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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 13  |  Issue : 1  |  Page : 59-63
Role of intraoperative echocardiography in surgical correction of the superior sinus venosus atrial septal defect


Department of Anaesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Click here for correspondence address and email

Date of Submission22-Nov-2008
Date of Acceptance17-Aug-2009
Date of Web Publication11-Jan-2010
 

   Abstract 

Superior type of sinus venosus atrial septal defect (SVASD) is invariably associated with the unroofing of right upper pulmonary vein (RUPV). Warden procedure and pericardial patch repair with rerouting of the RUPV are commonly performed operations for the superior SVASD. Both operations involve the risk of obstruction to the flow of superior vena cava or rerouted pulmonary vein in the postoperative period. The sinus venosus defects are well visualized on the transesophageal echocardiography (TEE) because of the proximity of the TEE probe to these structures. We are reporting two cases operated for the superior SVASD with unroofed RUPV, highlighting the intraoperative echocardiographic features before and after the surgery.

Keywords: Pericardial patch, sinus venosus defect, transesophageal echocardiography

How to cite this article:
Gadhinglajkar S, Sreedhar R, Jayakumar K, Misra M, Ganesh S, Mathew T. Role of intraoperative echocardiography in surgical correction of the superior sinus venosus atrial septal defect. Ann Card Anaesth 2010;13:59-63

How to cite this URL:
Gadhinglajkar S, Sreedhar R, Jayakumar K, Misra M, Ganesh S, Mathew T. Role of intraoperative echocardiography in surgical correction of the superior sinus venosus atrial septal defect. Ann Card Anaesth [serial online] 2010 [cited 2019 Sep 23];13:59-63. Available from: http://www.annals.in/text.asp?2010/13/1/59/58837



   Introduction Top


The sinus venosus defects are well visualized on the transesophageal echocardiography (TEE) because of the proximity of the TEE probe to these structures. We are reporting two cases of superior sinus venosus atrial septal defect (SVASD) with unroofed RUPV operated for the Warden procedure and pericardial patch repair with rerouting of the right upper pulmonary vein (RUPV) respectively. We are also describing the intraoperative echocardiographic features before and after the surgery


   Case Reports Top


Case 1

A three-year-old female patient, weighing 10 kg, presented to us with symptoms of recurrent respiratory infection since the age of four months, tachypnea and feeding difficulty. On examination, the heart rate was 98/min. A 4/6 ejection systolic murmur was heard at the pulmonary area. Preoperative transthoracic echocardiography (TTE) revealed a 10 mm SVASD anomalously draining RUPV, and pulmonary valve stenosis. She was subjected to catheter studies and angiography for further evaluation, that detected a step up in the oxygen saturation at right atrial level. The right ventricular systolic/diastolic pressures were 73/12 mmHg. A systolic pressure gradient of 48 mmHg was noticed across the pulmonary valve. Apart from confirming the diagnosis of SVASD and opening of the RUPV at junction of superior vena cava (SVC) and right atrium (RA) junction, the angiography also showed normal return of other pulmonary veins and presence of a persistent left superior vena cava (PLSVC) draining into coronary sinus.

The child was premedicated with 5 mg of oral midazolam one hour before surgery. After anesthesia induction a pediatric transesophageal echocardiography (TEE) probe was inserted and TEE examination was accomplished using Sonos 7500 machine (Philips ultrasound, US). A superior type of SVASD, shunting in left-to-right direction, was clearly seen during cardiac inspection in transverse plane (at zero sector-angle). This view was obtained by withdrawing the probe from mid esophageal (ME) 5-chamber view and slightly rotating it clockwise until it reached the junction between the SVC and right atrium (RA). On withdrawing the probe further from this level, we could visualize the unroofed RUPV opening in the SVC [Figure 1], [Video 1]-[Multimedia file 1]. The rounded appearance of the SVC was replaced with a "tear drop" appearance. On turning the navigation scan to (ME) bicaval view and withdrawing probe from this position to the level of right pulmonary artery (RPA) enabled us to visualize the SVC overriding the atrial septum [Figure 2]a. The sinus venosus defect was situated inferior to the RPA. On advancing the sector between 110 to 120 degrees and rotating the shaft a bit clockwise, we could identify the RUPV opening in the SVC [Figure 2]b, [Video 1]-[Multimedia file 2]. Applying more rightward twist on the probe could bring the right inferior pulmonary vein in focus, which was draining into the left atrium (LA). Left-sided pulmonary veins were found draining into the LA. A persistent left-sided SVC was detected on scanning in the midesophageal views near the left atrial appendage. The coronary sinus seen at 4-chamber view was dilated (>1 cm). The right ventricular outflow tract revealed stenosis of pulmonary valve and post-stenotic flow acceleration in the pulmonary artery.

After performing sternotomy and opening the pericardium, operating surgeon confirmed the presence of PLSVC. The right-sided SVC (RSVC) was small in size and no communicating vein was found between the RSVC and PLSVC. In view of unfavorable anatomy to carry out simple patch repair of the defect, we decided to perform the Warden procedure. After achieving electromechanical quiescence on cardiopulmonary bypass (CPB), pulmonary valvotomy was done. The unroofing of the RUPV and overriding of the RSVC were verified through an incision placed on the right atrial appendage. The RSVC was divided above the opening of RUPV and its caudal stump was oversewn using sutures. A pericardial baffle was stitched to redirect the RUPV flow via superior caval vein orifice into the LA that separated both atria as well. Later on, the cephalad end of SVC was anastomosed to the RA appendage. Patient was weaned from CPB with inotropic support of dobutamine 10 mcg/kg/min. On TEE examination in post-CPB period, a bolus injection of agitated saline revealed a free flow across the anastomosis between RSVC and RA appendage [Video 2]-[Multimedia file 3]. The pressure gradient between the RUPV and LA was less than 1.0 mmHg. The patient was weaned off ventilator after six hours of ventilation and discharged after an uneventful recovery.

Case 2

A three-year-old female patient, weighing 11 kg, was scheduled for pericardial patch closure of superior SVASD with rerouting of the RUPV. She presented with symptoms of recurrent respiratory infection since the age of one year. On auscultation, a 3/6 ejection systolic murmur was heard at the left sternal border. Diagnosis was established on TTE, which revealed SVASD with anomalous return of RUPV and mild pulmonary hypertension.

After anesthesia induction, the sinus venosus defect was inspected in a transverse plane at SVC-RA junction and also in the bicaval view with sweeping of the sector angle to 120 degrees. Scanning at the SVC-RA junction in the transverse plane revealed echo-lucent lateral border of SVC and its mirror image toward the right atrial side (mirror-image artifact). Further withdrawal of the probe from this position showed the unroofed RUPV opening in the SVC, which was also seen in the bicaval view. The SVC was seen overriding the atrial septum as in first patient. Right ventricular systolic pressure (RVSP) estimated using tricuspid regurgitation (TR) jet method was about 34 mmHg. Absent PLSVC and normal size of the RSVC were important findings on surgical inspection before the establishment of CPB. After cardioplegic arrest of the heart, an incision was placed on the antero-lateral aspect of the lower end of SVC and RA. Presence of the SVC override and unroofing of the RUPV were confirmed. A single pericardial patch was stitched around the margins of the defect that rerouted the RUPV to LA. Patient was weaned from CPB in a sinus rhythm and without any requirement for inotropic support. TEE inspection in the post-bypass period ruled out the presence of a residual ASD, and SVC or pulmonary venous flow obstruction [Video 3] - [Multimedia file 4]. The pericardial patch had rerouted the RUPV to the LA [Figure 3]. The RUPV-LA pressure gradient was less than 5 mmHg. Trachea was extubated five hours after artificial ventilation. The postoperative period was uneventful.


   Discussion Top


The sinus venosus defects are located either superiorly at the right atrial level and the superior caval vein, or inferiorly at the level of the inferior caval vein. The atrial communication in a SVASD is situated outside the true atrial septal structures, posterior to the fossa ovalis. The wall separating the SVC from LA is formed by the roof of RUPV entering the LA in normal subjects. A deficiency in this wall results in the superior type of defect associated with an unroofed RUPV, which drains into the SVC or RA. [1],[2] A great number of variations are observed in the anatomical drainage patterns of the pulmonary veins. Partial anomalous pulmonary venous return (PAPVR) is characterized by failure of one or more pulmonary veins to connect with the LA during fetal development. [3] Kirklin and Barrett-Boyes have described different patterns of anomalous connections of the right-sided pulmonary veins to the RA or the vena cavae. [4] The anomalous return of a pulmonary vein is termed as PAPVR in the surgical literature. However, the "anatomically displaced" pulmonary vein may not be equated with the "unroofed" pulmonary vein, which is anatomically in normal position. It would be more appropriate in a patient with SVASD, to consider the abnormal venous return of the RUPV occurring as sequelae of "unroofing" rather than describing it as a "PAPVR".

Diagnosis of the SVASD may sometimes be difficult on TTE because the atrial septum appears intact in most views. This limitation of TTE is more evident in adults than in children. Multiplane TEE is indispensable in imaging SVASD and is superior to the TTE in the diagnosis of this lesion. [1],[5],[6],[7] The proximity and orientation of the septum relative to the esophagus permits the entire structure to be adequately visualized in virtually every patient. The superior SVASD are best visualized in the ME bicaval view and in the transverse imaging plane at the SVC-RA junction. In an ME bicaval view, the RPA is usually seen in the superior aspect of the defect. The inferior aspect of the defect is formed by the superior aspect of the oval fossa of the atrial septum. In this view, the SVC appears to override the RA and LA across the border of atrial septum. A long axis image of this region is obtained by rotating the image plane from bicaval view to 120°. The defect between the LA and SVC is seen in immediate proximity to the RUPV, which enters the atrium on the right side of the septum. In the transverse imaging plane, the superior SVASD is seen as a communication between the SVC and the LA. The ultrasound waves strike the lateral border of the SVC and are reflected to the right, thereby creating a "mirror-image" artifact. [5] According to Van Praagh et al., [2] the unroofing of RUPV is invariably present with the SVASD, which however, is applicable more to the superiorly located sinus venosus defects than the inferior ones. A normally draining RUPV can be visualized in a transverse plane by turning the probe clockwise from 5-chamber view and in a longitudinal image plane developed from the bicaval view either by turning the probe to right or by rotating the transducer forward 10-20° from the standard position. [8] The entrance of vein into the LA usually appears on the far right of the screen as the SVC disappears from view. Although, the superior SVASD is seen in a transverse imaging plane at SVC-RA junction, visualization of the unroofed RUPV requires a further withdrawal of the probe. [9] Withdrawing the probe to the level of the RPA shows an anomalous connection of the RUPV as it enters the SVC, which results in a teardrop appearance of the normally round-appearing SVC. [10] All these features were present in both of our patients. Important echocardiographic features associated with a superior SVASD are summarized in [Table 1]. The examiner should take time to identify all four pulmonary veins entering the LA, as variable anatomy may change the course of the surgery. The view of right lower pulmonary vein is obtained by rotating the probe clockwise from the view of RUPV at modified bicaval view, which was obtained in our patient.

A PLSVC draining into the coronary sinus is the most frequent cause of enlargement of the coronary sinus. It is seen as an echo-free space between the left atrial appendage and left upper pulmonary vein, [11] with probe turned slightly counterclockwise. Its course can be traced in the midesophageal views to its entry into the coronary sinus, which is often enlarged (>1 cm). Identification of the PLSVC can be performed by injecting agitated saline through the left antecubital vein, which reaches the coronary sinus via PLSVC and subsequently drains into the RA. In the presence of a well formed persistent LSVC, the cardiac end of RSVC may be small in size, [4],[12] which has a surgical implication as in our case. Preoperative TEE examination of the cardiac end of RSVC may help predicting the necessity for the Warden repair.

The basic principle of surgical repair for the SVASD with unroofed RUPV is to redirect the flow of RUPV into the LA without creating a pulmonary venous or SVC obstruction, or injuring SA node or its supplying artery. In single-patch repair technique, the defect is closed by creating a pericardial tunnel that directs the flow from RUPV to the LA. However, as the pericardial tunnel is created within the SVC lumen, SVC flow obstruction may be sequelae in the postoperative period. Also, an incision crossing the SVC-RA junction puts the sinus node artery at risk with the consequence of permanent dysfunction of the sino-atrial node. The Warden procedure has been proposed particularly to prevent postoperative arrhythmias and the sinus node dysfunction. [13],[14] As the RSVC was small, the Warden procedure was indicated in our first patient. SVC and pulmonary venous obstruction are known complications after the internal patch closure of a SVASD. Hence, TEE verification of free flow across these vessels is necessary after the surgery. Anastomosis between the RA appendage and SVC is usually seen on the right side of the image, anterior to the native stump of SVC (conduit for the rerouted RUPV in the postoperative period), which is identified by injecting a bolus of agitated saline through internal jugular venous cannula. Flow obstruction may occur at this site and it should to be ruled out on TEE examination in the immediate postoperative period. The rerouted pulmonary vein is seen on turning the probe to right from the SVC-RAA anastomosis that may not be visualized simultaneously with the pulmonary vein. Although, pulmonary venous obstruction is uncommon after a Warden procedure, there are occasional reports in the literature suggesting the possibility of this complication. [15] Postoperative TEE examination in both patients demonstrated a gradient less than 2 mmHg across the pulmonary venous flow indicating that their rerouting was satisfactory.

In summary, with the help of TEE, we could recognize classical echocardiographic features associated with a superior type of SVASD like overriding of the SVC across the atrial septum and unroofing of the RUPV in the SVC. No flow obstruction was present in the SVC and rerouted pulmonary vein in the postoperative period. TEE is a useful diagnostic and monitoring tool during the surgeries involving a superior SVASD.

 
   References Top

1.Dinardo JA. Echocardiographic evaluation of intracardiac masses and septal defects. In Konstatd SN, Shernan SK, Oka Y, editors. Clinical transesophageal echocardiography. Philadelphia: Lippincott Williams and Wilkins; 2003. p. 191-202.  Back to cited text no. 1      
2.van Praagh S, Carrera ME, Sanders SP, Mayer JE, van Praagh R. Sinus venosus defects: unroofing of the right pulmonary veins--anatomic and echocardiographic findings and surgical treatment. Am Heart J 1994;128:365-79.  Back to cited text no. 2  [PUBMED]    
3.Alsoufi B, Cai S, van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg 2007;84:2020-6.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Atrial septal defect and partial anomalous pulmonary venous connection. In: Kirklin/ Barratt-Boyes, Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB, editors. Philadelphia, Pennsylvania: Churchill Livingstone; 2003. p. 715-51.  Back to cited text no. 4      
5.Adult congenital heart diseases. In: Oxorn DC, Otto CM, editors. Atlas of intraoperative transesophageal echocardiography. Philadelphia: Saunders Elsevier; 2007. p. 238-83.   Back to cited text no. 5      
6.Congenital heart diseases. In: Feigenbaum H, Armstrong WF, Ryan T, editors. Feigenbaum's Echocardiography. Philadelphia: Lippincott Williams and Wilkins; 2005. p. 559-636.  Back to cited text no. 6      
7.Hausmann D, Daniel WG, Mügge A, Ziemer G, Pearlman AS. Value of transesophageal color Doppler echocardiography for detection of different types of atrial septal defect in adults. J Am Soc Echocardiogr 1992;5:481-8.  Back to cited text no. 7      
8.Scott DA, Sutton DC. Image planes and standard views. In: Sidebotham D, Merry A, Legget M, editors. Practical perioperative transesophageal echocardiography. London: Butterworth Heinemann; 2003. p. 45-67.   Back to cited text no. 8      
9.Oliver JM, Gallego P, Gonzalez A, Dominguez FJ, Aroca A, Mesa JM. Sinus venosus syndrome: atrial septal defect or anomalous venous connection? A multiplane transesophageal approach. Heart 2002;88:634-8.  Back to cited text no. 9      
10.Pascoe RD, Oh JK, Warnes CA, Danielson GK, Tajik AJ, Seward JB. Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography. Circulation 1996;94:1049-55.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Kluger R. Common diagnostic pitfalls and cardiac masses. In: Sidebotham D, Merry A, Legget M, editors. Practical perioperative transesophageal echocardiography. London: Butterworth Heinemann; 2003. p. 69-87.  Back to cited text no. 11      
12.Nakahira A, Yagihara T, Kagisaki K, Hagino I, Ishizaka T, Koh M, et al. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 2006;82:978-82.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]  
13.Stewart RD, Bailliard F, Kelle AM, Backer CL, Young L, Mavroudis C. Evolving surgical strategy for sinus venosus atrial septal defect: Effect on sinus node function and late venous obstruction. Ann Thorac Surg 2007;84:1651-5.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]  
14.DiBardino DJ, McKenzie ED, Heinle JS, Su JT, Fraser CD Jr. The Warden procedure for partially anomalous pulmonary venous connection to the superior caval vein. Cardiol Young 2004;14:64-7.  Back to cited text no. 14  [PUBMED]  [FULLTEXT]  
15.Shahriari A, Rodefeld MD, Turrentine MW, Brown JW. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg 2006;81:224-30.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]  

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Correspondence Address:
Shrinivas Gadhinglajkar
Department of Anaesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.58837

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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

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