Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.

TitleImpact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.
Publication TypeJournal Article
Year of Publication2021
AuthorsGoldstein BH, Qureshi AM, Meadows JJ, Nicholson GT, Bauser-Heaton H, Petit CJ, Pater C, Kelleman MS, Morales DLS, Mery CM, Shashidharan S, Mascio CE, Mozumdar N, Aggarwal V, Agrawal H, R Ligon A, Christensen J, McCracken CE, Glatz AC
JournalPediatr Cardiol
Date Published2021 Jan 04
ISSN1432-1971
Abstract

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.

DOI10.1007/s00246-020-02511-y
Alternate JournalPediatr Cardiol
PubMed ID33394118