Ann Thorac Surg 2005;79:711-713
© 2005 The Society of Thoracic Surgeons
Case report
Novel Repair for Obstructed Total Anomalous Pulmonary Venous Connection to Coronary Sinus
Sajan Koshy, MCha,
Raman Krishna Kumar, DMb,
Rao Suresh Gururaja, MCha,
Krishnanaik Shivaprakasha, MCh*,a
a Division of Cardiac Surgery, Kochi, India
b Division of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Center, Kochi, India
Accepted for publication August 28, 2003.
* Address reprint requests to Dr Shivaprakasha, Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala 682 026, India
shivaprakashak{at}aimshospital.org
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Abstract
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A 4-month-old infant underwent surgery for total anomalous pulmonary venous connection to the coronary sinus with obstructions both at the entry of the common chamber into the coronary sinus and at the patent foramen ovale. Using a novel technique, the right atrium was opened with a transverse incision. The coronary sinus was de-roofed. The coronary sinuscommon chamber junction was split open into the common chamber. The incision extended up to the drainage of the right-sided pulmonary veins into the venous chamber. The common chamber was then anastomosed to left atrium thereby establishing an unobstructed pathway. The atrial septal defect was closed with a pericardial patch.
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Introduction
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Obstruction of the pulmonary veins in total anomalous pulmonary venous connections (TAPVC) to the coronary sinus (CS) is rare. When present, the usual treatment of de-roofing the coronary sinus leaves behind residual obstructions. We present an innovative technique that addresses this problem by establishing unrestrictive communication between the left atrium (LA) and the common venous chamber (CVC).
A 4 month-old male infant weighing 4,100 g presented with symptoms of heart failure. Echocardiography revealed obstructed TAPVC with severe pulmonary hypertension. All four pulmonary veins drained to a CVC, which was communicating to the CS through a communication measuring 6 mm (Fig 2b). The patent foramen ovale was 2 mms. The resting saturation at room air was 90%.

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Fig 2. (A) Postoperative echocardiogram showing unrestrictive anastomosis. (B) Preoperative echocardiogram displaying the obstruction at the coronary sinus junction with the common venous chamber. (CS = coronary sinus; LA = left atrium; RA = right atrium.)
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Through a median sternotomy, cardiopulmonary bypass was established with aorto-bicaval cannulation. Under deep hypothermic bypass, the common venous chamber was dissected free from the heart. The junction of the CVC with the CS was identified. After antegrade cardioplegic arrest, the right atrium was opened transversely (Fig 1a). The CS was de-roofed up to the junction of CVC with the CS (Fig 1b). The intervening atrial septum between the coronary sinus and the patent foramen ovale was excised. The restrictive opening of the CVC was confirmed. The right atrium incision was extended across the atrial septal defect into the posterior wall of the LA, similar to the technique used for correction of supracardiac TAPVC [1]. Working through the de-roofed CS, the CSCVC junction was slit open into the common chamber. The incision was then extended up to the right pulmonary veins (Fig 1c). The incision made in the common chamber was approximately 2 cm long in its axis. This was sutured to posterior wall of the LA, thereby ensuring an unobstructed channel between the two chambers. As the suture line reached interatrial groove, the atrial septal defect was closed with a redundant pretreated pericardium, thereby rerouting the CS to the LA. The suture line of the veno-atrial anastomosis was continued anteriorly to close the right atrium incision. The rest of the procedure was completed in standard fashion. Conventional ultrafiltration was done and cardiopulmonary bypass was discontinued. Postoperatively the pulmonary artery (PA) pressures were systemic in the first hour, which gradually came down to half-systemic levels with hyperventilation and administration of nitric oxide. The chest was left open with skin approximation due to the hypertrophic and enlarged right ventricle and high pulmonary pressures.

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Fig 1. (A) Transverse incision on the atrium. (B) Coronary sinus de-roofed to expose the restrictive communication of the vertical vein. (C) Common venous chamber slit open in continuity with the de-roofed coronary sinus. (CS = coronary sinus; IVC = inferior vena cava; LA = left atrium; RA = right atrium; SVC = superior vena cava.)
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The bypass time was 215 minutes and the cross-clamp time was 87 minutes. Delayed sternal closure was done 30 hours postoperatively. The patient had a smooth postoperative course, except for three episodes of pulmonary hypertension reaching systemic levels. He was extubated on postoperative day 3. Echocardiography showed unobstructed pulmonary venous pathway (Fig 2a) with mild pulmonary hypertension. On follow-up at 6 months, the child had gained weight and echocardiography confirmed unobstructed pulmonary venous flow with normalization of pulmonary hypertension.
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Comment
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Eighty-two patients were operated on for TAPVC in our center beginning in 1999 from which 66 patients were less than 1 year old (81%). Two of the 10 infants with coronary sinus TAPVC had obstruction in the pulmonary venous pathway (20%). The previous infant (who had been previously treated by conventional CS de-roofing) is having significant residual pulmonary venous obstruction at 3 months follow-up.
The cutback method is widely used for coronary sinus TAPVC repair. Another common technique involves fenestration between the CVC and the LA and direct closure of the atrial septal defect [1]. Progressive pulmonary venous obstruction occurs uncommonly in patients who undergo the cutback technique for cardiac TAPVC [2, 3]. The obstruction is more likely to occur whenever the pulmonary veins do not drain directly to the coronary sinus, but converge to form a short common vein as seen in this infant.
Our technique eliminates this complication by direct anastomosis between the CVC and LA, besides de-roofing. In addition, the transverse incision over the atria facilitates complete de-roofing of CS. Hence, we hypoth-esize that this complication is unlikely to occur in the long term. Applying the classic Shumacker approach [4], it is difficult to tackle this problem because interruption of the short communication between the common chamber and the LA is hazardous. Transection of vertical vein and CVCLA anastomosis through the superior approach has been advocated. Thus far this has only been performed in 1 patient to date [3]. Enlarging the obstructed pulmonary veins from within the heart carries the risk of going out of the heart near the atrioventricular groove where control of bleeding may be difficult.
Recently another technique of intraatrial rerouting is described [5]. The technique involves a cutback of the coronary sinus to develop a flap from the posterior LA wall. This is turned back to be anastomosed to the edge of the atrial septal defect. We would like to emphasize that these attempts highlight the unsatisfactory outcomes with conventional techniques.
In our case, we combined the two time-tested approaches to get a wide and unobstructed pulmonary venous pathway. We would stress the importance of transverse incision on the right atrium (Fig 1a). This incision allows extension into the posterior wall of the LA as in conventional repair of supracardiac TAPVC [1]. It also allows good exposure for de-roofing of the CS and visualization of the junction between the CVC and CS. The anastomosis has good growth potential as tissue-to-tissue approximation is achieved. A redundant pericardial patch to separate the atria ensured adequate left atrial volume.
In summary, this technique is fairly straightforward and easily reproducible. We do not anticipate long-term problems, because the technique is essentially a combination of the Shumacker approach and de-roofing of the CS.
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Acknowledgments
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We acknowledge the services of Dr Murugankutty Gopalan, Chief, Department of Medical Illustrations, and Prof Annamma Thomas, Department of English.
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References
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