Original article
Pediatric cardiac
Improving Outcomes of the Surgical Management of Right Atrial Isomerism

Presented at the Forty-seventh Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 31–Feb 2, 2011.
https://doi.org/10.1016/j.athoracsur.2011.05.110Get rights and content

Background

Patients with right atrial isomerism (RAI) have several cardiac malformations historically resulting in significant morbidity and mortality. We sought to assess whether current surgical strategies have improved the outcomes of patients with RAI.

Methods

A retrospective review of our database from 1997 to 2010 identified 60 consecutive patients with RAI who underwent initial palliation at Mt. Fuji Shizuoka Children's Hospital. All of the patients had a functional single ventricle. Of the 60 patients, 33 patients (51.7%) had obstructed total anomalous pulmonary venous drainage (TAPVD), 23 patients (40.3%) had significant (more than moderate) atrioventricular valvular regurgitation (AVVR), 7 patients (12.3%) had hiatus hernia, and 4 patients (6.7%) had major aortopulmonary collateral arteries. Pulmonary outflow atresia was present in 39 of the patients (65%), and 46 patients had systemic-to-pulmonary artery shunts. Of the 33 patients with TAPVD, 18 underwent repair of the condition at initial palliation, 9 others at the time of a Glenn operation, 2 more through a Fontan operation, and 4 at the interstage between palliative surgeries. Eight of the 23 patients with significant AVVR underwent atrioventricular valve repair at initial palliation, 8 others at the time of Glenn operation, 4 others at the time of Fontan operation, and 3 during the interstage between operations. An initial neonatal surgical procedure was performed in 30 patients (50%). The mean follow-up period for patients in the study was 53.1 months.

Results

Operative mortality after initial palliation was 15.4% (4 of 26 patients) before 2003 and 17.6% (6 of 34 patients) after 2004 (p = NS). Five-year survival was 53.8% before 2003 and 81.7% after 2004 (p = 0.035, log-rank test). A multivariate analysis identified persistent AVVR of more than moderate degree (p = 0.04) as a factor associated with late mortality.

Conclusions

The outcomes of surgery for RAI are improving. Neonatal palliative surgery for RAI carries a high operative risk of early mortality, and persistent significant AVVR remains a risk factor for late mortality.

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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