Outcomes of the Warden procedure for partial anomalous pulmonary venous drainage in children

Outcomes of the Warden procedure for partial anomalous pulmonary venous drainage in children Abstract OBJECTIVES When drainage of an anomalous pulmonary vein is high into the superior vena cava (SVC), traditional techniques of 1-patch or 2-patch repair may be challenging. The cavoatrial anastomosis technique (the Warden procedure) was developed to reduce the risk of sinus node dysfunction and SVC obstruction. Herein, we describe the outcomes of the Warden procedure in children at a single institution. METHODS A retrospective study was conducted on all children with partial anomalous pulmonary venous drainage (PAPVD) undergoing the Warden procedure from 1996 to 2015. RESULTS There were 42 PAPVD patients with a right upper pulmonary vein entering into the SVC undergoing the Warden procedure. The mean age of the patients at surgery was 5.5 years (71 days to 15.4 years). There was no operative mortality. Median hospital stay was 5 (3–25) days. One (2.4%) patient developed cavoatrial (SVC) obstruction. This patient required percutaneous intervention (stenting and balloon dilatation). There was 1 (2.4%) late death in a patient with PAPVD and pulmonary atresia due to a hypoxic brain injury following catheter intervention for a stenotic conduit. Survival was 97 ± 3% (95% confidence interval 83–100%) at 5 and 7 years. All patients were asymptomatic at a mean follow-up of 6 years (range 1 month to 21 years) after surgery. No patient had sick sinus syndrome, sinus node dysfunction or required permanent pacemaker at follow-up. CONCLUSIONS The Warden procedure for repair of PAPVD to the SVC can be performed with no operative mortality or permanent pacemaker requirement. Mid-term outcomes are excellent with a low occurrence of SVC obstruction. Surgery, Congenital heart disease, Pulmonary veins, Partial anomalous pulmonary venous drainage INTRODUCTION Numerous techniques have been described for repair of partial anomalous pulmonary venous drainage (PAPVD) of the right upper pulmonary veins to the superior vena cava (SVC) [1]. Repair aims at complete closure of the associated atrial septal defect (ASD) and redirection of the anomalous pulmonary veins into the left atrium. Partitioning the SVC into 2 pathways and manipulation of the right atrial-SVC junction increase the risk of pulmonary venous or SVC obstruction and injury to the sinus node. When entry of the pulmonary vein is high into the SVC, traditional techniques of 1-patch or 2-patch repair may be challenging. The cavoatrial anastomosis technique (the Warden procedure) was developed to reduce the risk of sinus node dysfunction and SVC obstruction in these cases [2]. Early outcomes of the procedure are promising [2]; however, numbers of patients are small, and no study in the reported literature exclusively studied outcomes in children. Herein, we describe the outcomes of the Warden procedure in children at a single institution. MATERIALS AND METHODS Patients The institutional research ethics board approved this study. All patients who underwent repair of PAPVD with the Warden procedure at the Royal Children’s Hospital between 1996 and 2015 were identified. Data were obtained by review of medical records from the initial admission until the last follow-up. All patients had a 12-lead electrocardiography and transthoracic echocardiography (to evaluate the cavoatrial anastomosis and pulmonary venous pathway) at annual cardiology review. A Holter monitor was performed if there were symptoms suggestive of dysrhythmias. Operative technique and patient selection The Warden procedure was selected based on surgeon preference and if there was concern that a 1-patch or 2-patch repair technique may have put the patient at risk of SVC obstruction. After sternotomy, the right SVC is circumferentially dissected and mobilized from the SVC-right atrial junction to the SVC-left innominate vein junction. The azygos vein is doubly ligated and divided to permit maximal mobilization of the SVC. Cardiopulmonary bypass (CPB) is instituted after cannulation of the ascending aorta, inferior vena cava and high SVC (near the SVC-innominate vein junction). If there is a large left SVC, additional venous drainage may be established. Two vascular clamps are placed across the SVC above the entrance of the highest anomalous right pulmonary vein. The SVC is then transected. The tip of the right atrial appendage is amputated and enlarged to the diameter of the cephalad SVC. Excision of the trabeculae in the right atrial appendage at the site of anastomosis is performed and reduces the risk of SVC obstruction. The caudal SVC stump is closed directly above the entry point of the anomalous pulmonary vein. The cephalad SVC is sewn to the amputated right atrial appendage with an interlocking continuous absorbable suture. The aortic cross-clamp is then applied and cardioplegic arrest induced. A high right atriotomy is performed to expose the sinus venosus or secundum ASD. If there is no defect, an interatrial septal defect is created. The anomalous right pulmonary venous drainage is baffled across the ASD into the left atrium with a fresh autologous pericardial patch. A Gore-Tex patch (W.L. Gore & Associates, Inc., Flagstaff, AZ, USA) may be used instead. The right atriotomy is then closed, and the patient is weaned off bypass as per standard protocol. All patients had intraoperative transoesophageal echocardiogram performed to confirm laminar flow through the baffle and cavoatrial anastomosis. Definitions Operative mortality was defined as death occurring within 30 days of surgery or later if the patient is still hospitalized (including patients transferred to other acute care facilities). All other deaths were considered as late. Postoperative cavoatrial (SVC) obstruction was defined as symptomatic (facial swelling, dyspnoea, headaches and venous engorgement of neck, trunk and upper extremities) right atrial-SVC anastomotic narrowing as identified on transthoracic echocardiogram and computerized tomography imaging. Postoperative pulmonary venous obstruction was identified during routine transthoracic echocardiogram or if the patient developed symptoms of heart failure and the stenosis was confirmed using computerized tomography imaging. Postoperative sinus node dysfunction was defined as persistent sinus bradycardia (<50 beats/min) or junctional/nodal rhythm, ectopic atrial rhythm or a wandering pacemaker (<60 beats/min) or pauses of more than 3 s. A low atrial rhythm was defined as inverted P waves in the inferior leads on electrocardiography. Patients were considered lost to follow-up if no medical information was available after hospital discharge. Data analysis All data were analyzed using Stata version 12 (Stata Corp, College Station, TX, USA). Data are expressed as mean ± standard deviation and median with interquartile range (IQR) as appropriate. The Kaplan–Meier curves were used for time to event (actuarial survival) analysis. RESULTS Detailed preoperative patient characteristics are listed in Table 1. Diagnosis of PAPVD was made by echocardiogram and computerized tomography imaging. Cardiac catheterization (n = 6) was used to confirm diagnosis earlier in the study period or when there were complex co-existing congenital heart defects. Surgery was performed with standard CPB. Mean CPB time was 93 ± 60 min (36–300 min), and mean cross-clamp time was 50 ± 41 min (10–198 min). Circulatory arrest was used in 1 patient. The mean lowest temperature during CPB was 33 ± 2°C (25–35°C). Concomitant repairs included ventricular septal defect closure in 4 (9.5%) patients, complete repair of pulmonary atresia (right ventricle to pulmonary artery conduit, ventricular septal defect closure and left/right pulmonary artery augmentation) in 1 (2.4%) patient, REV procedure in 1 (2.4%) patient, Ebstein valve repair in 1 (2.4%) patient, aortic valve repair in 1 (2.4%) patient, repair hypoplastic arch and coarctation in 1 (2.4%) patient and ASD closure in 38 (90%) patients. In 5 cases (12%), cavoatrial anastomosis could not be performed without excessive tension, hence, pericardial patch enlargement was performed. Median intubation time was 8 h (IQR 5–13 h), and median stay in the intensive care unit was 21 h (IQR 17–25 h). Median hospital stay was 5 (IQR 4–6) days. No patient developed dysrhythmias that required a temporary or permanent pacemaker in the immediate postoperative period. Table 1: Patient characteristics Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  ASD: atrial septal defect; BSA: body surface area; ECMO: extracorporeal membrane oxygenator; PDA: patent ductus arteriosus; SD: standard deviation; SVC: superior vena cava; VSD: ventricular septal defect. Table 1: Patient characteristics Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  ASD: atrial septal defect; BSA: body surface area; ECMO: extracorporeal membrane oxygenator; PDA: patent ductus arteriosus; SD: standard deviation; SVC: superior vena cava; VSD: ventricular septal defect. There were no hospital deaths. There was 1 late death (2.4%) in a patient with PAPVD and pulmonary atresia 1.2 years after surgery. The patient underwent catheter intervention for a stenotic conduit. During the procedure, there was a guide-wire perforation of the pulmonary artery resulting in a hypoxic arrest and hypoxic brain injury. The Kaplan–Meier analysis demonstrated actuarial survival of 97 ± 3% (95% confidence interval 83–100%) at 5 and 7 years (Fig. 1A). Figure 1: View largeDownload slide (A) The Kaplan–Meier survival curve. (B) The Kaplan–Meier freedom from reoperation or reintervention for the superior vena cava or pulmonary venous obstruction. CI: confidence interval. Figure 1: View largeDownload slide (A) The Kaplan–Meier survival curve. (B) The Kaplan–Meier freedom from reoperation or reintervention for the superior vena cava or pulmonary venous obstruction. CI: confidence interval. One (2.4%) patient developed SVC obstruction. This patient underwent surgery at 3.5 years of age (weight during surgery was 14.3 kg) after presenting with clinical features of a large shunt. The operation was uncomplicated, and the cavoatrial anastomosis was performed without any patch augmentation. The patient was discharged on Day 3 after surgery but developed SVC obstruction 3 years after the surgery. The patient was symptomatic and required percutaneous intervention (stenting and balloon dilatation). At the last follow-up, 8 years after the initial surgery, this patient was asymptomatic without any further evidence of SVC obstruction. No patient developed baffle or postoperative pulmonary venous obstruction. Freedom from reintervention or reoperation for SVC obstruction was 96 ± 4% (95% confidence interval 77–99%) at 5 and 7 years after surgery (Fig. 1B). No patient was lost to follow-up. The mean follow-up was 5.6 ± 4.3 years (1 month to 21 years) after surgery. All survivors were in New York Heart Association functional Class I or II. At follow-up, 12-lead electrocardiography was performed on all patients, and no patient developed sick sinus syndrome, sinus node dysfunction or atrial arrhythmias requiring permanent pacemaker implantation. Three (7.1%) patients had a low atrial rhythm but were asymptomatic. Three patients had a Holter monitor for palpitations: 1 patient had a low atrial rhythm, 1 had a normal sinus rhythm and 1 had intermittent self-resolving episodes of supraventricular tachycardia. Follow-up echocardiographic assessment of the right atrial-SVC anastomosis was available in 38 of the 41 (93%) survivors and demonstrated a mean velocity of 0.4 ± 0.59 (0–1.7) m/s. DISCUSSION Warden et al. [2] first described the cavoatrial anastomosis technique in 1984 as an alternative repair approach for PAPVD to the SVC. Early outcomes of the procedure were promising [3], but most series (Table 2) had a small number of patients, limited follow-up and included adult patients. Only 2 studies have reported on the Warden procedure exclusively in the paediatric population [1, 4]. Herein, we describe the largest study of the outcomes on the Warden procedure in children. Table 2: A summary of the current literature on the Warden procedure Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Only studies with >10 patients were included. a A part of a larger study of partial anomalous pulmonary venous drainage patients undergoing other types of repair. b Included 4 patients with total anomalous pulmonary venous drainage repaired with the Warden procedure. SVC: superior vena cava. Table 2: A summary of the current literature on the Warden procedure Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Only studies with >10 patients were included. a A part of a larger study of partial anomalous pulmonary venous drainage patients undergoing other types of repair. b Included 4 patients with total anomalous pulmonary venous drainage repaired with the Warden procedure. SVC: superior vena cava. The decision to perform the Warden procedure was based on surgeon preference and anatomical features when there was concern of SVC obstruction with the patch technique. The Warden procedure can be safely performed in children with excellent early and late outcomes. In our study, that included patients with other complex congenital cardiac defects, we report no operative mortality in children. Similarly, others have reported an operative mortality of 0–3% for the Warden procedure [4–6, 10] and patching technique [1], although anatomy of children with PAPVD undergoing the alternative technique (1-patch or 2-patch technique) might be different as mentioned above. Park et al. [10] reported an early death (3%) in a patient with co-existing congenital heart disease (absent pulmonary valve syndrome) among 30 patients undergoing the Warden procedure (mean age 4.9 years; range 1 month–55 years). The only late death in our study was unrelated to the Warden procedure but due to a procedural complication for the patient’s co-existing complex congenital cardiac defect. The reported incidence of long-term sinus node dysfunction with the use of the 1-patch or 2-patch technique has been inconsistent. Some authors have reported no sinus node dysfunction [1, 11], whereas other authors have reported significant sinus node dysfunction after patch techniques [12–14]. Stewart et al. [12] reported a 55% incidence of low atrial or junctional rhythm after the 2-patch technique that was significantly higher when compared with the 1-patch technique or Warden procedure. Iyer et al. [13] reported a 21% incidence of postoperative junctional rhythms among their 1-patch technique group, though none of these patients with junctional rhythms required permanent pacemakers. In comparison, the Warden procedure has demonstrated reproducible results with no postoperative sinus node dysfunction requiring permanent pacemaker [3, 4, 10]. A key principal of the Warden procedure is that it avoids incisions in the vicinity of the sinus node and the sinus nodal artery. We report no pacemaker requirement and a 7% incidence of low atrial rhythm on postoperative follow-up. The results of our study are reassuring and confirm that good results can also be achieved in children. This has likewise been reported in other studies where the majority of patients have remained free of sinus node dysfunction in the long term [4–6]. When the pulmonary venous ‘entry point was high’ in the SVC, traditional 1-patch technique can result in SVC narrowing. Hence, the 2-patch technique was developed to ensure that the SVC flow into the right atrium remains unobstructed. However, SVC narrowing may still occur if the patch is placed high in the SVC. The Warden technique mitigates this risk by allowing a generous anastomosis of the SVC to the right atrium. Furthermore, this technique may be useful in patients with bilateral SVC with a small-sized right SVC that may be at risk of narrowing with standard 2-patch techniques. However, the development of SVC obstruction at the site of anastomosis is an important complication of this procedure that may require reoperation [3]. The literature reports that 3–20% of patients will develop SVC obstruction [1, 3, 6, 10, 12]. Agarwal et al. [7] reported no SVC obstruction among 58 patients and demonstrated right atrial-SVC anastomotic gradients of <1 mm on echocardiography; however, the follow-up was limited to a mean of 1.2 years. Technical issues such as the absence of a tension-free cavoatrial anastomosis, purse stringing of the anastomosis or failure to resect right atrial trabeculae may be major determinants of SVC obstruction with the risk being higher in younger (<2 years) and smaller patients (<7 kg) [10]. These complications occurred within 1–2 years from surgery [10, 12]. Park et al. [10] highlighted the importance of a tension-free anastomosis between the cephalic end of the SVC and right-atrial appendage. Their technique involves extensive dissection and mobilization of the SVC and brachiocephalic vein as well as the resection of trabeculations in the right atrial appendage. In our experience, only 1 patient developed SVC obstruction requiring endovascular intervention. This low incidence may be attributed to the following strategies to achieve a tension-free cavoatrial anastomosis: (i) dissection and mobilization of the SVC and (ii) the use of interlocking stitches to prevent the purse-stringing effect of a continuous stitch. Furthermore, the use of an absorbable stitch may avoid potential strictures of the anastomosis with somatic growth of the patient. Modifications to reduce cavoatrial anastomosis tension, including patch augmentation or right atrial wall pedicle flap, have resulted in widely patent anastomoses at the last follow-up [10, 15]. Our experience with these modifications is limited, and, hence, no specific recommendations can be made. However, they are a useful adjunct when tension-free cavoatrial anastomosis cannot be achieved. Long-term durability of this anastomosis is unclear as most studies have small sample sizes with limited follow-up. This is important in the paediatric population where growth is expected over the ensuing years. In our cohort, 1 patient was followed up 21 years after the initial surgery at the age of 5 years and remained well with no symptoms of SVC obstruction. Our mid-term results are reassuring with few complications. However, an ongoing follow-up into adulthood is required to detect any late complications among patients operated during childhood. The development of postoperative pulmonary venous obstruction is likely a technical problem that may be related to a failure to enlarge a restrictive ASD or stenosis at the caudal end of the transected SVC. We routinely use autologous pericardium to baffle the pulmonary venous return through the ASD to the left atrium. Some authors suggest patch repair of the transected SVC to avoid stenosis [16]. However, we and other units have not observed any complications with direct suture closure of the caudal SVC [8, 10]. Limitations This study was limited by its retrospective design. Patient selection and surgical techniques have varied over the long study period. Because of the low incidence of adverse outcomes (mortality and morbidity), risk factor analysis could not be performed. There was no comparison group as patients who underwent patch repair (n = 21) had different anatomy, and selection was not uniform during the study period. CONCLUSIONS The Warden procedure for repair of PAPVD to the SVC can be performed with no operative mortality or permanent pacemaker requirement. Mid-term outcomes are excellent with a low occurrence of SVC obstruction. Funding Yves d’Udekem is a Career Development Fellow of the National Heart Foundation of Australia [CR 10M 5339]. Matthew Yong is supported by the David B. 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This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Interactive CardioVascular and Thoracic Surgery Oxford University Press

Outcomes of the Warden procedure for partial anomalous pulmonary venous drainage in children

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Oxford University Press
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© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
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1569-9293
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1569-9285
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10.1093/icvts/ivy097
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Abstract

Abstract OBJECTIVES When drainage of an anomalous pulmonary vein is high into the superior vena cava (SVC), traditional techniques of 1-patch or 2-patch repair may be challenging. The cavoatrial anastomosis technique (the Warden procedure) was developed to reduce the risk of sinus node dysfunction and SVC obstruction. Herein, we describe the outcomes of the Warden procedure in children at a single institution. METHODS A retrospective study was conducted on all children with partial anomalous pulmonary venous drainage (PAPVD) undergoing the Warden procedure from 1996 to 2015. RESULTS There were 42 PAPVD patients with a right upper pulmonary vein entering into the SVC undergoing the Warden procedure. The mean age of the patients at surgery was 5.5 years (71 days to 15.4 years). There was no operative mortality. Median hospital stay was 5 (3–25) days. One (2.4%) patient developed cavoatrial (SVC) obstruction. This patient required percutaneous intervention (stenting and balloon dilatation). There was 1 (2.4%) late death in a patient with PAPVD and pulmonary atresia due to a hypoxic brain injury following catheter intervention for a stenotic conduit. Survival was 97 ± 3% (95% confidence interval 83–100%) at 5 and 7 years. All patients were asymptomatic at a mean follow-up of 6 years (range 1 month to 21 years) after surgery. No patient had sick sinus syndrome, sinus node dysfunction or required permanent pacemaker at follow-up. CONCLUSIONS The Warden procedure for repair of PAPVD to the SVC can be performed with no operative mortality or permanent pacemaker requirement. Mid-term outcomes are excellent with a low occurrence of SVC obstruction. Surgery, Congenital heart disease, Pulmonary veins, Partial anomalous pulmonary venous drainage INTRODUCTION Numerous techniques have been described for repair of partial anomalous pulmonary venous drainage (PAPVD) of the right upper pulmonary veins to the superior vena cava (SVC) [1]. Repair aims at complete closure of the associated atrial septal defect (ASD) and redirection of the anomalous pulmonary veins into the left atrium. Partitioning the SVC into 2 pathways and manipulation of the right atrial-SVC junction increase the risk of pulmonary venous or SVC obstruction and injury to the sinus node. When entry of the pulmonary vein is high into the SVC, traditional techniques of 1-patch or 2-patch repair may be challenging. The cavoatrial anastomosis technique (the Warden procedure) was developed to reduce the risk of sinus node dysfunction and SVC obstruction in these cases [2]. Early outcomes of the procedure are promising [2]; however, numbers of patients are small, and no study in the reported literature exclusively studied outcomes in children. Herein, we describe the outcomes of the Warden procedure in children at a single institution. MATERIALS AND METHODS Patients The institutional research ethics board approved this study. All patients who underwent repair of PAPVD with the Warden procedure at the Royal Children’s Hospital between 1996 and 2015 were identified. Data were obtained by review of medical records from the initial admission until the last follow-up. All patients had a 12-lead electrocardiography and transthoracic echocardiography (to evaluate the cavoatrial anastomosis and pulmonary venous pathway) at annual cardiology review. A Holter monitor was performed if there were symptoms suggestive of dysrhythmias. Operative technique and patient selection The Warden procedure was selected based on surgeon preference and if there was concern that a 1-patch or 2-patch repair technique may have put the patient at risk of SVC obstruction. After sternotomy, the right SVC is circumferentially dissected and mobilized from the SVC-right atrial junction to the SVC-left innominate vein junction. The azygos vein is doubly ligated and divided to permit maximal mobilization of the SVC. Cardiopulmonary bypass (CPB) is instituted after cannulation of the ascending aorta, inferior vena cava and high SVC (near the SVC-innominate vein junction). If there is a large left SVC, additional venous drainage may be established. Two vascular clamps are placed across the SVC above the entrance of the highest anomalous right pulmonary vein. The SVC is then transected. The tip of the right atrial appendage is amputated and enlarged to the diameter of the cephalad SVC. Excision of the trabeculae in the right atrial appendage at the site of anastomosis is performed and reduces the risk of SVC obstruction. The caudal SVC stump is closed directly above the entry point of the anomalous pulmonary vein. The cephalad SVC is sewn to the amputated right atrial appendage with an interlocking continuous absorbable suture. The aortic cross-clamp is then applied and cardioplegic arrest induced. A high right atriotomy is performed to expose the sinus venosus or secundum ASD. If there is no defect, an interatrial septal defect is created. The anomalous right pulmonary venous drainage is baffled across the ASD into the left atrium with a fresh autologous pericardial patch. A Gore-Tex patch (W.L. Gore & Associates, Inc., Flagstaff, AZ, USA) may be used instead. The right atriotomy is then closed, and the patient is weaned off bypass as per standard protocol. All patients had intraoperative transoesophageal echocardiogram performed to confirm laminar flow through the baffle and cavoatrial anastomosis. Definitions Operative mortality was defined as death occurring within 30 days of surgery or later if the patient is still hospitalized (including patients transferred to other acute care facilities). All other deaths were considered as late. Postoperative cavoatrial (SVC) obstruction was defined as symptomatic (facial swelling, dyspnoea, headaches and venous engorgement of neck, trunk and upper extremities) right atrial-SVC anastomotic narrowing as identified on transthoracic echocardiogram and computerized tomography imaging. Postoperative pulmonary venous obstruction was identified during routine transthoracic echocardiogram or if the patient developed symptoms of heart failure and the stenosis was confirmed using computerized tomography imaging. Postoperative sinus node dysfunction was defined as persistent sinus bradycardia (<50 beats/min) or junctional/nodal rhythm, ectopic atrial rhythm or a wandering pacemaker (<60 beats/min) or pauses of more than 3 s. A low atrial rhythm was defined as inverted P waves in the inferior leads on electrocardiography. Patients were considered lost to follow-up if no medical information was available after hospital discharge. Data analysis All data were analyzed using Stata version 12 (Stata Corp, College Station, TX, USA). Data are expressed as mean ± standard deviation and median with interquartile range (IQR) as appropriate. The Kaplan–Meier curves were used for time to event (actuarial survival) analysis. RESULTS Detailed preoperative patient characteristics are listed in Table 1. Diagnosis of PAPVD was made by echocardiogram and computerized tomography imaging. Cardiac catheterization (n = 6) was used to confirm diagnosis earlier in the study period or when there were complex co-existing congenital heart defects. Surgery was performed with standard CPB. Mean CPB time was 93 ± 60 min (36–300 min), and mean cross-clamp time was 50 ± 41 min (10–198 min). Circulatory arrest was used in 1 patient. The mean lowest temperature during CPB was 33 ± 2°C (25–35°C). Concomitant repairs included ventricular septal defect closure in 4 (9.5%) patients, complete repair of pulmonary atresia (right ventricle to pulmonary artery conduit, ventricular septal defect closure and left/right pulmonary artery augmentation) in 1 (2.4%) patient, REV procedure in 1 (2.4%) patient, Ebstein valve repair in 1 (2.4%) patient, aortic valve repair in 1 (2.4%) patient, repair hypoplastic arch and coarctation in 1 (2.4%) patient and ASD closure in 38 (90%) patients. In 5 cases (12%), cavoatrial anastomosis could not be performed without excessive tension, hence, pericardial patch enlargement was performed. Median intubation time was 8 h (IQR 5–13 h), and median stay in the intensive care unit was 21 h (IQR 17–25 h). Median hospital stay was 5 (IQR 4–6) days. No patient developed dysrhythmias that required a temporary or permanent pacemaker in the immediate postoperative period. Table 1: Patient characteristics Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  ASD: atrial septal defect; BSA: body surface area; ECMO: extracorporeal membrane oxygenator; PDA: patent ductus arteriosus; SD: standard deviation; SVC: superior vena cava; VSD: ventricular septal defect. Table 1: Patient characteristics Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  Variables  Values  Total  42  Demographics     Male:female  21:21   Mean age at surgery (years), mean ± SD (range)  5.5 ± 4 (71 days to 15 years)   Weight at surgery (kg), mean ± SD (range)  19.2 ± 10.8 (3.8–48.4)   Height at surgery (cm), mean ± SD (range)  108 ± 29.3 (54–163)   BSA (m2), mean ± SD (range)  0.75 ± 0.32 (0.22–1.48)  Diagnosis, n (%)     Prematurity  1 (2.4)   ASD  38 (90)   PDA  2 (4.8)   VSD  5 (11.9)   Pulmonary atresia  1 (2.4)   Left SVC  4 (9.5)   Syndromal diagnosis  1 (2.4)  Interventions, n (%)     Preoperative intubation  1 (2.4)   Balloon atrial septostomy  1 (2.4)   Preoperative ECMO  0 (0)   Previous cardiac operation  4 (9.5)  ASD: atrial septal defect; BSA: body surface area; ECMO: extracorporeal membrane oxygenator; PDA: patent ductus arteriosus; SD: standard deviation; SVC: superior vena cava; VSD: ventricular septal defect. There were no hospital deaths. There was 1 late death (2.4%) in a patient with PAPVD and pulmonary atresia 1.2 years after surgery. The patient underwent catheter intervention for a stenotic conduit. During the procedure, there was a guide-wire perforation of the pulmonary artery resulting in a hypoxic arrest and hypoxic brain injury. The Kaplan–Meier analysis demonstrated actuarial survival of 97 ± 3% (95% confidence interval 83–100%) at 5 and 7 years (Fig. 1A). Figure 1: View largeDownload slide (A) The Kaplan–Meier survival curve. (B) The Kaplan–Meier freedom from reoperation or reintervention for the superior vena cava or pulmonary venous obstruction. CI: confidence interval. Figure 1: View largeDownload slide (A) The Kaplan–Meier survival curve. (B) The Kaplan–Meier freedom from reoperation or reintervention for the superior vena cava or pulmonary venous obstruction. CI: confidence interval. One (2.4%) patient developed SVC obstruction. This patient underwent surgery at 3.5 years of age (weight during surgery was 14.3 kg) after presenting with clinical features of a large shunt. The operation was uncomplicated, and the cavoatrial anastomosis was performed without any patch augmentation. The patient was discharged on Day 3 after surgery but developed SVC obstruction 3 years after the surgery. The patient was symptomatic and required percutaneous intervention (stenting and balloon dilatation). At the last follow-up, 8 years after the initial surgery, this patient was asymptomatic without any further evidence of SVC obstruction. No patient developed baffle or postoperative pulmonary venous obstruction. Freedom from reintervention or reoperation for SVC obstruction was 96 ± 4% (95% confidence interval 77–99%) at 5 and 7 years after surgery (Fig. 1B). No patient was lost to follow-up. The mean follow-up was 5.6 ± 4.3 years (1 month to 21 years) after surgery. All survivors were in New York Heart Association functional Class I or II. At follow-up, 12-lead electrocardiography was performed on all patients, and no patient developed sick sinus syndrome, sinus node dysfunction or atrial arrhythmias requiring permanent pacemaker implantation. Three (7.1%) patients had a low atrial rhythm but were asymptomatic. Three patients had a Holter monitor for palpitations: 1 patient had a low atrial rhythm, 1 had a normal sinus rhythm and 1 had intermittent self-resolving episodes of supraventricular tachycardia. Follow-up echocardiographic assessment of the right atrial-SVC anastomosis was available in 38 of the 41 (93%) survivors and demonstrated a mean velocity of 0.4 ± 0.59 (0–1.7) m/s. DISCUSSION Warden et al. [2] first described the cavoatrial anastomosis technique in 1984 as an alternative repair approach for PAPVD to the SVC. Early outcomes of the procedure were promising [3], but most series (Table 2) had a small number of patients, limited follow-up and included adult patients. Only 2 studies have reported on the Warden procedure exclusively in the paediatric population [1, 4]. Herein, we describe the largest study of the outcomes on the Warden procedure in children. Table 2: A summary of the current literature on the Warden procedure Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Only studies with >10 patients were included. a A part of a larger study of partial anomalous pulmonary venous drainage patients undergoing other types of repair. b Included 4 patients with total anomalous pulmonary venous drainage repaired with the Warden procedure. SVC: superior vena cava. Table 2: A summary of the current literature on the Warden procedure Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Authors  Year of publication  Years  Number of patients  Age  Operative mortality (%)  SVC obstruction (%)  Follow-up  Gustafson et al. [3]  1995  1964–1994  40  Mean 11 (1–52) years  2.5  2.5  Mean 11 years (6 months to 30 years)  DiBardino et al. [4]  2004  1995–2003  16  Mean 7 (0.2–14) years  0  0  Mean unknown (0.3–67 months)  Shahriari et al. [5]  2006  1991–2004  13a  Mean 13 (1.5–43) years  0  0  Mean 3.7 (1–12.8) years  Nakahira et al. [6]  2006  1993–2004  20  Mean 12 (4–26) years  0  10  Mean 6.5 (range unknown) years  Alsoufi et al. [1]  2007  1982–2006  14a  5.3 (0.47–18) years  0  7.1  Mean 11 (range unknown) years  Agarwal et al. [7]  2011  2008–2011  54  Median 11 (2–48) years  0  0  Mean 1.2 years (1 month to 2.8 years)  Kottayil et al. [8]  2011  2006–2010  32b  Median 4 years (3 months to 34 years)  0  0  Median 24 (6–46) months  Said et al. [9]  2011  1990–2009  18a  Mean 35 (1.3–76) years  0  5.5  Mean 2 (max 14) years  Park et al. [10]  2012  1994–2011  30  Median 4.9 years (1 month to 55 years)  3.3  10  Mean 5.3 years (1 month to 16 years)  Only studies with >10 patients were included. a A part of a larger study of partial anomalous pulmonary venous drainage patients undergoing other types of repair. b Included 4 patients with total anomalous pulmonary venous drainage repaired with the Warden procedure. SVC: superior vena cava. The decision to perform the Warden procedure was based on surgeon preference and anatomical features when there was concern of SVC obstruction with the patch technique. The Warden procedure can be safely performed in children with excellent early and late outcomes. In our study, that included patients with other complex congenital cardiac defects, we report no operative mortality in children. Similarly, others have reported an operative mortality of 0–3% for the Warden procedure [4–6, 10] and patching technique [1], although anatomy of children with PAPVD undergoing the alternative technique (1-patch or 2-patch technique) might be different as mentioned above. Park et al. [10] reported an early death (3%) in a patient with co-existing congenital heart disease (absent pulmonary valve syndrome) among 30 patients undergoing the Warden procedure (mean age 4.9 years; range 1 month–55 years). The only late death in our study was unrelated to the Warden procedure but due to a procedural complication for the patient’s co-existing complex congenital cardiac defect. The reported incidence of long-term sinus node dysfunction with the use of the 1-patch or 2-patch technique has been inconsistent. Some authors have reported no sinus node dysfunction [1, 11], whereas other authors have reported significant sinus node dysfunction after patch techniques [12–14]. Stewart et al. [12] reported a 55% incidence of low atrial or junctional rhythm after the 2-patch technique that was significantly higher when compared with the 1-patch technique or Warden procedure. Iyer et al. [13] reported a 21% incidence of postoperative junctional rhythms among their 1-patch technique group, though none of these patients with junctional rhythms required permanent pacemakers. In comparison, the Warden procedure has demonstrated reproducible results with no postoperative sinus node dysfunction requiring permanent pacemaker [3, 4, 10]. A key principal of the Warden procedure is that it avoids incisions in the vicinity of the sinus node and the sinus nodal artery. We report no pacemaker requirement and a 7% incidence of low atrial rhythm on postoperative follow-up. The results of our study are reassuring and confirm that good results can also be achieved in children. This has likewise been reported in other studies where the majority of patients have remained free of sinus node dysfunction in the long term [4–6]. When the pulmonary venous ‘entry point was high’ in the SVC, traditional 1-patch technique can result in SVC narrowing. Hence, the 2-patch technique was developed to ensure that the SVC flow into the right atrium remains unobstructed. However, SVC narrowing may still occur if the patch is placed high in the SVC. The Warden technique mitigates this risk by allowing a generous anastomosis of the SVC to the right atrium. Furthermore, this technique may be useful in patients with bilateral SVC with a small-sized right SVC that may be at risk of narrowing with standard 2-patch techniques. However, the development of SVC obstruction at the site of anastomosis is an important complication of this procedure that may require reoperation [3]. The literature reports that 3–20% of patients will develop SVC obstruction [1, 3, 6, 10, 12]. Agarwal et al. [7] reported no SVC obstruction among 58 patients and demonstrated right atrial-SVC anastomotic gradients of <1 mm on echocardiography; however, the follow-up was limited to a mean of 1.2 years. Technical issues such as the absence of a tension-free cavoatrial anastomosis, purse stringing of the anastomosis or failure to resect right atrial trabeculae may be major determinants of SVC obstruction with the risk being higher in younger (<2 years) and smaller patients (<7 kg) [10]. These complications occurred within 1–2 years from surgery [10, 12]. Park et al. [10] highlighted the importance of a tension-free anastomosis between the cephalic end of the SVC and right-atrial appendage. Their technique involves extensive dissection and mobilization of the SVC and brachiocephalic vein as well as the resection of trabeculations in the right atrial appendage. In our experience, only 1 patient developed SVC obstruction requiring endovascular intervention. This low incidence may be attributed to the following strategies to achieve a tension-free cavoatrial anastomosis: (i) dissection and mobilization of the SVC and (ii) the use of interlocking stitches to prevent the purse-stringing effect of a continuous stitch. Furthermore, the use of an absorbable stitch may avoid potential strictures of the anastomosis with somatic growth of the patient. Modifications to reduce cavoatrial anastomosis tension, including patch augmentation or right atrial wall pedicle flap, have resulted in widely patent anastomoses at the last follow-up [10, 15]. Our experience with these modifications is limited, and, hence, no specific recommendations can be made. However, they are a useful adjunct when tension-free cavoatrial anastomosis cannot be achieved. Long-term durability of this anastomosis is unclear as most studies have small sample sizes with limited follow-up. This is important in the paediatric population where growth is expected over the ensuing years. In our cohort, 1 patient was followed up 21 years after the initial surgery at the age of 5 years and remained well with no symptoms of SVC obstruction. Our mid-term results are reassuring with few complications. However, an ongoing follow-up into adulthood is required to detect any late complications among patients operated during childhood. The development of postoperative pulmonary venous obstruction is likely a technical problem that may be related to a failure to enlarge a restrictive ASD or stenosis at the caudal end of the transected SVC. We routinely use autologous pericardium to baffle the pulmonary venous return through the ASD to the left atrium. Some authors suggest patch repair of the transected SVC to avoid stenosis [16]. However, we and other units have not observed any complications with direct suture closure of the caudal SVC [8, 10]. Limitations This study was limited by its retrospective design. Patient selection and surgical techniques have varied over the long study period. Because of the low incidence of adverse outcomes (mortality and morbidity), risk factor analysis could not be performed. There was no comparison group as patients who underwent patch repair (n = 21) had different anatomy, and selection was not uniform during the study period. CONCLUSIONS The Warden procedure for repair of PAPVD to the SVC can be performed with no operative mortality or permanent pacemaker requirement. Mid-term outcomes are excellent with a low occurrence of SVC obstruction. Funding Yves d’Udekem is a Career Development Fellow of the National Heart Foundation of Australia [CR 10M 5339]. Matthew Yong is supported by the David B. Rosenthal Scholarship (University of Melbourne), Australian Government Research Training Program Scholarship and National Health and Medical Research Council Postgraduate Scholarship [APP1133977]. Conflict ofinterest: none declared. REFERENCES 1 Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg  2007; 84: 2020– 6. Google Scholar CrossRef Search ADS PubMed  2 Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg  1984; 38: 601– 5. Google Scholar CrossRef Search ADS PubMed  3 Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg  1995; 60: S614– 7. 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Google Scholar CrossRef Search ADS PubMed  8 Walker RE, Mayer JE, Alexander ME, Walsh EP, Berul CI. Paucity of sinus node dysfunction following repair of sinus venosus defects in children. Am J Cardiol  2001; 87: 1223– 6; A8. Google Scholar CrossRef Search ADS PubMed  9 Stewart RD, Bailliard F, Kelle AM, Backer CL, Young L, Mavroudis C. Evolving surgical strategy for sinus venosus atrial septal defect: effect on sinus node function and late venous obstruction. Ann Thorac Surg  2007; 84: 1651– 5. Google Scholar CrossRef Search ADS PubMed  10 Iyer AP, Somanrema K, Pathak S, Manjunath PY, Pradhan S, Krishnan S. Comparative study of single- and double-patch techniques for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. J Thorac Cardiovasc Surg  2007; 133: 656– 9. Google Scholar CrossRef Search ADS PubMed  11 Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen WK et al.   Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients. Circulation  2005; 112: 1953– 8. Google Scholar CrossRef Search ADS PubMed  12 Agarwal V, Okonta KE, Abubakar U, Gichuhi S. Impact of Warden's procedure on the sinus rhythm: our experience. Heart Lung Circ  2011; 20: 718– 21. Google Scholar CrossRef Search ADS PubMed  13 Tao K, Pan W, Lin K, Shi Y, Zhu P, Guo Y et al.   Modified cavoatrial anastomosis in Warden procedure. Ann Thorac Surg  2010; 89: 2047– 8. Google Scholar CrossRef Search ADS PubMed  14 Baron O, Roussel JC, Videcoq M, Guerin P, Gournay V, Lefevre M. Partial anomalous pulmonary venous connection: correction by intra-atrial baffle and cavo-atrial anastomosis. J Cardiac Surgery  2002; 17: 166– 9. Google Scholar CrossRef Search ADS   15 Kottayil BP, Dharan BS, Menon S, Bijulal S, Neema PK, Gopalakrishnan SK et al.   Anomalous pulmonary venous connection to superior vena cava: Warden technique. Eur J Cardiothorac Surg  2011; 39: 388– 91. Google Scholar CrossRef Search ADS PubMed  16 Said SM, Burkhart HM, Dearani JA, Eidem B, Stensrud P, Phillips SD et al.   Outcome of caval division techniques for partial anomalous pulmonary venous connections to the superior vena cava. Ann Thorac Surg  2011; 92: 980– 4. Google Scholar CrossRef Search ADS PubMed  © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

Journal

Interactive CardioVascular and Thoracic SurgeryOxford University Press

Published: Mar 23, 2018

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