Improving Outcomes of the Surgical Management of Right Atrial Isomerism

摘要

Study DesignPatientsOperative ProcedureOperative Procedure for TAPVD RepairOperative Procedure for Atrioventricular Valve RepairStatistical AnalysisResultsPatientsSurgical TreatmentOperative Mortality With Initial Palliative OperationFollow-UpFactors Associated With Initial Operative MortalityFactors Associated With Late MortalityCommentFactors Associated With OutcomesCommon Atrioventricular Valve RepairRepair of TAPVDStudy LimitationsDiscussionReferencesPatients 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.MethodsA 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.ResultsOperative 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.ConclusionsThe 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.CTSNet classification:21Pediatric Cardiac SurgeryThe Annals of Thoracic Surgery CME Program is located online at http://cme.ctsnetjournals.org. To take the CME activity related to this article, you must have either an STS member or an individual non-member subscription to the journal.Visceral heterotaxy syndromes are characterized by abnormal development of left- or right-sided structures. Although in heterotaxy there are distinct anatomic abnormalities in the lungs and various intra-abdominal organs, cardiac anomalies largely dictate the long-term outcome [