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Table of Contents
Year : 2012  |  Volume : 15  |  Issue : 2  |  Page : 166-168
Sinus venosus atrial septal defect in a patient with Pentalogy of Fallot

1 Department of Cardiac Anaesthesia and Cardiothoracic, AIIMS, New Delhi, India
2 Department of Vascular Surgery, AIIMS, New Delhi, India

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Date of Web Publication16-Apr-2012

How to cite this article:
Singh SP, Chauhan S, Talwar S. Sinus venosus atrial septal defect in a patient with Pentalogy of Fallot. Ann Card Anaesth 2012;15:166-8

How to cite this URL:
Singh SP, Chauhan S, Talwar S. Sinus venosus atrial septal defect in a patient with Pentalogy of Fallot. Ann Card Anaesth [serial online] 2012 [cited 2023 Jan 27];15:166-8. Available from:

The Editor,

Pentalogy of Fallot is the association of Tetralogy of Fallot (TOF) with an atrial septal defect (ASD). The most common type of ASD found with TOF is ostium secundum (OS), followed by the primum ASD. [1] The association of a sinus venosus ASD (SV ASD) with TOF is rare. We, hereby, describe a case of SV ASD detected using transesophageal echocardiography (TEE) after the intracardiac repair of TOF.

A 5-year-old male child, weighing 15 kg, diagnosed with TOF and an OS ASD, underwent an uneventful intracardiac repair with the aid of moderately hypothermic cardiopulmonary bypass (CPB) and cardioplegic arrest of the heart. A TEE probe was not used because of non-availability. A, trans-right atrial, dacron patch VSD closure with right ventricular infundibular resection and pulmonary valvotomy was performed. The OS ASD was closed with a dacron patch and a patent foramen ovale was electively created. Post surgery, the ratio of right and left ventricular pressures was 0.5. One hour later, in the intensive care unit (ICU), the patient had systemic arterial desaturation and, on a fractional-inspired concentration of oxygen (FiO 2 ) of 100%, the PaO 2 was 44 mmHg only. On TEE, there was no residual VSD and the right ventricular outflow tract was found to be wide open. In the midesophageal (ME) 4-chamber view, air bubbles were seen entering the left atrium (LA) [Figure 1] continuously as fluid was being administered through a peripheral intravenous cannula placed on the dorsum of the left hand. On performing an agitated saline injection, microair bubbles were seen entering the left atrium and left ventricle [Figure 2]. In the ME bicaval view, no blood flow could be appreciated entering the right atrium (RA) through the superior vena cava (SVC). A possibility of SVC being misdirected into the left atrium while closure of OS ASD or a "missed" SV ASD was considered. On reoperation, a high SV ASD was unveiled and the patch used to close the OS ASD was extended further to close the SV defect. On saline contrast TEE, air bubbles were seen entering both the RA and the LA, with only minimal bubbles in the LA [Figure 3]. The patient was shifted to the ICU with stable hemodynamics and a PaO 2 of 65 mmHg on a FiO 2 of 100%. Thereafter, the patient was successfully extubated on postoperative day (POD) 7 and discharged on POD 12.
Figure 1: Fluid administered through the peripheral intravenous cannula entering the left atrium

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Figure 2: Saline contrast performed through the central venous cannula placed in the right internal jugular vein

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Figure 3: Saline contrast performed after closure of the sinus venosus atrial septal defect showing inflow of saline into both sides of the heart (very less on the left side)

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In the present case, it was difficult to prove whether the right to left shunting of saline contrast into the LA was because of a SV ASD or erroneous placement of the OS ASD patch as TEE was not done preoperatively. It appears that while snugging the SVC cannula, the SV ASD was missed and when the OS ASD was closed with a patch, the SVC was directed into the left atrium. The above case emphasizes two major issues in cardiac surgery: One, the use of TEE [2] and two: Missed anatomic defects on diagnosis. In this patient, if a TEE probe had been placed, not only would it have detected the inadvertent mistake of surgically draining SVC into the LA, postoperatively, but it might have also lead to the diagnosis of a SV ASD, preoperatively. [3] Because the association of a SV ASD with TOF is uncommon, the latter assertion may not have been fulfilled, but it would have definitely prevented the delay between the first and the second surgery. The question that arises is that whether an unreported high-lying SV ASD can be detected on the surgical table, especially if the surgeon is not looking for it? The answer lies in the technique of SVC cannulation and snugging. It is ideal to snug a SVC cannula just above the entry of the right upper pulmonary vein into the left atrium so that any SV ASD may be detected. However, it is difficult to contemplate why a surgeon would be so careful in a routine TOF patient when a preoperative TTE has clearly delineated the anatomy. Therefore, it is prudent to take precautions to rule out common anomalies, but consideration must be given to uncommon ones as well because they do occur.

   References Top

1.Dabizzi RP, Teodori G, Barletta GA, Caprioli G, Baldrighi G, Baldrighi V. Associated coronary and cardiac anomalies in the tetralogy of Fallot. An angiographic study. Eur Heart J 1990;11:692-704.  Back to cited text no. 1
2.Kamra K, Russell I, Miller-Hance WC. Role of transesophageal echocardiography in the management of pediatric patients with congenital heart disease. Paediatr Anaesth 2011;21:479-93.  Back to cited text no. 2
3.John J, Abrol S, Sadiq A, Shani J. Mixed atrial septal defect coexisting ostium secundum and sinus venosus atrial septal defect. J Am Coll Cardiol 2011;58:e9.  Back to cited text no. 3

Correspondence Address:
Sarvesh P Singh
112, Doctors Hostel, J P N A Trauma Centre, A.I.I.M S, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.95087

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  [Figure 1], [Figure 2], [Figure 3]