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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 12  |  Issue : 1  |  Page : 53-56
Superior vena cava syndrome after pulsatile bidirectional Glenn shunt procedure: Perioperative implications

1 Department of Anaesthesiology Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, Kerala, India
2 Department of Cardiothoracic and Vascular Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, Kerala, India

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Date of Submission13-May-2008
Date of Acceptance28-Aug-2008


Bidirectional superior cavopulmonary shunt (bidirectional Glenn shunt) is generally performed in many congenital cardiac anomalies where complete two ventricle circulations cannot be easily achieved. The advantages of BDG shunt are achieved by partially separating the pulmonary and systemic venous circuits, and include reduced ventricular preload and long-term preservation of myocardium. The benefits of additional pulsatile pulmonary blood flow include the potential growth of pulmonary arteries, possible improvement in arterial oxygen saturation, and possible prevention of development of pulmonary arteriovenous malformations. However, increase in the systemic venous pressure after BDG with additional pulsatile blood flow is known. We describe the peri-operative implications of severe flow reversal in the superior vena cava after pulsatile BDG shunt construction in a child who presented for surgical interruption of the main pulmonary artery.

Keywords: Airway management, bidirectional Glenn shunt, superior vena cava syndrome, weaning from ventilator

How to cite this article:
Neema PK, Sethuraman M, Krishnamanohar S R, Rathod RC. Superior vena cava syndrome after pulsatile bidirectional Glenn shunt procedure: Perioperative implications. Ann Card Anaesth 2009;12:53-6

How to cite this URL:
Neema PK, Sethuraman M, Krishnamanohar S R, Rathod RC. Superior vena cava syndrome after pulsatile bidirectional Glenn shunt procedure: Perioperative implications. Ann Card Anaesth [serial online] 2009 [cited 2022 Dec 8];12:53-6. Available from:

Bidirectional superior cavopulmonary shunt (bidirectional Glenn shunt; (BDG)) is generally performed as a first- or second-stage procedure in situations of left or right heart hypoplasia in which complete two ventricle circulations cannot easily be achieved, as part of one and a half ventricle repair with pulsatile BDG in situations of hypoplastic but potentially partially usable right ventricle [1] and in patients presenting with tricuspid atresia having single ventricle physiology. [2],[3] The additional pulsatile pulmonary blood flow (PBF) is generally obtained by the construction of systemic-pulmonary artery anastomosis (Blalock-Tausig shunt) or through a stenosed native pulmonary artery or by controlled banding of the native pulmonary artery. We describe anaesthetic implications of severe reversal of flow in the superior vena cava (SVC) in a child who earlier had undergone pulsatile BDG shunt construction and presented for surgical interruption of the main pulmonary artery (MPA).

   Case Report Top

A one-and-half-year old male child weighing 12 kg presented for surgical interruption of the MPA. The child underwent balloon atrial septostomy at 27th day of life; later at 6 months of age, the child underwent pulsatile BDG shunt construction with the interruption of patent ductus arteriosus and atrial septostomy. At birth, the child was diagnosed to have d-transposition of great arteries (d-TGA) with severe pulmonary stenosis and considered unsuitable for biventricular repair. On examination, the child was tachypneic and showed dilated tortuous veins over the face, chest and abdomen [Figure 1a] and severe swelling of the upper half of the body. The external jugular veins were pulsatile and grossly dilated. The heart rate was 120/min, blood pressure 100/60 mmHg and peripheral saturation 84%. Transthoracic echocardiography examination showed d-TGA, a 3.5-mm atrial septal defect, and a 3 mm subpulmonic ventricular septal defect. The left ventricular ejection fraction was 49%. The BDG shunt showed severe flow reversal. The Doppler-estimated gradient across the pulmonary valve was 65 mmHg. The right ventricle (RV) angiography and aortic root injection showed good RV function with mild tricuspid regurgitation and aortopulmonary collaterals from right internal thoracic artery. The left ventricular angiogram showed a very large pulmonary artery [Figure 2]. On Cardiac catheterization, the measured RV end-diastolic and pulmonary venous wedge pressures were 20 and 13 mmHg, respectively.

The child was premedicated with oral atropine 0.5 mg. In the operating room, monitoring of ECG, pulse oximetry and non-invasive blood pressure was started. General anaesthesia was induced with oxygen, sevoflurane (3%-8%), and intravenous fentanyl 50 µg; pancuronium 2 mg, was administered to facilitate tracheal intubation with a 3.5-mm ID plain endotracheal tube (ETT). Initial attempt at tracheal intubation with a 4-mm ID ETT had failed. Controlled mechanical ventilation started with a tidal volume (V T ) of 110 ml and respiratory rate of 18 per minute. Anaesthesia was maintained with isoflurane (0%-2%) in oxygen, fentanyl 50 µg, midazolam 2 mg, pancuronium 1 mg and morphine infusion 40 µg/kg/h. Femoral artery and vein were cannulated for monitoring the arterial blood pressure (ABP) and central venous pressure (CVP). Left external jugular vein was cannulated; its transduction showed a good pulmonary artery pressure (PAP) waveform. The monitored ABP was 90/55, PAP 36/20, and CVP 10 mmHg. The MPA was approached through fourth intercostal space through left lateral thoracotomy. The MPA and the hemiazygous vein were dilated (approximately 2.5-3 cm and 1.0-1.5 cm, respectively). After their interruption, the PAP decreased to 25/16 mmHg. During surgical dissection and lung retraction, systolic PAP frequently increased to 45-50 mmHg; presumably due to increased pulmonary vascular resistance secondary to surgical retraction and compression of the left lung, and compression of the hemiazygous vein and its tributaries, compromising the decompression of systemic venous system and indirectly increasing systemic venous pressure. After the surgical procedure, the patient was transferred to intensive care unit and after elective overnight ventilation, weaning from mechanical ventilation was started with synchronized intermittent mandatory ventilation (SIMV) breath rate of 18 per minute and pressure support (PS) of 10 cm H 2 O; the V T generated was 20-30 ml. A few minutes after the initiation of weaning, the patient became restless. Immediately, weaning was discontinued and controlled ventilation was recommenced. Two hours later, weaning from mechanical ventilation was restarted with a SIMV rate of 18 per minute and a PS of 15 cm H 2 O; with an increase in the PS, the spontaneous V T increased to 40-50 ml; the PS was further increased to 18 cm H 2 O, and the spontaneous V T was increased to 50-60 ml; this time the patient remained comfortable. Within half an hour, without weaning, the child was extubated. Post-operatively, the upper body oedema reduced significantly [Figure 1b], but peripheral oxygen saturation decreased to 76%.

   Discussion Top

At hindsight, it appears that this patient may have been suitable for arterial switch or Rastelli or Nikaidoh repair at the time when he was diagnosed with d-TGA with severe pulmonary stenosis. The author had no control over the past surgical decisions, but had to plan the anaesthetic management on the basis of pre-operative evaluation and the proposed surgical procedure.

BDG is an anastomosis between SVC and right pulmonary artery (RPA) [Figure 3a]. The MPA is left uninterrupted if a pulsatile flow is desired. However, if, because of the forward flow, the PA pressure is high (>18 mmHg), it is either banded or interrupted [Figure 3b]. The main physiologic advantages of BDG shunt are an increase in PBF without an increase in ventricular volume load, less pulmonary artery distortion, and prevention of pulmonary artery hypertension. [4] The benefits of additional pulsatile PBF include the potential growth of pulmonary arteries, possible improvement in arterial oxygen saturation, and possible prevention of development of pulmonary arteriovenous malformations. [5],[6] With pulsatile BDG shunt, early Fontan repair is reported. [7] However, several studies report the development of increased systemic venous pressure with additional PBF and emphasises excluding all other sources of PBF. [8],[9] Caspi et al . [4] reported an increase in native pulmonary artery stenosis with time and shutting-off of additional pulsatile PBF. However, in our patient, PBF through the native pulmonary artery increased over time and resulted in severe reversal of flow in SVC and congestion and swelling of the upper half of the body. Presumably, the preoperative tachypnea was due to decreased lung compliance secondary to increased PBF and due to breathing through narrowed and congested airways. High SVC pressure, additionally, lead to the opening of venous collaterals (hemiazygous system of veins) between the left innominate vein and the inferior vena cava [Figure 3c] and decompression of SVC through the hemiazygous venous system. Apparently, the SVC drained partly through the collaterals (hemiazygous venous system) into the right atrium via the inferior vena cava.

The pathophysiologic effects of pulsatile BDG and the associated peri-operative issues are secondary to raised SVC pressure and include the congestion and narrowing of the airways, oedema of the tongue, prolonged circulation time and cerebral congestion. These changes may result in airway trauma on airway instrumentation, delayed systemic effects of intravenously administered drugs and delayed recovery from the anaesthetic agents. In our patient, the appropriate tracheal tube as indicated by age formula is 4.5-mm ID; earlier, 1 year ago, at six months of age during BDG Shunt construction, this child was intubated with a 4-mm ID ETT. However, during this procedure, at one-and-half years of age, the trachea could be intubated only with a 3.5-mm ID ETT. Evidently, in situation of reversal of flow in SVC due to additional pulsatile PBF, one should anticipate narrowed airway. Selection of the ETT based on the age formula or previous anaesthetic record may result in tracheal intubation with an oversized ETT and airway trauma. The other important implication is the weaning of the patient from ventilatory support; spontaneous/assisted breathing through a small diameter ETT imposes significant airway resistance. Presumably, because of high airway resistance during weaning, the child did not get sufficient V T at a PS of 10 cm H 2 O and became restless. With an increase in PS to 18 cm H 2 O, V T increased and the patient became comfortable. Evidently, in presence of a disproportionately small ETT, weaning should start at a higher pressure support. It should be noted that fighting with the ventilator may result in airway injury that can be devastating in presence of a narrowed airway. In such a situation, it may be prudent to extubate the patient without prolonged weaning trial; however, one should ensure adequate breathing efforts, acceptable blood gases, muscle power, level of consciousness and readiness to reintubate (if required).

To summarise, peri-operative implications due to severe flow reversal in SVC secondary to the presence of pulsatile BDG are described. Possibility of intubation with a smaller-sized ETT than the one recommended on the basis of age or previous anaesthetic record should be considered. In situation of intubation with a disproportionately small ETT, it is prudent to start weaning with a higher pressure support. Weaning from ventilatory support with a normally recommended pressure support level may result in inadequate V T and associated complications. The possibility of delayed systemic effects of intravenously administered drugs should be kept in mind.

   References Top

1.Chowdhury UK, Airan B, Sharma R, Bhan A, Kothari SS, Saxena A, et al . One and half ventricle repair with pulsatile Glenn: Results and guidelines for patient selection. Ann Thorac Surg 2001;71:1995-2002.   Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Hopkins RA, Armstrong BE, Serwer GA, Peterson RJ, Oldham HN Jr. Physiologic rationale for a bidirectional cavopulmonary shunt: A versatile complement to the Fontan principle. J Thorac Cardiovasc Surg 1985;90:391-8.  Back to cited text no. 2  [PUBMED]  
3.Mazzera E, Corno A, Picardo S, Di Donato R, Marino B, Costa D, et al . Bidirectional cavopulmonary shunts: Clinical applications as staged or definite palliation. Ann Thorac Surg 1989;47:415-20.  Back to cited text no. 3  [PUBMED]  
4.Caspi J, Pettitt TW, Ferguson TB Jr, Stopa AR, Sandhu SK. Effects of controlled antegrade pulmonary blood flow on cardiac function after Bidirectional cavopulmonary anastomosis. Ann Thorac Surg 2003;76:1917-21.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Penny DJ, Pawade A, Wilkinson JL, Karl TR. Pulmonary artery size after bidirectional cavopulmonary connection. J Card Surg 1995;10:21-6.  Back to cited text no. 5  [PUBMED]  
6.Uemura H, Yagihara T, Kawashima Y, Okada K, Kamiya T, Anderson RH. Use of bidirectional Glenn procedure in the presence of forward flow from the ventricles to the pulmonary arteries. Circulation 1995;92:II228-32.   Back to cited text no. 6  [PUBMED]  
7.Van de Wal HJ, Ouknine R, Tamisier D, Lιvy M, Vouhι PR, Leca F. Bi-directional cavopulmonary shunt: Is accessory pulsatile flow, good or bad? Eur J Cardiothorac Surg 1999;16:104-10.   Back to cited text no. 7    
8.Chang AC, Hanley FL, Wernovsky G, Rosenfeld HM, Wessel DL, Jonas RA, et al . Early bidirectional cavopulmonary shunt in young infants: Postoperative course and early results. Circulation 1993;88:II149-58.  Back to cited text no. 8  [PUBMED]  
9.Frommelt MA, Frommelt PC, Berger S, Pelech AN, Lewis DA, Tweddell JS, et al . Does an additional source of pulmonary blood flow alter outcome after a bidirectional cavopulmonary shunt? Circulation 1995;92:II240-4.  Back to cited text no. 9  [PUBMED]  

Correspondence Address:
Praveen Kumar Neema
B-9, NFH, Sree Chitra Residential Quarters, Poonthi Road, Kumarpuram, Trivandrum - 695 011, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.45014

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  [Figure 1a], [Figure 1b], [Figure 2], [Figure 3a], [Figure 3b], [Figure 3c]

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