We report a case of an unusual congenital triad consisting of absence of coronary sinus, persistent left superior vena cava and scimitar
syndrome incidentally found in a CT-scan performed on a female complaining of exertional dyspnea

Bastarrika, G. (Gorka)

Gavira, J.J. (Juan José)

Simon-Yarza, I. (Isabel)

Universidad de Navarra

Incidental computed tomography diagnosis of a rare triad consisting of absence of coronary sinus, persistent left superior vena cava, and scimitar syndrome

Dadun, University of Navarra

ARTICLE IN PRESSwww.icvts.orgdoi:10.1510/icvts.2010.252510Interactive CardioVascular and Thoracic Surgery 12 (2011) 490–491 2011 Published by European Association for Cardio-Thoracic SurgeryCase report - CongenitalIncidental computed tomography diagnosis of a rare triad consistingof absence of coronary sinus, persistent left superior vena cava,and scimitar syndromeGorka Bastarrika *, Isabel Simo´n-Yarza , Juan J. Gaviraa, a bDepartment of Radiology, Clı´nica Universidad de Navarra, Avenida Pı´o XII, 36, 31008 Pamplona, SpainaDepartment of Cardiology, Clı´nica Universidad de Navarra, Pamplona, SpainbReceived 23 August 2010; received in revised form 1 December 2010; accepted 3 December 2010AbstractWe report a case of an unusual congenital triad consisting of absence of coronary sinus, persistent left superior vena cava and scimitarsyndrome incidentally found in a CT-scan performed on a female complaining of exertional dyspnea. 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.Keywords: Computed tomography scanner; X-ray; Heart defects; Congenital; Scimitar syndromeFig. 1. Volume rendered ECG-gated DSCT angiography. (a) Right oblique view.A large vertical scimitar-like vein formed by the three right pulmonary veinsdraining into the inferior vena cava near the confluence of the hepatic veinsis shown (arrow). (b) Left oblique view. Left persistent superior vena cava(LPSVC) draining directly (*) into the left atrium (LA) is demonstrated. Inde-pendent drainage of all the coronary veins (GCV, great cardiac vein; PLMV,posterolateral marginal vein; MCV, middle cardiac vein) into the left atriumis clearly seen. Note confluence (arrow) of the right anomalous pulmonaryvenous drainage (APVD) and inferior vena cava (IVC) on the right side. RUL,right upper lobe pulmonary vein; RML, right middle lobe pulmonary vein;RLL, right lower lobe pulmonary vein; SVC, superior vena cava; HV, hepaticveins; RA, right atrium; RV, right ventricle; LV, left ventricle.1. Case reportA 73-year-old female presented with a five-years’ historyof exertional dyspnea. She had a past history of arterialhypertension and hypercholesterolemia. She did not havecyanosis. A chest radiograph showed cardiomegaly anddextroposition of the heart, right lung hypoplasia, and signsof pulmonary hypertension. Transthoracic echocardiogramshowed normal left and right ventricular morphology andsystolic function, mild-to-moderate tricuspid regurgitationwestimated systolic pulmonary artery pressure (sPAP)80 mmHgx, biatrial dilation, and marked dilation of theinferior vena cava (IVC) and hepatic veins (HV) with inver-sion of the systolic flow. An echocardiography (ECG)-gateddual-source computed tomography (DSCT) (Somatom Defi-nition, Siemens Healthcare, Forchheim, Germany) angiog-raphy was performed to evaluate the status of pulmonaryvasculature. The chest CT angiogram revealed hypoplasiaof the right lung and a large vertical scimitar-like veinformed by the three right pulmonary veins draining intothe IVC near the confluence of the HV, just above thediaphragm (Fig. 1a). The two left pulmonary veins drainednormally into the left atrium (LA). On the left side, a leftpersistent superior vena cava (SVC) formed by the leftsubclavian vein and the left internal jugular vein wasobserved. This vein descended vertically into the left sideof the mediastinum and drained directly into the LA w1x(Fig. 1b). Incidentally the study revealed an absence ofthe coronary sinus with the great cardiac vein draining inthe anterolateral aspect of the LA (Fig. 2a), and the middlecardiac vein, the posterior vein and the posterolateral*Corresponding author. Tel.: q34 948 255400; fax: q34 948 296500.E-mail address: bastarrika@unav.es (G. Bastarrika).marginal vein draining independently into the posteriorregion of the LA (Fig. 2b). The atrial situs was solitus, andboth left and right appendages showed normal morphology.No interatrial septal defect was found.Prior case reports have documented sporadic cases ofscimitar syndrome with persistent left SVC w2x or totalcoronary vein–left atrial drainage with an associated per-sistent left SVC w3, 4x. This case report is unique as itgathers an unusual triad consisting of absence of coronarysinus with drainage of all of the coronary veins into the LA,ARTICLE IN PRESS491G. Bastarrika et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 490–491NewIdeasInstitutionalReportWorkinProgressReportESCVSArticleNegativeResultsState-of-the-artBestEvidenceTopicBriefCommunicationCaseReportFollow-upPaperEditorialProtocolProposalforBail-outProcedureNomenclatureHistoricalPagesFig. 2. Volume rendered ECG-gated DSCT angiography. (a) Anterior-cranialview. The cardiac study revealed absence of the coronary sinus with the greatcardiac vein and the posterolateral marginal vein draining independently inthe anterolateral and posterolateral aspect of the left atrium, respectively.(b) Inferior view. Note direct drainage of the middle cardiac vein, the pos-terior vein and the posterolateral marginal vein into the posterior region ofthe left atrium. This view showed nicely the convergence of the scimitarvein (right anomalous pulmonary venous drainage, APVD) and inferior venacava (IVC).Video 1. Volume rendered ECG-gated DSCT angiography. The absence of cor-onary sinus with drainage of all of the coronary veins into the left atrium,persistent left superior vena cava (in blue) and right anomalous pulmonaryvenous drainage is shown.persistent left SVC and scimitar syndrome (Video 1). More-over, the case presented here emphasizes the usefulness ofCT to assess complex congenital heart diseases, oftenconfirming and completing information available from echo-cardiography and not infrequently demonstrating anatomi-cal malformations misses by the latter.Referencesw1x Goyal SK, Punnam SR, Verma G, Ruberg FL. Persistent left superiorvena cava: a case report and review of literature. Cardiovasc Ultra-sound 2008;6:50.w2x Sun J, Zhang S, Jiang D, Yang G. Scimitar syndrome with the leftpersistent superior vena cava. Surg Radiol Anat 2009;31:307–309.w3x Pizarro G, Castillo JG, Gaztanaga J, Garcia MJ. Images in cardiovascularmedicine. Total coronary vein-left atrial drainage. Circulation 2009;120:914–917.w4x Miraldi F, di Gioia CR, Proietti P, De Santis M, d’Amati G, Gallo P.Cardinal vein isomerism: an embryological hypothesis to explain apersistent left superior vena cava draining into the roof of the leftatrium in the absence of coronary sinus and atrial septal defect.Cardiovasc Pathol 2002;11:149–152.

Incidental computed tomography diagnosis of a rare triad consisting of absence of coronary sinus, persistent left superior vena cava, and scimitar syndrome

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