European Journal of Cardio-Thoracic Surgery Advance Access published April 7, 2015
ORIGINAL ARTICLE
European Journal of Cardio-Thoracic Surgery (2015) 1–8 doi:10.1093/ejcts/ezv115
A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect† Vladimir Sojak*, Marlotte Kooij, Aria Yazdanbakhsh, Dave R. Koolbergen, Eline F. Bruggemans and Mark G. Hazekamp Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands * Corresponding author. Department of Cardiothoracic Surgery, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands. Tel: +31-715262348; fax: +31-715248284; e-mail: [email protected] (V. Sojak). Received 22 September 2014; received in revised form 26 January 2015; accepted 3 February 2015
Abstract OBJECTIVES: To evaluate our experience with patients reoperated after primary repair of atrioventricular septal defect (AVSD) and identify predictors of poor outcome. METHODS: Between 1976 and 2014, 69 patients were reoperated after primary repair of partial (n = 28), intermediate (n = 15) or complete (n = 26) AVSD. RESULTS: Median age at first reoperation was 62.4 (range, 1.6–845) months, median interval to first reoperation was 22.3 (range, 0.2–598) months. Main indications for first reoperation included left atrioventricular valve (LAVV) pathology (66%), residual septal defect (19%) and left ventricle outflow tract obstruction (LVOTO; 4%). Procedures to address LAVV pathology included various valvuloplasties in 47 (77%) patients and valve replacement in 14 (23%) patients. A second, third, fourth and fifth reoperation was required in 27, 12, 4 and 1 patient, respectively. Most common procedures were LAVV replacement (LAVVR), LVOTO relief, pacemaker implantation and right atrioventricular valve procedure. Freedom from reoperation after LAVV valvuloplasty (LAVVP) was 84 and 62% at 1 and 10 years, respectively. There were 10 early and 4 late deaths. Estimated overall survival at 1, 5 and 10 years was 87, 83 and 83%, respectively. Double orifice LAVV (DOLAVV) was a risk factor for in-hospital and overall mortality [odds ratio (OR) = 14.5; 95% confidence interval (CI) = 1.2–178.7; P = 0.037 and hazard ratio (HR) = 6.8; 95% CI = 1.5–31.7; P = 0.015, respectively]. Patients with LAVVP and LAVVR differed significantly in overall survival (P = 0.014). At the last follow-up (median, 9.8; range, 0–34 years), 84% survivors were in New York Heart Association Class I or II. CONCLUSIONS: Many patients reoperated after primary AVSD repair needed surgical reintervention. LAVV pathology was the most common indication for reoperation. DOLAVV was a risk factor for mortality. Particular AVSD type did not appear to be a risk factor for mortality or LAVVP failure. There is some evidence for the close relationship between LAVV pathology and LVOTO in subjects undergoing reoperation after primary AVSD repair as some patients with initial LAVV problems needed LVOTO repair later on and vice versa. Keywords: Atrioventricular septal defect • Valve repair • Valve replacement • Left ventricle outflow tract obstruction • Reoperation • Risk factors
INTRODUCTION Atrioventricular septal defect (AVSD) represents a spectrum of congenital heart defects characterized by incomplete development of the septal tissue at atrial and/or ventricular levels along with abnormalities of atrioventricular valves (AVVs). The results of surgical treatment of AVSD have improved significantly in the last 60 years [1, 2]. However, despite excellent survival, a significant number of patients need a surgical reintervention mainly because of severe left AVV (LAVV) regurgitation or left ventricular outflow tract obstruction (LVOTO) [3–5]. Recently, † Presented at the 28th Annual Meeting of the European Association for CardioThoracic Surgery, Milan, Italy, 11–15 October 2014.
we published a 30-year experience with surgical correction of AVSD and long-term results of reoperation for LAVV regurgitation after correction of AVSD [6, 7]. The objective of our current study is to evaluate experience in patients undergoing reoperation after primary AVSD repair from all causes and to identify predictors of poor outcome.
PATIENTS AND METHODS From January 1975 to April 2014, 457 consecutive patients received biventricular repair of AVSD at Centre for Congenital Heart Defects Amsterdam Leiden, Netherlands. The review of our surgical database has revealed 79 patients undergoing AVSD redo
© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CONGENITAL
Cite this article as: Sojak V, Kooij M, Yazdanbakhsh A, Koolbergen DR, Bruggemans EF, Hazekamp MG. A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect. Eur J Cardiothorac Surg 2015; doi:10.1093/ejcts/ezv115.
V. Sojak et al. / European Journal of Cardio-Thoracic Surgery
2
surgery at our institution. Inpatient and outpatient data were retrieved from individual medical records. Ten patients were lost to the follow-up. The complete data are available for 69 patients who enrolled in the current study. The study was approved with a waiver of consent by the local Ethics Committee because of its retrospective nature.
Patient characteristics In the study cohort, 28 patients (40.6%) had partial AVSD (PAVSD), 15 patients (21.7%) had intermediate AVSD (IAVSD) and 26 patients (37.7%) had complete AVSD (CAVSD). There were 29 males and 40 females. The incidence of Down syndrome was 40.6%. The frequency of LAVV dysplasia and double orifice LAVV (DOLAVV) was 21.7 and 4.3%, respectively. Two of 3 patients with DOLAVV had also pseudoparachute LAVV. Three patients (4.3%) had coarctation of the aorta. The median age at primary operation was 17.2 (range, 1.6–830) months, the median age at first reoperation was 62.4 (range, 1.6–845) months and the median time to first reoperation was 22.3 (range, 0.2–598) months (Table 1). The indications (25% of patients had multiple redo indications) for first reoperation after primary AVSD repair were as follows: LAVV pathology (n = 61, 65.6%), residual septal defect (n = 18, 19.4%), LVOTO (n = 4, 4.3%), right AVV (RAVV) pathology (n = 4,
4.3%), cardiac arrhythmia requiring pacemaker (PM) implantation (n = 3, 3.2%), right ventricle outflow tract obstruction (RVOTO, n = 2, 2.2%) and endocarditis (n = 1, 1.1%) (Table 2). At first reoperation, 47 patients (77%) and 14 patients (23%) who were reoperated on for LAVV pathology underwent LAVV valvuloplasty (LAVVP) and replacement (LAVVR), respectively. Residual septal defects (ASD = 7, VSD = 11) were closed in 17 patients. Four patients underwent RAVV repair. Four patients underwent LVOTO relief (membrane resection = 3, septal myectomy = 1). Three patients received PM. Two patients had RVOTO relief (muscle bundle resection + transannular patch). One patient with damaged LAVV due to endocarditis underwent LAVVR.
Surgical technique All reoperations for residual structural defects were performed via median sternotomy using cardiopulmonary bypass under mild or moderate hypothermia. Myocardial protection was achieved by repeated administration of cold St Thomas crystalloid cardioplegia every 30 min. The choice of particular surgical technique was based on the indication for reoperation, intraoperative transoesophageal echocardiography and anatomical findings. In case of LAVV pathology, the valve was inspected for possible valvuloplasty. The LAVV ‘cleft’ dehiscence was addressed by direct
Table 1: Patient characteristics
n (%) M/F Down syndrome, n (%) LAVV dysplasia, n (%) DOLAVV, n (%) Heterotaxy, n (%) Coarctation, n (%) Tetralogy of Fallot, n (%) Age at primary repair (median, range) in months Age at first redo (median, range) in months Time to first redo (median, range) in months
PAVSD
IAVSD
CAVSD
Total
P-values
28 (40.6) 13/15 3 (10.7) 5 (17.9) 2 (7.1) 5 (17.9) 2 (7.1) 0 (0) 52.5 (1.6–830) 184.8 (1.6–845) 69.6 (1.5–598)
15 (21.7) 4/11 3 (20) 4 (26.7) 0 (0) 3 (20) 0 (0) 0 (0) 40.9 (2.3–293) 54.0 (3.1–631) 22.1 (0.6–511)
26 (37.7) 12/14 22 (84.6) 6 (23.1) 1 (3.8) 0 (0) 1 (3.8) 5 (19.2) 4.8 (1.8–90.4) 30.8 (2.2–353) 12.6 (0.2–350)
69 (100) 29/40 28 (40.6) 15 (21.7) 3 (4.3) 8 (11.6) 3 (4.3) 5 (7.2) 17.2 (1.6–830) 62.4 (1.6–845) 22.3 (0.2–598)
0.395
ORIGINAL ARTICLE
European Journal of Cardio-Thoracic Surgery (2015) 1–8 doi:10.1093/ejcts/ezv115
A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect† Vladimir Sojak*, Marlotte Kooij, Aria Yazdanbakhsh, Dave R. Koolbergen, Eline F. Bruggemans and Mark G. Hazekamp Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands * Corresponding author. Department of Cardiothoracic Surgery, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands. Tel: +31-715262348; fax: +31-715248284; e-mail: [email protected] (V. Sojak). Received 22 September 2014; received in revised form 26 January 2015; accepted 3 February 2015
Abstract OBJECTIVES: To evaluate our experience with patients reoperated after primary repair of atrioventricular septal defect (AVSD) and identify predictors of poor outcome. METHODS: Between 1976 and 2014, 69 patients were reoperated after primary repair of partial (n = 28), intermediate (n = 15) or complete (n = 26) AVSD. RESULTS: Median age at first reoperation was 62.4 (range, 1.6–845) months, median interval to first reoperation was 22.3 (range, 0.2–598) months. Main indications for first reoperation included left atrioventricular valve (LAVV) pathology (66%), residual septal defect (19%) and left ventricle outflow tract obstruction (LVOTO; 4%). Procedures to address LAVV pathology included various valvuloplasties in 47 (77%) patients and valve replacement in 14 (23%) patients. A second, third, fourth and fifth reoperation was required in 27, 12, 4 and 1 patient, respectively. Most common procedures were LAVV replacement (LAVVR), LVOTO relief, pacemaker implantation and right atrioventricular valve procedure. Freedom from reoperation after LAVV valvuloplasty (LAVVP) was 84 and 62% at 1 and 10 years, respectively. There were 10 early and 4 late deaths. Estimated overall survival at 1, 5 and 10 years was 87, 83 and 83%, respectively. Double orifice LAVV (DOLAVV) was a risk factor for in-hospital and overall mortality [odds ratio (OR) = 14.5; 95% confidence interval (CI) = 1.2–178.7; P = 0.037 and hazard ratio (HR) = 6.8; 95% CI = 1.5–31.7; P = 0.015, respectively]. Patients with LAVVP and LAVVR differed significantly in overall survival (P = 0.014). At the last follow-up (median, 9.8; range, 0–34 years), 84% survivors were in New York Heart Association Class I or II. CONCLUSIONS: Many patients reoperated after primary AVSD repair needed surgical reintervention. LAVV pathology was the most common indication for reoperation. DOLAVV was a risk factor for mortality. Particular AVSD type did not appear to be a risk factor for mortality or LAVVP failure. There is some evidence for the close relationship between LAVV pathology and LVOTO in subjects undergoing reoperation after primary AVSD repair as some patients with initial LAVV problems needed LVOTO repair later on and vice versa. Keywords: Atrioventricular septal defect • Valve repair • Valve replacement • Left ventricle outflow tract obstruction • Reoperation • Risk factors
INTRODUCTION Atrioventricular septal defect (AVSD) represents a spectrum of congenital heart defects characterized by incomplete development of the septal tissue at atrial and/or ventricular levels along with abnormalities of atrioventricular valves (AVVs). The results of surgical treatment of AVSD have improved significantly in the last 60 years [1, 2]. However, despite excellent survival, a significant number of patients need a surgical reintervention mainly because of severe left AVV (LAVV) regurgitation or left ventricular outflow tract obstruction (LVOTO) [3–5]. Recently, † Presented at the 28th Annual Meeting of the European Association for CardioThoracic Surgery, Milan, Italy, 11–15 October 2014.
we published a 30-year experience with surgical correction of AVSD and long-term results of reoperation for LAVV regurgitation after correction of AVSD [6, 7]. The objective of our current study is to evaluate experience in patients undergoing reoperation after primary AVSD repair from all causes and to identify predictors of poor outcome.
PATIENTS AND METHODS From January 1975 to April 2014, 457 consecutive patients received biventricular repair of AVSD at Centre for Congenital Heart Defects Amsterdam Leiden, Netherlands. The review of our surgical database has revealed 79 patients undergoing AVSD redo
© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CONGENITAL
Cite this article as: Sojak V, Kooij M, Yazdanbakhsh A, Koolbergen DR, Bruggemans EF, Hazekamp MG. A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect. Eur J Cardiothorac Surg 2015; doi:10.1093/ejcts/ezv115.
V. Sojak et al. / European Journal of Cardio-Thoracic Surgery
2
surgery at our institution. Inpatient and outpatient data were retrieved from individual medical records. Ten patients were lost to the follow-up. The complete data are available for 69 patients who enrolled in the current study. The study was approved with a waiver of consent by the local Ethics Committee because of its retrospective nature.
Patient characteristics In the study cohort, 28 patients (40.6%) had partial AVSD (PAVSD), 15 patients (21.7%) had intermediate AVSD (IAVSD) and 26 patients (37.7%) had complete AVSD (CAVSD). There were 29 males and 40 females. The incidence of Down syndrome was 40.6%. The frequency of LAVV dysplasia and double orifice LAVV (DOLAVV) was 21.7 and 4.3%, respectively. Two of 3 patients with DOLAVV had also pseudoparachute LAVV. Three patients (4.3%) had coarctation of the aorta. The median age at primary operation was 17.2 (range, 1.6–830) months, the median age at first reoperation was 62.4 (range, 1.6–845) months and the median time to first reoperation was 22.3 (range, 0.2–598) months (Table 1). The indications (25% of patients had multiple redo indications) for first reoperation after primary AVSD repair were as follows: LAVV pathology (n = 61, 65.6%), residual septal defect (n = 18, 19.4%), LVOTO (n = 4, 4.3%), right AVV (RAVV) pathology (n = 4,
4.3%), cardiac arrhythmia requiring pacemaker (PM) implantation (n = 3, 3.2%), right ventricle outflow tract obstruction (RVOTO, n = 2, 2.2%) and endocarditis (n = 1, 1.1%) (Table 2). At first reoperation, 47 patients (77%) and 14 patients (23%) who were reoperated on for LAVV pathology underwent LAVV valvuloplasty (LAVVP) and replacement (LAVVR), respectively. Residual septal defects (ASD = 7, VSD = 11) were closed in 17 patients. Four patients underwent RAVV repair. Four patients underwent LVOTO relief (membrane resection = 3, septal myectomy = 1). Three patients received PM. Two patients had RVOTO relief (muscle bundle resection + transannular patch). One patient with damaged LAVV due to endocarditis underwent LAVVR.
Surgical technique All reoperations for residual structural defects were performed via median sternotomy using cardiopulmonary bypass under mild or moderate hypothermia. Myocardial protection was achieved by repeated administration of cold St Thomas crystalloid cardioplegia every 30 min. The choice of particular surgical technique was based on the indication for reoperation, intraoperative transoesophageal echocardiography and anatomical findings. In case of LAVV pathology, the valve was inspected for possible valvuloplasty. The LAVV ‘cleft’ dehiscence was addressed by direct
Table 1: Patient characteristics
n (%) M/F Down syndrome, n (%) LAVV dysplasia, n (%) DOLAVV, n (%) Heterotaxy, n (%) Coarctation, n (%) Tetralogy of Fallot, n (%) Age at primary repair (median, range) in months Age at first redo (median, range) in months Time to first redo (median, range) in months
PAVSD
IAVSD
CAVSD
Total
P-values
28 (40.6) 13/15 3 (10.7) 5 (17.9) 2 (7.1) 5 (17.9) 2 (7.1) 0 (0) 52.5 (1.6–830) 184.8 (1.6–845) 69.6 (1.5–598)
15 (21.7) 4/11 3 (20) 4 (26.7) 0 (0) 3 (20) 0 (0) 0 (0) 40.9 (2.3–293) 54.0 (3.1–631) 22.1 (0.6–511)
26 (37.7) 12/14 22 (84.6) 6 (23.1) 1 (3.8) 0 (0) 1 (3.8) 5 (19.2) 4.8 (1.8–90.4) 30.8 (2.2–353) 12.6 (0.2–350)
69 (100) 29/40 28 (40.6) 15 (21.7) 3 (4.3) 8 (11.6) 3 (4.3) 5 (7.2) 17.2 (1.6–830) 62.4 (1.6–845) 22.3 (0.2–598)
0.395
