Case Reports

Closure of Unroofed Coronary Sinus With a CoveredStent in a Symptomatic Infant

Alejandro Torres,* MD, Welton M. Gersony, MD, and William Hellenbrand, MD

A covered stent was used for percutaneous closure of an unroofed coronary sinus inan infant with congestive heart failure secondary to increased pulmonary flow. Prior tothe stent deployment, the location of the entrance of the coronary vein into the coro-nary sinus was demonstrated with a selective left coronary artery angiogram to facili-tate placement of the stent. The procedure was well tolerated without complications.The infant’s symptoms improved significantly after the procedure. ' 2007 Wiley-Liss, Inc.

Key words: unroofed coronary sinus; pediatric interventions; covered stent

INTRODUCTION

Heart failure in infants with atrial left to rightshunts, although rare, has been reported [1–3]. Somestudies have shown improvement in growth after earlyatrial septal defect (ASD) closure even in asymptom-atic infants and children [2,3]. Unroofed coronary sinus(UCS) is a rare type of ASD, in which a communica-tion occurs between the coronary sinus and the leftatrium as a consequence of an absent or deficient coro-nary sinus septum. This entity is frequently associatedwith a persistent left superior vena cava (LSVC) [4–6].In the majority of cases, left to right shunting is welltolerated and young patients remain asymptomatic, butas with other types of ASD, exceptions occur. Untilnow, surgical repair has been the treatment for sympto-matic patients with UCS. In this report we introduce anovel approach for transcatheter closure of an UCS inan infant presenting with congestive heart failure andfailure to thrive.

CASE REPORT

A 7-month-old boy was referred for cardiac evalua-tion due to a history of respiratory distress, frequentrespiratory infections, excessive sweating with activity,and failure to thrive. He had been diagnosed withAxenfeld–Rieger syndrome (chromosome 6 microdele-tion associated with congenital glaucoma, craniofacial,and dental anomalies and very rarely, congenital heartdefects) and moderate gastroesophageal reflux. On ini-tial physical examination, the patient was tachypneicwith mild subcostal retractions with an oxygen satura-

tion of 95% on room air. His weight was 6.8 kg (5thpercentile). Cardiac examination demonstrated a quietprecordium, normal first and second sound, and a II/VIsystolic ejection murmur loudest along the left sternalborder. Pulses were strong and symmetrical. Bilateralcoarse rales were heard over both lung fields. The liverwas palpable 2 cm below the right costal margin.The ECG showed normal sinus rhythm, right axis

deviation, RA enlargement, and biventricular hypertro-phy, Chest X-ray showed a normal sized cardiac sil-houette and increased pulmonary vascular markings.The transthoracic echocardiogram demonstrated situssolitus of the atria, d-ventricular looping and normallyrelated great arteries. Pulmonary venous return wasnormal. There were bilateral superior vena cavae(SVC) with no apparent bridging vein. The left SVCdrained into an enlarged coronary sinus. A 1-cm defectwas demonstrated on the roof of the coronary sinuswith left to right shunting. The right ventricle wasmildly dilated, with mild diastolic flattening of theventricular septum. The mitral valve annulus appearedsomewhat small (8.5 mm, Z-score¼ �3) with a mean

Children’s Hospital of New York-Presbyterian, Columbia Uni-versity, Pediatric Cardiology, New York, New York

*Correspondence to: Alejandro Torres, Children’s Hospital of New

York-Presbyterian, Columbia University, Pediatric Cardiology, New

York, NY. E-mail: ajtorres@mac.com

Received 8 January 2007; Revision accepted 3 March 2007

DOI 10.1002/ccd.21189

Published online 11 June 2007 in Wiley InterScience (www.

interscience.wiley.com).

' 2007 Wiley-Liss, Inc.

Catheterization and Cardiovascular Interventions 70:745–748 (2007)

predicted gradient of 6 mm Hg; the leaflets were nor-mally formed. Biventricular function was normal.Cardiac catheterization was performed to assess the

anatomy and hemodynamics of the UCS, as well asthe possible significance of the mitral stenosis as deter-mined with balloon occlusion of the defect. The studydemonstrated the following intracardiac pressures: LA:A wave: 9 mm Hg, V wave: 10 mm Hg, mean: 7 mmHg; RA: mean: 5 mm Hg; left ventricular end diastolicpressure: 8 mm Hg. Right ventricular systolic pressurewas mildly elevated (35/6 mm Hg); there was a10 mm Hg gradient to both pulmonary arteries, whichwas considered to be flow related. Aortic saturationwas 99% on room air. There was a significant O2 satu-ration step up from the R-SVC (53%) to the RPA andLPA (82% in both), indicating a QP/QS of 2.7:1.L-SVC saturation was 80% likely secondary to refluxinto the vessel from the LA. The mitral valve flowwas assessed with transesophageal echocardiography atbaseline and with balloon occlusion of the coronarysinus septal defect. At baseline mean gradient acrossthe valve was 2–4 mm Hg and with balloon occlusion5–6 mm Hg. The annulus measured 11 mm in diame-ter and the excursion of the mitral leaflets was normal.There was no mitral regurgitation. It was consideredthat the degree of mitral valve stenosis was not signifi-cant enough to affect possible post UCS closuredynamics.Several angiograms were performed into the L-SVC

to assess the site and size of the atrial defect (Fig. 1).

The defect measured 1 cm at its longest diameter. TheL-SVC measured 7 mm in diameter and the coronarysinus 9 mm in the area of the defect and 8 mm at itsRA orifice. The distance between the cephalic edge ofthe defect and the L subclavian vein was �5 cm. Aselective injection into the left coronary artery wasperformed to assess the site of entrance of the coro-nary veins into the coronary sinus. The coronary veinwas visualized draining into the coronary sinusbetween the defect and the RA, �3 mm from thedefect. On the basis of these findings, it was decidedto attempt to close the coronary sinus defect with aself-expandable covered stent.A 0.0350 Rosen wire (Cook, Bloomington, IN) was

advanced from the RA into the L-SVC. A 9 Fr,30 mm length 3 12 mm diameter self-expandable stent(Cordis, Hialeah, FL) was advanced over the wire intothe L-SVC. The position of the stent was confirmed byangiography from a catheter in the L internal jugularvein. The stent was carefully uncovered under TEEand angiographic guidance. As the stent was beingdeployed, great care was taken to avoid covering theostium of the coronary vein. The stent formed a coneshape with its apex in the LSVC. After the procedure,TEE evaluation revealed a small residual defect withleft to right shunting at the proximal portion of thestent. A L-SVC angiogram demonstrated the proximal(RA) end of the stent distal to the coronary vein orifice(Fig. 2). Pressures and oxygen saturation across the

Fig. 1. LSVC angiogram demonstrates the unroofed coronarysinus. LAA, left atrial appendage; LV, left ventricle.

Fig. 2. LSVC angiogram after stent deployment. The angio-gram of the coronary artery has been superimposed demon-strating the ostium of the coronary vein in the coronary sinus(arrow) far from the edge of the stent.

746 Torres et al.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

right heart were obtained and showed QP/QS of 1.1:1and unchanged normal PA pressures. The risk/benefitof placing a second covered stent to close the residualdefect was considered. It was decided that deploying asecond stent, which carried the risk of occluding theostium of the coronary vein, outweighed the potentialbenefit, given the small residual left to right shunt.The patient was observed overnight for observation

and discharged the following day on aspirin, whichwill continue for 6 months. Almost immediately his re-spiratory status showed remarkable improvement; hewas no longer tachypneic nor was retractions noted onphysical examination. The infant’s feeding patternimproved and he was not sweating excessively withfeedings. At 1-month follow-up, the patient’s improve-ment was sustained. Echocardiogram showed a smallresidual defect (Fig. 3) and was otherwise normal.

DISCUSSION

To our knowledge, this is the first report of trans-catheter closure of a UCS defect with a covered stent.From a technical standpoint, the main concern to beaddressed is the distance between the coronary veinostium and the edge of the coronary sinus defect.Obstruction of coronary vein drainage has been associ-ated with complete atrioventricular block, ventriculardysfunction and hemodynamic decompensation [7–9].Two anatomic aspects of the L-SVC-coronary sinus

should be considered: (1) The anatomy is not that of ahomogenous cylindrical structure; (2) There is a con-cave to convex longitudinal shape from the left to theright at the entrance into the atrium. These characteris-tics make it difficult to measure the exact length/diam-eter and therefore to estimate the degree of stent short-ening once deployed. A different alternative wouldhave been to deliver the stent from the left internaljugular vein to facilitate its accurate deployment in thearea of the coronary vein. However, angiographic guid-ance with this approach would have not been adequateduring the stent placement, and would have requiredthe insertion of a 9-Fr catheter through the neck of asmall infant. However, this technique might be usefulin an older patient.The use and reliability of self-expandable and non

self-expandable covered stents has been reported forthe treatment of other vascular lesions in pediatricsand adult patients in several studies [10–12].A 30-mm long stent was chosen instead of a shorter

one to assure proper anchoring in the L-SVC. Sincethe radial force of self-expandable stents is not high, alonger surface of contact between the stent and thevessel walls was considered necessary to decrease therisk of migration. The stent did not cover any signifi-cant vessel.The decision of not pursuing the placement of a sec-

ond stent to close the small residual defect was basedon the potential risk of occluding the coronary veinduring the procedure. A residual Qp/Qs in the range of1.1:1 is hemodynamically insignificant, and presumablywill never require reintervention.Unroofed CS defects are not suitable to be closed

by other devices, such as the atrial septal occluder in apatient of this size. The morphology of the defect ofthe unroofed CS is not necessary circular or ovale asin most secundum atrial defects and defining its exactmorphology and the size of the rims by either TEE orballoon sizing is difficult. In addition, catheter manipu-lation would be extremely challenging due to lack ofroom within the CS to accomodate the delivery sheathand the right disk of the device.In summary it has been demonstrated that closure of

a UCS type defect is feasible with a covered stent.The anatomy of the defect must be defined by TEEand angiography prior to the procedure. The exactlocation of the coronary vein ostium into the coronarysinus must be noted by selective coronary angiography.A covered stent can be deployed from either the femo-ral region or the neck depending on the anatomic fea-tures and age of the patient. On the rare instance ofcongestive heart failure associated with a left to rightshunt via an UCS, this nonsurgical approach may be auseful management tool.

Fig. 3. Transthoracic echocardiogram shows the residualatrial defect at the edge of the stent. LA, left atrium; RA, rightatrium.

Closure of Unroofed Coronary Sinus 747

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

REFERENCES

1. Rhee EK, Evangelista JK, Nigrin DJ, et al. Impact of anatomic clo-

sure on somatic growth among small, asymptomatic children with

secundum atrial septal defect. Am J Cardiol 2000;85:1472–1475.

2. Lammers A, Hager A, Eicken A, et al. Need for closure of

secundum atrial septal defect in infancy. J Thorac Cardiovasc

Surg 2005;129:1353–1357.

3. Andrews R, Tulloh R, Magee A, et al. Atrial septal defect with

failure to thrive in infancy: Hidden pulmonary vascular disease?

Pediatr Cardiol 2002;23:528–530.

4. Kirklin JW, Barrat-Boyes. Cardiac Surgery. New York: Wiley;

1993.

5. Ootaki Y, Yamaguchi M, Yoshimura N, et al. Unroofed coro-

nary sinus syndrome: Diagnosis, classification, and surgical

treatment. J Thorac Cardiovasc Surg 2003;126:1655–1656.

6. Takahashi H, Kaminishi Y, Saito T, Misawa Y. Anatomical repair

of partially unroofed coronary sinus syndrome through the coronary

sinus orifice. Ann Thorac Cardiovasc Surg 2005;11:208–210.

7. Watson GH. Atresia of the coronary sinus orifice. Pediatr Car-

diol 1985;6:99–101.

8. Yokota M, Kyoku I, Kitano M, Shimada I, Mizuhara H, Saka-

moto K, Nakano H, Hamazaki M. Atresia of the coronary sinus

orifice. Fatal outcome after intraoperative division of the drain-

age left superior vena cava. J Thorac Cardiovasc Surg 1989;98:

30–32.

9. Fulton JO, Mas C, Brizard CP, et al. The surgical importance of

coronary sinus orifice atresia. Ann Thorac Surg 1998;66: 2112–

2114.

10. Pedra CA, Fontes VF, Esteves CA, et al. Use of covered stents

in the management of coarctation of the aorta. Pediatr Cardiol

2005;26:431–439.

11. Palma JH, de Souza JA, Alves CMR, et al. Self-expandable aor-

tic stent-grafts for treatment of descending aortic dissections.

Ann Thorac Surg 2002;73:1138–1141.

12. Brunkwall J, Lindblad B, Ivancev K, et al. AV-fistula treated by

a graft-covered self-expandable stent. Eur J Vasc Endovasc Surg

1996;12:243–245.

748 Torres et al.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).