Ann Thorac Surg 2009;88:1348-1350. doi:10.1016/j.athoracsur.2009.02.088
© 2009 The Society of Thoracic Surgeons
Case Reports
Modified Primary Sutureless Repair of Total Anomalous Pulmonary Venous Connection in Heterotaxy
Yoshihiro Oshima, MD*,
Masahiro Yoshida, MD,
Ayako Maruo, MD,
Chikashi Shimazu, MD,
Tomonori Higuma, MD,
Takeshi Inoue, MD
Department of Cardiovascular Surgery, Kobe Children's Hospital, Kobe, Japan
Accepted for publication February 20, 2009.
* Address correspondence to Dr Oshima, Department of Cardiovascular Surgery, Kobe Children's Hospital, 1-1-1 Takakura-dai, Suma-ku, Kobe, 654-0081, Japan (Email: ooshima_kch{at}hp.pref.hyogo.jp).
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Abstract
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Early and late mortality remain high after palliative open heart surgery in low-weight neonates. In addition, the need for neonatal repair and the presence of pulmonary venous obstruction are risk factors for mortality in right isomerism. We describe a modified primary sutureless repair of infracardiac total anomalous pulmonary venous connection in a 1,600 g neonate with heterotaxy. Endarterectomy of intimal hyperplasia localized to the anastomotic site was required at bilateral bidirectional cavopulmonary connection 6 months after the initial repair. The patient underwent a successful fenestrated Fontan procedure using an extracardiac conduit at the age of 2 years.
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Introduction
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Mortality remains high among heterotaxy patients treated in the modern surgical era, particularly those with obstructed total anomalous pulmonary venous connection (TAPVC). Pulmonary vein stenosis is common after repair of TAPVC in heterotaxy, and it is associated with poor outcomes [1]. We report primary sutureless repair of TAPVC in a low-weight neonate with heterotaxy.
A female newborn with a birth weight of 1,722 g and gestational age of 36 weeks had mild subcostal retraction and moderate cyanosis with oxygen saturations at 75% to 80% immediately after birth. Echocardiography confirmed single ventricle, heterotaxia syndrome (asplenia), double-outlet right ventricle, common atrioventricular valve, bilateral superior vena cavae, and infracardiac TAPVC with mild pulmonary venous obstruction. She was carefully managed in the neonatal unit and had hemodynamic and respiratory stability for the first week after birth. Thereafter, she had severe respiratory distress and cyanosis develop. Echocardiography showed severe pulmonary venous obstruction (peak velocity, 2.5 m/sec). Therefore she was intubated with respiratory support being administered. At 9 days of age (body weight, 1,600 g), she underwent primary sutureless repair of infracardiac TAPVC, ligation of patent ductus arteriosus, and pulmonary artery banding. After initiation of cardiopulmonary bypass with cannulation of the ascending aorta and a single right atrial venous cannulation, deep hypothermic circulatory arrest was instigated. Adequate exposure of the vertical pulmonary venous confluence located in the posterior mediastinum was provided. The posterior pericardium was incised over the pulmonary venous confluence. Under complete circulatory arrest, the pulmonary venous confluence was longitudinally incised across its entire length. The incision was not carried into each pulmonary vein and the vertical vein was left patent. The incision in the left-sided atrium was extended transversely across the back of the atrium, and the part of the atrial wall was excised. The divided edge of the atrial wall was sutured to the pericardium using a running 7-0 polypropylene suture, with the suture line located 4 to 5 mm from the divided edge of the pulmonary venous confluence (Fig 1A). The deep hypothermic circulatory arrest time was 24 minutes. Immediately after the operation, the patient had an arterial oxygen saturation level of 80%, and mild congestive heart failure. She was extubated 12 days after surgery. Early postoperative echocardiography showed unobstructed pulmonary venous return to the atrium. Because the pulmonary venous obstruction was suspected on echocardiography (peak velocity, 1.5 m/sec; anastomotic site, 4 to 5 mm in diameter), catheterization was performed at 6 months of age (body weight, 4 kg). Mean pulmonary artery pressure was 15 mm Hg; pulmonary vascular resistance was 2 Um–2. Mild to moderate pulmonary venous stenosis was detected at the anastomotic site. Therefore we performed endarterectomy of intimal hyperplasia at the anastomotic site, which had an ostium of 6 to 7 mm in diameter, with normal-looking individual veins. The scar-like tissue around the anastomotic region was removed, and through an intra-atrial approach, the incision was carried into each pulmonary vein, not beyond the second branch. Bilateral bidirectional cavopulmonary connections, division of the main pulmonary artery, and closure of the pulmonary valve orifice were performed at the same time. Postoperative echocardiography showed laminar caval flow in the bilateral superior vena cavae, normal right ventricular systolic function, and mild atrioventricular valve regurgitation. Her postoperative oxygen saturations ranged between 80% and 85% with oxygen by nasal cannula. After the surgery she had significant gastroesophageal reflux develop, and Nissen fundoplication was performed accordingly, which was done at 8 months of age. She underwent a successful fenestrated Fontan procedure with an extracardiac conduit at 2 years of age. At operation, there was no intimal hyperplasia surrounding the pulmonary vein ostia with normal-looking individual veins, with the exception of the hypoplastic left upper vein, which could not be corrected. At her most recent follow-up at 30 months of age, she is doing well clinically with oxygen saturations at 93%, with oxygen by nasal cannula and with echocardiography demonstrating no pulmonary venous stenosis (Fig 2).
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Fig 1. (A) The pulmonary venous confluence is longitudinally incised. The incision is not extended into each branch of the pulmonary vein, and the vertical vein is left open. Part of the back of the atrial wall is excised, and the divided edge of the atrial wall is sutured to the pericardium surrounding the pulmonary venous confluence using a running suture. The dotted line indicates the suture line of the atrium to the pericardium (arrow). (SVC = superior vena cavae.) (B) New modification of the primary sutureless technique. The divided edge of the pulmonary vein confluence is attached to the divided edge of the pericardium using five interrupted sutures of 7-0 polypropylene (arrows). The suture line of the atrium to the pericardium is the same as previously described.
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Fig 2. (A) Two-dimensional echocardiogram showing the connection between the atrium and the pulmonary vein (arrow). (B) Doppler image showing unobstructed pulmonary venous return to the atrium.
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Comment
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The TAPVC associated with heterotaxy syndrome is a risk factor for poor outcome [1–3]. Other risk factors for mortality include younger age at the time of the initial operation, obstructed TAPVC, and need for TAPVC repair at the initial operation [3]. Recently, early survival in patients with single ventricle and TAPVC has significantly improved, but intermediate survival remains poor [4]. Conversely, primary sutureless repair of the pulmonary veins has been associated with a low incidence of subsequent pulmonary venous obstruction (PVO) [5]. Hypoplasia of the pulmonary veins is an important risk factor for later development of PVO after conventional TAPVC repair [6], and also early and late mortality after palliation in low-weight neonates remain high. Hence we decided to perform primary sutureless repair of TAPVC. Our method was modified from the original technique described by Caldarone [7]. The incision was localized to the pulmonary vein confluence and did not extend beyond each pulmonary branch. This modification might have contributed to postoperative PVO, but this seems unlikely because the intimal hyperplasia was localized at the anastomotic site, which had normal-looking individual veins. Because the main cause of PVO appears to be turbulence of pulmonary venous flow around the pulmonary vein confluence caused by blood coming into contact with the rough surface of the adventitia, we performed a new modification in another patient. The divided edge of the pulmonary vein confluence was attached to the divided edge of the pericardium using four or five interrupted stitches of 7–0 polypropylene to reduce the rough zone (Fig 1B). Although that patient died of systemic-to-pulmonary artery shunt failure, postoperative echocardiography showed a wide pulmonary vein–atrial junction and no accelerated pulmonary venous flow at the anastomotic site. In primary sutureless repair, trauma to the venous wall can be minimized, and the risk of distortion can be prevented once the heart is repositioned in the pericardial well [8]. We do not think that the efficacy of the sutureless technique would be impaired by interrupted sutures of the pulmonary venous confluence to the edge of the divided pericardium.
Although this patient had mild-to-moderate PVO after initial surgery, she underwent the Fontan procedure without any complications, including recurrent PVO. However, as we have used this technique in few patients, we recognize that it needs to be applied in a large number of patients with long-term follow-up to ascertain its efficacy in preventing PVO. However, it appears that TAPVC repair using this sutureless technique did not increase the risk of poor long-term outcome, even in such a low-weight neonate with heterotaxy. We have also reported the modified primary sutureless technique involving limited sutures of the pulmonary vein to the pericardium. We advocate primary sutureless repair of TAPVC in heterotaxy patients.
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References
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- Foerster SR, Gauvreau K, McElhinney DB, Geva T. Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome Pediatr Cardiol 2008;29:536-544.[Medline]
- Heinemann MK, Hanley FL, Van Praagh S, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy Ann Thorac Surg 1994;57:88-91.[Abstract/Free Full Text]
- Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection J Thorac Cardiovasc Surg 1999;117:506-514.[Abstract/Free Full Text]
- Lodge AJ, Rychik J, Nicolson SC, Ittenbach RF, Spray TL, Gaynor JW. Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection Ann Thorac Surg 2004;78:1688-1695.[Abstract/Free Full Text]
- Yun TJ, Al-Radi OO, Adatia I, et al. Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies J Thorac Cardiovasc Surg 2006;131:1108-1113.[Abstract/Free Full Text]
- Jenkins KJ, Sanders SP, Orav EJ, Coleman EA, Mayer JE, Colan SD. Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection J Am Coll Cardiol 1993;22:201-206.[Abstract]
- Caldarone CA. "Sutureless" pulmonary vein stenosis repair. The Cardiothoracic Surgery Network. Clinical resources congenital cardiac expert technique (2004). www.ctsnet.org 1993Accessed August 26, 2009.
- Buitrago E, Panos AL, Ricci M. Primary repair of infracardiac total anomalous pulmonary venous connection using a modified sutureless technique Ann Thorac Surg 2008;86:320-322.[Abstract/Free Full Text]
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Abstract
Introduction
