Journal of Cardiology Cases 10 (2014) 73–77

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Journal of Cardiology Cases

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Case Report

Persistent right superior vena cava in a patient with dextrocardia:

Case report and review of the literature

Keerthana Karumbaiah (MD), Susan Choe (DO), Morhaf Ibrahim (MD),Bassam Omar (MD, PhD)*

Division of Cardiology, University of South Alabama, Mobile, AL 36617, USA

A R T I C L E I N F O

Article history:

Received 25 February 2014

Received in revised form 11 April 2014

Accepted 8 May 2014

Keywords:

Dextrocardia

Anomalous venous connections

Echocardiography

A B S T R A C T

Introduction: Systemic venous circulation anomalies are uncommon; they are often incidental findings

during echocardiography.

Case: A 56-year-old man, with dextrocardia, was evaluated for dyspnea. The patient’s medical history

included diabetes mellitus requiring insulin treatment, hypertension, and tobacco use. Physical

examination revealed normal jugular venous pulsations and clear lungs. Cardiac examination revealed

normal heart sounds, and grade II/VI systolic ejection murmur over the right precordium.

Echocardiography revealed normal chamber size and systolic function, without significant valvular

lesions. The coronary sinus was dilated. It was evaluated using intravenous agitated saline contrast to

rule out anomalous venous drainage or shunting. When injected into the left antecubital vein, contrast

appeared initially in the right atrium followed by the right ventricle. However, when injected into the

right antecubital vein, contrast appeared initially in the dilated coronary sinus followed by the right

atrium and right ventricle. There was no evidence of intracardiac shunting. These findings were

consistent with persistent right superior vena cava in the setting of situs inversus dextrocardia, with

normally draining left superior vena cava.

Conclusion: Persistent superior vena cava connection to the coronary sinus is often incidental but an

important finding which helps in planning safe invasive procedures.

<Learning objective: Understand the importance of identifying anomalous venous connections with

regard to catheter-based procedures. Appreciate the incidence of these vascular anomalies in the normal

population and in congenital heart disease. Understand how echocardiography with intravenous

agitated saline contrast can be helpful in the diagnosis of such anomalous venous connections.>

� 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Introduction

Anomalies of the systemic venous circulation are not uncom-mon in patients with congenital heart disease, and can pose a risk ifunrecognized prior to diagnostic or therapeutic catheter proce-dures requiring the use of systemic veins. We present a case of a56-year-old man with dextrocardia and situs inversus who wasfound to have a persistent right superior vena cava (PRSVC) by 2Dechocardiography using intravenous agitated saline contrastperformed to evaluate his dilated coronary sinus.

* Corresponding author. Tel.: +1 251 471 7923; fax: +1 251 470 5888.

E-mail address: bomar@health.southalabama.edu (B. Omar).

http://dx.doi.org/10.1016/j.jccase.2014.05.005

1878-5409/� 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights

Case report

A 56-year-old man, with situs inversus dextrocardia, wasevaluated for dyspnea. The patient’s medical history includeddiabetes mellitus requiring insulin treatment, hypertension, andtobacco use. Physical examination revealed normal jugular venouspulsations and clear lungs. Cardiac examination revealed normalright-sided point of maximal impulse, normal heart sounds, andgrade II/VI systolic ejection murmur over the right precordium.Electrocardiogram revealed sinus rhythm and dextrocardia,without ischemia (Fig. 1). Chest X-ray revealed no acute pathology(Fig. 2). Cardiac catheterization revealed normal coronary arteriesand normal ejection fraction. Echocardiography revealed normalchamber size and left ventricular systolic function, withoutsignificant valvular lesions. There was an incidental dilatedcoronary sinus (Fig. 3), which was evaluated using intravenous

reserved.

[(Fig._1)TD$FIG]

Fig. 1. Electrocardiogram showing sinus rhythm with features of dextrocardia.

[(Fig._3)TD$FIG]

K. Karumbaiah et al. / Journal of Cardiology Cases 10 (2014) 73–7774

(IV) agitated saline contrast to rule out anomalous venous drainageor shunting. When injected into the left antecubital vein, thecontrast appeared initially in the right atrium followed by the rightventricle (Fig. 4). However, when injected into the right antecubitalvein, the contrast appeared initially in the dilated coronary sinusfollowed by the right atrium and right ventricle (Fig. 5). There wasno evidence of intracardiac shunting. These findings wereconsistent with persistent right superior vena cava in the settingof situs inversus dextrocardia, with normally draining left superiorvena cava. Due to the absence of an obvious cardiac cause of hisdyspnea and his tobacco use history, pulmonary function testswere performed, which revealed moderate restrictive lung diseaseand increase in airway resistance. This was thought to explain hissymptoms; therefore, pulmonary clinic follow-up was arranged forfurther diagnosis and therapy.

[(Fig._2)TD$FIG]

Fig. 2.Chest X-ray with situs inversus dextrocardia showing part of the

stomach air on the right side.

Fig. 3.Parasternal long-axis 2D echocardiographic view showing the dilated

coronary sinus, which measured 2.5 cm in transverse diameter.

Discussion

Anomalous connection of systemic venous circulation to thecoronary sinus has been previously reported. The most common isa persistent left superior vena cava (PLSVC), which has beenreported in approximately 0.5% of the general population, andabout 3–10% in patients with congenital heart disease [1]. Theseanomalies are usually asymptomatic and are discovered inciden-tally. They have also been reported in patients with dextrocardia, ararer anomaly in itself. Reports of PLSVC, in addition to pertinentstudies of anomalous superior vena cava connections in dextro-cardia, will be reviewed to demonstrate the clinical implications ofthese often incidental findings, especially in relation to catheter-based procedures. The following four case reports will demon-strate the difficulties encountered during pacemaker insertion, andcentral venous line and Swan–Ganz placement in patients withpreviously unrecognized PLSVC.

Bhatti and colleagues [2] reported a PLSVC draining into adilated coronary sinus, associated with anomalous left hepatic veindrainage into the right atrium, in an 86-year-old man undergoingpacemaker implantation for complete heart block. The left

[(Fig._4)TD$FIG]

Fig. 4.

Apical four-chamber view angled to reveal the coronary sinus following

injection of intravenous agitated saline in the left arm. Note the

appearance on the contrast in the right atrium and right ventricle

without opacification of the coronary sinus, indicating normal drainage

of the left superior vena cava into the right atrium.

K. Karumbaiah et al. / Journal of Cardiology Cases 10 (2014) 73–77 75

subclavian approach had to be abandoned due to difficulty ininserting the pacing catheter, and a right subclavian approach wassuccessful. The diagnosis was confirmed by echocardiography withagitated saline and coronal computed tomography (CT) of the chestwith contrast.

Higgs et al. [3] described an anomalous course of a transvenouscatheter inserted through the external jugular vein, correspondingto PLSVC course, in a 3-year-old boy requiring venous access for thetreatment of scald injury complicated by disseminated intravas-cular coagulation. The course of the catheter was confirmed withechocardiography; the catheter was retracted to avoid irritation ofthe coronary sinus and was successfully used for total parenteralnutrition administration.

Goyal et al. [4] reported the case of a 19-year-old man with amotor vehicle accident who was incidentally found to have adilated coronary sinus on echocardiography. A Swan–Ganzcatheter inserted via the left subclavian approach was shown to

[(Fig._5)TD$FIG]

Fig. 5.

Apical four-chamber view angled to reveal the coronary sinus following

injection of intravenous agitated saline in the right arm. Note the

appearance on the contrast in the coronary sinus before opacification of

the right atrium and right ventricle, indicating anomalous drainage of

the right superior vena cava into the coronary sinus.

pass through the coronary sinus by echocardiography. A contrast-enhanced CT study of thorax performed to assess the bone andvascular injuries associated with the motor vehicle accident alsorevealed PLSVC as an incidental finding.

Walpot et al. [5] reported a case of a 29-year-old man whounderwent an emergent laparotomy for multiple intra-abdominalabscesses complicated by sepsis. Postoperatively the tip of acentral line catheter inserted through the left subclavian vein wasin a left paramediastinal position on chest X-ray, instead ofcrossing the midline to the superior vena cava. A transthoracicechocardiography study performed after injection of agitatedsaline in the catheter showed opacification of the coronary sinus,the right atrium, and the right ventricle, confirming the diagnosisof PLSVC.

Next, the seven case reports and case series in which theincidental finding of PLSVC was made by diagnostic echocardiog-raphy, as in our patient, will be discussed to demonstrateassociated findings and for additional help in testing.

Stoevesandt and colleagues [6] reported a 42-year-old manwith primary sclerosing cholangitis and colitis ulcerosa, screenedfor liver transplantation, who was found to have a dilated coronarysinus by echocardiography. The diagnosis of PLSVC draining into adilated coronary sinus was made by cardiac magnetic resonanceimaging (MRI).

Kong and Ahmad [7] reported the case of a 65-year-old womanwith hemoptysis due to chest infection. Transthoracic echocardi-ography showed dilated atria, right ventricle, and main pulmonaryartery; volume overload with diastolic flattening of the interven-tricular septum was noted. Left-to-right shunting from the leftatrium into a dilated unroofed coronary sinus was seen. Agitatednormal saline injected into the patient’s left antecubital veinappeared in the coronary sinus before appearing in the right heartthus confirming a PLSVC. On transesophageal echocardiography,the right pulmonary veins were noted to drain into the superiorvena cava, while the left pulmonary veins drained normally.

Guarnieri et al. [8] reported two cases of echocardiographicdiagnosis of the absence of the right superior vena cava, withPLSVC and a large coronary sinus in structurally normal heart in afetus of 20 weeks’ gestation and in a newborn. Upon injection ofagitated saline into the right antecubital vein in the newborn,apical four-chamber views showed earlier enhancement of thedilated coronary sinus than the right cardiac chambers. Similarfindings after birth confirmed PLSVC in the fetus.

El-Chami et al. [9] reported the case of a 64-year-old womanwith progressive shortness of breath who was found to havepreserved ejection fraction with marked pulmonary hypertensionand severe decrease in right ventricular function by echocardiog-raphy. An incidental finding was a dilated coronary sinus. Rightheart catheterization and pulmonary angiogram were done forfurther diagnostic and therapeutic purposes, revealing pruning ofthe distal vessels consistent with thromboembolic disease.Cannulation and dye injection of the coronary sinus revealedthe presence of the incidental PLSVC.

Recupero et al. [10] used contrast echocardiography todemonstrate PLSVC in five patients; the results were confirmedby multislice CT (MCT) or MRI. Four patients revealed contrastappearing first in the coronary sinus after either right or leftantecubital injection. These were all shown to have agenesis of theright superior vena cava in addition to PLSVC. The last patient hadcontrast appearing in the coronary sinus after left antecubetalinjection and in the right atrium after right antecubital injection,and was confirmed to have PLSVC associated with right superiorvena cava by MCT.

Gonzalez-Juanatey et al. [11] reported 10 cases of PLSVCdraining into the coronary sinus diagnosed by transthoracicechocardiogram using echo-contrast enhancement and confirmed

[(Fig._6)TD$FIG]

Fig. 6.Chest X-ray from Fig. 2 with an overlay of a diagrammatic

representation of the patient’s anticipated major venous connections.

K. Karumbaiah et al. / Journal of Cardiology Cases 10 (2014) 73–7776

by MRI. Three cases were associated with other congenital heartdisease including secundum atrial septal defect (ASD), sinusvenosus ASD, and severe aortic coarctation. The remaining sevencases were associated with arrhythmia, ischemia, or hypertensiveheart disease. In three cases, the right superior vena cava wasabsent and demonstrated a significant increase in the size of themain coronary sinus.

Hsiao et al. [12] reported four cases of PLSVC diagnosed bycontrast echocardiography, transesophageal echocardiographyand later confirmed by invasive angiography. They demonstrated100% diagnostic accuracy with contrast echocardiography usingthree criteria: (1) dilated coronary sinus in parasternal long-axisview; (2) enhancement of the dilated coronary sinus earlier thanthe right cardiac chambers after contrast infusion into the left arm;(3) enhancement of the right cardiac chambers earlier than thedilated coronary sinus after contrast infusion into the right armvein.

Rigatelli [13] reviewed the embryogenesis and diagnosticmodalities of PLSVC. The author discussed the related technicalproblems in the face of invasive cardiovascular procedures, takinginto consideration the points of view of anesthesiologists(placement of central vein lines), nephrologists (placement ofnoncuffed dialysis catheters), electrophysiologists (insertion ofright or left ventricular pacemaker leads), invasive/interventionalcardiologists (right heart catheterization and placement of ASDclosure devices), and cardiothoracic surgeons (use of retrogradecardioplegia and in cardiac transplantation). The author concludedthat the recognition of PLSVC before invasive medical–surgicalprocedures is important in order to avoid medical errors, lost time,and poor results.

Dextrocardia is a rare condition, which often coexists with othercongenital anomalies. Bohun et al. [14] reported the prevalence ofdextrocardia to be 1 in 12,019 pregnancies. The most prevalentconfiguration, also corroborated by Garg et al. [15], was situsinversus, which was defined as a left inferior vena cava and a leftsuperior vena cava connecting to the systemic right atrium on theleft side of the heart, with a left-sided liver and a right-sidedstomach. Situs inversus was also associated with the least cardiacand noncardiac pathology. Few reports of anomalous venousconnections in patients with dextrocardia have been published.The following most pertinent five case reports will demonstrateincidentally found anomalous venous connection and associatedlesions in patients with dextrocardia during diagnostic ortherapeutic procedures.

Murayama et al. [16] reported the case of a 5-year-old girl withprogressive cyanosis who had situs inversus dextrocardia withother complex congenital heart anomalies. She was found to have aPRSVC draining into the left-sided right atrium via the coronarysinus, with an absent left superior vena cava.

Nakagawa et al. [17] reported the case of a 56-year-old womanwith known situs inversus admitted with dyspnea due tocongestive heart failure caused by severe biatrioventricular valveregurgitation. Transesophageal echocardiography and cardiac MRIperformed in preparation for surgery revealed the presence of aPRSVC draining into the left atrium via a dilated coronary sinus.

Pott et al. [18] reported the case of a 40-year-old man withdextrocardia and idiopathic dilated cardiomyopathy requiringimplantation of a biventricular pacemaker-defibrillator. His leftsuperior vena cava was found to be draining into the coronarysinus, which caused difficulties with multiple lead insertions to theright atrium, right ventricle, and into a left ventricular branch ofthe dilated coronary sinus, using selective angiography.

Manohar and Tharakan [19] reported a 3-year-old woman withdextrocardia and total anomalous pulmonary venous connectionto the coronary sinus, who presented with recurrent respiratoryinfections and delayed development. Echocardiography revealed

normal atrial situs with dextroversion, all four pulmonary veinsdraining into a large coronary sinus, secundum ASD, and mildpulmonary hypertension. PLSVC draining into the coronary sinuswas identified by cardiac catheterization, necessitating the use ofthe right superior vena cava for central venous access duringsurgery.

Tripathi et al. [20] reported a 37-year-old man with situs solitusdextrocardia who underwent pulmonary artery catheter insertionin preparation for mitral valve replacement. The patient sufferedsevere mitral regurgitation following a failed balloon valvotomyfor rheumatic mitral valve stenosis associated with pulmonaryhypertension. The catheter was introduced through the rightinternal jugular vein; however, it was shown to take the path of aleft-sided superior vena cava which drained into the inferior venacava. The long course before pulmonary artery pressure waveformwas obtained prevented the catheter from wedging, and raised aninitial fear of catheter coiling.

The above discussion underscores the importance of identifyinganomalous venous drainage especially with regard to catheter-based procedures. In most reports, as in our patient, an incidentalfinding of a dilated coronary sinus raised the suspicious of ananomalous venous connection, especially PLSVC, to the coronarysinus. Our patient had dextrocardia with clinical featurescompatible with situs inversus, which is expected to have a leftsuperior vena cava connecting to the systemic right atrium on theleft side of the heart [14]. This was demonstrated by theappearance of IV agitated saline contrast through his left armdirectly into the right atrium. The appearance of the IV agitatedsaline injected through his right arm initially into the coronarysinus, however, was indicative of a PRSVC. PRSVC is anticipated tostart at the junction of the right subclavian vein and right internaljugular vein and drain into the coronary as illustrated in thediagrammatic overlay in Fig. 6. It is thought to be due to a lack ofobliteration of the right anterior cardinal vein during normal fetaldevelopment. Our patient the three contrast echocardiographycriteria described by Hsiao et al. [12]; but modified to account forthe dextrocardia. This incidental, seemingly benign, finding will be

K. Karumbaiah et al. / Journal of Cardiology Cases 10 (2014) 73–77 77

important if our patient is to require any diagnostic or therapeuticcentral venous access procedures in the future, which would haveto be limited to his left superior vena cava, avoiding the anomalousconnection of the right superior vena cava.

Sources of support

None.

Conflict of interest

The authors declare no conflict of interest.

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