Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

Christopher J. Petit; Andrew C. Glatz; Andrew B. Goldstone; Mark A. Law; Jennifer C. Romano; Shiraz A. Maskatia; Paul J. Chai; Jeffrey D. Zampi; Jeffery J. Meadows; George T. Nicholson; Shabana Shahanavaz; Athar M. Qureshi; Courtney E. McCracken; Christopher E. Mascio; Sarosh P. Batlivala; Ivor B. Asztalos; Steven J. Healan; Justin D. Smith; Joelle A. Pettus; Asaad Beshish; James E.B. Raulston; Krissie M. Hock; Amy L. Pajk; Bryan H. Goldstein

doi:10.1016/j.jacc.2023.05.051

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

Christopher J. Petit, Andrew C. Glatz, Andrew B. Goldstone, Mark A. Law, Jennifer C. Romano, Shiraz A. Maskatia, Paul J. Chai, Jeffrey D. Zampi, Jeffery J. Meadows, George T. Nicholson, Shabana Shahanavaz, Athar M. Qureshi, Courtney E. McCracken, Christopher E. Mascio, Sarosh P. Batlivala, Ivor B. Asztalos, Steven J. Healan, Justin D. Smith, Joelle A. Pettus, Asaad BeshishJames E.B. Raulston, Krissie M. Hock, Amy L. Pajk, Bryan H. Goldstein

Research output: Contribution to journal › Article › peer-review

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Abstract

Background: Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. Objectives: The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Methods: Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. Results: We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Conclusions: Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

Original language	English
Pages (from-to)	615-627
Number of pages	13
Journal	Journal of the American College of Cardiology
Volume	82
Issue number	7
DOIs	https://doi.org/10.1016/j.jacc.2023.05.051
State	Published - Aug 15 2023

Keywords

congenital
pulmonary artery
tetralogy

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10.1016/j.jacc.2023.05.051

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Petit, C. J., Glatz, A. C., Goldstone, A. B., Law, M. A., Romano, J. C., Maskatia, S. A., Chai, P. J., Zampi, J. D., Meadows, J. J., Nicholson, G. T., Shahanavaz, S., Qureshi, A. M., McCracken, C. E., Mascio, C. E., Batlivala, S. P., Asztalos, I. B., Healan, S. J., Smith, J. D., Pettus, J. A., ... Goldstein, B. H. (2023). Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot. Journal of the American College of Cardiology, 82(7), 615-627. https://doi.org/10.1016/j.jacc.2023.05.051

@article{c89b4ff85bcb42c0a69884556a76d84d,

title = "Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot",

abstract = "Background: Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. Objectives: The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Methods: Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. Results: We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Conclusions: Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.",

keywords = "congenital, pulmonary artery, tetralogy",

author = "Petit, {Christopher J.} and Glatz, {Andrew C.} and Goldstone, {Andrew B.} and Law, {Mark A.} and Romano, {Jennifer C.} and Maskatia, {Shiraz A.} and Chai, {Paul J.} and Zampi, {Jeffrey D.} and Meadows, {Jeffery J.} and Nicholson, {George T.} and Shabana Shahanavaz and Qureshi, {Athar M.} and McCracken, {Courtney E.} and Mascio, {Christopher E.} and Batlivala, {Sarosh P.} and Asztalos, {Ivor B.} and Healan, {Steven J.} and Smith, {Justin D.} and Pettus, {Joelle A.} and Asaad Beshish and Raulston, {James E.B.} and Hock, {Krissie M.} and Pajk, {Amy L.} and Goldstein, {Bryan H.}",

note = "Publisher Copyright: {\textcopyright} 2023 American College of Cardiology Foundation",

year = "2023",

month = aug,

day = "15",

doi = "10.1016/j.jacc.2023.05.051",

language = "English",

volume = "82",

pages = "615--627",

journal = "Journal of the American College of Cardiology",

issn = "0735-1097",

number = "7",

}

Petit, CJ, Glatz, AC, Goldstone, AB, Law, MA, Romano, JC, Maskatia, SA, Chai, PJ, Zampi, JD, Meadows, JJ, Nicholson, GT, Shahanavaz, S, Qureshi, AM, McCracken, CE, Mascio, CE, Batlivala, SP, Asztalos, IB, Healan, SJ, Smith, JD, Pettus, JA, Beshish, A, Raulston, JEB, Hock, KM, Pajk, AL & Goldstein, BH 2023, 'Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot', Journal of the American College of Cardiology, vol. 82, no. 7, pp. 615-627. https://doi.org/10.1016/j.jacc.2023.05.051

TY - JOUR

T1 - Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

AU - Petit, Christopher J.

AU - Glatz, Andrew C.

AU - Goldstone, Andrew B.

AU - Law, Mark A.

AU - Romano, Jennifer C.

AU - Maskatia, Shiraz A.

AU - Chai, Paul J.

AU - Zampi, Jeffrey D.

AU - Meadows, Jeffery J.

AU - Nicholson, George T.

AU - Shahanavaz, Shabana

AU - Qureshi, Athar M.

AU - McCracken, Courtney E.

AU - Mascio, Christopher E.

AU - Batlivala, Sarosh P.

AU - Asztalos, Ivor B.

AU - Healan, Steven J.

AU - Smith, Justin D.

AU - Pettus, Joelle A.

AU - Beshish, Asaad

AU - Raulston, James E.B.

AU - Hock, Krissie M.

AU - Pajk, Amy L.

AU - Goldstein, Bryan H.

PY - 2023/8/15

Y1 - 2023/8/15

N2 - Background: Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. Objectives: The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Methods: Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. Results: We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Conclusions: Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

AB - Background: Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. Objectives: The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Methods: Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. Results: We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Conclusions: Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

KW - congenital

KW - pulmonary artery

KW - tetralogy

UR - http://www.scopus.com/inward/record.url?scp=85166076349&partnerID=8YFLogxK

U2 - 10.1016/j.jacc.2023.05.051

DO - 10.1016/j.jacc.2023.05.051

M3 - Article

C2 - 37558375

AN - SCOPUS:85166076349

SN - 0735-1097

VL - 82

SP - 615

EP - 627

JO - Journal of the American College of Cardiology

JF - Journal of the American College of Cardiology

IS - 7

ER -

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

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