{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T13:25:41Z","timestamp":1771248341366,"version":"3.50.1"},"reference-count":26,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"University of Medicine and Pharmacy and Carol Davila"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Background: An unroofed coronary sinus (UCS) is a rare congenital cardiac anomaly, accounting for less than 1% of atrial septal defects and frequently associated with a persistent left superior vena cava (PLSVC). Its coexistence with Tetralogy of Fallot (TOF) is exceptionally uncommon and has been reported almost exclusively in isolated case reports. Case Presentation: We report the case of a 20-year-old woman with a history of surgically corrected TOF in infancy, who presented with progressive exertional dyspnea. Multimodality imaging, including transthoracic echocardiography and cardiac magnetic resonance imaging, revealed a large atrial-level shunt caused by a type I unroofed coronary sinus associated with a persistent left superior vena cava, leading to significant right-sided chamber dilation and pulmonary hypertension. Notably, this anomaly had not been identified at the time of the initial TOF repair. The patient underwent a successful surgical correction with interatrial compartmentation and tricuspid annuloplasty, with an uneventful postoperative course. Conclusions: This case underscores the extreme rarity of the UCS\u2013TOF association and highlights the potential for UCSs with PLSVC to remain clinically silent for years after complex congenital heart surgery. A comprehensive reassessment of the systemic venous and atrial anatomy using advanced multimodality imaging is essential in symptomatic adults with repaired TOF, as late-presenting venous anomalies may have significant hemodynamic and clinical implications.<\/jats:p>","DOI":"10.3390\/life16020342","type":"journal-article","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T12:37:28Z","timestamp":1771245448000},"page":"342","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Late Diagnosis of Unroofed Coronary Sinus Associated with Persistent Left Superior Vena Cava in Patient with Repaired Tetralogy of Fallot: Case Report"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5374-1924","authenticated-orcid":false,"given":"Oana","family":"Gheorghe-Fronea","sequence":"first","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Cardiology, Clinical Emergency Hospital Bucharest, 014461 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3599-129X","authenticated-orcid":false,"given":"Mircea","family":"Robu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian","family":"Onciul","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Cardiology, Clinical Emergency Hospital Bucharest, 014461 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Nica","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Cardiovascular Surgery, Clinical Emergency Hospital Bucharest, 014461 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristian","family":"Voica","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Clinical Emergency Hospital Bucharest, 014461 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"\u021aig\u0103na\u0219u","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Cardiovascular Surgery, Clinical Emergency Hospital Bucharest, 014461 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5340-8929","authenticated-orcid":false,"given":"Gabriel-Petre","family":"Gorecki","sequence":"additional","affiliation":[{"name":"Department of Anesthesia and Intensive Care, Faculty of Medicine, Titu Maiorescu University, 040051 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2241-279X","authenticated-orcid":false,"given":"Hora\u021biu","family":"Moldovan","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Cardiovascular Surgery, Clinical Emergency Hospital Bucharest, 014461 Bucharest, Romania"},{"name":"Academy of Romanian Scientists, 050711 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,2,16]]},"reference":[{"key":"ref_1","first-page":"1","article-title":"Coronary Sinus Atrial Septal Defect (Unroofed Coronary Sinus) with Total Anomalous Pulmonary Venous Connection\u2014A Case Report","volume":"6","author":"Muthiah","year":"2017","journal-title":"Case Rep. Clin. Med."},{"key":"ref_2","first-page":"1","article-title":"The coronary sinus and its tributaries","volume":"5","year":"1992","journal-title":"Clin. Anat."},{"key":"ref_3","first-page":"92","article-title":"Tetralogy of Fallot associated with cor triatriatum, unroofed coronary sinus, and persistent left superior vena cava","volume":"45","author":"Nomura","year":"1997","journal-title":"J. Jpn. Assoc. Thorac. Surg."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1016\/S0003-4975(10)63339-5","article-title":"Surgical experience with unroofed coronary sinus","volume":"27","author":"Quaegebeur","year":"1979","journal-title":"Ann. Thorac. Surg."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1055\/s-0029-1185336","article-title":"Rare association of tetralogy of Fallot with partially unroofed coronary sinus and persistent left superior vena cava","volume":"58","author":"Jian","year":"2010","journal-title":"Thorac. Cardiovasc. Surg."},{"key":"ref_6","first-page":"35","article-title":"Dextrose 5% contrast echocardiography for diagnosis of unroofed coronary sinus in a child with tetralogy of Fallot","volume":"3","author":"Molaei","year":"2019","journal-title":"Indian Heart J. Cardiovasc. Case Rep."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1161\/01.CIR.31.6.906","article-title":"Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus","volume":"31","author":"Raghib","year":"1965","journal-title":"Circulation"},{"key":"ref_8","first-page":"347.e5","article-title":"Unroofed coronary sinus: Diagnosis by echocardiography and computed tomography","volume":"19","author":"Suresh","year":"2006","journal-title":"J. Am. Soc. Echocardiogr."},{"key":"ref_9","first-page":"342","article-title":"Unroofed coronary sinus: Pathological anatomy, clinical features, and surgical implications","volume":"62","author":"Carrera","year":"1988","journal-title":"Am. J. Cardiol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1111\/echo.12514","article-title":"Persistent left superior vena cava with absent right superior vena cava: Review of the literature and clinical implications","volume":"31","author":"Sheikh","year":"2014","journal-title":"Echocardiography"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1111\/j.1540-8191.2008.00578.x","article-title":"Radical repair of cor triatriatum with partially unroofed coronary sinus and persistent left superior vena cava 35 years after operation for tetralogy of Fallot","volume":"23","author":"Ueno","year":"2008","journal-title":"J. Card. Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/s13244-014-0330-8","article-title":"Computed Tomography and Magnetic Resonance Imaging of the Coronary Sinus: Anatomic Variants and Congenital Anomalies","volume":"5","author":"Chen","year":"2014","journal-title":"Insights Imaging"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"A208","DOI":"10.1136\/heartjnl-2011-300867.609","article-title":"Echocardiagraphic diagnosis of unroofed coronary sinus syndrome","volume":"97","author":"Yali","year":"2011","journal-title":"Heart"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"121","DOI":"10.3348\/kjr.2001.2.3.121","article-title":"MR imaging of congenital heart diseases in adolescents and adults","volume":"2","author":"Choe","year":"2001","journal-title":"Korean J. Radiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1148\/radiology.199.3.8637985","article-title":"Congenital heart disease in adults and adolescents: Comparative value of transthoracic and transesophageal echocardiography and MR imaging","volume":"199","author":"Hoppe","year":"1996","journal-title":"Radiology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"314","DOI":"10.2214\/AJR.17.19128","article-title":"Evaluation of Unroofed Coronary Sinus Syndrome Using Cardiovascular CT Angiography: An Observational Study","volume":"211","author":"Sun","year":"2018","journal-title":"Am. J. Roentgenol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1093\/icvts\/ivt268","article-title":"Evaluation of a tissue-engineered bovine pericardial patch in paediatric patients with congenital cardiac anomalies: Initial experience with the ADAPT-treated CardioCel(R) patch","volume":"17","author":"Neethling","year":"2013","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1148\/rg.324105220","article-title":"Imaging of the coronary sinus: Normal anatomy and congenital abnormalities","volume":"32","author":"Shah","year":"2012","journal-title":"Radiographics"},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Margarint, I.M., Youssef, T., Robu, M., Rotaru, I., Popescu, A., Untaru, O., Filip, C., Stiru, O., Iliescu, V.A., and Vladareanu, R. (2024). The Management of Aortic Coarctation Associated with Hypoplastic Arches and Particular Arch Anatomies: A Literature Review. J. Pers. Med., 14.","DOI":"10.3390\/jpm14070732"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/s10396-015-0648-y","article-title":"Utility of three-dimensional transesophageal echocardiography for diagnosis of unroofed coronary sinus","volume":"43","author":"Kawamukai","year":"2016","journal-title":"J. Med. Ultrason."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1186\/s40792-020-00978-8","article-title":"Minimally invasive surgical repair for unroofed coronary sinus syndrome directed by three-dimensional transesophageal echocardiography","volume":"6","author":"Handa","year":"2020","journal-title":"Surg. Case Rep."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Pojar, M., Timbilla, S., Cerny, S., Karalko, M., and Vojacek, J. (2022). Case report: Robotic repair of unroofed coronary sinus. Front. Cardiovasc. Med., 9.","DOI":"10.3389\/fcvm.2022.974089"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1017\/S1047951114000353","article-title":"Percutaneous management of coronary sinus atrial septal defect: Two cases representing the spectrum for device closure and a review of the literature","volume":"24","author":"Sandeep","year":"2014","journal-title":"Cardiol. Young"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1161\/01.CIR.33.2.317","article-title":"Congenital anomalies involving the coronary sinus","volume":"33","author":"Mantini","year":"1966","journal-title":"Circulation"},{"key":"ref_25","first-page":"518","article-title":"Multimodality imaging guidelines for patients with repaired congenital heart disease: A scientific statement from the American Heart Association","volume":"130","author":"Valente","year":"2014","journal-title":"Circulation"},{"key":"ref_26","first-page":"947","article-title":"Superior vena cava drainage into the left atrium: Radiographic and angiographic features","volume":"146","author":"Buirski","year":"1986","journal-title":"AJR Am. J. Roentgenol."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/16\/2\/342\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T12:49:38Z","timestamp":1771246178000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/16\/2\/342"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,16]]},"references-count":26,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,2]]}},"alternative-id":["life16020342"],"URL":"https:\/\/doi.org\/10.3390\/life16020342","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,16]]}}}