CONGENITAL HEART
Is Restrictive Atrial Septal Defect a Risk in Partial Anomalous Pulmonary Venous Drainage Repair? Jiaquan Zhu, MD, PhD, Yasuhiro Kotani, MD, PhD, Devin Chetan, HBA, Lisa Zhao, John G. Coles, MD, Christopher A. Caldarone, MD, Glen S. Van Arsdell, MD, and Osami Honjo, MD, PhD Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada; and Cardiothoracic Surgery Department, Xinhua Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China
Background. The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair may pose a risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and atrial rhythm disturbances. Methods. 155 children who underwent repair of right PAPVD between 1990 and 2010 were reviewed. PVS and SVCS were defined by mean gradients on echocardiography: mild [ 3 to 5 mm Hg; severe [ 6 mm Hg or higher. Postoperative cardiac rhythms were categorized as sinus, transient nonsinus, and persistent nonsinus rhythms. Outcomes were compared between patients who underwent the creation or superior enlargement of an ASD (group A) and those who did not (group B). Results. There was no early or late death. Freedom from any PVS at 15 years after operation was lower in group A than in group B (76.1% vs 96.5%, p [ 0.002), and no differences were found in freedom from severe PVS (p [ 0.103), any SVCS (p [ 0.419), or severe SVCS
(p [ 0.373). Group A patients had more PVS-related reoperations (p [ 0.022). Nineteen patients had nonsinus rhythm, and 4 patients experienced first-degree atrioventricular block, but no significant difference was found between the groups. Cox regression revealed the creation or superior enlargement of an ASD as a predictor for postoperative PVS (p [ 0.032). A case-match analysis confirmed a higher risk of PVS in patients with the creation or superior enlargement of an ASD (p [ 0.018). Conclusions. Late outcomes after repair of PAPVD are excellent. The subgroup that requires creation or superior enlargement of an ASD in repair of a right PAPVD is at a higher risk of late PVS and a subsequent increase in PVSrelated reoperation. The presence of restrictive ASD did not increase SVCS, sinus node, or atrial conduction dysfunction.
T
the PV pathway at the level of the atrial septum and may also impair sinus node or atrial conduction or both. However, this has not been well described in the literature [4, 6, 11, 14]. We hypothesized that IAS or restrictive ASD in children with right PAPVD who require the creation or enlargement of an ASD may be related to late PVS or atrial rhythm disturbances. Therefore, we reviewed our experience over the past 20 years to analyze the late outcomes of this entity.
he principle of repair for right partial anomalous pulmonary venous drainage (PAPVD) is to redirect the anomalous pulmonary vein (PV) into the left atrium through a baffle without compromising the pathway from the superior vena cava (SVC) to the right atrium (RA). Various surgical techniques, including one-patch or twopatch repair and caval division technique, have been effective, with very low operative mortality [1–10]. However, the risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and sinus node dysfunction in the follow-up period are not negligible, with estimates ranging up to 24%, 21.6%, and 35%, respectively [4, 9, 11–18]. About 8% to 20% of all PAPVD patients [2, 4, 11, 13, 17, 19] have an intact atrial septum (IAS), requiring ASD creation during operation. ASD enlargement is also needed in PAPVD patients with a restrictive ASD. The creation or enlargement of an ASD may pose a risk of obstruction in
Accepted for publication Jan 17, 2014. Address correspondence to Dr Honjo, Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada, M5G 1X8; e-mail: osami.honjo@ sickkids.ca.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2014;97:1664–70) Ó 2014 by The Society of Thoracic Surgeons
Patients and Methods This retrospective study was approved by the research ethics board at our institution, and the need for patient consent was waived. One hundred fifty-five patients who underwent right PAPVD repair from January 1990 to December 2010 were identified from the surgical database at the Hospital for Sick Children. Patients with scimitar syndrome or right PAPVD as part of mixed total anomalous pulmonary venous drainage were excluded.
Surgical Indication and Techniques Surgical repair is indicated when the pulmonary-to-systemic flow ratio is greater than 2:1 by magnetic resonance 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.01.051
imaging (MRI). The surgical technique chosen depends on the anatomy, particularly the location of the right PAPVD insertion, and partly depends on the surgeon’s discretion. Multiple surgical procedures were selected, as described in our previous study [13]. Briefly, for PAPVD draining into the RA, the one-patch technique was performed, with autologous pericardium used as an intraatrial baffle. When PAPVD was connected to the SVC-RA junction or the low or middle SVC, a second patch was placed to augment the SVC-RA junction [20]. For PAPVD draining into the upper portion of the SVC, the caval division technique (Warden procedure) was used, including SVC division with reimplantation to the RA appendage. The intracardiac baffle was sutured to the margin of the sinus venosus ASD, redirecting the PV blood from the SVC orifice into the left atrium. The associated heart defects were repaired simultaneously. An ASD was created in patients with IAS. The atrial septum within the fossa ovalis was completely excised, and the incision was extended superiorly close to or into the limbus until an ASD of appropriate size was created. The resected edge was typically approximated with a fine polypropylene suture. The same enlargement technique was used in patients with highly restrictive patent foramen ovale (PFO), secundum, or sinus venosus ASD. Cephalic enlargement of sinus venosus ASD is indicated when the size of the sinus venosus ASD is significantly smaller than the PV orifice, there is a prominent superior rim that potentially makes an intraatrial baffle longer, or both these features are present. The superior enlargement was performed until the size of the sinus venosus ASD became equivalent to the PV orifice, and the resected edge was reendocardialized. The cohort was divided into two groups: group A (patients with creation or superior enlargement of an ASD, n ¼ 34) and group B (patients without ASD superior enlargement, n ¼ 121). The patients’ preoperative profiles are shown in Table 1. Reintervention for PV stenosis was indicated when the PV pathway had a significant pressure gradient (mean gradient >6 mm Hg) with a progressive nature. Surgical revision rather than catheter-based intervention has been our strategy during the study period.
Outcome Assessment All surgical and catheter-based reinterventions after PAPVD repair were recorded. One hundred thirty-one patients received complete follow-up, and 24 patients were lost to follow-up (n ¼ 5 in group A and n ¼ 19 in group B). All echocardiography reports were reviewed to identify PV or SVC obstruction. Grading was based on mean gradient and was performed as follows: none or trivial, under 3 mm Hg; mild, 3 to 5 mm Hg; severe, 6 mm Hg or higher, or total occlusion. Some patients underwent postoperative MRI if PVS was suspected. Inasmuch as only a few patients underwent postoperative MRI, these data were not included in the statistical analysis. The perioperative and follow-up electrocardiograms were reviewed to identify the heart rhythm and atrial rhythm disturbance. We categorized the heart rhythms as normal
ZHU ET AL PAPVD/RESTRICTIVE ASD
1665
sinus rhythm, transient nonsinus rhythm, and persistent nonsinus rhythm. Patients with transient nonsinus rhythm were those who had both sinus and nonsinus rhythm during follow-up, whereas patients with persistent nonsinus rhythm had only nonsinus rhythm.
Case-Matched Control Patients Case-matched control analysis was designed to more accurately define the effects of the creation or superior enlargement of an ASD on clinical outcomes after PAPVD repair. Control patients were selected from those who did not need the creation or superior enlargement of an ASD. Matching was based on age, body weight, and surgical repair technique. Freedom from reintervention, any PVS, any SVCS, and heart rhythm were compared between the study cohort and matched control patients.
Statistical Analysis Data were presented as frequency (percentage), median (range), or mean standard deviation. Differences between groups were tested with the c2 test or Fisher’s exact test for discrete variables. The Mann-Whitney U test was used for continuous variables between groups. Longterm survival or freedom from PVS, SVCS, and reinterventions were analyzed by the Kaplan-Meier method and the log-rank test. Cox regression was used to determine predictors of postoperative morbidities. Variables significant in univariable analysis (p < 0.10) were taken into a forward stepwise multivariable Cox regression model. Data analysis was performed with SPSS 19.0 software (IBM Corporation, Armonk, NY).
Results The clinical data from these 155 patients are summarized in Table 1. Group A contains 34 patients who had superior ASD enlargement resulting from an intact atrial septum (n ¼ 15), restrictive PFO or secundum ASD (n ¼ 10), and restrictive sinus venosus ASD (n ¼ 9). The Warden procedure was used more frequently in group A than in group B (p ¼ 0.002). Three patients had previous operations before PAPVD repair (ventricular septal defect closure, hemitruncus repair, and repair of coarctation of the aorta). The PAPVD lesion was diagnosed preoperatively in the first two children before their primary operations and was electively repaired later, whereas the PAPVD diagnosis in the third child was missed at the time of his initial operation.
Survival and Reinterventions There was no early or late death and no permanent pacemaker implantation during the follow-up period. The patients were followed up for 1 month to 18 years (median, 2.6 years; mean, 5.1 5.5 years). All patients were in New York Heart Association class I. Three patients (3/155, 1.9%) required reintervention (Table 2). Two of them were in group A because of PVS; the other was in group B because of SVCS. The mechanism of PVS in both patients was related to a thickened pericardial baffle, not the restriction of the enlarged ASD. These patients
CONGENITAL HEART
Ann Thorac Surg 2014;97:1664–70
1666
ZHU ET AL PAPVD/RESTRICTIVE ASD
Ann Thorac Surg 2014;97:1664–70
Table 1. Demographic Data Clinical Characteristics CONGENITAL HEART
Age at PAPVD repair (years) Gender (M) Body weight (kg) Body surface area (m2) Follow-up (years) Drainage position SVC SVC-RA junction RA Azygous vein Anomalous right pulmonary vein(s) Right upper pulmonary vein Right middle pulmonary vein Right lower pulmonary vein Cardiac incision Transcavoatrial Nontrancavoatrial Intracardiac baffle Untreated autologous pericardium Treated autologous pericardium Bovine pericardium Operative technique One-patch repair Two-patch repair Warden procedure Associated diagnosis Sinus venosus ASD Secundum septal defect or PFO Intact atrial septum Persistent left SVC Pulmonary stenosis Left PAPVD Patent duct arteriosis Ventricular septal defect Pulmonary artery sling Coarctation of the aorta
Group A (n ¼ 34)
Group B (n ¼ 121)
6.72 4.36 21 26.01 17.3 0.83 0.38 6.9 5.8
6.83 4.76 60 25.53 18.17 0.83 0.40 4.5 5.3
0.87 0.21 0.68 0.66 0.016
18 9 6 1
55 37 29 0
0.44 0.64 0.44 0.22
34 22 9
119 79 29
1.00 0.95 0.76
13 21
55 66
0.45 0.45
33 1 0
115 4 2
1.00 1.00 1.00
17 9 8
75 39 7
0.21 0.52
Is Restrictive Atrial Septal Defect a Risk in Partial Anomalous Pulmonary Venous Drainage Repair? Jiaquan Zhu, MD, PhD, Yasuhiro Kotani, MD, PhD, Devin Chetan, HBA, Lisa Zhao, John G. Coles, MD, Christopher A. Caldarone, MD, Glen S. Van Arsdell, MD, and Osami Honjo, MD, PhD Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada; and Cardiothoracic Surgery Department, Xinhua Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China
Background. The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair may pose a risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and atrial rhythm disturbances. Methods. 155 children who underwent repair of right PAPVD between 1990 and 2010 were reviewed. PVS and SVCS were defined by mean gradients on echocardiography: mild [ 3 to 5 mm Hg; severe [ 6 mm Hg or higher. Postoperative cardiac rhythms were categorized as sinus, transient nonsinus, and persistent nonsinus rhythms. Outcomes were compared between patients who underwent the creation or superior enlargement of an ASD (group A) and those who did not (group B). Results. There was no early or late death. Freedom from any PVS at 15 years after operation was lower in group A than in group B (76.1% vs 96.5%, p [ 0.002), and no differences were found in freedom from severe PVS (p [ 0.103), any SVCS (p [ 0.419), or severe SVCS
(p [ 0.373). Group A patients had more PVS-related reoperations (p [ 0.022). Nineteen patients had nonsinus rhythm, and 4 patients experienced first-degree atrioventricular block, but no significant difference was found between the groups. Cox regression revealed the creation or superior enlargement of an ASD as a predictor for postoperative PVS (p [ 0.032). A case-match analysis confirmed a higher risk of PVS in patients with the creation or superior enlargement of an ASD (p [ 0.018). Conclusions. Late outcomes after repair of PAPVD are excellent. The subgroup that requires creation or superior enlargement of an ASD in repair of a right PAPVD is at a higher risk of late PVS and a subsequent increase in PVSrelated reoperation. The presence of restrictive ASD did not increase SVCS, sinus node, or atrial conduction dysfunction.
T
the PV pathway at the level of the atrial septum and may also impair sinus node or atrial conduction or both. However, this has not been well described in the literature [4, 6, 11, 14]. We hypothesized that IAS or restrictive ASD in children with right PAPVD who require the creation or enlargement of an ASD may be related to late PVS or atrial rhythm disturbances. Therefore, we reviewed our experience over the past 20 years to analyze the late outcomes of this entity.
he principle of repair for right partial anomalous pulmonary venous drainage (PAPVD) is to redirect the anomalous pulmonary vein (PV) into the left atrium through a baffle without compromising the pathway from the superior vena cava (SVC) to the right atrium (RA). Various surgical techniques, including one-patch or twopatch repair and caval division technique, have been effective, with very low operative mortality [1–10]. However, the risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and sinus node dysfunction in the follow-up period are not negligible, with estimates ranging up to 24%, 21.6%, and 35%, respectively [4, 9, 11–18]. About 8% to 20% of all PAPVD patients [2, 4, 11, 13, 17, 19] have an intact atrial septum (IAS), requiring ASD creation during operation. ASD enlargement is also needed in PAPVD patients with a restrictive ASD. The creation or enlargement of an ASD may pose a risk of obstruction in
Accepted for publication Jan 17, 2014. Address correspondence to Dr Honjo, Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada, M5G 1X8; e-mail: osami.honjo@ sickkids.ca.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2014;97:1664–70) Ó 2014 by The Society of Thoracic Surgeons
Patients and Methods This retrospective study was approved by the research ethics board at our institution, and the need for patient consent was waived. One hundred fifty-five patients who underwent right PAPVD repair from January 1990 to December 2010 were identified from the surgical database at the Hospital for Sick Children. Patients with scimitar syndrome or right PAPVD as part of mixed total anomalous pulmonary venous drainage were excluded.
Surgical Indication and Techniques Surgical repair is indicated when the pulmonary-to-systemic flow ratio is greater than 2:1 by magnetic resonance 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.01.051
imaging (MRI). The surgical technique chosen depends on the anatomy, particularly the location of the right PAPVD insertion, and partly depends on the surgeon’s discretion. Multiple surgical procedures were selected, as described in our previous study [13]. Briefly, for PAPVD draining into the RA, the one-patch technique was performed, with autologous pericardium used as an intraatrial baffle. When PAPVD was connected to the SVC-RA junction or the low or middle SVC, a second patch was placed to augment the SVC-RA junction [20]. For PAPVD draining into the upper portion of the SVC, the caval division technique (Warden procedure) was used, including SVC division with reimplantation to the RA appendage. The intracardiac baffle was sutured to the margin of the sinus venosus ASD, redirecting the PV blood from the SVC orifice into the left atrium. The associated heart defects were repaired simultaneously. An ASD was created in patients with IAS. The atrial septum within the fossa ovalis was completely excised, and the incision was extended superiorly close to or into the limbus until an ASD of appropriate size was created. The resected edge was typically approximated with a fine polypropylene suture. The same enlargement technique was used in patients with highly restrictive patent foramen ovale (PFO), secundum, or sinus venosus ASD. Cephalic enlargement of sinus venosus ASD is indicated when the size of the sinus venosus ASD is significantly smaller than the PV orifice, there is a prominent superior rim that potentially makes an intraatrial baffle longer, or both these features are present. The superior enlargement was performed until the size of the sinus venosus ASD became equivalent to the PV orifice, and the resected edge was reendocardialized. The cohort was divided into two groups: group A (patients with creation or superior enlargement of an ASD, n ¼ 34) and group B (patients without ASD superior enlargement, n ¼ 121). The patients’ preoperative profiles are shown in Table 1. Reintervention for PV stenosis was indicated when the PV pathway had a significant pressure gradient (mean gradient >6 mm Hg) with a progressive nature. Surgical revision rather than catheter-based intervention has been our strategy during the study period.
Outcome Assessment All surgical and catheter-based reinterventions after PAPVD repair were recorded. One hundred thirty-one patients received complete follow-up, and 24 patients were lost to follow-up (n ¼ 5 in group A and n ¼ 19 in group B). All echocardiography reports were reviewed to identify PV or SVC obstruction. Grading was based on mean gradient and was performed as follows: none or trivial, under 3 mm Hg; mild, 3 to 5 mm Hg; severe, 6 mm Hg or higher, or total occlusion. Some patients underwent postoperative MRI if PVS was suspected. Inasmuch as only a few patients underwent postoperative MRI, these data were not included in the statistical analysis. The perioperative and follow-up electrocardiograms were reviewed to identify the heart rhythm and atrial rhythm disturbance. We categorized the heart rhythms as normal
ZHU ET AL PAPVD/RESTRICTIVE ASD
1665
sinus rhythm, transient nonsinus rhythm, and persistent nonsinus rhythm. Patients with transient nonsinus rhythm were those who had both sinus and nonsinus rhythm during follow-up, whereas patients with persistent nonsinus rhythm had only nonsinus rhythm.
Case-Matched Control Patients Case-matched control analysis was designed to more accurately define the effects of the creation or superior enlargement of an ASD on clinical outcomes after PAPVD repair. Control patients were selected from those who did not need the creation or superior enlargement of an ASD. Matching was based on age, body weight, and surgical repair technique. Freedom from reintervention, any PVS, any SVCS, and heart rhythm were compared between the study cohort and matched control patients.
Statistical Analysis Data were presented as frequency (percentage), median (range), or mean standard deviation. Differences between groups were tested with the c2 test or Fisher’s exact test for discrete variables. The Mann-Whitney U test was used for continuous variables between groups. Longterm survival or freedom from PVS, SVCS, and reinterventions were analyzed by the Kaplan-Meier method and the log-rank test. Cox regression was used to determine predictors of postoperative morbidities. Variables significant in univariable analysis (p < 0.10) were taken into a forward stepwise multivariable Cox regression model. Data analysis was performed with SPSS 19.0 software (IBM Corporation, Armonk, NY).
Results The clinical data from these 155 patients are summarized in Table 1. Group A contains 34 patients who had superior ASD enlargement resulting from an intact atrial septum (n ¼ 15), restrictive PFO or secundum ASD (n ¼ 10), and restrictive sinus venosus ASD (n ¼ 9). The Warden procedure was used more frequently in group A than in group B (p ¼ 0.002). Three patients had previous operations before PAPVD repair (ventricular septal defect closure, hemitruncus repair, and repair of coarctation of the aorta). The PAPVD lesion was diagnosed preoperatively in the first two children before their primary operations and was electively repaired later, whereas the PAPVD diagnosis in the third child was missed at the time of his initial operation.
Survival and Reinterventions There was no early or late death and no permanent pacemaker implantation during the follow-up period. The patients were followed up for 1 month to 18 years (median, 2.6 years; mean, 5.1 5.5 years). All patients were in New York Heart Association class I. Three patients (3/155, 1.9%) required reintervention (Table 2). Two of them were in group A because of PVS; the other was in group B because of SVCS. The mechanism of PVS in both patients was related to a thickened pericardial baffle, not the restriction of the enlarged ASD. These patients
CONGENITAL HEART
Ann Thorac Surg 2014;97:1664–70
1666
ZHU ET AL PAPVD/RESTRICTIVE ASD
Ann Thorac Surg 2014;97:1664–70
Table 1. Demographic Data Clinical Characteristics CONGENITAL HEART
Age at PAPVD repair (years) Gender (M) Body weight (kg) Body surface area (m2) Follow-up (years) Drainage position SVC SVC-RA junction RA Azygous vein Anomalous right pulmonary vein(s) Right upper pulmonary vein Right middle pulmonary vein Right lower pulmonary vein Cardiac incision Transcavoatrial Nontrancavoatrial Intracardiac baffle Untreated autologous pericardium Treated autologous pericardium Bovine pericardium Operative technique One-patch repair Two-patch repair Warden procedure Associated diagnosis Sinus venosus ASD Secundum septal defect or PFO Intact atrial septum Persistent left SVC Pulmonary stenosis Left PAPVD Patent duct arteriosis Ventricular septal defect Pulmonary artery sling Coarctation of the aorta
Group A (n ¼ 34)
Group B (n ¼ 121)
6.72 4.36 21 26.01 17.3 0.83 0.38 6.9 5.8
6.83 4.76 60 25.53 18.17 0.83 0.40 4.5 5.3
0.87 0.21 0.68 0.66 0.016
18 9 6 1
55 37 29 0
0.44 0.64 0.44 0.22
34 22 9
119 79 29
1.00 0.95 0.76
13 21
55 66
0.45 0.45
33 1 0
115 4 2
1.00 1.00 1.00
17 9 8
75 39 7
0.21 0.52
![[Partial anomalous right pulmonary venous return into the coronary sinus without atrial septal defect (author's transl)].](/assets/img/hold.png)
