HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Christian Kreutzer Gabriel Santiago Andres J. Schlichter Guillermo O. A. Kreutzer Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kreutzer, C. Articles by Kreutzer, G. O. A. Search for Related Content PubMed PubMed Citation Articles by Kreutzer, C. Articles by Kreutzer, G. O. A.

J Thorac Cardiovasc Surg 1998;115:462-463
© 1998 Mosby, Inc.

BRIEF COMMUNICATIONS

Persistent left superior vena cava: An unusual cause of subdividedleft atrium

Buenos Aires, Argentina

From the Division of Cardiovascular Surgery,^aCardiac Intensive Care Unit,^b Cardiology Services,^cEchocardiography Section,^d and Bazterrica Clinic, Children'sHospital "Ricardo Gutierrez" of Buenos Aires, Buenos Aires, Argentina.

Received for publication July 9, 1997 Accepted for publication July 17, 1997. Address for reprints: Christian Kreutzer, MD, Instructor inCardiovascular Surgery, Children's Hospital "Ricardo Gutierrez" of Buenos Aires,Gallo 1330 (1425), Buenos Aires, Argentina.

The persistence of a left superior vena cava (SVC) draining into thecoronary sinus has been described as the most common thoracic venous anomaly andis present in about 3% to 10% of patients with congenital heartdisease. Moreover, it has been reported as an anatomic finding withouthemodynamic consequences unless associated with an unroofed coronary sinus. In1987, however, Ascuitto and colleagues ¹reported a case in which a left SVC produced a subdivided left atrium.

We report five consecutive cases and one early case in which a persistentleft SVC produced a ridge in the left atrium, causing a subdivided left atrium.The aim of this report is to draw attention to this rare diagnosis as an unusualcause of obstruction to pulmonary venous flow.

Patients and methods. From April 1993 toDecember 1995, five patients with atrial septal defect (ASD), persistent leftSVC draining in the coronary sinus, and diverse associated anomalies wereoperated on in the Children's Hospital "Ricardo Gutierrez" of BuenosAires and Bazterrica Clinic. Demographic and anatomic characteristics of thepatients are summarized in Table I.The diagnosis of persistent left SVC was made by two-dimensionalechocardiography (ECHO-2D) in these five patients and confirmed by cardiaccatheterization and angiography in two. ECHO-2D showed a dilated coronary sinusand a ridge coursing diagonally along the inner aspect of the posterior wall ofthe left atrium. This divided the left atrium into two chambers: one proximal,where all the pulmonary veins drained normally, and one distal, which containedthe left atrial appendage and mitral valve. ECHO-2D in one patient withassociated tetralogy of Fallot revealed a "small" left ventriclewith an end-diastolic volume of 20 ml/m² body surface. At follow-up,all patients were studied with color-Doppler echocardiography, and the diastolicfunction was evaluated by transmitral flow.

Surgical technique. Through a mediansternotomy, the left SVC was carefully evaluated, as was the presence of theinnominate vein. We have observed that the left SVC is shorter in this entity,producing some degree of traction on the posterior wall of the left atrium. Whenthe innominate vein was absent and the right SVC was hypoplastic, the dominantleft SVC was temporary occluded, and if the pressure in the venous line of theleft arm rose above 20 mm Hg, a Pacifico DLP cannula (Medtronic DLP, GrandRapids, Mich.) was placed in the left SVC, leaving enough room for the placementof a conduit between the two SVCs. Ascending aorta, SVC, and inferior vena cavawere cannulated, and cardiopulmonary bypass was started. After aorticcrossclamping and administration of cardioplegic solution, the right atrium wasopened, the ASD was inspected, and a markedly dilated coronary sinus wasconfirmed. At close inspection of the left atrium through the ASD, a ridgeproduced by the impingement of the left SVC was noted. The ridge divided theleft atrium into two chambers: one proximal, with the pulmonary veins, and onedistal, with the left atrial appendage and mitral valve.

The coronary sinus was completely unroofed (Fig. 1, A), and the tissueforming the ridge was completely excised. The ASD was closed with an autologouspericardial patch, and the coronary sinus was closed with continuous suture (Fig. 1, B). Complete repairof the associated anomalies was performed after that. Once the aortic crossclamphad been released, the left SVC was sectioned and divided to eliminate thetension on the posterior aspect of the left atrium. It is remarkable that oncedivided the distance between the two ends was usually more than 10 mm. In twopatients with absence of the innominate vein, an 8 mm polytetrafluoroethyleneconduit was placed between the two SVCs.

View larger version (31K): [in this window] [in a new window]   Fig. 1. A,The coronary sinus iscompletely unroofed and the tissue that formed the ridge is completely excised.B, The ASD is closed with an autologouspericardial patch and the coronary sinus is closed with continuous suture.RSVC, Right SVC; LSVC,left SVC; Ao, aorta; Mv,mitral valve; CS, coronary sinus;Tv, tricuspid valve.

The autopsy of the patient operated on in 1983 showed a subdivided leftatrium and a severe flow obstruction to the mitral chamber produced by the ridgein the posterior aspect and by the ASD patch in the anterior aspect, a goodmitral valve repair, and no residual ASD. Left ventricular dimensions were smallbut within normal ranges.

Comments. It has been postulated that theabnormal membrane of the typical cor triatriatum may be induced by persistentleft SVC impinging on the posterior wall of the primitive left atrium. ^2,3In the particular group of patients described here, a ridge produced by the leftSVC subdivided the left atrium into two chambers, as in classic cor triatriatumtype Ab: one proximal, with the pulmonary veins, and one distal, with the mitralvalve. These patients had a preoperative diagnosis of persistent left SVCdraining into the coronary sinus. In this series, two patients with ASD hadsevere congestive heart failure. This could be explained by an obligatory shuntat the level of the left atrium. In the patient with tetralogy of Fallot, ⁴ the presence of the anomaly produceda "small" left ventricle according to ECHO-2D, and it was importantto have the preoperative diagnosis to achieve a successful correction. Whentetralogy of Fallot is associated with small left ventricle, the presence of aleft SVC should be considered. In the case without preoperative diagnosis ofsubdivided left atrium, the consequence was fatal; the patient died in thecardiac intensive care unit of pulmonary edema with high left atrial pressure onthe first postoperative day. We hypothesize that the obstruction was created bythe ASD closure. Furthermore, we believe that this anomaly should be consideredwhen pulmonary venous flow obstruction is suspected and is not explained bypulmonary vein stenosis, total anomalous pulmonary venous return, typical cortriatriatum, left atrial myxomas, or mitral valve anomalies. ⁵

In conclusion, we advocate the close inspection of the left atrium inpatients with persistent left SVC. In the presence of a ridge, the coronarysinus should be unroofed and the left SVC should be divided to completelyeliminate the tension on the posterior aspect of the left atrium.

References

1. Ascuitto RJ, Ross-Ascuitto NT, Kopf GS, FaheyJ, Kleinmann CS, Hellenbrand WE, et al. Persistent left superior vena cavacausing subdivided left atrium: diagnosis, embryological implications, andsurgical management. Ann Thorac Surg 1987;44:546-9.[Abstract]
   
2. Gharagozloo F, Bulkley BH, Hutchings GM. Aproposed pathogenesis of cor triatriatum: impingement of the left superior venacava on the developing left atrium. Am Heart J 1977;94:618-26.[Medline]
   
3. Thilenius OJ, Bharati S, Lev M. Subdividedleft atrium: an expanded concept of cor triatriatum sinistrum. Am J Cardiol 1976;37:743-52.[Medline]
   
4. Binotto MA, Aiello VD, Ebaid M. Coexistenceof divided left atrium (cor triatriatum) and tetralogy of Fallot. Int J Cardiol 1991;31:97-9.[Medline]
   
5. Radermecker MA, Grenade T, Jalali H, LegrandV, de Leval MR. An unusual case of cor triatriatum: clinical presentation andhaemodynamics of a rare anomaly. Cardiovasc Surg 1993;1:83-5.[Medline]
   

This article has been cited by other articles:

				F. J. Vargas, J. Rozenbaum, R. Lopez, M. Granja, A. De Dios, B. Zarlenga, E. Flores, E. Fischman, and E. Kreutzer Surgical approach to left ventricular inflow obstruction due to dilated coronary sinus. Ann. Thorac. Surg., July 1, 2006; 82(1): 191 - 196. [Abstract] [Full Text] [PDF]

				J. A. M. van Son and J. Hambsch Repair of subdivided left atrium associated with persistent left superior vena cava J. Thorac. Cardiovasc. Surg., September 1, 1998; 116(3): 535 - 535. [Full Text]

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Christian Kreutzer Gabriel Santiago Andres J. Schlichter Guillermo O. A. Kreutzer Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kreutzer, C. Articles by Kreutzer, G. O. A. Search for Related Content PubMed PubMed Citation Articles by Kreutzer, C. Articles by Kreutzer, G. O. A.