Abstract

Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions. Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not. Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P < .001). Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted.

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