{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T05:45:34Z","timestamp":1788587134219,"version":"build-2803163510"},"reference-count":29,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T00:00:00Z","timestamp":1718841600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"University of Medicine, Pharmacy, Science and Technology \u201cGeorge Emil Palade\u201d of T\u00e2rgu Mure\u0219 Research","award":["10126\/4\/17.12.2020"],"award-info":[{"award-number":["10126\/4\/17.12.2020"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Raghib syndrome is a rare malformation complex consisting of the drainage of the left superior vena cava (LSVC) into the left atrium, ostial atresia of the coronary sinus and an atrial septal defect (ASD). Case Report: This report aims to present the case of a child newly diagnosed with Raghib syndrome, complicated by pulmonary arterial hypertension, and to review previously published cases with the same diagnosis. A six-year-old female patient presented with signs and symptoms of heart failure (Ross III), reduced exercise tolerance and severe delay in stature and ponderal development. The imagistic work-up included echocardiography, followed by computer tomography (CT) and magnetic resonance imaging (MRI), through which a diagnosis of Raghib syndrome was established, complicated by pulmonary hypertension. As in other cases presented in the literature, MRI allowed for an accurate diagnosis, detecting the absent coronary sinus. The decision regarding the surgical closure of the ASD was made, with the patient having a favorable clinical evolution but with the persistence of elevated pulmonary artery pressure, for which Sildenafil therapy was instituted. Conclusions: The malformation complex consisting of an atrial septal defect, ostium atresia of the coronary sinus, uncovered coronary sinus, and persistent left superior vena cava, as identified through multiple imagistic investigations, was suggestive of the rare diagnosis of Raghib syndrome in this case. Among the limited number of cases of Raghib syndrome available in the literature, the present case is distinguished by the severity of the pulmonary artery hypertension at a very young age and in the absence of other concurrent cardiac malformations.<\/jats:p>","DOI":"10.3390\/jcm13123623","type":"journal-article","created":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T11:42:03Z","timestamp":1718883723000},"page":"3623","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Raghib Syndrome and Pulmonary Arterial Hypertension in a Pediatric Patient: Case Report and Literature Review"],"prefix":"10.3390","volume":"13","author":[{"given":"Liliana","family":"Gozar","sequence":"first","affiliation":[{"name":"Department of Pediatrics 3, \u201cGeorge Emil Palade\u201d University of Medicine, Pharmacy, Sciences and Technology from T\u00e2rgu Mure\u0219, Gheorghe Marinescu Street No. 38, 540136 T\u00e2rgu Mure\u0219, Romania"},{"name":"Pediatric Cardiology Clinic, Emergency Institute for Cardiovascular Diseases and Transplantation, Gheorghe Marinescu Street No. 50, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8156-7460","authenticated-orcid":false,"given":"Maria Oana","family":"S\u0103s\u0103ran","sequence":"additional","affiliation":[{"name":"Department of Pediatrics 3, \u201cGeorge Emil Palade\u201d University of Medicine, Pharmacy, Sciences and Technology from T\u00e2rgu Mure\u0219, Gheorghe Marinescu Street No. 38, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius C\u0103t\u0103lin","family":"Cosma","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Clinic, Emergency Institute for Cardiovascular Diseases and Transplantation, Gheorghe Marinescu Street No. 50, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Toma","sequence":"additional","affiliation":[{"name":"Department of Pediatrics 3, \u201cGeorge Emil Palade\u201d University of Medicine, Pharmacy, Sciences and Technology from T\u00e2rgu Mure\u0219, Gheorghe Marinescu Street No. 38, 540136 T\u00e2rgu Mure\u0219, Romania"},{"name":"Pediatric Cardiology Clinic, Emergency Institute for Cardiovascular Diseases and Transplantation, Gheorghe Marinescu Street No. 50, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9295-807X","authenticated-orcid":false,"given":"Andreea Georgiana","family":"Nan","sequence":"additional","affiliation":[{"name":"First Department of Psychiatry, Clinical County Hospital Mure\u0219, Gheorghe Marinescu Street No. 38, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Horea","family":"Gozar","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, \u201cGeorge Emil Palade\u201d University of Medicine, Pharmacy, Sciences and Technology from T\u00e2rgu Mure\u0219, Gheorghe Marinescu Street No. 38, 540136 T\u00e2rgu Mure\u0219, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,20]]},"reference":[{"key":"ref_1","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; a developmental complex","volume":"31","author":"Raghib","year":"1965","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1514","DOI":"10.1007\/s00246-012-0620-1","article-title":"Chronic Cyanosis Due to Persistent Left Superior Vena Cava Draining into the Left Atrium in the Absence of a Coronary Sinus","volume":"34","author":"Singh","year":"2013","journal-title":"Pediatr. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"553","DOI":"10.21037\/qims-22-465","article-title":"Prenatal Diagnosis of a Left Superior Vena Cava Draining into the Left Atrium and Atrial Septal Defect with Ventricular Septal Defect","volume":"13","author":"Shen","year":"2023","journal-title":"Quant. Imaging Med. Surg."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1186\/s13244-020-00906-2","article-title":"Persistent Left Superior Vena Cava: Clinical Importance and Differential Diagnoses","volume":"11","author":"Azizova","year":"2020","journal-title":"Insights Imaging"},{"key":"ref_5","first-page":"921247","article-title":"Raghib Syndrome Presenting as a Cryptogenic Stroke: Role of Cardiac MRI in Accurate Diagnosis","volume":"2015","author":"Daruwalla","year":"2015","journal-title":"Case Rep. Cardiol."},{"key":"ref_6","first-page":"1824","article-title":"Impact of Persistent Left Superior Vena Cava on Radiofrequency Catheter Ablation in Patients with Atrial Fibrillation","volume":"21","author":"Kim","year":"2019","journal-title":"EP Eur."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1002\/uog.2704","article-title":"Prenatal Diagnosis of Persistent Left Superior Vena Cava and Its Associated Congenital Anomalies","volume":"27","author":"Berg","year":"2006","journal-title":"Ultrasound Obstet. Gynecol."},{"key":"ref_8","first-page":"bcr-2013","article-title":"Case Report: Persistent Left Superior Vena Cava Draining into the Left Atrium","volume":"2013","author":"Tobbia","year":"2013","journal-title":"BMJ Case Rep."},{"key":"ref_9","first-page":"e13041","article-title":"A Rare Case of an Unroofed Coronary Sinus With a Persistent Left Superior Vena Cava Diagnosed by Two-Dimensional Transthoracic Echocardiography","volume":"13","author":"Khadkikar","year":"2021","journal-title":"Cureus"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/S0022-5223(19)38083-3","article-title":"Coronary Sinus Septal Defect. Surgical Considerations","volume":"78","author":"Lee","year":"1979","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1007\/BF02469635","article-title":"Raghib\u2019s Syndrome: A Report of Two Cases","volume":"12","author":"Okumori","year":"1982","journal-title":"Jpn. J. Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e159","DOI":"10.1016\/j.jcin.2020.04.057","article-title":"Percutaneous Repair of Raghib Syndrome","volume":"13","author":"Wang","year":"2020","journal-title":"JACC Cardiovasc. Interv."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1177\/2150135113515933","article-title":"Cortriatriatum with Classical Raghib Complex: A Rare Anatomic Association","volume":"5","author":"Nair","year":"2014","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1007\/s11748-019-01159-7","article-title":"Cor Triatriatum with Raghib Complex in Partial Atrioventricular Septal Defect and Common Atrium: A Rare Combination","volume":"68","author":"Baek","year":"2020","journal-title":"Gen. Thorac. Cardiovasc. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1186\/s43044-021-00183-4","article-title":"Cor Triatriatum with Supramitral Ring: \u201cCor Tetratriatum\u201d, Associated with Raghib Syndrome with Eisenmenger Syndrome: Multimodality Imaging Approach in This Exceedingly Rare Case Report","volume":"73","author":"Kumar","year":"2021","journal-title":"Egypt. Heart J."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1177\/2150135116673017","article-title":"An Unusual Case of Raghib Defect and Double-Orifice Tricuspid Valve","volume":"9","author":"Egbe","year":"2018","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"suac121.187","DOI":"10.1093\/eurheartjsupp\/suac121.187","article-title":"540 RAGHIB SYNDROME WITH VERY LATE CLINICAL PRESENTATION","volume":"24","author":"Frecentese","year":"2022","journal-title":"Eur. Heart J. Suppl."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/j.recesp.2015.04.002","article-title":"Raghib Syndrome. Surgical Treatment","volume":"69","year":"2016","journal-title":"Rev. Esp. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1016\/j.ijcard.2012.11.113","article-title":"Coronary Sinus Septal Defect (Unroofed Coronary Sinus): Echocardiographic Diagnosis and Surgical Treatment","volume":"168","author":"Xie","year":"2013","journal-title":"Int. J. Cardiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1007\/s10554-010-9670-8","article-title":"MR Findings of a Rare Defect, Coronary Sinus ASD","volume":"28","author":"Chaturvedi","year":"2012","journal-title":"Int. J. Cardiovasc. Imaging"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1016\/j.ejrad.2011.11.042","article-title":"Cardiac CT Angiography in Children with Congenital Heart Disease","volume":"82","author":"Siripornpitak","year":"2013","journal-title":"Eur. J. Radiol."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Kakucs, Z., Heidenhoffer, E., and Pop, M. (2022). Detection of Coronary Artery and Aortic Arch Anomalies in Patients with Tetralogy of Fallot Using CT Angiography. J. Clin. Med., 11.","DOI":"10.3390\/jcm11195500"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"3946","DOI":"10.21037\/jtd.2017.09.03","article-title":"CT Angiography for Diagnosis and Subcategorization of Unroofed Coronary Sinus Syndrome","volume":"9","author":"Zhi","year":"2017","journal-title":"J. Thorac. Dis."},{"key":"ref_24","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_25","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.jus.2012.06.001","article-title":"Unroofed Coronary Sinus and Persistent Left Superior Vena Cava: A Case Report","volume":"15","author":"Bonardi","year":"2012","journal-title":"J. Ultrasound"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"S2953","DOI":"10.21037\/jtd.2018.08.92","article-title":"Atrial Septal Defects and Pulmonary Arterial Hypertension","volume":"10","author":"Nashat","year":"2018","journal-title":"J. Thorac. Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1093\/ehjci\/jet261","article-title":"Persistent Left Superior Vena Cava Draining into Left Atrium, Unroofed Coronary Sinus, and Coronary Sinus Atrial Septal Defect as Rare Cause for Severe Right Heart Dilatation: Diagnostic Steps and Therapy","volume":"15","author":"Speiser","year":"2014","journal-title":"Eur. Heart J.\u2014Cardiovasc. Imaging"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1002\/j.2205-0140.2012.tb00228.x","article-title":"Persistent Left Superior Vena Cava\u2014Considerations in Fetal, Pediatric and Adult Populations","volume":"15","author":"Saha","year":"2012","journal-title":"Australas. J. Ultrasound Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1111\/jocs.13240","article-title":"Correction of Cortriatriatum Sinister with Classical Raghib\u2019s Complex Using an Extracardiac Conduit","volume":"32","author":"Ak","year":"2017","journal-title":"J. Card. Surg."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/12\/3623\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:01:53Z","timestamp":1760108513000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/12\/3623"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,20]]},"references-count":29,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2024,6]]}},"alternative-id":["jcm13123623"],"URL":"https:\/\/doi.org\/10.3390\/jcm13123623","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,20]]}}}