CONGENITAL HEART DISEASE WITH DOUBLE-OUTLET RIGHT VENTRICLE IN AN ADULT: A RARE CASE
Main Article Content
Abstract
- Background: Double outlet right ventricle (DORV) is a rare congenital heart defect, usually diagnosed in childhood. We report an unrepaired DORV case persisting into adulthood. 2. Case presentation: A 30-year-old patient was admitted with hemoptysis. Imaging revealed DORV with subpulmonary ventricular septal defect, a large VSD (~22 mm), severe pulmonary arterial hypertension, and associated lung disease. 3. Conclusion: This case highlights the complex interaction between congenital cardiac anatomy, chronic pulmonary arterial hypertension, and underlying lung pathology. Despite survival into adulthood, severe complications had developed, emphasizing the importance of comprehensive cardiopulmonary evaluation and multidisciplinary management.
Keywords
Double outlet right ventricle, congenital heart disease, pulmonary arterial hypertension.
Article Details
References
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Published 2023 Sep 25. doi:10.3389/fped.2023.1244558
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7. Baroutidou A, Arvanitaki A, Hatzidakis A, et al. Haemoptysis in Pulmonary Arterial Hypertension Associated with
Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management. J Clin Med. 2022;11(3):633.
Published 2022 Jan 26. doi:10.3390/jcm11030633
8. Neufeld HN, Lucas RV JR, Lester RG, et al. Origin of both great vessels from the right ventricle without pulmonary
stenosis. Br Heart J. 1962;24(4):393-408. doi:10.1136/hrt.24.4.393
9. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563-645. doi:10.1093/eurheartj/ehaa554
10. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital
Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical
Practice Guidelines. J Am Coll Cardiol. 2019;73(12):e81-e192. doi:10.1016/j.jacc.2018.08.1029
2. Zhao M, Liu J, Xin M, et al. Pulmonary arterial hypertension associated with congenital heart disease: An omics
study. Front Cardiovasc Med. 2023;10:1037357. Published 2023 Mar 10. doi:10.3389/fcvm.2023.1037357.
3. Bell-Cheddar Y, Devine WA, Diaz-Castrillon CE, et al. Double outlet right ventricle. Front Pediatr. 2023;11:1244558.
Published 2023 Sep 25. doi:10.3389/fped.2023.1244558
4. Okabe M, Sasaguri M, Ohba H, et al. A rare adult case of double-outlet right ventricle without pulmonary stenosis:
an autopsy case. Kokyu To Junkan. 1990;38(12):1253-1258.
5. Khalil I, Abrar S, Hossain MI. Unraveling the complexities of double outlet right ventricle (DORV): A rare case of congenital heart disease with bidirectional shunting in a young girl. Radiol Case Rep. 2025;20(8):3930-3937. Published 2025 May 22. doi:10.1016/j.radcr.2025.04.075
6. Wu QY, Li DH, Li HY, et al. Surgical Treatment of Double Outlet Right Ventricle Complicated by Pulmonary Hypertension. Chin Med J (Engl). 2017;130(4):409-413. doi:10.4103/0366-6999.199827
7. Baroutidou A, Arvanitaki A, Hatzidakis A, et al. Haemoptysis in Pulmonary Arterial Hypertension Associated with
Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management. J Clin Med. 2022;11(3):633.
Published 2022 Jan 26. doi:10.3390/jcm11030633
8. Neufeld HN, Lucas RV JR, Lester RG, et al. Origin of both great vessels from the right ventricle without pulmonary
stenosis. Br Heart J. 1962;24(4):393-408. doi:10.1136/hrt.24.4.393
9. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563-645. doi:10.1093/eurheartj/ehaa554
10. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital
Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical
Practice Guidelines. J Am Coll Cardiol. 2019;73(12):e81-e192. doi:10.1016/j.jacc.2018.08.1029