{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T00:10:41Z","timestamp":1767139841123,"version":"build-2065373602"},"reference-count":25,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T00:00:00Z","timestamp":1696982400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left ventricle. The aim of our paper is to describe the morphological and imaging features of DOLV and to assess the prevalence of the associated malformations and their surgical outcomes. METHODS From 2011 to 2022, we retrospectively reviewed the electronic case records of patients diagnosed with DOLV at the Bambino Gesu Children\u2019s Hospital. A systematic search was developed in MEDLINE, Web of Science, and EMBASE databases to identify reports assessing the morphology and outcomes of DOLV between 1975 and 2023. RESULTS: Over a median follow-up of 9.9 years (IQR 7.8\u201311.7 y), four cases of DOLV were identified at our institution. Two patients were diagnosed with (S,D,D) DOLV subaortic VSD and pulmonary stenosis (PS): one patient had (S,D,D) DOLV with doubly committed VSD and hypoplastic right ventricle, and another patient had (S,D,L) DOLV with subaortic VSD and PS (malposition type). Pulmonary stenosis was the most commonly associated lesion (75%). LITERATURE REVIEW: After systematic evaluation, a total of 12 reports fulfilled the eligibility criteria and were included in our analysis. PS or right ventricular outflow tract obstruction was the most commonly associated lesion (69%, 95% CI 62\u201376%). The most common locations of VSD were subaortic (pooled prevalence: 75%, 95% CI 68\u201381), subpulmonary (15%, 95% CI 10\u201321), and doubly committed (7%, 95% CI 4\u201312). The position of the great arteries showed that d-transposition of the aorta was present in 128 cases (59% 95% CI 42\u201374), and l-transposition was present in 77 cases (35%, 95% CI 29\u201343).<\/jats:p>","DOI":"10.3390\/diagnostics13203175","type":"journal-article","created":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T08:07:12Z","timestamp":1697011632000},"page":"3175","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Double-Outlet Left Ventricle: Case Series and Systematic Review of the Literature"],"prefix":"10.3390","volume":"13","author":[{"given":"Michele","family":"Lioncino","sequence":"first","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3187-0829","authenticated-orcid":false,"given":"Giulio","family":"Calcagni","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fausto","family":"Badolato","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Antonelli","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6715-3472","authenticated-orcid":false,"given":"Benedetta","family":"Leonardi","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"de Zorzi","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2611-8061","authenticated-orcid":false,"given":"Aurelio","family":"Secinaro","sequence":"additional","affiliation":[{"name":"Advanced Cardiothoracic Imaging Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianluca","family":"Brancaccio","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonia","family":"Albanese","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3908-5091","authenticated-orcid":false,"given":"Adriano","family":"Carotti","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1888-373X","authenticated-orcid":false,"given":"Fabrizio","family":"Drago","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriele","family":"Rinelli","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology and Cardiac Arrhythmias and Syncope Unit, Bambino Ges\u00f9 Children\u2019s Hospital, IRCSS, 00146 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1161\/01.CIR.41.1.129","article-title":"Double-Outlet Left Ventricle with an Intact Ventricular Septum","volume":"41","author":"Paul","year":"1970","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1136\/hrt.36.6.554","article-title":"Double outlet left ventricle","volume":"36","author":"Anderson","year":"1974","journal-title":"Br. Heart J."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1016\/S0003-4975(99)01281-3","article-title":"Congenital Heart Surgery Nomenclature and Database Project: Double outlet left ventricle","volume":"69","author":"Tchervenkov","year":"2000","journal-title":"Ann. Thorac. Surg."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"e36","DOI":"10.1161\/CIRCULATIONAHA.106.629923","article-title":"Images in cardiovascular medicine. Double-outlet left ventricle","volume":"115","author":"Lilje","year":"2007","journal-title":"Circulation"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1161\/01.CIR.56.2.139","article-title":"Terminology of congenital heart disease. Glossary and commentary","volume":"56","year":"1977","journal-title":"Circulation"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 statement: An updated guideline for reporting systematic reviews","volume":"372","author":"Page","year":"2021","journal-title":"BMJ"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1136\/bmjebm-2017-110853","article-title":"Methodological quality and synthesis of case series and case reports","volume":"23","author":"Murad","year":"2018","journal-title":"BMJ Evid. Based Med."},{"unstructured":"Van Praagh, R., Weinberg, P.M., and Srebro, J. (1989). Double Outlet Left Ventricle. Moss Heart Disease in Infants, Children, and Adolescents, Williams & Wilkins. [1989th ed.].","key":"ref_8"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"311","DOI":"10.2459\/JCM.0000000000000101","article-title":"Current management of double-outlet left ventricle: Towards biventricular repair in infancy","volume":"18","author":"Luciani","year":"2017","journal-title":"J. Cardiovasc. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1161\/01.CIR.58.3.558","article-title":"The morphologic spectrum of double outlet left ventricle and its surgical significance","volume":"58","author":"Bharati","year":"1978","journal-title":"Circulation"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1111\/j.1747-0803.2010.00456.x","article-title":"Characteristics and Outcomes of Double Outlet Left Ventricle","volume":"5","author":"Elmi","year":"2010","journal-title":"Congenit. Hear. Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1016\/0002-9149(76)90803-1","article-title":"Double outlet left ventricle: Morphology, cineangiocardiographic diagnosis and surgical treatment","volume":"38","author":"Brandt","year":"1976","journal-title":"Am. J. Cardiol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/S0003-4975(94)01036-6","article-title":"Alternatives in biventricular repair of double-outlet left ventricle","volume":"60","author":"DeLeon","year":"1995","journal-title":"Ann. Thorac. Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1347","DOI":"10.1016\/S0003-4975(00)02541-8","article-title":"Pulmonary root translocation for biventricular repair of double-outlet left ventricle","volume":"71","author":"Ootaki","year":"2001","journal-title":"Ann. Thorac. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1016\/S0022-5223(97)70204-6","article-title":"Pulmonary root translocation for biventricular repair of double-outlet left ventricle with absent subpulmonic conus","volume":"114","author":"McElhinney","year":"1997","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1865","DOI":"10.1111\/jocs.14768","article-title":"Bi-ventricular repair of double outlet left ventricle: Experience and review of the literature","volume":"35","author":"Raja","year":"2020","journal-title":"J. Card. Surg."},{"key":"ref_17","first-page":"413","article-title":"Double outlet left ventricle: How should we repair it?","volume":"20","author":"Villani","year":"1979","journal-title":"J. Cardiovasc. Surg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/S0003-4975(10)63251-1","article-title":"Surgical Treatment of Double-Outlet Left Ventricle in 2 Patients with d-Position and l-Position of the Aorta","volume":"27","author":"Stegmann","year":"1979","journal-title":"Ann. Thorac. Surg."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1385","DOI":"10.1016\/S0002-9149(84)80113-7","article-title":"Double-outlet left ventricle with atrioventricular discordance","volume":"54","author":"Subirana","year":"1984","journal-title":"Am. J. Cardiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1016\/0003-4975(92)90398-N","article-title":"Arterial correction of double-outlet left ventricle by pulmonary artery translocation","volume":"53","author":"Chiavarelli","year":"1992","journal-title":"Ann. Thorac. Surg."},{"unstructured":"Van Praagh, R. (2022). DOLV: Congenital Heart Disease: A Clinical, Pathological, Embryological and Segmental Analysis, Elsevier.","key":"ref_21"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1177\/2150135116651114","article-title":"Development and Morphology of the Ventricular Outflow Tracts","volume":"7","author":"Anderson","year":"2016","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1390","DOI":"10.1016\/j.amjcard.2016.07.050","article-title":"What Determines Whether the Great Arteries Are Normally or Abnormally Related?","volume":"118","year":"2016","journal-title":"Am. J. Cardiol."},{"key":"ref_24","first-page":"580","article-title":"Taussig-Bing anomaly: From original description to the current era","volume":"36","author":"Konstantinov","year":"2009","journal-title":"Tex. Heart Inst. J."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1016\/S0003-4975(10)60969-1","article-title":"Intraventricular repair of double-outlet left ventricle","volume":"31","author":"Murphy","year":"1981","journal-title":"Ann. Thorac. 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