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Minimally Invasive Robotically Assisted Repair of Partial Anomalous Venous Connection

Pirelli, Luigi MD; Kliger, Chad A. MD; Patel, Nirav C. MD; Bono, Marcella MS; Ruiz, Carlos E. MD, PhD; Jelnin, Vladimir MD; Fontana, Gregory P. MD

Innovations: Technology & Techniques in Cardiothoracic & Vascular Surgery: January/February 2017 - Volume 12 - Issue 1 - p 71–73
doi: 10.1097/IMI.0000000000000323
How-To-Do-It Articles

Abstract: We describe a novel robotically assisted minimally invasive surgical technique for repair of partial anomalous pulmonary vein connection (PAPVC). Partial anomalous pulmonary vein connection is a rare congenital anomaly that consists in drainage of one or more pulmonary veins into the systemic venous system. Traditionally, large thoracotomy incision and sometimes establishment of cardiopulmonary bypass are needed to redirect the abnormal pulmonary vein to the left-sided reservoir. We describe a robotically assisted, minimally invasive, off-pump technique for the treatment of the left PAPVC in a 57-year-old patient with signs of progressive right ventricular dilatation. The Da Vinci robot was used for mediastinal dissection and isolation of the distal aspect of the left superior anomalous pulmonary vein from the brachiocephalic vein. Through a left minithoracotomy, under direct vision, the pulmonary vein was reanastomosed to the left atrial appendage, thus reconstituting a normal venous return pattern. The use of the da Vinci robot is a valid adjunct for correction of the left PAPVC. It helps mediastinal dissection and allows reconnection of the pulmonary vein to the left venous system via a small thoracotomic incision and without the use of cardiopulmonary bypass.

From the Heart and Vascular Institute of New York, Lenox Hill Hospital, Northwell Health System, New York, NY USA.

Accepted for publication August 7, 2016.

Disclosures: Chad A. Kliger, MD, is on the speaker's bureau for St. Jude Medical, Inc, St. Paul, MN USA, and Philips Healthcare, Amsterdam, The Netherlands. Luigi Pirelli, MD, Nirav C. Patel, MD, Marcella Bono, MS, Carlos E. Ruiz, MD, PhD, Vladimir Jelnin, MD, and Gregory P. Fontana, MD, declare no conflicts of interest.

Address correspondence and reprint requests to Luigi Pirelli, MD, 130 East 77th Street, 4th Floor, New York, NY 10075 USA. E-mail:

Partial anomalous pulmonary venous connection (PAPVC) consists of an abnormal drainage of one or more pulmonary veins into the systemic venous system.1 Commonly associated with atrial septal defects (ASDs), the incidence of PAPVC at autopsy has been reported between 0.6% and 0.7% of the congenital cardiac defects; the clinical frequency may be lower, indicating that many patients remain asymptomatic. In the absence of associated anomalies, symptoms depend on the number of anomalous veins, their site of connection, the presence and the size of ASD, and pulmonary venous resistance. Patients can present in adulthood with symptoms of fatigue, dyspnea, palpitation, tachycardia, and/or chest pain. We describe a patient with a left superior pulmonary vein (LSPV) draining into the brachiocephalic trunk, who underwent minimally invasive, robotically assisted, off-pump diversion of the anomalous pulmonary vein to the left atrial appendage (LAA).

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A 57-year-old man presented with shortness of breath and echocardiographic findings of progressive dilation of the right ventricle (RV) associated with normal pulmonary pressures. Previous cardiac magnetic resonance imaging revealed the PAPVC with Qp/Qs of 1.4, RVEDV of 258 mL (RVEDVi 150 mL/m2), RV major axis of 8.3 cm, and right ventricular ejection fraction of 44%. Three-dimensional reconstructed chest computed tomographic angiography showed visceral and atrial situs solitus, concordant atrioventricular and ventriculoarterial connections, normal systemic venous return, and anomalous drainage of the LSPV into the left brachiocephalic vein (Fig. 1). The right heart catheterization confirmed a Qp/Qs of 2.1/1 with RV and pulmonary pressures recorded as 24/3 (5) and 24/9 (16) mm Hg, respectively. Left heart catheterization revealed normal LVEF and absence of significant coronary disease. Considering the progressive dilation of the RV associated with worsening of left-to-right shunt, decision was made to proceed with the surgical correction.

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The patient was brought to the operating room and intubated with a double-lumen endotracheal tube for isolation of the left lung. The Da Vinci robot was docked in and its arms introduced through three ports in the second, fifth, and seventh intercostals space, along the left anterior axillary line. The chest cavity was filled with carbon dioxide. The anomalous LSPV was easily visualized and isolated (Fig. 2). Particular attention was devoted to the phrenic nerve and adjacent vascular structures. After complete mobilization of the vein, heparin was administered, the robot was docked out, and the middle port incision was extended medially for approximately 2 in. Once the patient was fully anticoagulated, the pulmonary vein was stapled and transected under direct vision with a Thoracoscopic GIA 45 stapler at the connection to the innominate vein (IV, Fig. 3A). The pericardium was then opened under direct vision, and the LAA was visualized and retracted. The left pulmonary artery was controlled and snared down to avoid blood overflow. A C-clamp was applied to the base of the LAA, and the pulmonary vein was anastomosed with a 5-0 Prolene running suture in an end-to-side fashion (Fig. 3B). After deairing of the left heart, the clamp was removed and hemostasis was achieved. Pressures were checked in the pulmonary vein and left atrium simultaneously, and no gradient was noted. Protamine was administered, and the small incision sutured in the usual fashion after introduction of a small chest tube in the pleural space. The patient tolerated the procedure well and was extubated in the operating room. Postoperative transesophageal echocardiogram showed stable RV function and normal pulmonary vein return pattern. The patient was discharged from the hospital 3 days after surgery in New York Heart Association functional class 1.

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Partial anomalous pulmonary venous connection is a rare congenital anomaly resulting from a partial disconnection between the canalizing pulmonary vein and the venous plexus. Anastomoses between unconnected pulmonary segments and the systemic venous plexus persist and develop. In pediatric studies, PAPVC has been reported to be as twice more common in males than in females and arising in 90% of cases from the right upper lobe, with only 10% from the left upper lobe. Most commonly, the right upper or middle lobe connects to the right atrium or the superior vena cava. This form is almost always associated with a sinus venosus type ASD.2,3 Rarely, venous drainage is into the inferior vena cava, the IV, the coronary sinus, the cavae, the right atrium, or the left subclavian vein. Cases in which the left pulmonary veins drain into the systemic circulation are even less common. Partial anomalous pulmonary venous connection can also be associated with congenital heart disease such as heterotaxia syndromes and scimitar syndrome.4

Partial anomalous pulmonary venous connection can be asymptomatic for decades and present later in life with signs of progressive RV dilation and pulmonary hypertension.1 A standard approach to correct the most common form of PAPVC (right upper or middle pulmonary vein draining to the SVC and associated ASD) is to baffle the pulmonary vein inside the SVC to and through the ASD to the left atrium. The patch separates the right and the left atria. This technique implies median sternotomy and establishment of cardiopulmonary bypass with bicaval cannulation.5 Potential complications of this procedure are SVC stenosis, arrhythmias, and damage to the sinus node. A left anomalous pulmonary vein connection can be repaired via a median sternotomy or via a large left thoracotomy with or without cardiopulmonary bypass. The incision in the latter should be wide enough to enable the surgeons to reach the IV in the superior mediastinum. The Da Vinci robot allowed the dissection, isolation, and separation of the distal part of the pulmonary vein from the brachiocephalic vein. Without the robotic long arms, a wider incision and the spread of intercostal spaces would have been necessary to complete the dissection.

ElBardissi et al6 described the largest series of surgical correction of PAPVC. They analyzed data from 376 patients with PAPVC from 1954 until 2006. Although the surgical technique was similar to our case, only 13 patients were treated with a minimally invasive approach and none with a robotically assisted technique. Repair of left-sided PAPVC was performed via a left thoracotomy without cardiopulmonary bypass in only 13 patients. The robot allowed us to minimize the width of the lateral thoracotomy, thus reducing overall surgical trauma and promoting a faster recovery.

The approach described is a novel example of minimally invasive, robotically assisted, off-pump technique for correction of PAPVR. The procedure is feasible and safe, with the potential of reducing postoperative pain and shortening overall length of stay.

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1. Kirklin JW, Barrett-Boyes BG. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kirklin JW, Barrett-Boyes BG, eds. Cardiac Surgery. 2nd ed. Vol 1. New York, NY: Churchill Livingstone, 1993;1:627–630.
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Cardiac anatomy/pathologic anatomy; CHD in adult; Minimally invasive surgery; Pulmonary veins; Robotics

©2017 by the International Society for Minimally Invasive Cardiothoracic Surgery