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Robotic Repair of Sinus Venosus Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return and Persistent Left Superior Vena Cava

Lewis, Clifton T.P. MD*; Bethencourt, Daniel M. MD; Stephens, Richard L. PAC*; Cline, Jennifer L. PhD, ARNP‡§; Tyndal, Charles M. CCP

Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery: September/October 2014 - Volume 9 - Issue 5 - p 388–390
doi: 10.1097/IMI.0000000000000093
Case Reports

The presence of partial anomalous pulmonary venous return and/or persistent left superior vena cava (LSVC) is usually viewed as a contraindication for robotic repair of complex atrial septal defects, such as those of the sinus venosus type. Three patients, aged 29, 73, and 23 years, successfully underwent totally endoscopic, robotic-assisted repair of sinus venosus–type atrial septal defect with partial anomalous pulmonary venous return and persistent LSVC. Two different techniques—direct cannulation or placement of a sump sucker—were successfully used to manage venous return from the persistent LSVC.

From the *Division of Cardiothoracic Surgery Princeton Baptist Medical Center, Birmingham, AL USA; †Division of Cardiothoracic Surgery, Long Beach Memorial Medical Center, Long Beach, CA USA; ‡Sarasota Memorial Hospital; §Sarasota Vascular Specialists, Sarasota, FL USA; and ∥University of Alabama at Birmingham Hospitals, Birmingham, AL USA.

Accepted for publication January 9, 2014.

Disclosures: Clifton T.P. Lewis, MD, is a consultant to Edwards Life Sciences Corp, Irvine, CA USA; Intuitive Surgical, Sunnyvale, CA USA; Medtronic, Inc, Minneapolis, MN USA; and St. Jude Medical, Inc, St Paul, MN USA. Daniel M. Bethencourt, MD, is a consultant to Edwards Life Sciences Corp, Irvine, CA USA; Intuitive Surgical, Sunnyvale, CA USA; and St. Jude Medical, Inc, St Paul, MN USA. Richard L. Stephens, PAC; Jennifer L. Cline, PhD, ARNP; and Charles M. Tyndal, CCP, declare no conflicts of interest.

Address correspondence and reprint requests to Clifton T.P. Lewis, MD, Princeton Baptist Medical Center, 833 Princeton Ave, SW, Birmingham, AL 35211 USA. E-mail:

In cardiac surgery, the most common applications for robotic surgery have thus far been mitral valve repairs and coronary revascularization. However, several centers have reported experience with robotic techniques for atrial septal defect (ASD) repairs.1–5 Published series have comprised approximately 100 total ASD repairs, with most cases requiring simple suture closures and not patch closure. Most excluded complex ASD repairs, including sinus venosus type with or without partial anomalous pulmonary venous return (PAPVR), and also those in patients with persistent left superior vena cava (LSVC).

Persistent LSVC is a relatively common congenital anomaly of systemic venous return.6 Although it is usually a benign condition, configurations in which the LSVC drains into the coronary sinus and subsequently into the right atrium (RA) can complicate cardiopulmonary bypass if not properly managed. Therefore, persistent LSVC represents a challenge for totally endoscopic robotic surgery.

We report the successful repair of three sinus venosus–type ASDs with PAPVR and persistent LSVC, using a totally endoscopic, robotically assisted approach, and describe two different operative techniques for managing the LSVC during bypass.

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Patient 1 was a 29-year-old Hispanic man who presented to the emergency department with symptoms of congestive heart failure (New York Heart Association functional class 3-4). Transthoracic echocardiography revealed a sinus venosus–type ASD with massive right atrial and right ventricular dilatation as well as evidence of pulmonary vascular hypertension. Cardiac catheterization revealed normal coronary arteries, a pulmonary-systemic blood flow ratio (Qp/Qs) of 2.2:1, and pulmonary artery systolic pressure of 92 mm Hg. Transesophageal echocardiography (TEE) confirmed the presence of a large sinus venosus ASD with PAPVR. Computed tomography angiography further revealed a persistent LSVC draining into the coronary sinus and a normal but small aortoiliac tree.

Patient 2 was a 73-year-old white man who was referred to our office for surgical closure of an ASD, 6 months after it was identified during a workup for cerebrovascular accident. The patient, who had recovered from the stroke, presented to us with mild congestive heart failure (New York Heart Association functional class 2). Catheterization demonstrated normal coronary arteries and a Qp/Qs of 1.8:1. Transesophageal echocardiography showed a large sinus venosus–type ASD with PAPVR. Computed tomography angiography showed a normal aortoiliac tree and a persistent LSVC draining into the coronary sinus.

Patient 3 was a 23-year-old white woman in whom a sinus venosus ASD was discovered by transthoracic echocardiography during treatment of bronchitis. Preoperative TEE, computed tomography angiography, and magnetic resonance imaging identified a persistent LSVC draining into an enlarged coronary sinus, a Qp/Qs of 2.72:1, pulmonary artery systolic pressure of 38 mm Hg, mildly elevated right ventricular systolic pressure, and no evidence of pulmonary hypertension. Ejection fraction was 65%.

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Operative Technique

All operations were performed using the da Vinci S surgical system (Intuitive Surgical, Inc, Sunnyvale, CA USA). For patients 1 and 2 (console surgeon: CTPL), a dual-lumen endotracheal tube was placed along with bilateral brachial arterial lines for use in monitoring occlusion balloon deployment and position. Bilateral, 16-gauge angiocatheters were placed in the right and left internal jugular veins for later cannulation. No other necklines were used.

Port placement was configured for a totally endoscopic “CO2-tight” approach and included a 12-mm camera port in the fourth intercostal space (ICS) just lateral to the midclavicular line; a 2-cm working port placed 2 cm lateral to the camera port in the fourth ICS; and left, right, and third arm retractor ports in the second, sixth, and fifth ICS, respectively (Fig. 1).



Two angiocatheters were placed lateral to the working port for use in retracting the pericardium (see below), and one was placed medially in the sixth ICS to retract the diaphragm.

Systemic heparinization was administered. The LSVC was cannulated through the left internal jugular vein and the right SVC was cannulated through the right internal jugular vein, using 15F arterial cannulas. Both were preformed percutaneously, via guide wire through the previously placed 16-gauge angiocatheter. The inferior vena cava was cannulated through the right femoral vein with a 25F cannula, and then all three were extended into the venous circuit using a double Y connector.

To prepare for cross-clamping, the femoral artery was cannulated with an introducer (arterial perfusion cannula) and an occlusion balloon advanced into the ascending aorta. A 23F cannula with a side arm for the occlusion balloon was used for patient 2; because of small femoral artery size in patient 1, a bilateral approach was used with a 19F introducer in the right femoral artery and a 17F arterial return in the left femoral artery. All cannulations were done under TEE guidance using a modified Seldinger technique.

The robot was docked, pericardial fat was removed, and cardiopulmonary bypass was initiated at normothermia. The pericardium was opened vertically, and retraction sutures were placed in the pericardium and brought out through the lateral angiocatheters to form a “landing pad” to hold the lung back and facilitate instrument exchange during the intracardiac repair.

The inferior and superior venae cavae were dissected circumferentially, and caval tapes were prepositioned for later use. The occlusion balloon was inflated in the ascending aorta, the aorta was clamped, and the heart was arrested with 1 L of cold-blood cardioplegia. Intermittent doses of cardioplegia were given at 15-minute intervals. Caval tapes were knotted, and a vertical right atriotomy was made. The dynamic left atrial robotic retractor was positioned to hold the anterior wall of the RA, and the posterior edge was sewn to the pericardial flap to hold this edge back and to keep a sump sucker positioned in the coronary sinus. This approach allowed excellent exposure of the defect, as seen in Figure 2. After careful inspection of the atrium, the ASD, and the relationship of the pulmonary vein ostia, a bovine pericardial patch was sewn to the edges of the ASD in such a way as to direct pulmonary vein flow into the left atrium.



The cross-clamp was released by deflating the balloon. No deairing was required because a CO2-tight approach introduces no air into the heart. The RA was then closed in a two-layer fashion with 4-0 ePTFE suture (Gore-tex; W. L. Gore & Associates, Inc. Flagstaff, AZ USA) and the caval tapes were released. The patient was weaned from bypass and decannulated. A single chest tube was placed through the right arm port, and a Blake drain (Ethicon, Somerville, NJ USA) was placed through the third arm port, and the remaining ports were closed.

In patient 3 (console surgeon: DMB), port placement and operative techniques were similar except for the following differences, which reflected individual surgeon preference: Instead of directly cannulating the LSVC through the internal jugular vein, it was instead left to drain into the coronary sinus, with excess return volume managed by placement of a plastic vented sucker. A 21F Edwards cannula and Edwards endoballoon were used for aortic clamping, venting, and cardioplegia. Finally, autologous pericardium was used, and a second pericardial patch was used to close the right atrial incision to decrease the possibility of stenosis at the RA-SVC junction.

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In all three cases, the sinus venosus ASD was successfully repaired with robotic techniques, and in each case, a pericardial patch was used to close the defect. In patients 1 and 2, the LSVC was cannulated directly via the internal jugular vein, whereas in patient 3, the RA-SVC junction was closed with a second patch, and the increased drainage from the persistent LSVC was managed by placing a sump sucker into the coronary sinus. Both of these approaches seemed suitable and reliable. Cross-clamp times for patients 1, 2, and 3 were 66, 65, and 137 minutes, respectively. Bypass times were 98, 138, and 155 minutes, respectively. Intensive care unit length of stay was less than 24 hours for each patient. Patient 1 was discharged on postoperative day 3; patient 2, on day 4; and patient 3, on day 3. There were no transfusions and no postoperative complications. Discharge echocardiograms showed no evidence of residual shunt in any of the three patients.

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There have been a number of reports of robotic ASD repairs published in the literature1–5; however, the presence of additional congenital anomalies, such as PAPVR and/or persistent LSVC, has been viewed by some surgeons as a contraindication for robotic ASD repair because of the added surgical complexity. To our knowledge, this is the first report to describe the successful repair of sinus venosus ASD with PAPVR and a persistent LSVC.

We describe two different techniques for addressing the persistent LSVC, one by directly cannulating it through the left internal jugular vein and one in which a sump is placed into the coronary sinus to scavenge the increased drainage. Retraction of the posterior right atrial wall by sewing it to the pericardial flap helped provide stable exposure of the defect and the other structures in the RA and allowed successful repair of these more complicated ASDs. All types of ASDs are now routinely approached in this manner at our institutions.

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The authors thank Jeanne McAdara-Berkowitz, PhD, for expert editorial assistance.

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1. Argenziano M, Oz MC, Kohmoto T, et al. Totally endoscopic atrial septal defect repair with robotic assistance. Circulation. 2003; 108 (suppl 1): II191–II194.
2. Bonaros N, Schachner T, Oehlinger A, et al. Robotically assisted totally endoscopic atrial septal defect repair: insights from operative times, learning curves, and clinical outcome. Ann Thorac Surg. 2006; 82: 687–693.
3. Morgan JA, Peacock JC, Kohmoto T, et al. Robotic techniques improve quality of life in patients undergoing atrial septal defect repair. Ann Thorac Surg. 2004; 77: 1328–1333.
4. Torracca L, Ismeno G, Alfieri O. Totally endoscopic computer-enhanced atrial septal defect closure in six patients. Ann Thorac Surg. 2001; 72: 1354–1357.
5. Wimmer-Greinecker G, Dogan S, Aybek T, et al. Totally endoscopic atrial septal repair in adults with computer-enhanced telemanipulation. J Thorac Cardiovasc Surg. 2003; 126: 465–468.
6. de Leval MR, Ritter DG, McGoon DC, Danielson GK. Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc. 1975; 50: 599–610.

Minimally invasive surgery; Robotic surgery; Sinus venosus atrial septal defect; Partial anomalous pulmonary venous return; Persistent left superior vena cava

©2014 by the International Society for Minimally Invasive Cardiothoracic Surgery