 boys and 28 girls) with RAI who underwent a first palliative operation at Mt. Fuji Shizuoka Children's Hospital in Shizuoka, Japan, from September 1997 to October 2010. The institutional review board of the hospital approved this study, and individual consent for the study was waived. None of the patients in the study withdrew from treatment. A patient (n = 1) who had undergone biventricular repair as
Patients
The patients' median age at initial palliation was 29.0 days (range, 0 to 765 days); 13 patients (21.7%) were less than 7 days old at the time of their initial operation, and 30 (50%) of the 60 patients in the study were less than 28 days old. The patients' median weight at their initial operation was 3.25 kg (range, 2.0 to 12.1 kg). Twenty-two patients (36.7%) weighed less than 3.0 kg at the time of their initial operation, including 6 patients (10%) who weighed less than 2.5 kg. Two patients
Comment
Surgical outcomes in visceral heterotaxy syndromes are improving, especially in the case of RAI. In contrast to the relatively favorable prognosis in left atrial isomerism [4, 15, 16] infants with RAI have a poor outcome. Foerster and colleagues [17] reported a poorer survival over time for patients with a phenotype of asplenia syndrome than for those with a phenotype of polysplenia. Given the frequency of withdrawal from treatment [2, 18] and the high surgical mortality after palliative
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