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Congress: ECR25
Poster Number: C-11671
Type: Poster: EPOS Radiologist (educational)
Authorblock: C. Marin Rodriguez, T. Alvarez Martin, A. Lancharro, L. Vara De Andrés, A. Aguado Del Hoyo, M. Sánchez Pérez, Y. Ruiz Martin; Madrid/ES
Disclosures:
Carlos Marin Rodriguez: Nothing to disclose
Teresa Alvarez Martin: Nothing to disclose
Angel Lancharro: Nothing to disclose
Loreto Vara De Andrés: Nothing to disclose
Alejandra Aguado Del Hoyo: Nothing to disclose
María Sánchez Pérez: Nothing to disclose
Yolanda Ruiz Martin: Nothing to disclose
Keywords: Cardiac, Paediatric, CT, MR, Ultrasound, Computer Applications-3D, Contrast agent-intravenous, Congenital, Haemodynamics / Flow dynamics
Background
            • DORV is an uncommon congenital heart defect, occurring in 0.1% of newborns and representing 1.5% of all congenital heart malformations. It is part of the conotruncal anomalies (malformations of the ventricular outlet) that include
              • Truncus arteriosus and aortopulmonary window
              • Interrupted aortic arch
              • Tetralogy of Fallot (TOF)
              • Transposition of the great arteries
              • DORV
            • The term DORV refers to a group of associated conotruncal anomalies that involve the right ventricular outflow tract, in which both great arteries arise entirely or predominantly from the right ventricle. DORV represents a diverse group of disorders with diverse etiologies including several chromosomal abnormalities, mutations and genetic variants, that suggest different pathogenic mechanisms including neural crest abnormalities and anomalies of situs and loop (1)
            • The theorical principle that no mitroaortic (or mitrolunar) continuity should be present is controversial (2). Currently, the term “DORV” is accepted when more than 50% of both great arteries arise from the right ventricle (3). Embryologically, there is a lack of alignment between the conal septum and the ventricular septum, arising both great arteries from the right ventricle  (Fig.1)
              • In DORV there is invariably a ventricular septal defect (VSD) that permits flow exit from the right ventricle.
              • DORV is classified according to the relationship between the VSD and the closest great artery (or its semilunar valve). Surgery and prognosis depend on DORV characteristics (4) (Fig. 2)
                :
                • Subaortic VSD, or VSD type DORV (the VSD lies under the aortic valve)
                • Subaortic VSD with pulmonary stenosis, or Fallot type DORV
                • Subpulmonary VSD, or transposition type (the VSD lies under the pulmonary valve) AKA Taussig-Bing malformation
                • Doubly committed VSD DORV (the VSD is equally related to both great arteries)
                • Non-committed VSD DORV (the VSD lies away from both great arteries) 
              • There are numerous cardiac and non-cardiac anomalies associated with DORV, including, but not limited to:
                • VSD (invariably)
                • Pulmonary valve stenosis and atresia (20-40%)
                • Atrial septal defect (ASD) (20%)
                • Persistent ductus arteriosus (PDA) (15%)
                • Endocardial cushion defect (8%)
                • Subaortic stenosis (3-30%)
                • Aortic coarctation and aortic arch hypoplasia (2-45%)
                • Ventricular malformations
                • Coronary arteries anomalies
                • Anomalous pulmonary venous connections
                • Heterotaxy syndromes
                • Diaphragmatic hernia
              • The role of imaging in DORV includes:
                • Pre and post-natal cardiac ultrasound (CUS) for diagnosis
                • Pre-surgical advanced imaging with cardiac computed tomography (CCT) or cardiac magnetic resonance (CMR) for anatomic characterization and functional assessment in complex cases (particularly in subpulmonary and non-committed types) and evaluation of associated anomalies. Pre-surgical CCT could be more suitable for neonates than CMR (5).
                  • Single post-contrast arterial phase usually suffices as systemic and pulmonary arteries show diagnostic enhancement simultaneously due to the VSD
                • CT and MR angiography with 3D rendering and 3D printing in complex cases
                • Post-surgical follow-up, preferably with CMR for radiation protection and for a comprehensive anatomical and functional evaluation including morphologic and functional sequences
                  • Coronal T2 single shot
                  • Whole heart 3D balanced-steady state free precession (b-SSFP)
                  • MR angiography when peripheral vessels examination is required
                  • Cine MR in usual planes (two, three and four chamber views, RVOT). Ventricular function analysis
                  • Phase contrast imaging for shunts and valvular assessment
                  • Late enhancement if myocardial infarction is suspected
                • The subsequent section will address the anatomical, physiological, radiological, and therapeutic characteristics of these DORV types, in addition to their association with other malformations.
GALLERY