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).


    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





    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

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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



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



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



    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/

    Conflict of interest. Onbehalfofallauthors,thecorrespondingauthorstatesthattherearenocon-flictsofinterest.

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

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