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Blackwell Publishing IncMalden, USACHDCongenital Heart Disease1747-079X© 2007, the Authors; Journal compilation © 2007 Blackwell Publishing, Inc.? 200724265269CASE REPORT

Interventional Rerouting of Scimitar Vein to Left AtriumSingh et al.

CASE REPORTS

Interventional Rerouting of Scimitar Vein to Left Atrium Using an Amplatzer Vascular Plug

Harminder Singh, MD, FACC, Manoj Luthra, MCh, Prashant Bharadwaj, MD, and Ravi Kumar, MD

Military Hospital, Cardio-thoracic Center, Pune, Maharashtra, India

A B S T R A C T

Scimitar syndrome is a rare congenital anomaly accounting for about 0.5% of all congenital heart diseases. In itsmost common form, it is characterized by anomalous drainage of one or all right side pulmonary veins into inferiorvena cava or rarely into hepatic, portal, or azygous veins. Other associated anomalies can be hypoplasia or agenesisof right lung, hypoplasia of right pulmonary artery, bronchial anomalies, aberrant blood supply and (or) sequestra-tion of right lung, and variety of intracardiac defects which commonly includes atrial or ventricular septal defectsand tetrology of Fallot. The usual treatment of these patients is surgical rerouting of Scimitar vein into left atrium(LA) by baffle formation, lobectomy, embolization or ligation of aberrant blood supply and various combinationsof these. Nonsurgical and interventional treatments are rarely possible. We report a rare case where completererouting of anomalous venous drainage to LA could be achieved by interventional treatment by a simple deploy-ment of a vascular plug in combination with embolization of aberrant vascular supply to the same area, thus avoidingsurgical intervention.

Key Words.

Scimitar Vein; Amplatzer Vascular Plug; Left Atrium; Pulmonary Veins; Left to Right Shunt

Case Report

he patient is a 3-year-old boy born out ofnonconsanguineous marriage, who had re-

current chest infections since infancy. Clinically hehad cardiomegaly and signs of right ventricularvolume overload and mid-systolic murmur in leftupper sternal edge for which he was referred toour hospital. Echocardiography showed situssolitus, levocardia, with no intracardiac defects.There was dilatation of right atrium (RA), rightventricle, and pulmonary arteries (PA). A contin-uous flow was seen in inferior vena cava (IVC)near its entry into RA. Interatrial and interventric-ular septa were intact. Only left-sided pulmonaryveins were seen entering into left atrium (LA).Overall picture was diagnostic of Scimitar syn-drome; however, the Scimitar vein (SV) could notbe well profiled on echocardiography. Magneticresonance imaging, however, readily confirmedthe diagnosis. Magnetic resonance imaging alsorevealed stenosis of the SV near its entry point

T

into IVC. The child was taken up for diagnosticcatheterization. Catheterization revealed pulmo-nary to systemic flow ratio of 1.7:1 with a PApressure of 42/18 (mean 29 mm Hg) and pulmo-nary vascular resistance of 1.9 woods/m

2

body sur-face area. Diagnostic right PA angiogram showednormal sized PA and during the levo-phaseshowed both right upper and lower pulmonaryveins forming SV which drained into the IVC(Figure 1A). Stenosis of the SV could be well dem-onstrated on selective injections in SV (Figure 1Band C). Selective angiogram also revealed atortuous collateral connecting SV and the LA(Figure 1B and C). Abdominal aortic angiogramshowed an aberrant vessel supplying the basal seg-ments of the right lower lobe. These segments haddual supply from the pulmonary artery andabdominal aorta.

Occlusion of the SV at the stenotic site justbefore the entry point into the IVC would reroutethe pulmonary venous drainage into the LAthrough the collateral communication as reported

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earlier.

1–3

Our hesitation was would the small col-lateral vessel provide unobstructed pulmonaryvenous drainage?

The procedure was performed under sedationwith midazolam, ketamine, and fantanyl which wassubsequently changed to general anesthesia. Twovenous accesses were taken in both femoral veins

and arterial access in right femoral artery. A 5-FCournand catheter was kept in the right PA tomonitor PA pressure. Balloon occlusion of thestenotic site in SV was performed for 30 minuteswith 5-F Swan Ganz catheter which produced nochange in PA pressure (Figure 2A and B). A 6-FRenal Double Curve (Medtronic Inc., Minneapo-

Figure 1.

(A) Levo-phase of right pulmonary artery angiogram showing formation of Scimitar vein by right upper and lowerpulmonary veins, which crosses the diaphragm to drain into IVC (arrow). (B) and (C) Selective PA view and lateral view ofScimitar vein angiogram respectively, showing the site of stenosis (arrow) large and sizable collateral connecting Scimitarvein to LA (double arrow). IVC, inferior vena cava; PA, pulmonary artery; LA, left atrium.

A B C

Figure 2.

(A) and (B) Balloon occlusion of the stenotic site in Scimitar vein. (C) Pulmonary angiogram after deployment ofvascular plug (arrow) showing stasis and delayed clearance of contrast due to venous obstruction. The vascular plug wasremoved subsequently.

A B C

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lis, MN, USA) catheter was passed to hook the SVfrom the right femoral route. A 10 mm Amplatzervascular plug (AGA Medical Corporation, GoldenValley, MN, USA) was deployed astride thestenotic segment producing a good waist(Figure 2C). Pulmonary artery pressure was con-tinuously monitored which measured 36/16 (mean24 mm Hg). The child developed tachypnoea andstarted coughing after 15 minutes of deploymentof the device. Pulmonary artery angiogram per-formed at the time showed poor clearance of thedye suggesting obstruction to the pulmonaryvenous drainage (Figure 2C) necessitating extrac-tion of the vascular plug. Cause of failure of theprocedure, we feel, was inability of the collateralvessel to accommodate all venous return of theright lung. We were very sure during deploymentthat the vascular plug was not occluding the originof the collateral channel. Case was discussed withsurgeons and parents. As the child had no otherintracardiac anomaly requiring surgery, it wasjointly decided that another attempt at interven-tional management would be in the best interestof the patient.

We decided to redo the procedure along withcoil embolization of the aberrant blood supply tothe right lower lobe. We felt, once this is per-formed, the small vascular collateral may be ableto sustain reduced venous drainage withoutobstruction. We performed coil embolization ofthe aberrant blood supply to right lower lobe asfirst step (Figure 3). A 4-F Cobra catheter was used

to hook the aberrant vessel arising from the abdom-inal aorta and 3

×

40 mm 0.035 inch free fiberedPlatinum coils (Boston Scientific, Target Vascular,Cork, Ireland) were deployed with immediateocclusion of the vessel. Subsequent deployment ofthe vascular plug was exactly as the previousattempt (Figure 4A, B, and D). This time the pro-cedure was successful. There was no change in thePA pressure. Pulmonary artery angiogram showedunobstructed clearance of dye entirely into LAwithout opacification of RA (Figure 4C). Thepatient was electively ventilated for 12 hours andPA pressure was monitored which showed noincrease. Subsequent course was uneventful. Thechild is asymptomatic since 6 months. Repeatechocardiogram has shown unobstructed flow inIVC and hepatic veins and no flow via SV into IVC.

Absence of any intracardiac defects, presence ofa sizable collateral providing alternative drainageinto LA and stenosis of SV at a convenient loca-tion made our patient suitable for interventionalrerouting of SV.

Discussion

Scimitar syndrome has varied presentations andrequires individualization of treatment based onthe anatomy and symptomatology.

4

Surgical man-agement includes creating a baffle to route the SVinto LA, ligation of aberrant vessels, lobectomyor pneumonectomy, and repair of any associatedintracardiac defects or a combination of these.

Figure 3.

Aberrant supply of basal segments of right lower lobe from abdominal aorta was coil occluded to reduce arterialsupply to the region.

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Interventional procedures usually include coilembolization of the aberrant vessels and closureof ASD.

5

Rarely interventional rerouting of SVinto LA is possible.

1–3

Rerouting the SV into LAdepends upon presence of dual drainage via a col-lateral vessel connecting the two. Such channelsare rarely reported.

1–3,6

Natural history of suchcollateral channels is not settled. Roman et al.reported 2 cases similar to ours with dual drain-age.

6

They managed one by creating a surgicalbaffle which subsequently got occluded but thepatient did well because of drainage into LA viathe collateral. Their second case had spontaneousclosure of the SV to IVC connection with com-plete drainage to LA via the collateral. This ledthem to believe that such cases of dual drainageshould be followed up without any surgery. How-ever, Forbess et al. had 2 similar cases with dualdrainage that could not be left untreated and hadto be coiled at 53 and 65 years of age suggestingthat outcome of these cases is unpredictable.

2

Asthe natural history of such cases is not settled, wedecided to proceed with closure of the SV whichwas easily possible and benefited the patient.However, given the small size of the collateral, weare concerned about the long-term outcome,which has been uneventful so far. The child is onregular follow-up.

Embolization of the sequestered segments andaberrant vessel is helpful in reducing symptoms ofchest infections. In our case, embolization washelpful by reducing the blood flow and thereforevenous return load on the small collateral channel.A previously unsuccessful procedure could suc-ceed after embolization of aberrant vessels.

Choice of material for occluding the SV hasbeen variable. Gianturco-Grifka vascular occlu-sion coils

2

and Amplatzer duct occluder

3

have beenused. We used a vascular plug, for the first time,for its ease of deployment, small size of sheathrequired and easy extractability and redeploymentin case required. Presence of stenosis in the SVmade it a suitable choice.

To conclude, we report a rare case of Scimitarsyndrome treated by interventional deployment ofa vascular plug to occlude the SV-IVC junction,thus achieving complete venous drainage into LAby a collateral.

Corresponding Author:

Harminder Singh, MD,FACC, Military Hospital (Cardio-Thoracic Center),Department of Cardiology, Golibar Maidan, KondwaRoad, Pune, Maharashtra 411040, India. Tel: (

+

91)2026306172; Fax: (

+

91) 202-427-5877; E-mail:harminder62@yahoo.com

Accepted in final form: May 3, 2007

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Figure 4.

(A) and (B) Lateral and PA views respectively during positioning and deployment of the vascular plug. (C) Levo-phase of the right pulmonary angiogram showing free flow of dye into LA via the Scimitar vein and the collateral. (D) Vascularplug released. PA, pulmonary artery; LA, left atrium.

LA

B C DA

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