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Aortopulmonary window, aortic arch interruption, and anomalous origin of the right pulmonary artery in a neonate with Goldenhar syndrome

Published online by Cambridge University Press:  18 October 2022

Kaitlyn Foreman*
Affiliation:
USC Keck School of Medicine, LAC+USC Medical Center, Los Angeles, CA, USA
Lindsey Miller
Affiliation:
USC Keck School of Medicine, LAC+USC Medical Center, Los Angeles, CA, USA Department of Pediatrics, LAC+USC Medical Center, Los Angeles, CA, USA
S. Ram Kumar
Affiliation:
USC Keck School of Medicine, LAC+USC Medical Center, Los Angeles, CA, USA Heart Institute, Children’s Hospital Los Angeles, Los Angeles, CA, USA
Merujan Uzunyan
Affiliation:
USC Keck School of Medicine, LAC+USC Medical Center, Los Angeles, CA, USA Department of Pediatrics, LAC+USC Medical Center, Los Angeles, CA, USA
*
Author for correspondence: Kaitlyn Foreman, USC Keck School of Medicine, LAC+USC Medical Center, 1975 Zonal Avenue, Los Angeles, CA 90033, USA. E-mail: kmforeman16@gmail.com

Abstract

The combination of aortopulmonary window, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the ascending aorta is a rare and complex congenital cardiac malformation. Despite good prenatal care in our case, this cardiac anomaly was not detected prior to birth. Untreated infants who do not undergo surgical correction have a mortality rate of 70% in their first year, and 30% will die within the first 3 months of life.

Type
Brief Report
Copyright
© The Author(s), 2022. Published by Cambridge University Press

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References

Berry, TE, Bharati, S, Muster, AJ, et al. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol 1982; 49: 108116.CrossRefGoogle ScholarPubMed
Carrel, T, Pfammatter, JP. Interrupted aortic arch, aorto-pulmonary window and aortic origin of the right pulmonary artery: single stage repair in the neonate. Eur J Cardiothrac Surg 1997; 12: 668680.CrossRefGoogle ScholarPubMed
Hu, R, Zhang, W, Liu, X, Dong, W, Zhu, H, Zhang, H. Current outcomes of one-stage surgical correction for Berry syndrome. Thorac Cardiovasc Surg 2017; 153: 11391147.CrossRefGoogle ScholarPubMed
Kutsche, LM, Van Mierop, LHS. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol 1987; 59: 443447.CrossRefGoogle ScholarPubMed
Kutsche, LM, Van Mierop, LHS. Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis. Am J Cardiol 1988; 61: 850856.CrossRefGoogle ScholarPubMed
Kumar, SR. A potential window into surgical outcomes for Berry syndrome. J Thorac Cardiovasc Surg 2017; 153: 11481149.CrossRefGoogle ScholarPubMed
Fontana, GP, Spach, MS, Effmann, EL, Sabiston, DC. Origin of the right pulmonary artery from the ascending aorta. Ann Surg 1987; 206: 102113.CrossRefGoogle ScholarPubMed
Digilio, MC, Calzolari, F, Capolino, R, et al. Congenital heart defects in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome). Am J Med Genet A 2008; 146: 18151819.CrossRefGoogle Scholar