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Ann Thorac Surg 2007;83:2205-2207
© 2007 The Society of Thoracic Surgeons

Case Reports

Endoscopic Correction of the Adult Form of Scimitar Syndrome and Mitral Regurgitation: Anatomic and Technical Considerations

Oren Lev-Ran, MDa, Filip Casselman, MDa,*, Jose Coddens, MDb, Geert van Vaerenbergh, EBCPa, Hugo Vanermen, MDa,1

a Department of Cardiovascular and Thoracic Surgery, OLV Clinic, Aalst, Belgium
b Department of Anesthesiology and Critical Care Medicine, OLV Clinic, Aalst, Belgium

Accepted for publication December 29, 2006.

* Address correspondence to Dr Casselman, Department of Cardiovascular and Thoracic Surgery, OLV Clinic, Moorselbaan 164, Aalst, 9300, Belgium (Email: filip.casselman{at}olvz-aalst.be).


Dr Vanermen discloses that he has a financial relationship with Cardiovations.

 

   Abstract
Top
 Abstract
Introduction
 Comment
 Footnotes
 References
 
Scimitar syndrome is a congenital cardiac anomaly characterized by anomalous venous drainage of the right lung into the inferior vena cava. We report the combination of scimitar syndrome and mitral regurgitation and describe port-access correction for the adult form by means of an intraatrial baffle combined with mitral valve repair. Related considerations and modifications required in the standard Heartport (Cardiovations, Somerville, NJ) technique are discussed.

Scimitar syndrome is an infrequent congenital cardiac anomaly defined by anomalous venous drainage of the right lung to the inferior vena cava (IVC) [1, 2]. The resulting hemodynamic state is functionally similar to that of a large atrial septal defect. Extracardiac anomalies are frequent and include hypoplastic malformed right lung (abnormal bronchial branching and segmentation), dextrocardia, and pulmonary vascular lesions [1, 2]. These anomalies render distinct problems for a port-access approach for anatomic and technical reasons. We describe endoscopic reconstruction of an intraatrial baffle for the adult form combined with mitral valve repair. Alterations required in the standard technique are discussed.

A 37-year-old patient with previously diagnosed scimitar syndrome presented with congestive heart failure at New York Heart Association functional class II. The aberrant right pulmonary vein was supradiaphragmatic and drained at the junction of the right atrium–IVC. The right lung was hypoplastic, and the heart was severely dextropositioned to the right. Pulmonary vascular anomalies were excluded. The resulting left-to-right shunt (Qp/Qs) was 1.5:1. New grade IV mitral regurgitation was detected on transesophageal echocardiography (TEE).

Corrective surgery was undertaken with the goals of simultaneous repair of the mitral regurgitation and of the anomalous pulmonary venous drainage. Our management of Heartport (Cardiovations, Somerville, NJ) operations has previously been detailed [3]. A standard 21F superior vena cava cannula was introduced percutaneously. After preparation of the right femoral vessels, a 5-cm skin incision in the right inframammary groove was made. Contrary to normal anatomy, the rightward cardiac shift resulted in the heart being dangerously close to the chest wall and prevented safe completion of the anterolateral minithoracotomy. Standard wire-guided femoral arterial (21F) and venous cannulas (25F) were therefore introduced (Seldinger technique; TEE-guided) at this stage [3].

The initiation of the pump resulted in the backward withdrawal of the right ventricle and enabled placement of the ports and a soft-tissue retractor. Three standard ports of 5 to 7 mm were created for scope insertion, for insertion of carbon dioxide and a vent cannula, and for introducing atrial retractor handle [3].

Upon opening the pericardium, severe scimitar-related leftward deviation of the atrial septum and left atrium precluded standard access to the mitral valve through a left atriotomy [3] and warranted unplanned transseptal approach through a right atriotomy. Attempts to encircle the IVC for snaring were complicated by the position of the aberrant scimitar vein and adhesions and were eventually unsuccessful. As an alternative, a Chitwood clamp was introduced through a nonstandard right parasternal 10-mm port to occlude the IVC. Endoclamping of the aorta was performed [3], and transseptal mitral valve exposure was attained through a right atriotomy by using a long-blade atrial retractor.

P2 prolapse secondary to chordal rupture was repaired by segment resection and ring annuloplasty (Cosgrove #34, Edwards Lifesciences Inc, Irvine, CA; Fig 1A). An intraatrial baffle was then constructed from the scimitar vein orifice to the iatrogenic septal defect using two connected pericardial patches (Synovis, St. Paul, MN; Fig 1B).


Figure 1
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  		Fig 1. (A) Mitral valve ring annuloplasty (endoscopic transseptal view). (B) Intraatrial baffle (right atrial view).

 
Deep hypothermia (18°C) and retrograde cerebral perfusion were used during 50 minutes of circulatory arrest. Total pump time was 260 minutes (planned reperfusion time, 70 minutes). There was no residual mitral regurgitation or atrial shunt. The patient’s postoperative course was uneventful.


   Comment
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 Abstract
 Introduction
 Comment
Footnotes
 References
 
Contrary to the infantile form, the adult form usually consists of a left-to-right shunt volume of less than 50% [2]. Recent data suggest that this variant is well tolerated and that patients may sustain normal life without surgical correction [2]. Operative intervention, however, has been recommended in symptomatic or in asymptomatic patients with a Qp:Qs shunt exceeding 1.5:1 [4]. In this case, we report the combination of scimitar syndrome and mitral regurgitation. In our patient, there was no evidence supporting a common etiologic origin for both pathologies, and the mitral regurgitation was most likely a result of coexisting independent degenerative mitral valve disease unrelated to the syndrome. The primary cause of symptoms could not be distinguished and may be ascribed to severe mitral regurgitation.

Reflecting anatomic variability, several techniques have been used for redirecting the anomalous pulmonary venous drainage into the left atrium, with or without circulatory arrest. Confluence is restored by an intraatrial baffle from the scimitar vein orifice to the left atrium through an atrial septal defect [4] or by reimplantation of the scimitar vein (directly or indirectly) [5].

We used an endoscopic repair. Alterations in the standard management are addressed because they may facilitate future cases:

1 The scimitar-related rightward displacement of the heart occasioned by the small ipsilateral lung [1, 2] results in immediate proximity to the chest wall. This complicates the right anterolateral minithoracotomy and introduction of endoscopic instruments. Early initiation of pump vents causes the ventricle to relocate backwards enough to allow safe placements of standard ports. Although this strategy prolongs pump time, no other ready solution presents itself.
2 Consistent with previous observations, the atrial septum was deviated left, precluding left atriotomy. To access the mitral valve preparations for a transseptal mitral valve approach through a right atriotomy should therefore be made. Despite the limited experience and our concerns about the adequacy of a nondesignated endoscopic left atrial retractor to provide sufficient transseptal exposure, comfortable access was maintained throughout the procedure.
3 Failure to encircle and snare the IVC in the presence of the scimitar vein should be taken into consideration. Contributing factors are the existence of typical prominent right-sided pleuropericardial adhesions [1, 2] and the position of the scimitar vein. The use of an external occluding clamp introduced through separate parasternal port, as was done in this case, is an option. If prepared for, however, this distinct problem can be addressed by inserting an intracaval balloon occlusion catheter through nonstandard Y connections in the IVC cannula, as is occasionally used in reoperative endoscopic Heartport cases. In this technique, the over-the-wire balloon occluder is inserted through the Y piece and advanced and positioned under TEE guidance, excluding the need for complicated external dissection.

In conclusion, intracardiac repair of adult-form scimitar syndrome can be performed through a port access; however, modifications are required in the standard technique for anatomic reasons. Concomitant repair of mitral regurgitation can be performed.


   Footnotes
Top
 Abstract
 Introduction
 Comment
 Footnotes
References
 
1 Dr Vanermen discloses that he has a financial relationship with Cardiovations. Back


   References
Top
 Abstract
 Introduction
 Comment
 Footnotes
 References
 

  1. Brody H. Drainage of the pulmonary veins into the right side of the heart Arch Path 1942;33:221-240.
  2. Dupuis C, Charaf LA, Breviere GM, Abou P, Remy-Jardin M, Helmius G. The adult form of the scimitar syndrome Am J Cardiol 1992;70:502-507.[Medline]
  3. Casselman FP, Van Slycke S, Dom H, Lambrechts DL, Vermeulen Y, Vanermen H. Endoscopic mitral valve repair: feasible; reproducible and durable J Thorac Cardiovas Surg 2003;125:273-282.[Abstract/Free Full Text]
  4. Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom HM. Scimitar syndrome: twenty years’ experience and results of repair J Thorac Cardiovas Surg 1996;112:1161-1169.[Abstract/Free Full Text]
  5. Brown JW, Ruzmetov MR, Minniich DJ, et al. Surgical management of scimitar syndrome: an alternative approach J Thorac Cardiovas Surg 2003;125:238-245.[Abstract/Free Full Text]




This Article
Right arrow Abstract Freely available
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Right arrow Author home page(s):
Oren Lev-Ran
Filip Casselman
Hugo Vanermen
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Right arrow Articles by Lev-Ran, O.
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Right arrow Articles by Lev-Ran, O.
Right arrow Articles by Vanermen, H.
Related Collections
Right arrow Lung - other
Right arrow Valve disease

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