Ann Thorac Surg. 2024 Aug 3:S0003-4975(24)00635-0. doi: 10.1016/j.athoracsur.2024.07.021. Online ahead of print.
ABSTRACT
BACKGROUND: The outcomes of single ventricle palliation in unbalanced atrioventricular canal defect with coarctation of aorta (uAVC+CoA) have not been well studied. These patients have a propensity to develop systemic ventricle outflow tract obstruction following aortic arch repair with pulmonary artery banding (arch-PAB); which may adversely affect survival and Fontan candidacy.
METHODS: A retrospective review was performed of patients who underwent single ventricle palliation for uAVC+CoA from 2000 to 2022. Patients were divided into two groups based on initial palliation – (1) arch-PAB and (2) Norwood procedure. Demographic and clinical characteristics were analyzed and compared, along with survival data.
RESULTS: 41 patients underwent stage 1 palliation for uAVC+CoA. Arch-PAB was performed in 14 infants and Norwood in 27 infants. Arch-PAB patients had more chromosomal abnormalities (28.6 vs 7.4%, p<0.009) and less severe systemic ventricle outflow tract obstruction on baseline echocardiogram (0.0 vs. 70.4%, p<0.001). Survival to stage 3 palliation was lower for the arch-PAB group (28.6 vs. 66.6%, p=0.02). Arch-PAB remained a significant risk factor for mortality (HR 2.93 [CI 1.05-8.53], p=0.04) after adjusting for chromosomal abnormalities and atrioventricular valve regurgitation. Following arch-PAB, systemic ventricle outflow tract obstruction was diagnosed in 13/14 patients. Echocardiography underestimated the degree of outflow tract obstruction in 10/13 arch-PAB patients.
CONCLUSIONS: Arch-PAB has worse outcomes than Norwood for uAVC+CoA. Almost all patients develop systemic ventricle outflow tract obstruction following arch-PAB. Outflow tract obstruction is underestimated by echocardiogram, and requires a high index of suspicion along with advanced imaging to ensure timely diagnosis and management.
PMID:39102934 | DOI:10.1016/j.athoracsur.2024.07.021
