European Journal of Cardio-Thoracic Surgery Advance Access published January 30, 2014
The Yasui operation for patients with adequate-sized ventricles and ventricular septal defect associated with obstructions of the aortic arch and left ventricular outflow tract† Toshihide Nakano* ,a, Hideaki Kadoa, Hideki Tatewakia, Kazuhiro Hinokiyamaa, Daisuke Machidaa, Noriyoshi Ebuokaa and Hisataka Yasuib a b
Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, Fukuoka, Japan Hamanomachi Hospital, Fukuoka, Japan
* Corresponding author. Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, 2-5-1 Tojin-machi, Chuo-ku, Fukuoka 810-0063, Japan. Tel: +81-92-7133111; fax: +81-92-7133122; e-mail: [email protected] (T. Nakano). Received 19 September 2013; received in revised form 25 November 2013; accepted 4 December 2013
Abstract OBJECTIVE: To review the surgical outcome of the Yasui operation in patients with adequate-sized ventricles and ventricular septal defect (VSD) associated with obstructions of the aortic arch and left ventricular outflow tract (LVOT). METHODS: Since 1985, 17 patients have undergone the Yasui operation at our institution. Interrupted aortic arch was present in 11 patients and coarctation of the aorta/hypoplastic arch was present in 6. Twelve patients had aortic stenosis, and 5 patients had aortic atresia. The minimum diameter of the LVOT and the z-score in patients with aortic stenosis were 3.7 ± 0.4 mm and −9.2 ± 1.2, respectively. Primary repair was performed in 6 patients, and 11 patients were staged, with bilateral pulmonary artery banding (PAB) in 8, arch repair with PAB in 2 and Norwood operation in 1. The mean age and body weight at the time of the Yasui operation was 4.7 ± 5.3 months and 4.5 ± 1.8 kg, respectively. The ascending aorta and aortic arch were reconstructed by Damus–Kaye–Stansel (DKS) anastomosis with graft interposition in 2, DKS with direct anastomosis in 6 and Norwood-type reconstruction in 9. VSD was enlarged in 6 patients. Right ventricle to pulmonary artery continuity was established with a valved conduit in 14 patients, the Lecompte manoeuvre in 2 patients and another method in 1 patient. The mean duration of the follow-up was 7.6 ± 9.2 years. RESULTS: There was 1 early death due to myocardial infarction and 1 late death due to non-cardiac cause. The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively. In the last echo study, LVOT flow velocity was 1.2 ± 0.8 m/s, and neoaortic valve regurgitation was mild in 1 patient and trivial or absent in the remaining patients. CONCLUSIONS: The results of the Yasui operation were excellent, showing low mortality and good mid-term left ventricular function without outflow tract stenosis or neoaortic valve insufficiency. Bilateral PAB as initial palliation is a useful option in symptomatic neonates. Keywords: Yasui operation • Left ventricular outflow tract obstruction • Biventricular repair • Bilateral pulmonary artery banding
INTRODUCTION The first report of primary biventricular repair by Yasui in 2 neonates with interrupted aortic arch (IAA) and severe left ventricular outflow tract obstruction (LVOTO) was in 1987 at our institution [1]. Since then, several modifications have been implemented [2–7], and satisfactory surgical results have been reported [8–12]. The utility of the Yasui operation has been well recognized; however, because of the complicated and extensive nature of this surgery, controversy exists regarding the surgical strategy of this procedure. Some leading institutions recommend primary repair in the neonatal period [9, 10]; however, staged repair following an initial palliative procedure should be considered depending on the † Presented at the 27th Annual Meeting of the European Association for CardioThoracic Surgery, Vienna, Austria, 5-9 October 2013.
patient’s condition [12]. In this study, we summarized our experience of the Yasui operation to assess the surgical outcome and efficacy of the staged strategy.
MATERIALS AND METHODS Patients From 1985 to 2012, 17 patients underwent the Yasui operation at Fukuoka Children’s Hospital. The characteristics of the patients are summarized in Table 1. Eleven patients had IAA, and 6 patients had coarctation of the aorta (CoA) or hypoplastic arch. Among the types of LVOTO, aortic atresia was present in 5 patients and aortic valvular stenosis and/or subaortic stenosis was observed in 12
© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CONGENITAL
ORIGINAL ARTICLE
European Journal of Cardio-Thoracic Surgery (2014) 1–7 doi:10.1093/ejcts/ezt658
T. Nakano et al. / European Journal of Cardio-Thoracic Surgery
2
Table 1: Patient characteristics and echocardiographic measurements before the Yasui operation No.
Arch anatomy
Type of LVOTO
Cardiac anatomy
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
IAA(B) IAA(A) CoA IAA(B) Hypo arch IAA(B) IAA(B) IAA(B) IAA(B) IAA(C) CoA IAA(B) IAA(B) IAA(A) CoA CoA Hypo arch
AS AS AA AS AA AS AS AS AA AA AS AS AS AS AS AS AA
VSD DORV VSD VSD VSD DOLV VSD DORV VSD DORV VSD VSD VSD DORV DORV DORV DORV
Non-cardiac diagnosis
Minimum LVOT diameter (mm)
22q11.2deletion
CHARGE synd CHARGE synd 22q11.2deletion
CHARGE synd
Minimum LVOT diameter (z-score)
Mitral annular diameter (mm)
Mitral annular diameter (z-score)
−11.7 −8.7
3 3.6 – 3.5 – 3.5 3.8 3.7 – – 3.9 3.3 3.8 4.1 4.4 3.6 –
−8.3 −8.7 −11.5 −8.3 −8.8 −8.7 −8.5 −9.3 −9.7 −10.3
12.6 13.4 19.4 9.9 13.6 18.5 12.1 12.3 12.9 17.3 14.4 12.1 13.8
0.5 0.5 1.5 −2.2 −0.6 2.5 −1.6 0.2 0.7 1.1 −0.9 −3.1 −0.8
IAA, interrupted aortic arch; CoA, coarctation of aorta; AS, aortic stenosis; AA, aortic atresia; VSD, ventricular septal defect; DORV, double outlet right ventricle; DOLV, double outlet left ventricle; LVOT, left ventricular outflow tract.
patients. The cardiac anatomy was ventricular septal defect (VSD) in 9 patients, double outlet right ventricle (DORV) in 7 patients and double outlet left ventricle (DOLV) in 1 patient. The type of VSD was perimembranous outlet in 9 patients, including inlet extension in 3 patients, subarterial in 7 patients and muscular outlet in 1 patient. Five patients had chromosomal abnormalities, including CHARGE syndrome in 3 patients and the 22q11.2 deletion syndrome in 2 patients. The echocardiographic measurements taken before the Yasui operation showed that the mean minimum left ventricular outflow tract (LVOT) diameter was 3.7 ± 0.4 mm (range, 3.0–4.4) and the mean z-score was −9.4 ± 1.2 (range, −11.7 to −8.3). The mean mitral annulus diameter was 14.1 ± 2.8 mm (range, 9.9– 19.4) and the z-score was −0.2 ± 1.6 (range, −3.1 to 2.5). Prior to the Yasui operation in 11 patients, palliative surgery was performed, including bilateral pulmonary artery banding (PAB) in 8 patients, aortic arch repair with PAB in 2 patients and Norwood operation in 1 patient. The 2 patients who initially underwent aortic arch repair with PAB were patients with DORV (Taussig–Bing anomaly) with IAA, and DORV (subarterial VSD) with CoA, who were presented with severe congestive heart failure. We decided to perform a staged biventricular repair; however, subaortic stenosis developed after the initial operation, which made them candidates for the Yasui operation. In the staged group, age and body weight of the patients at the time of palliative surgery were 12.2 ± 7.1 days (range, 4–26) and 3.2 ± 0.3 kg (range, 2.8–3.6), respectively, and the values at the time of the Yasui operation were 200.1 ± 176.4 days (range, 41–669) and 5.3 ± 1.9 kg (range, 2.6–8.2), respectively. The interval between the palliative surgery and the Yasui operation was 188.7 ± 175.3 days (range, 23–652) in the staged group. Six patients underwent a primary Yasui operation at a mean age and body weight of 34.3 ± 22.1 days (range, 16–74) and 3.2 ± 0.3 kg (range, 2.8–3.69), respectively. The complete follow-up was performed for all survivors, and the mean duration of the follow-up was 7.6 ± 9.2 years (range, 0.7–28.1).
Operation Cardiopulmonary bypass technique. During the aortic arch reconstruction in our earlier series, 33 min of deep hypothermic circulatory arrest was used in 1 patient, and a short period (18, 19 and 22 min) of cerebral circulatory arrest was employed in 3 patients using the revised perfusion technique developed at our institution [13]. In the last 13 cases from 2003, the total body perfusion technique was employed to avoid circulatory arrest during arch reconstruction [14]. Briefly, by inserting another arterial cannula to the descending thoracic aorta through the posterior pericardium for lower body perfusion in addition to selective cerebral perfusion, mild hypothermic high-flow total body perfusion was maintained throughout the operation.
Aortic reconstruction. In the first 2 patients in this series, the interrupted arch was repaired by the interposition of polytetrafluoroethylene (PTFE) graft (8 mm in 1 patient and 6 mm in the other patient) from the ascending aorta to the descending aorta in addition to Dames–Kaye–Stansel (DKS) connection between the proximal pulmonary trunk and the ascending aorta. Of the remaining 15 patients, proximal DKS connection combined with direct arch anastomosis was performed in 6 patients, and Norwood-type reconstruction was performed in 9 patients. Intraventricular rerouting. A longitudinal incision was made approximately 10 mm beneath the pulmonary annulus on the right ventricular outflow tract, rather than on the body of the right ventricle. Intraventricular baffling can be performed through this RV outflow incision when the type of VSD is perimembranous outlet, subarterial or muscular outlet. The lower part of the VSD is closed through the right atrium when the VSD extends towards the inlet. We are extremely careful to ensure that the incision is not close to the left anterior descending artery.
T. Nakano et al. / European Journal of Cardio-Thoracic Surgery
A Dacron patch (n = 4) or PTFE patch (n = 13) was used to baffle the left ventricular outflow through the VSD to the pulmonary and aortic valves in patients with aortic stenosis or to the pulmonary valve in patients with aortic atresia. The VSD was enlarged anterolaterally in 6 patients.
Statistical analysis
Right ventricle to pulmonary artery connection. A RV to
RESULTS
pulmonary artery (PA) connection was established using a valved conduit in 14 patients (Hancock 14 mm in 2 patients, Carpenter-Edwards 12 mm in 1 patient, a handmade valved conduit from the xenopericardium in 1 patient and a handmade PTFE valved conduit [15] in 10 patients [10 mm in 1 patient, 12 mm in 7 patients, 14 mm in 1 patient, and 16 mm in 1 patient]). The right ventricular outflow tract reconstruction (RVOTR) was performed by direct posterior anastomosis with an anterior monocusp patch in 1 patient, and the Lecompte manoeuvre [6, 7] was employed in 2 patients. The mean duration of cardiopulmonary bypass and myocardial ischaemia were 330.5 ± 79.8 min and 135.2 ± 23.2 min, respectively. Sternal closure was possible in 4 of 6 patients in the primary group and 9 of 11 patients in the staged group. The perioperative data are summarized in Table 2.
There was no interstage death after the initial palliation in the staged group. After the Yasui operation, 1 patient required extracorporeal membrane oxygenation (ECMO) due to severe ventricular dysfunction. The patient was successfully weaned off ECMO after 5 days later. Operative death occurred in 1 patient. The patient was a 3.2 kg neonate with a diagnosis of aortic atresia with VSD associated with CoA. The patient underwent a primary Yasui operation at the age of 20 days. The ascending aorta was 1.5 mm in diameter, and Norwood-type aortic reconstruction using a xenopericardial roll was performed. Acute haemodynamic deterioration occurred immediately after admission to the intensive care unit (ICU). Autopsy confirmed the acute myocardial infarction. There was 1 late death, 11 months after the operation, where the patient died of acute viral encephalitis. The
All data were expressed as mean ± standard deviation. Actuarial survival and freedom from reoperation were calculated using the Kaplan–Meier formula.
Table 2: Perioperative data of the Yasui operation No. Staged Initial procedure
Age (days)
Body weight (kg)
Aortic reconstruction
VSD enlargement
RVOTR
CPB time (min)
MC ischaemic time (min)
Valved conduit [14 mm] Valved conduit [14 mm] Xenopericardial conduit Valved conduit [12 mm] Lecompte Anterior patch PTFE conduit [12 mm] PTFE conduit [10 mm] Lecompte PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [14 mm] PTFE conduit [16 mm]
270
123
18
Survived
276
138
19
Survived
329
144
33
Died
310
146
22
Survived
506 294 396
146 144 108
0 0 0
Survived Survived Survived
321
113
0
Survived
308 480
137 182
0 0
Survived Survived
439
160
0
Survived
310
165
0
Survived
319
112
0
Survived
267
147
0
Survived
307
90
0
Survived
202
124
0
Survived
284
119
0
Survived
1
0
36
3.4
DKS + grafting
0
2
0
18
3.1
DKS + grafting
0
3
0
20
3.2
NWD type
0
4
0
42
2.8
DKS + direct
1
5 6 7
1 0 1
Bil.PAB Bil.PAB
41 16 669
2.6 2.9 7.8
NWD type DKS + direct NWD type
0 0 1
8
1
Bil.PAB
120
3.3
NWD type
0
9 10
0 1
Bil.PAB
74 91
3.6 4.5
NWD type NWD type
0 1
11
1
Bil.PAB
102
4.5
DKS + direct
0
12
1
Bil.PAB
115
5.3
DKS + direct
1
13
1
Bil.PAB
182
4.8
NWD type
1
14
1
Arch repair + PAB
261
7.6
DKS + direct
0
15
1
Bil.PAB
4
NWD type
0
16
1
Arch repair + PAB
207
5.6
DKS + direct
0
17
1
NWD
326
8.2
NWD type
1
96
CA time Result (min)
Bil.PAB, bilateral pulmonary artery banding; NWD, Norwood; DKS, Damus–Kaye–Stansel; VSD, ventricular septal defect; RVOTR, right ventricular outflow tract reconstruction; PTFE, polytetrafluoroethylene; CPB, cardiopulmonary bypass; MC, myocardial; CA, circulatory arrest.
CONGENITAL
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reoperation for a significant pressure gradient. In the first patient with an 8 mm graft, another graft interposition between the left common carotid artery and descending aorta with a 12-mm PTFE graft was performed 7.8 years after the primary Yasui operation. The second patient underwent replacement of the 6-mm interposing graft with an 18-mm graft 8.7 years later. Two procedures for the branch pulmonary artery (resection of the fibrous ridge in 1 patient and PTFE graft interposition to the left pulmonary artery in 1 patient who underwent the Lecompte manoeuvre) were performed concomitantly with RVOTR. The first patient in our series underwent a second RV-PA conduit exchange. The freedom from reoperation for all causes at 5 and 10 years in our series were 71.3 and 28.5%, respectively, and 81.5 and 48.9% for reoperation excluding the RV-PA conduit exchange (Fig. 2). Figure 1: Actuarial survival. The survival for the entire group was 87.8% at 10 years.
Table 3: Reoperation (n = 6) RV-PA conduit exchange (n = 4) Second RV-PA conduit exchange (n = 1) RVOTR (anterior monocusp patch, n = 1) LVOTR (n = 2) Mitral valve plasty (n = 1) Graft replacement in aortic arch (n = 1) Graft interpose in aortic arch (n = 1) PA plasty (n = 2) RV-PA: right ventricle to pulmonary artery; RVOTR: right ventricular outflow tract reconstruction; RVOTR: right ventricular outflow tract reconstruction; LVOTR: left ventricular outflow tract reconstruction; PA: pulmonary artery.
haemodynamic status of the patient had been stable. The actuarial survival at 10 years was 87.8% in the entire group (Fig. 1).
Postoperative course In the staged group, 5 patients were extubated within 24 h after the Yasui operation. Excluding a patient who required ECMO and another patient with multiple chromosomal disorder, the mean time to the extubation and the mean ICU stay were 1.9 ± 1.5 days (range, 0–5) and 5.8 ± 2.4 days (range, 3–10) in the staged group, and 15.4 ± 16.8 days (range, 3–39) and 30.4 ± 26.2 days (range, 16– 75) in the primary group, respectively. Inotropes were discontinued after 5.5 ± 3.6 days (range, 1–13) in the staged group and 12.2 ± 3.0 days (range, 8–15) in the primary group.
Echocardiogram The last postoperative cardiac echo study was performed in 16 patients at 5.6 ± 7.6 years after the Yasui operation. The left ventricular ejection fraction was 70.5 ± 12.1%, and the LVOT flow velocity was 1.2 ± 0.8 m/s (range, 0–3.9). The patient with an LVOT flow velocity of 3.9 m/s recurrently developed subaortic fibrous ridge after surgical resection in the reoperation as described above. The degree of regurgitation from the neoaortic valve (anatomical aortic valve or pulmonary valve) was absent in 11 patients, trivial in 5 patients, and mild in 1 patient. All survivors are in New York Heart Association Class I.
Reoperation Six patients underwent 8 reoperations, including 13 procedures (Table 3). Six RVOTRs (RV-PA conduit exchange in 5 patients and anterior monocusp patch in 1 patient) were performed 5.3 ± 2.5 years after the Yasui operation. Two procedures for recurrent LVOTO were performed. The first patient originally had a perimembranous outlet with inlet extension VSD with an accessory mitral valve. At the time of the Yasui operation, the accessory mitral valve was resected, and the VSD was enlarged, and a long intraventricular route using a PTFE patch was created. The patient developed recurrent LVOTO and mitral regurgitation and underwent redo-intraventricular baffling with concomitant mitral valve repair using PTFE artificial chordae at 28 months after the Yasui operation. The other patient developed discrete fibrous stenosis at the subaortic area and underwent resection of the subaortic fibrous tissue with VSD enlargement after 42 months. The first 2 patients who had an interposing PTFE graft between the ascending and descending aorta required
DISCUSSION In the presence of a VSD with two adequate-sized ventricles, biventricular repair is possible in patients with severe LVOTO and arch obstruction. The first report of a successful primary biventricular repair in 2 neonates with IAA and severe aortic stenosis was performed by Yasui at our institution in 1987 [1]. Since then, the concept of the Yasui operation (a combination of integrated aortic arch reconstruction, redirection of LV outflow through the VSD to both the semilunar valves and establishment of RV-PA continuity using a valved conduit) has been applied for patients with aortic atresia and VSD with normal left ventricle [2, 3, 6]. Several modifications have been made in terms of the surgical technique. Aortic arch reconstruction may be performed by proximal anastomosis of the pulmonary trunk and the ascending aorta by means of DKS connection with direct repair of the obstructed arch or Norwood-type arch reconstruction with or without the use of a homograft [2–6], depending on the individual anatomy. To
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CONGENITAL
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Figure 2: Freedom from reoperation. The freedom from reoperation for all causes was 71.3% at 5 years and 28.5% at 10 years (A). The freedom from reoperation other than exchange of the right ventricle to the pulmonary artery conduit was 81.5% at 5 years and 48.9% at 10 years (B).
establish RV-PA continuity, the Lecompte manoeuvre has been expected to reduce or delay the risk of reoperation in patients with a certain anatomy [6, 7]. The surgical results of the Yasui operation in a relatively small series of patients have been reported with satisfactory results. An early report by Ohye et al. [11] described their experience with 20 patients (11 underwent primary repair and 9 underwent staged repair) with a 5-year actuarial survival of 78%. Pearl et al. [12] reported the results of 8 patients (6 were staged with the initial Norwood operation) without early and late mortality. More recently, Kanter et al. [8] reported their experience of the Yasui operation in 21 patients (6 underwent primary repair and 15 underwent staged repair) with no early mortality, 3 late deaths and a 5-year actuarial survival of 75%. The result of the current study demonstrating an actuarial survival of 87.8% at 10 years is satisfactory for this group of patients with complex heart defects. The Yasui operation requires a valved conduit for RV-PA continuity; therefore, reoperation is frequently required. The Yasui operation is indicated in patients with significant aortic valvular stenosis or subaortic stenosis that is unable to accommodate systemic output without a pressure gradient in the presence of a normal left ventricle and VSD. In determining the indication for the Yasui operation in terms of the severity of LVOTO, we set the minimum LVOT diameter of body weight plus 1 mm or less. For example, an LVOT diameter of
The Yasui operation for patients with adequate-sized ventricles and ventricular septal defect associated with obstructions of the aortic arch and left ventricular outflow tract† Toshihide Nakano* ,a, Hideaki Kadoa, Hideki Tatewakia, Kazuhiro Hinokiyamaa, Daisuke Machidaa, Noriyoshi Ebuokaa and Hisataka Yasuib a b
Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, Fukuoka, Japan Hamanomachi Hospital, Fukuoka, Japan
* Corresponding author. Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, 2-5-1 Tojin-machi, Chuo-ku, Fukuoka 810-0063, Japan. Tel: +81-92-7133111; fax: +81-92-7133122; e-mail: [email protected] (T. Nakano). Received 19 September 2013; received in revised form 25 November 2013; accepted 4 December 2013
Abstract OBJECTIVE: To review the surgical outcome of the Yasui operation in patients with adequate-sized ventricles and ventricular septal defect (VSD) associated with obstructions of the aortic arch and left ventricular outflow tract (LVOT). METHODS: Since 1985, 17 patients have undergone the Yasui operation at our institution. Interrupted aortic arch was present in 11 patients and coarctation of the aorta/hypoplastic arch was present in 6. Twelve patients had aortic stenosis, and 5 patients had aortic atresia. The minimum diameter of the LVOT and the z-score in patients with aortic stenosis were 3.7 ± 0.4 mm and −9.2 ± 1.2, respectively. Primary repair was performed in 6 patients, and 11 patients were staged, with bilateral pulmonary artery banding (PAB) in 8, arch repair with PAB in 2 and Norwood operation in 1. The mean age and body weight at the time of the Yasui operation was 4.7 ± 5.3 months and 4.5 ± 1.8 kg, respectively. The ascending aorta and aortic arch were reconstructed by Damus–Kaye–Stansel (DKS) anastomosis with graft interposition in 2, DKS with direct anastomosis in 6 and Norwood-type reconstruction in 9. VSD was enlarged in 6 patients. Right ventricle to pulmonary artery continuity was established with a valved conduit in 14 patients, the Lecompte manoeuvre in 2 patients and another method in 1 patient. The mean duration of the follow-up was 7.6 ± 9.2 years. RESULTS: There was 1 early death due to myocardial infarction and 1 late death due to non-cardiac cause. The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively. In the last echo study, LVOT flow velocity was 1.2 ± 0.8 m/s, and neoaortic valve regurgitation was mild in 1 patient and trivial or absent in the remaining patients. CONCLUSIONS: The results of the Yasui operation were excellent, showing low mortality and good mid-term left ventricular function without outflow tract stenosis or neoaortic valve insufficiency. Bilateral PAB as initial palliation is a useful option in symptomatic neonates. Keywords: Yasui operation • Left ventricular outflow tract obstruction • Biventricular repair • Bilateral pulmonary artery banding
INTRODUCTION The first report of primary biventricular repair by Yasui in 2 neonates with interrupted aortic arch (IAA) and severe left ventricular outflow tract obstruction (LVOTO) was in 1987 at our institution [1]. Since then, several modifications have been implemented [2–7], and satisfactory surgical results have been reported [8–12]. The utility of the Yasui operation has been well recognized; however, because of the complicated and extensive nature of this surgery, controversy exists regarding the surgical strategy of this procedure. Some leading institutions recommend primary repair in the neonatal period [9, 10]; however, staged repair following an initial palliative procedure should be considered depending on the † Presented at the 27th Annual Meeting of the European Association for CardioThoracic Surgery, Vienna, Austria, 5-9 October 2013.
patient’s condition [12]. In this study, we summarized our experience of the Yasui operation to assess the surgical outcome and efficacy of the staged strategy.
MATERIALS AND METHODS Patients From 1985 to 2012, 17 patients underwent the Yasui operation at Fukuoka Children’s Hospital. The characteristics of the patients are summarized in Table 1. Eleven patients had IAA, and 6 patients had coarctation of the aorta (CoA) or hypoplastic arch. Among the types of LVOTO, aortic atresia was present in 5 patients and aortic valvular stenosis and/or subaortic stenosis was observed in 12
© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CONGENITAL
ORIGINAL ARTICLE
European Journal of Cardio-Thoracic Surgery (2014) 1–7 doi:10.1093/ejcts/ezt658
T. Nakano et al. / European Journal of Cardio-Thoracic Surgery
2
Table 1: Patient characteristics and echocardiographic measurements before the Yasui operation No.
Arch anatomy
Type of LVOTO
Cardiac anatomy
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
IAA(B) IAA(A) CoA IAA(B) Hypo arch IAA(B) IAA(B) IAA(B) IAA(B) IAA(C) CoA IAA(B) IAA(B) IAA(A) CoA CoA Hypo arch
AS AS AA AS AA AS AS AS AA AA AS AS AS AS AS AS AA
VSD DORV VSD VSD VSD DOLV VSD DORV VSD DORV VSD VSD VSD DORV DORV DORV DORV
Non-cardiac diagnosis
Minimum LVOT diameter (mm)
22q11.2deletion
CHARGE synd CHARGE synd 22q11.2deletion
CHARGE synd
Minimum LVOT diameter (z-score)
Mitral annular diameter (mm)
Mitral annular diameter (z-score)
−11.7 −8.7
3 3.6 – 3.5 – 3.5 3.8 3.7 – – 3.9 3.3 3.8 4.1 4.4 3.6 –
−8.3 −8.7 −11.5 −8.3 −8.8 −8.7 −8.5 −9.3 −9.7 −10.3
12.6 13.4 19.4 9.9 13.6 18.5 12.1 12.3 12.9 17.3 14.4 12.1 13.8
0.5 0.5 1.5 −2.2 −0.6 2.5 −1.6 0.2 0.7 1.1 −0.9 −3.1 −0.8
IAA, interrupted aortic arch; CoA, coarctation of aorta; AS, aortic stenosis; AA, aortic atresia; VSD, ventricular septal defect; DORV, double outlet right ventricle; DOLV, double outlet left ventricle; LVOT, left ventricular outflow tract.
patients. The cardiac anatomy was ventricular septal defect (VSD) in 9 patients, double outlet right ventricle (DORV) in 7 patients and double outlet left ventricle (DOLV) in 1 patient. The type of VSD was perimembranous outlet in 9 patients, including inlet extension in 3 patients, subarterial in 7 patients and muscular outlet in 1 patient. Five patients had chromosomal abnormalities, including CHARGE syndrome in 3 patients and the 22q11.2 deletion syndrome in 2 patients. The echocardiographic measurements taken before the Yasui operation showed that the mean minimum left ventricular outflow tract (LVOT) diameter was 3.7 ± 0.4 mm (range, 3.0–4.4) and the mean z-score was −9.4 ± 1.2 (range, −11.7 to −8.3). The mean mitral annulus diameter was 14.1 ± 2.8 mm (range, 9.9– 19.4) and the z-score was −0.2 ± 1.6 (range, −3.1 to 2.5). Prior to the Yasui operation in 11 patients, palliative surgery was performed, including bilateral pulmonary artery banding (PAB) in 8 patients, aortic arch repair with PAB in 2 patients and Norwood operation in 1 patient. The 2 patients who initially underwent aortic arch repair with PAB were patients with DORV (Taussig–Bing anomaly) with IAA, and DORV (subarterial VSD) with CoA, who were presented with severe congestive heart failure. We decided to perform a staged biventricular repair; however, subaortic stenosis developed after the initial operation, which made them candidates for the Yasui operation. In the staged group, age and body weight of the patients at the time of palliative surgery were 12.2 ± 7.1 days (range, 4–26) and 3.2 ± 0.3 kg (range, 2.8–3.6), respectively, and the values at the time of the Yasui operation were 200.1 ± 176.4 days (range, 41–669) and 5.3 ± 1.9 kg (range, 2.6–8.2), respectively. The interval between the palliative surgery and the Yasui operation was 188.7 ± 175.3 days (range, 23–652) in the staged group. Six patients underwent a primary Yasui operation at a mean age and body weight of 34.3 ± 22.1 days (range, 16–74) and 3.2 ± 0.3 kg (range, 2.8–3.69), respectively. The complete follow-up was performed for all survivors, and the mean duration of the follow-up was 7.6 ± 9.2 years (range, 0.7–28.1).
Operation Cardiopulmonary bypass technique. During the aortic arch reconstruction in our earlier series, 33 min of deep hypothermic circulatory arrest was used in 1 patient, and a short period (18, 19 and 22 min) of cerebral circulatory arrest was employed in 3 patients using the revised perfusion technique developed at our institution [13]. In the last 13 cases from 2003, the total body perfusion technique was employed to avoid circulatory arrest during arch reconstruction [14]. Briefly, by inserting another arterial cannula to the descending thoracic aorta through the posterior pericardium for lower body perfusion in addition to selective cerebral perfusion, mild hypothermic high-flow total body perfusion was maintained throughout the operation.
Aortic reconstruction. In the first 2 patients in this series, the interrupted arch was repaired by the interposition of polytetrafluoroethylene (PTFE) graft (8 mm in 1 patient and 6 mm in the other patient) from the ascending aorta to the descending aorta in addition to Dames–Kaye–Stansel (DKS) connection between the proximal pulmonary trunk and the ascending aorta. Of the remaining 15 patients, proximal DKS connection combined with direct arch anastomosis was performed in 6 patients, and Norwood-type reconstruction was performed in 9 patients. Intraventricular rerouting. A longitudinal incision was made approximately 10 mm beneath the pulmonary annulus on the right ventricular outflow tract, rather than on the body of the right ventricle. Intraventricular baffling can be performed through this RV outflow incision when the type of VSD is perimembranous outlet, subarterial or muscular outlet. The lower part of the VSD is closed through the right atrium when the VSD extends towards the inlet. We are extremely careful to ensure that the incision is not close to the left anterior descending artery.
T. Nakano et al. / European Journal of Cardio-Thoracic Surgery
A Dacron patch (n = 4) or PTFE patch (n = 13) was used to baffle the left ventricular outflow through the VSD to the pulmonary and aortic valves in patients with aortic stenosis or to the pulmonary valve in patients with aortic atresia. The VSD was enlarged anterolaterally in 6 patients.
Statistical analysis
Right ventricle to pulmonary artery connection. A RV to
RESULTS
pulmonary artery (PA) connection was established using a valved conduit in 14 patients (Hancock 14 mm in 2 patients, Carpenter-Edwards 12 mm in 1 patient, a handmade valved conduit from the xenopericardium in 1 patient and a handmade PTFE valved conduit [15] in 10 patients [10 mm in 1 patient, 12 mm in 7 patients, 14 mm in 1 patient, and 16 mm in 1 patient]). The right ventricular outflow tract reconstruction (RVOTR) was performed by direct posterior anastomosis with an anterior monocusp patch in 1 patient, and the Lecompte manoeuvre [6, 7] was employed in 2 patients. The mean duration of cardiopulmonary bypass and myocardial ischaemia were 330.5 ± 79.8 min and 135.2 ± 23.2 min, respectively. Sternal closure was possible in 4 of 6 patients in the primary group and 9 of 11 patients in the staged group. The perioperative data are summarized in Table 2.
There was no interstage death after the initial palliation in the staged group. After the Yasui operation, 1 patient required extracorporeal membrane oxygenation (ECMO) due to severe ventricular dysfunction. The patient was successfully weaned off ECMO after 5 days later. Operative death occurred in 1 patient. The patient was a 3.2 kg neonate with a diagnosis of aortic atresia with VSD associated with CoA. The patient underwent a primary Yasui operation at the age of 20 days. The ascending aorta was 1.5 mm in diameter, and Norwood-type aortic reconstruction using a xenopericardial roll was performed. Acute haemodynamic deterioration occurred immediately after admission to the intensive care unit (ICU). Autopsy confirmed the acute myocardial infarction. There was 1 late death, 11 months after the operation, where the patient died of acute viral encephalitis. The
All data were expressed as mean ± standard deviation. Actuarial survival and freedom from reoperation were calculated using the Kaplan–Meier formula.
Table 2: Perioperative data of the Yasui operation No. Staged Initial procedure
Age (days)
Body weight (kg)
Aortic reconstruction
VSD enlargement
RVOTR
CPB time (min)
MC ischaemic time (min)
Valved conduit [14 mm] Valved conduit [14 mm] Xenopericardial conduit Valved conduit [12 mm] Lecompte Anterior patch PTFE conduit [12 mm] PTFE conduit [10 mm] Lecompte PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [12 mm] PTFE conduit [14 mm] PTFE conduit [16 mm]
270
123
18
Survived
276
138
19
Survived
329
144
33
Died
310
146
22
Survived
506 294 396
146 144 108
0 0 0
Survived Survived Survived
321
113
0
Survived
308 480
137 182
0 0
Survived Survived
439
160
0
Survived
310
165
0
Survived
319
112
0
Survived
267
147
0
Survived
307
90
0
Survived
202
124
0
Survived
284
119
0
Survived
1
0
36
3.4
DKS + grafting
0
2
0
18
3.1
DKS + grafting
0
3
0
20
3.2
NWD type
0
4
0
42
2.8
DKS + direct
1
5 6 7
1 0 1
Bil.PAB Bil.PAB
41 16 669
2.6 2.9 7.8
NWD type DKS + direct NWD type
0 0 1
8
1
Bil.PAB
120
3.3
NWD type
0
9 10
0 1
Bil.PAB
74 91
3.6 4.5
NWD type NWD type
0 1
11
1
Bil.PAB
102
4.5
DKS + direct
0
12
1
Bil.PAB
115
5.3
DKS + direct
1
13
1
Bil.PAB
182
4.8
NWD type
1
14
1
Arch repair + PAB
261
7.6
DKS + direct
0
15
1
Bil.PAB
4
NWD type
0
16
1
Arch repair + PAB
207
5.6
DKS + direct
0
17
1
NWD
326
8.2
NWD type
1
96
CA time Result (min)
Bil.PAB, bilateral pulmonary artery banding; NWD, Norwood; DKS, Damus–Kaye–Stansel; VSD, ventricular septal defect; RVOTR, right ventricular outflow tract reconstruction; PTFE, polytetrafluoroethylene; CPB, cardiopulmonary bypass; MC, myocardial; CA, circulatory arrest.
CONGENITAL
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T. Nakano et al. / European Journal of Cardio-Thoracic Surgery
4
reoperation for a significant pressure gradient. In the first patient with an 8 mm graft, another graft interposition between the left common carotid artery and descending aorta with a 12-mm PTFE graft was performed 7.8 years after the primary Yasui operation. The second patient underwent replacement of the 6-mm interposing graft with an 18-mm graft 8.7 years later. Two procedures for the branch pulmonary artery (resection of the fibrous ridge in 1 patient and PTFE graft interposition to the left pulmonary artery in 1 patient who underwent the Lecompte manoeuvre) were performed concomitantly with RVOTR. The first patient in our series underwent a second RV-PA conduit exchange. The freedom from reoperation for all causes at 5 and 10 years in our series were 71.3 and 28.5%, respectively, and 81.5 and 48.9% for reoperation excluding the RV-PA conduit exchange (Fig. 2). Figure 1: Actuarial survival. The survival for the entire group was 87.8% at 10 years.
Table 3: Reoperation (n = 6) RV-PA conduit exchange (n = 4) Second RV-PA conduit exchange (n = 1) RVOTR (anterior monocusp patch, n = 1) LVOTR (n = 2) Mitral valve plasty (n = 1) Graft replacement in aortic arch (n = 1) Graft interpose in aortic arch (n = 1) PA plasty (n = 2) RV-PA: right ventricle to pulmonary artery; RVOTR: right ventricular outflow tract reconstruction; RVOTR: right ventricular outflow tract reconstruction; LVOTR: left ventricular outflow tract reconstruction; PA: pulmonary artery.
haemodynamic status of the patient had been stable. The actuarial survival at 10 years was 87.8% in the entire group (Fig. 1).
Postoperative course In the staged group, 5 patients were extubated within 24 h after the Yasui operation. Excluding a patient who required ECMO and another patient with multiple chromosomal disorder, the mean time to the extubation and the mean ICU stay were 1.9 ± 1.5 days (range, 0–5) and 5.8 ± 2.4 days (range, 3–10) in the staged group, and 15.4 ± 16.8 days (range, 3–39) and 30.4 ± 26.2 days (range, 16– 75) in the primary group, respectively. Inotropes were discontinued after 5.5 ± 3.6 days (range, 1–13) in the staged group and 12.2 ± 3.0 days (range, 8–15) in the primary group.
Echocardiogram The last postoperative cardiac echo study was performed in 16 patients at 5.6 ± 7.6 years after the Yasui operation. The left ventricular ejection fraction was 70.5 ± 12.1%, and the LVOT flow velocity was 1.2 ± 0.8 m/s (range, 0–3.9). The patient with an LVOT flow velocity of 3.9 m/s recurrently developed subaortic fibrous ridge after surgical resection in the reoperation as described above. The degree of regurgitation from the neoaortic valve (anatomical aortic valve or pulmonary valve) was absent in 11 patients, trivial in 5 patients, and mild in 1 patient. All survivors are in New York Heart Association Class I.
Reoperation Six patients underwent 8 reoperations, including 13 procedures (Table 3). Six RVOTRs (RV-PA conduit exchange in 5 patients and anterior monocusp patch in 1 patient) were performed 5.3 ± 2.5 years after the Yasui operation. Two procedures for recurrent LVOTO were performed. The first patient originally had a perimembranous outlet with inlet extension VSD with an accessory mitral valve. At the time of the Yasui operation, the accessory mitral valve was resected, and the VSD was enlarged, and a long intraventricular route using a PTFE patch was created. The patient developed recurrent LVOTO and mitral regurgitation and underwent redo-intraventricular baffling with concomitant mitral valve repair using PTFE artificial chordae at 28 months after the Yasui operation. The other patient developed discrete fibrous stenosis at the subaortic area and underwent resection of the subaortic fibrous tissue with VSD enlargement after 42 months. The first 2 patients who had an interposing PTFE graft between the ascending and descending aorta required
DISCUSSION In the presence of a VSD with two adequate-sized ventricles, biventricular repair is possible in patients with severe LVOTO and arch obstruction. The first report of a successful primary biventricular repair in 2 neonates with IAA and severe aortic stenosis was performed by Yasui at our institution in 1987 [1]. Since then, the concept of the Yasui operation (a combination of integrated aortic arch reconstruction, redirection of LV outflow through the VSD to both the semilunar valves and establishment of RV-PA continuity using a valved conduit) has been applied for patients with aortic atresia and VSD with normal left ventricle [2, 3, 6]. Several modifications have been made in terms of the surgical technique. Aortic arch reconstruction may be performed by proximal anastomosis of the pulmonary trunk and the ascending aorta by means of DKS connection with direct repair of the obstructed arch or Norwood-type arch reconstruction with or without the use of a homograft [2–6], depending on the individual anatomy. To
5
CONGENITAL
T. Nakano et al. / European Journal of Cardio-Thoracic Surgery
Figure 2: Freedom from reoperation. The freedom from reoperation for all causes was 71.3% at 5 years and 28.5% at 10 years (A). The freedom from reoperation other than exchange of the right ventricle to the pulmonary artery conduit was 81.5% at 5 years and 48.9% at 10 years (B).
establish RV-PA continuity, the Lecompte manoeuvre has been expected to reduce or delay the risk of reoperation in patients with a certain anatomy [6, 7]. The surgical results of the Yasui operation in a relatively small series of patients have been reported with satisfactory results. An early report by Ohye et al. [11] described their experience with 20 patients (11 underwent primary repair and 9 underwent staged repair) with a 5-year actuarial survival of 78%. Pearl et al. [12] reported the results of 8 patients (6 were staged with the initial Norwood operation) without early and late mortality. More recently, Kanter et al. [8] reported their experience of the Yasui operation in 21 patients (6 underwent primary repair and 15 underwent staged repair) with no early mortality, 3 late deaths and a 5-year actuarial survival of 75%. The result of the current study demonstrating an actuarial survival of 87.8% at 10 years is satisfactory for this group of patients with complex heart defects. The Yasui operation requires a valved conduit for RV-PA continuity; therefore, reoperation is frequently required. The Yasui operation is indicated in patients with significant aortic valvular stenosis or subaortic stenosis that is unable to accommodate systemic output without a pressure gradient in the presence of a normal left ventricle and VSD. In determining the indication for the Yasui operation in terms of the severity of LVOTO, we set the minimum LVOT diameter of body weight plus 1 mm or less. For example, an LVOT diameter of
