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An extreme case of cor triatriatum mimicking hypoplastic left heart syndrome and combined pulmonary vein stenosis

Published online by Cambridge University Press:  29 November 2023

Hee Ju Hong ,
Hye Won Kwon Open the ORCID record for Hye Won Kwon [Opens in a new window] ,
Jae Gun Kwak ,
Sang Yun Lee  and
Yoon Seong Lee
Hee Ju Hong
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, South Korea
Hye Won Kwon*
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, South Korea
Jae Gun Kwak
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, South Korea
Sang Yun Lee
Affiliation:
Department of Pediatrics, Seoul National University Hospital, Seoul, South Korea
Yoon Seong Lee
Affiliation:
Department of Radiology, Seoul National University Hospital, Seoul, South Korea
*
Corresponding author: Hye Won Kwon; Email: drhwkwon@snu.ac.kr
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Abstract

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

Keywords

Cor triatriatumpulmonary vein stenosisangioplastyballoon
Type
Brief Report
Information
Cardiology in the Young , Volume 34 , Issue 1 , January 2024 , pp. 205 - 208
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Copyright
© The Author(s), 2023. Published by Cambridge University Press

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References

Patel, JD, Briones, M, Mandhani, M, et al. Systemic sirolimus therapy for infants and children with pulmonary vein stenosis. J Am Coll Cardiol 2021; 77: 2807–2818.CrossRefGoogle ScholarPubMed
Saxena, P, Burkhart, HM, Schaff, HV, Daly, R, Joyce, LD, Dearani, JA. Surgical repair of cor triatriatum sinister: the Mayo Clinic 50-year experience. Ann Thorac Surg 2014; 97: 1659–1663.CrossRefGoogle Scholar
Kim, D, Kwon, BS, Kim, DH, Choi, ES, Yun, TJ, Park, CS. Surgical outcomes of cor triatriatum sinister: a single-center experience. J Chest Surg 2022; 55: 151–157.CrossRefGoogle ScholarPubMed
Song, MK, Bae, EJ, Jeong, SI, et al. Clinical characteristics and prognostic factors of primary pulmonary vein stenosis or atresia in children. Ann Thorac Surg 2013; 95: 229–234.CrossRefGoogle ScholarPubMed
Pazos-López, P, García-Rodríguez, C, Guitián-González, A, et al. Pulmonary vein stenosis: etiology, diagnosis and management. World J Cardiol 2016; 8: 81–88.CrossRefGoogle ScholarPubMed
Herlong, JR, Jaggers, JJ, Ungerleider, RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies. Ann Thorac Surg 2000; 69: S56–S69.CrossRefGoogle ScholarPubMed
Yoon, JK, Kim, GB, Song, MK, et al. Hybrid pulmonary vein stenting in infants with refractory to surgical pulmonary vein stenosis repair. Pediatr Cardiol 2018; 39: 1642–1649.CrossRefGoogle ScholarPubMed
Bharat, A, Epstein, DJ, Grady, M, et al. Lung transplant is a viable treatment option for patients with congenital and acquired pulmonary vein stenosis. J Heart Lung Transplant 2013; 32: 621–625.CrossRefGoogle ScholarPubMed
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