Persistent left superior vena cava, absence of the innominate vein, and upper sinus venosus defect

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  • Introduction

    Echocardiography plays an important role in the evaluation of congenital heart disease. In patients with a dilated coro-nary sinus (CS), a persistent left superior vena cava (PLSVC) should be considered. A bubble study with agitated saline is a simple, practical method to evaluate ad-ditional anomalies. Here, we report a pa-tient diagnosed with a PLSVC, absence of the innominate vein, and upper sinus venosus defect based on a bubble study and confirmed with magnetic resonance imaging (MRI).

    Case

    A 60-year-old woman was admitted to our cardiology department complaining of palpitations. She had a rhythm dis-turbance for the last 5 years and Holt-er monitoring detected paroxysmal atrial fibrillation. The patient was tak-ing propafenone 200 mg, metoprolol 50 mg, and acetylsalicylic acid 300 mg once a day. Her arterial blood pressure was 125/80 mm Hg. The physical exam-ination was normal except for a 2/6 sys-tolic murmur in the apical and left up-per parasternal areas. The electrocardio-

    gram showed normal sinus rhythm and her heart rate was 72 beats/min. The transthoracic echocardiogram revealed normal left ventricular systolic function, with mild mitral and tricuspid regurgi-tation. There was no significant enlarge-ment of the cardiac chambers. Her sys-tolic pulmonary pressure was 30 mmHg and color Doppler echocardiography re-

    vealed no defect in the interatrialinter-ventricular septum. A significantly dilat-ed CS (2.2 cm in diameter) was detect-ed in the atrioventricular groove. Sus-pecting a PLSVC, a bubble study with agitated saline was performed via the left antecubital vein. First, the bubbles filled the CS and then the right cardiac chambers. The bubbles filled both atria

    Herz201338:317320DOI10.1007/s00059-012-3704-zReceived:27June2012Revised:24September2012Accepted:27September2012Publishedonline:14December2012Urban&Vogel2012

    I.Akpinar1M.R.Sayin1T.Karabag1S.M.Dogan1S.T.Sen2N.E.Gudul1M.Aydin11FacultyofMedicine,DepartmentofCardiology,BulentEcevitUniversity,Kozlu/Zonguldak2DepartmentofRadiology,AtaturkStateHospital,Zonguldak

    Persistentleftsuperiorvenacava,absenceoftheinnominatevein,anduppersinusvenosusdefect

    Arareanomalydetectedusingbubbles

    Fig. 18Transthoracicechocardiographicimages.a,c,Videos 1, 3Whentheagitatedsalinewasad-ministeredviatheleftantecubitalvein,bubbleswereseeninrightatriumandventricleviathecoro-narysinus(CS;arrow).Sincetherewerenobubblesintheleftatrium,theroofoftheCSwasintact.b,d,Videos 2, 4Whentheagitatedsalinewasgivenviatherightantecubitalvein,bubbleswerede-tectedthroughouttheheart,indicatingarighttoleftshunt.a,bParasternallong-axisviewoftheheart;c,dapicalfour-chamberviewoftheheart.LAleftatrium,LVleftventricle,RArightatrium,RVrightventricle,Aoaorta

    Additional material online

    ThisarticleincludesfouradditionalVideos.Thissupplementalmaterialisavailableatdx.doi.org/10.1007/s00059-012-3704-z.

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  • simultaneously via the right antecubital vein before the ventricles (.Fig.1, Vid-eos14). Transesophageal echocardiog-raphy was recommended, but the pa-tient refused. Venous return anomalies with congenital heart disease were con-sidered and MRI was performed. After the patient was administered a contrast agent simultaneously in both arms, ab-sence of the left innominate vein was de-tected, in addition to the PLSVC. The

    vena cava superior dextra (VCSD) was found overriding the interatrial septum with communication to both atria via an upper sinus venosus defect (.Fig.2, ar-row). There was no significant shunt in the echocardiographic assessment (Qp/Qs =1.4). Follow-up with medical treat-ment was planned.

    Abstract Zusammenfassung

    Herz201338:317320DOI10.1007/s00059-012-3704-zUrban&Vogel2012

    I.AkpinarM.R.SayinT.KarabagS.M.DoganS.T.SenN.E.GudulM.Aydin

    Persistent left superior vena cava, absence of the innominate vein, and upper sinus venosus defect. A rare anomaly detected using bubbles

    AbstractSuperiorvenacavaanomaliesareraremal-formationsthataretypicallyseenwithothercongenitalcardiacdefects.Althoughapersis-tentleftsuperiorvenacavaisthemostcom-monanomalyofthesystemicvenousreturninthethorax,itscombinationwithanuppersinusvenosusdefectandabsenceofthein-nominateveinisextremelyrare.Here,were-portapatientdiagnosedwiththeseanoma-liesbasedonabubblestudyandconfirmedwithmagneticresonanceimaging.

    KeywordsDiagnosisVenacavaUppersinusvenosusdefectHeartCongenitalabnormalities

    Persistierende V. cava superior sinistra, Fehlen der V. innominata und oberer Sinus-venosus-Defekt. Eine seltene, mittels Gasblschen diagnostizierte Anomalie

    ZusammenfassungAnomalienderV.cavasuperiorsindselteneFehlbildungen,diegewhnlichzusammenmitanderenangeborenenHerzfehlernfest-gestelltwerden.EinepersistierendeV.ca-vasuperiorsinistrastelltzwardiehufigsteAnomaliebeidenStrukturendessystemi-schenRckflussesindenThoraxdar,aberdieKombinationmiteinemoberenSinus-veno-sus-DefektunddemFehlenderV.innomina-taistuerstselten.HierwirdeinPatientvor-gestellt,beidemdieseAnomalienmithilfeeinerEchokardiographiemitGasblschenalsKontrastmitteldiagnostiziertunddurchMag-netresonanztomographiebesttigtwurden.

    SchlsselwrterDiagnosestellungV.cavaObererSinus-venosus-DefektHerzKongenitaleAnomalien

    Fig. 29Magneticres-onanceimagingofthepersistentleftsu-periorvenacava(PLS-VC)showstheabsenceoftheleftinnominatevein(*),andthevenacavasuperiordextra(VCSD)connectedwithbothatriaviaanuppersinusvenosusdefect(arrow).RArightatri-um,LA leftatrium

    Fig. 38Schematicrepresentationofourpatientscongenitalanomalies:anuppersinusvenosusde-fect,persistentleftsuperiorvenacava(PLSVC),andabsentinnominatevein.VCSVenacavasuperi-or,VCSSvenacavasuperiorsinister,VCSDvenacavasuperiordextra,RArightatrium,LAleftatrium,RVrightventricle,LVleftventricle,CScoronarysinus

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  • Discussion

    Although a PLSVC is not encountered of-ten on echocardiography, it is the most common congenital anomaly of the ve-nous return in the thorax [1]. PLSVC is present in approximately 0.30.5% of the general population [2, 3]. During intra-uterine life, the right and left anterior cardinal veins develop into the ipsilater-al subclavian, internal jugular, and bra-chiocephalic veins. During the eighth week, the left brachiocephalic vein (in-nominate vein) combines with the right brachiocephalic vein and gives rise to the vena cava superior (VCS; normal), which is connected to the right atrium. During this time, the distal portion of the vena cava superior sinister (VCSS) regress-es; if not, the remnant VCSS is called a PLSVC in adults (.Fig.3). A PLSVC of-ten drains into the right atrium through the CS. More rarely, it may be connect-ed with the left atrium. In a study of 1205 patients undergoing cardiac catheteriza-tion for suspected congenital heart dis-ease, the incidence of PLSVC was 6.1% (74 patients). The most common con-genital heart diseases associated with a PLSVC are ventricular septal defect, atri-al septal defect (ASD), and pulmonary valve stenosis [4].

    In a study of 9075 patients who un-derwent an echocardiographic eval-uation, the prevalence of PLSVC was

    0.001%. Since most cases are asymp-tomatic, the true prevalence of isolated PLSVC is unclear. Congenital anoma-lies, such as an ASD and coarctation of the aorta, may be combined. If a patient presents with dyspnea and transthorac-ic echocardiography indicates only dila-tation of the CS, contrast echocardiogra-phy should be performed to detect oth-er underlying defects. In addition, fur-ther investigations should be considered, such as MRI and computed tomography (CT). When associated with an upper si-nus venosus defect, a partial anomalous pulmonary venous connection (PAPVC; mainly affecting the upper right pulmo-nary vein) should be evaluated cautious-ly [5, 6].

    During the course of central venous catheterization, a PLSVC may be not-ed incidentally, and it can result in right atrial injury during permanent pace maker implantation [7, 8].

    With an apparent dilated CS located in the posterior atrioventricular groove on echocardiography, a PLSVC should be suspected, although right ventricular dysfunction, right atrial hypertension, severe pulmonary hypertension, abnor-mal pulmonary venous drainage into the CS, and coronary AV fistula are oth-er causes of CS dilatation [9]. A contrast study with agitated saline should be per-formed in both arms because a PLSVC

    may be associated with other venous re-turn anomalies.

    This manuscript presents a rare con-genital venous anomaly: an upper si-nus venosus defect and PLSVC connect-ed to the CS without a connection to the VCSD, as the innominate vein is ab-sent. Echo-contrast imaging of the VCSS showed bubbles only in the right atrium and right ventricle, whereas contrast in-jection via the VCSD showed bubbles in the right and left heart structures via an atrial septal defect. This phenomenon is impossible in the presence of a nor-mal interatrial communication (secun-dum ASD), whereas in the presence of pulmonary hypertension a right-to-left shunt must exist via both venous routes. The only explanation for this phenom-enon is that the interatrial communi-cation is not interatrial, but between the VCSD and left atrium. This is a so-called sinus venosus defect overriding the VC-SD over the interatrial septum (.Fig.3), which is nearly almost associated with a PAPVC (mostly the right upper pulmo-nary vein). In our patient, however, no PAPVC was detected on MRI (.Fig.4).

    If we observe many bubbles in the left cardiac chambers in addition to the right chambers in a saline study via the right arm, further evaluations are required, such as transesophageal echocardiogra-phy, CT, and MRI. Finally, the accurate detection of such significant anomalies of the venous return in patients with a dilated coronary sinus is of vital impor-tance for preventing complications dur-ing pacemaker implantation and central venous catheterization.

    Corresponding address

    I. AkpinarFacultyofMedicine,DepartmentofCardiology,BulentEcevitUniversity67600Kozlu/ZonguldakTurkeydr.ibrahimakpinar@gmail.com

    Conflict of interest. Onbehalfofallauthors,thecorrespondingauthorstatesthattherearenocon-flictsofinterest.

    Fig. 49Nopartialanomalouspulmo-naryvenousconnec-tionwasdetectedonMRI.Allofthepulmo-naryveinsdrainin-totheleftatrium(LA).Thearrowindicatestherightupperpulmo-naryvein(Pv).

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