• Jeffrey D Zampi, Jennifer C Hirsch-Romano, and Aimee K Armstrong.
• Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.
• World J Pediatr Congenit Heart Surg. 2013 Oct 1;4(4):367-72.

BackgroundIn the early postoperative period after stage II palliation, patients with single ventricle physiology can have cyanosis due to a variety of causes. This cyanosis can be significant and necessitate cardiac catheterization to determine etiology and attempt treatment. Our objective was to determine the etiology of early postoperative cyanosis and outcomes in patients referred to the catheterization laboratory from the cardiac intensive care unit (CICU) after stage II palliation.MethodsWe performed a retrospective analysis of all patients referred for cardiac catheterization from the CICU for evaluation of early cyanosis after stage II palliation. Etiology for hypoxemia, treatment strategy, and patient outcomes were examined for each patient.ResultsBetween January 1, 2006, and December 31, 2011, 244 patients underwent stage II palliation of which 22 required cardiac catheterization during the early postoperative period because of severe cyanosis. The etiologies for cyanosis were venovenous collaterals (n = 12), cavopulmonary pathway thrombosis (n = 3), hemi-Fontan pathway baffle leak (n = 2), and undetermined (n = 5). Overall, transplant-free survival to hospital discharge was 50% and survival to hospital discharge with stage II physiology was 32%. Venovenous collateral occlusion, cavopulmonary anastomosis takedown, and addition of a second source of pulmonary blood flow were not associated with improved outcome.ConclusionsRegardless of the etiology or treatment strategy, severe cyanosis in the early postoperative period after stage II palliation imparts high mortality and usually indicates failing stage II physiology. Venovenous collateral occlusion and thrombectomy are usually futile, and those who survive have a low likelihood of having stage II physiology at hospital discharge.

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