Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot

Mark A Law; Andrew C Glatz; Jennifer C Romano; Paul J Chai; Christopher E Mascio; Christopher J Petit; Courtney E McCracken; Michael S Kelleman; George T Nicholson; Jeffery J Meadows; Jeffrey D Zampi; Shabana Shahanavaz; Sarosh P Batlivala; Joelle Pettus; Amy L Pajk; Kristal M Hock; Bryan H Goldstein; Athar M Qureshi; Congenital Cardiac Research Collaborative (CCRC) Investigators

doi:10.1007/s00246-022-02886-0

Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot

Pediatr Cardiol. 2022 Oct;43(7):1587-1598. doi: 10.1007/s00246-022-02886-0. Epub 2022 Apr 5.

Authors

Mark A Law¹, Andrew C Glatz², Jennifer C Romano³, Paul J Chai⁴, Christopher E Mascio², Christopher J Petit⁵, Courtney E McCracken⁴, Michael S Kelleman⁴, George T Nicholson⁶, Jeffery J Meadows⁷, Jeffrey D Zampi³, Shabana Shahanavaz⁸, Sarosh P Batlivala⁸, Joelle Pettus⁴, Amy L Pajk⁸, Kristal M Hock⁹, Bryan H Goldstein^{8

10}, Athar M Qureshi¹¹; Congenital Cardiac Research Collaborative (CCRC) Investigators

Collaborators

Congenital Cardiac Research Collaborative (CCRC) Investigators:
Lindsay F Eilers, Hala Q Khan, Justin D Smith, Ivor B Asztalos, Alicia M Kamsheh, R Allen Ligon, Sarina Juma, Stephan Juergensen, George T Nicholson, Fatuma Ayann Rinderknecht, Taylor C Merritt, Matthew Candor, Amy L Pajk, Steven J Healan

Affiliations

¹ Department of Pediatrics, University of Alabama at Birmingham, 1700 6th Ave S, Suite 9100, Birmingham, AL, 35233, USA. mlaw@uabmc.edu.
² Cardiac Center, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
³ C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, Atlanta, USA.
⁴ Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA.
⁵ Columbia University Irving Medical Center, New York, NY, USA.
⁶ Division of Cardiology, Monroe Carrell Jr. Children's Hospital, Vanderbilt University School of Medicine, Nashville, TN, USA.
⁷ Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA.
⁸ Department of Pediatrics, Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, OH, USA.
⁹ Department of Pediatrics, University of Alabama at Birmingham, 1700 6th Ave S, Suite 9100, Birmingham, AL, 35233, USA.
¹⁰ Department of Pediatrics, The Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburg School of Medicine, Pittsburgh, PA, USA.
¹¹ Department of Pediatrics, Lillie Frank Abercrombie Section on Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.

PMID: 35381860
DOI: 10.1007/s00246-022-02886-0

Abstract

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005-2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18 months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5 kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < - 2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18 months (HR = 0.87, 95% CI = 0.63-1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93-4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36-3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI.

Keywords: Blalock–Taussig–Thomas Shunt; Congenital heart disease; Right ventricle outflow tract intervention; Tetralogy of Fallot.

Publication types

Multicenter Study

MeSH terms

Blalock-Taussig Procedure*
Humans
Infant
Infant, Newborn
Palliative Care
Pulmonary Artery / surgery
Retrospective Studies
Tetralogy of Fallot*
Treatment Outcome