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Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous?
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Double Outlet right Ventricle with Non-Committed Ventricular Septal Defect and two Adequate Ventricles: is Anatomical Repair Advantageous? Olivier Villemain MD, Damien Bonnet MD, PhD, Lucile Houyel MD, Mathieu Vergnat MD, Magalie Ladouceur MD, Virginie Lambert MD, Zakaria Jalal MD, PhD, Pascal Vouhé MD, PhD, Emre Belli MD www.elsevier.com/locate/buildenv
PII: DOI: Reference:
S1043-0679(16)00024-1 http://dx.doi.org/10.1053/j.semtcvs.2016.01.007 YSTCS823
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Semin Thoracic Surg
Cite this article as: Olivier Villemain MD, Damien Bonnet MD, PhD, Lucile Houyel MD, Mathieu Vergnat MD, Magalie Ladouceur MD, Virginie Lambert MD, Zakaria Jalal MD, PhD, Pascal Vouhé MD, PhD, Emre Belli MD, Double Outlet right Ventricle with NonCommitted Ventricular Septal Defect and two Adequate Ventricles: is Anatomical Repair Advantageous?, Semin Thoracic Surg, http://dx.doi.org/10.1053/j.semtcvs.2016.01.007 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Double Outlet Right Ventricle with Non-Committed Ventricular Septal Defect and Two Adequate Ventricles: is Anatomical Repair Advantageous?
Authors : Olivier Villemain, MD1; Damien Bonnet, MD, PhD1; Lucile Houyel, MD2; Mathieu Vergnat, MD2; Magalie Ladouceur, MD1; Virginie Lambert, MD2 ; Zakaria Jalal, MD, PhD1; Pascal Vouhé, MD, PhD1; and Emre Belli, MD3.
1
M3C-Necker Enfants malades, Pediatric and Congenital Cardiology and Cardiac Surgery, Université Paris Descartes, Sorbonne Paris Cité, Paris, France 2 Centre Chirurgical Marie Lannelongue, M3C, Pediatric Cardiac Surgery, Université Paris Sud, Le Plessis Robinson, France 3 Institut Hospitalier Jacques Cartier. Pediatric and Congenital Cardiac Surgery, Massy, France
Corresponding Author : Emre Belli, MD Institut Hospitalier Jacques Cartier. Pediatric Cardiac Surgery, Massy, France Telephone: 00.33.1.60 13 46 56 Fax: 00.33.1.60 13 46 07
No potential conflicts of interest exist, for all authors No funding sources
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Abbreviations list
ASO: Arterial switch operation AV: Atrioventricular AVSD: Atrioventricular septal defect CPB: Cardiopulmonary bypass DORV: Double outlet right ventricle IVR: Intraventricular baffle repair ncVSD: non committed ventricular septal defect RVOT: Right ventricle outflow tract VSD: Ventricular septal defect
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Abstract Objective: The management of Double Outlet Right Ventricle (DORV) associated with anatomically non-committed Ventricular Septal Defect (NCVSD) constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical versus univentricular repair still remain to be established. Methods: Between 1993 and 2011, 36 consecutive patients presenting with DORV/NC-VSD (21 inlet, 10 muscular and 5 central perimembranous) and two adequately sized ventricles underwent surgical repair at two centers. Right ventricular outflow tract (RVOT) obstruction was present in 18/36 (50%). Twenty-one patients had undergone previous palliative procedures. Results: Anatomical repair (Group l) by means of intraventricular baffle construction was performed in 24 (associated RVOT reconstruction in 12 and Arterial Switch in 5) at a median age of 10.5 months. VSD was surgically enlarged in 12 (50%). The remaining 12 patients underwent univentricular repair (Group ll). There were 4 hospital deaths (11%), all in Group l (p=.30 versus group II). 8/20 group l survivors underwent 13 reoperations after a median delay of 24 months: subaortic stenosis was the main cause for reoperation in 6/8. There was one late death in group I and two late deaths in group II. The median follow-up was 5.6 years (CI95%; 0.2-9.8). Ten years actuarial survival rate and freedom from reoperation were respectively 74.7 ± 5% and 58 ± 5 % in Group l, and, 71 ± 7% and 70 ± 7% in Group ll. At last visit, all survivors were in NYHA class l-ll. Univariate analysis showed that AVSD and/or isolated mitral cleft were associated with death (p=.04) and need for reoperation (p=.038). Conclusion: Anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution. Associated AVSD and/or isolated mitral cleft were the only risk factors for mortality and reoperation.
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Perspective Statement: The surgical management of DORV with ncVSD constitutes a challenge. Between 1993 and 2011, 36 consecutive patients presenting with DORV/NC DORV/NC-VSD and two adequately sized ventricles underwent surgical repair at two centers. Anatomical repair in DORV with NCVSD should be considered with caution. caution Associated AVSD and/or isolated mitral cleft were the only risk factors for mortality and reoperation.
Central Picture: Overall survival (2A) and freedom of reoperation (2B) according to surgical strategy
Central Message: Anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution.
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INTRODUCTION The term Double Outlet Right Ventricle (DORV) is a type of ventriculo-arterial connection (or alignment) of the great arteries in which both great arteries arise entirely or predominantly from the right ventricle1,2. The classification according to the location of the ventricular septal defect (VSD) introduced by Lev and Bharati contributed significantly to the understanding of this malformation and helped the evolution of management strategies3. The term “noncommitted” (nc) was generally used to define the intra-cardiac anatomy where the VSD was anatomically far from both great arteries precluding the difficulties to properly redirect the blood flow of the left ventricle to the systemic or pulmonary circulation. The term “noncommitted” had been subject to debates and different definitions. On the other hand, DORV with subarterial VSD are nowadays routinely managed by anatomical repair strategy using surgical intra-cardiac baffle construction, however, the feasibility and the justification of anatomical repair in this rare subgroup of ncDORV is still under debate: the preference for univentricular palliation despite the presence of two adequate ventricles is not uncommon4. The purpose of the present study was, in light of recent anatomical description of the VSD, to precisely define the anatomy of ncDORV, to assess surgical strategies and outcomes in this specific subgroup of DORV.
METHODS Patients We reviewed all records of 498 consecutive patients presenting with DORV and considered for anatomical repair in two Parisian centers between 1993 and 2011. DORVs with ncVSD associated with univentricular heart or severely imbalanced ventricles precluding biventricular repair were excluded. The diagnosis of DORV with two adequate ventricles (or mild asymmetry not precluding biventricular consideration) was confirmed after review of echocardiography, MRI and/or CT-scan, angiography and surgical reports. If the exact type of DORV could not be defined with enough accuracy from the preoperative data, the anatomical description of the surgical report was considered as the final diagnosis. 5
Demographic data included gender, prenatal or postnatal diagnosis, center, and associated genetic anomalies. Three anatomical groups were defined according to the location of the VSD: 1- Inlet VSD, 2- Muscular VSD, 3- Central perimembranous VSD. Associated cardiac anomalies were noted: VSD [number, restriction], right and left outflow tracts obstruction, atrioventricular septal defect (ASVD), mitral valve cleft and other mitral valve anomalies, straddling of the mitral or of the tricuspid valve, and coronary artery anomalies. Two surgical strategies were used: 1- Anatomical repair by means of intraventricular baffle construction (group I), including: -
Intraventricular baffle
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Intraventricular baffle + arterial switch operation (ASO)
-
Intraventricular baffle + right ventricular outflow tract (RVOT) reconstruction (patch or conduit) and the Yasui procedure.
2- Univentricular palliative repair (group II), performed when biventricular anatomy was considered unattainable in light of both preoperative investigations and/or intraoperative evaluation. The univentricular palliative repair was acted at the end of the surgical program (with planned staged reoperation). Concomitant surgical procedures during anatomical repair were noted: VSD enlargement, atrioventricular valve repair, coarctation repair, subvalvular apparatus mobilization to divide or displace subvalvular structures precluding baffle construction, sub-aortic stenosis release and pulmonary artery branch stenosis repair. Surgical data included prior palliative intervention, age and weight at time of repair, surgical strategy, duration of cardiopulmonary bypass (CPB), duration of mechanical ventilation, reoperation and delay between repair and reoperation. Anatomical description
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Double outlet right ventricle was defined as a type of ventriculo-arterial connection in which both great arteries arise entirely or predominantly from the right ventricle2. According to Lev, the VSD in DORV can be defined as committed, when the blood coming from the left ventricle goes directly or preferentially in one or both great vessels, or non committed3. The definition of non-committed VSD has been the subject of many controversies. Some authors have advocated an anatomic distinction between “truly non committed VSD” (inlet or muscular, Figure 1) and “not directly committed VSD” (VSD opening in the outlet of the right ventricle, located between the two limbs of the septal band, but “remote” from the aorta and the pulmonary artery)5,6. To solve these ambiguities, we chose to include in this category all the VSD which are not located in the outlet of the right ventricle, between the two limbs of the septal band7. The blood flow through a non-committed VSD is therefore not directed towards the outflow tract, but directly towards the right ventricular cavity, running perpendicular to the ventricular septum. These VSD include inlet and muscular VSD, and also central perimembranous VSD, located below the posterior limb of the septal band, behind the septal leaflet of the tricuspid valve8. Statistical Analysis Continuous variables were presented as mean ± standard deviation or median with minimum and maximum range, and categorical variables were presented as percentage. Comparisons of categorical variables were made using chi-square test, or Fisher exact test when appropriate. Time to death and time to reoperation are shown in Kaplan–Meier curves. Univariate analyses of continuous variables were performed with the Student’s t-test (normal distribution) or Wilcoxon rank test (abnormal distribution). Univariate comparisons for categorical variables were performed with the two-tailed χ2 test or, when necessary (one or
more of the cells have an expected frequency of five or less), the Fisher’s exact test. Cox proportional hazard regression analysis, with the date of repair used as start date was performed as univariate analysis to investigate risk factors for early death, late death, early reoperation and late reoperation, when 5 or more events were associated with the variable tested, otherwise a logistic regression was used to determine the predicted value of the
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variable. All entered variables were selected on the basis of clinical experience, univariate analysis, and previously published data. The level of significance was set at an alpha level of 0.05 or less. Analysis was conducted using Medcalc (MedCalc Software, Mariakerke, Belgium). Finally, we collected as early mortality and reoperation that occurring less than 30 days after repair, and, as late mortality and reoperation occurring beyond that period.
RESULTS Population and Anatomic Characteristics Overall, 42 patients with DORV and ncVSD have been identified (42/498; 8,4%). Four patients with notable hypoplasia of the left ventricle were excluded. One patient died just after a previous palliative procedure (PA banding) and one patient died before surgery (associated genetic disorder). Finally, 36 DORV with ncVSD with two balanced ventricles constituted the study group. Their demographic and anatomical characteristics are summarized in Table 1. This study had been approved by our ethics board (Comité de Protection des Personnes – Ile-de-France VI) Surgical procedures The number and the type of prior palliative interventions according to the anatomical groups are shown in Table 2. The decision making process often included intraoperative evaluation of the cardiac anatomy in order to privilege the anatomical repair option as much as possible. Anatomical repair was performed in 24 patients (Group I) at a median age of 0.9 years (CI95%, 0.1-8.6). Seven patients underwent an intraventricular baffle, five patients underwent an intraventricular baffle + ASO, twelve patients underwent a intraventricular baffle + RVOT reconstruction (six RVOT enlargements, five Rastelli operations and one Yasui procedure9). For group I, 28 concomitant surgical procedures during anatomical repair were done: 7 enlargements of VSD, 6 atrioventricular valve repairs (2 atrioventricular valve repairs, 4 mitral cleft closures), 2 coarctation repairs, 6 chordae mobilizations, 5 resections of sub-aortic stenosis, and 2 pulmonary artery branch stenosis repairs. 8
Table 1. Patient characteristics Total cohort n=36
Group I (Anatomical) n=24
Group II (Univentricular) n=12
P-value
Demographic Characteristics Male/Female Center (Necker/Marie Lannelongue) Prenatal diagnosis Chromosomal/genetic abnormalities
19/17 12/24 10 (28%) 4 (11%)
13/11 6/18 8 (33%) 3 (13%)
6/6 6/6 2 (17%) 1 (8%)
1 0.26 0.43 1
Location of the VSD Inlet Muscular Perimembranous
21 (58%) 10 (28%) 5 (14%)
12 (50%) 8 (33%) 4 (17%)
9 (75%) 2 (17%) 1 (8%)
0.45 0.44 0.64
28 (77%) 3 (8%) 5 (14%) 9 (23%)
21 (87%) 2 (9%) 1 (4%) 7 (29%)
7 (58%) 1 (8%) 4 (33%) 2 (17%)
0.09 1 0.10 0.68
23 (64%) 3 (8%) 10 (28%) 18 (50%) 7 (19%) 12 (33%) 9 (25%) 3 (8%) 0 6 (17%) 8 (22%) 4 (11%) 4 (11%) 8 (23%) 1 (3%) 3 (9%) 5 (14%) 5 (14%)
18 (75%) 2 (8%) 4 (17%) 9 (38%) 3 (13%) 6 (25%) 7 (29%) 2 (8%) 0 5 (21%) 6 (25%) 2 (9%) 4 (17%) 7 (30%) 1 (4%) 2 (9%) 4 (17%) 5 (28%)
5 (42%) 1 (8%) 6 (50%) 9 (75%) 4 (33%) 5 (42%) 2 (17%) 1 (8%) 0 1 (8%) 2 (17%) 2 (17%) 0 1 (8%) 0 1 (8%) 1 (8%) 0
0.08 1 0.06 0.07 0.20 0.45 0.69 1 0.64 0.69 0.59 0.27 0.21 1 1 0.64 0.14
Variable
Anatomical characteristics VSD Number of VSD One Two Multiple Restrictive VSD Ventriculo-arterial spatial relationship D-Malposition L-Malposition Undetermined # RVOTO Pulmonary atresia Stenosis (Infundibular/Valvular/Supravulvular) Left Outflow Tract Obstruction Coarctation of the aorta Interrupted aortic arch Valvular or sub-valvular aortic stenosis Atrio-ventricular septal defect (ASVD) Complete Isolated mitral cleft Straddling Mitral Tricuspid Bivalvulvar Coronary artery anomalies
VSD: Ventricle septal defect; RVOTO: Right ventricle outflow tract obstruction; ASVD: Atrioseptal ventricular defect
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Table 2. Surgical Strategy and early outcomes Total cohort n=36 21 9 10 1 1
Group I (Anatomical) n=24 12 8 4 1 0
Group II (Univentricular) n=12 8 1 6 0 1
Median age at repair (years, [CI95%]) Median weight at repair (kg)
1.6 [0.1-11] 7.1 [2.6-21]
0.9 [0.1-8.6] 5 [2.6-21]
7.4 [0.5-16]† 15 [4.2-43]†
0.01 0.01
Major repair Duration of CPB (minutes, mean) Aortic clamping time (minutes, mean) Inotropic support (days, median [CI95%]) Duration of intubation (days, median [CI95%]) % Reoperation (< 30 days) % Mortality (< 30 days)
151±68 92±89 4 [0-13] 3.5 [0-20] 3% (1/36) 11% (4/36)
172±59 101±32 6 [1-13] 6.5 [2-20] 4% (1/24) 17% (4/24)
105±97 * 55±33 * 2 [0-4] 0 [0-5] 0 0
0.17 0.08 0.27 0.03 1 0.28
Previous palliative procedure PA banding Modified Blalock-Taussig shunt Atrial septectomy pAPVR repair
P-value 0.71 0.20 0.06 1 1
CPB: cardiopulmonary bypass; PA: pulmonary artery; pAPVR: partial anomalous pulmonary venous return † Concerning the Univentricular Group, the median age of the first stage of the repair was at 2.6 [0.5-7] years and the median weight was at 10 [4.2-19] kg. * 55% of univentricular repair were performed without aortic clamp and 21% without CPB
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Univentricular repair was performed in 12 patients (Group II), with eleven total cavopulmonary connections with extracardiac conduit (ten in two stages, one in one stage) and one with Puga’s operation. For group II, 8 concomitant surgical procedures during anatomical repair were done: 1 enlargement of VSD, 2 atrioventricular valve repairs, 1 coarctation repair, 1 sub-aortic stenosis release and 3 pulmonary artery branch stenosis repairs. The surgical data are summarized in Table 2. Overall survival and reoperation The median follow-up was 5.6 years (CI95%; 0.2-9.8). The overall early survival and freedom of reoperation rate were respectively 89% and 97%. The overall survival and freedom from reoperation rate were respectively 81.2% and 95% at five year, 73.1% and 63.9% at 10 years. Ten years actuarial survival rate and freedom from reoperation were respectively 74.7 ± 5% and 58 ± 5 % in Group l, and, 71 ± 7% and 70 ± 7% in Group ll. No significant statistical difference was found in overall mortality (p=0.57, Figure 2A) according to the surgical strategies (anatomical or univentricular repair). At ten years, reoperation was more frequent (Hazard Ratio [HR] with 95% Confidence Interval [CI95] = 5.62 [1.57-20.1], with p < 0.01 on the Logrank Test) in anatomical repair than in univentricular repair (Figure 2B). At last followup, 22/29 (76%) survivors were in NYHA functional class I, and 7/29 (24%) in NYHA functional class II. Univariate analysis showed that AVSD and/or isolated mitral cleft were associated with death (p=.04) and need for reoperation (p=.038). Anatomical repair (Group I) Early mortality or reoperation. 4/24 patients (16.6%) died within 30 days after repair. These four cases of early death are summarized in Table 3. The only risks factors associated with early mortality, in univariate regression logistic analysis, were the presence of an AVSD (p=0.035). One patient (1/24, 4%) needed a reoperation, for a residual VSD, ten days after surgery. It was an inlet VSD with mitral cleft and with RVOTO (pulmonary stenosis). This patient underwent initially an interventricular baffle + RVOT reconstruction (RVOT enlargement).
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Late mortality or reoperation. One patient died, 51 days after surgery. The VSD was perimembranous. This patient underwent initially an interventricular baffle + ASO. The coronary artery pattern was usual. The cause of his death was a sudden cardiac arrest. Eight/19 survivors (42%) underwent 13 reoperations at a median time interval of 2 years after repair (CI95 0.1-8.8 years). Five patients underwent only one reoperation, one patient underwent two reoperations and two patients underwent three reoperations. 6/8 patients (75%) have been reoperated for subaortic stenosis. The second main indication for reoperation was RVOTO in 3/8 patients (37%). Risk factors associated with reoperation in univariate analysis are shown in Table 4. The presence of associated ASVD was the only significant risk factor (p<0.01). Univentricular repair (Group II) Early mortality or reoperation. No early event was observed in this group of patients. Late mortality or reoperation. Two patients (2/12, 17%) died during follow-up. The first patient had an inlet VSD with RVOTO (infundibular and valvar pulmonary stenosis) in the setting of a complete ASVD, operated on at 3 months of life and had bidirectional cavo-pulmonary connection (associated with a TAPVC reparation). He died 68 days after surgery from low cardiac output. The second patient had an inlet restrictive VSD with pulmonary atresia and a Kartagener syndrome. He underwent a previous palliative procedure (a modified BlalockTaussig-Thomas shunt) and a total cavopulmonary connection at the age of 11 years. He was reoperated on 7.9 years later for severe aortic valve regurgitation (mechanical aortic valve replacement). He died 31 days after his reoperation. This patient was the only one late reoperation in group II.
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Table 3. Early death after anatomical repair (n=4) Case 1 Perimembranous
Case 2 Inlet
Case 3 Inlet
Case 4 Inlet
Coarctation of aorta
Complete ASVD
13
28
Complete ASVD Pulmonary stenosis 92
Partial ASVD Pulmonary atresia 1351
3.1
2.9
3.4
13
Unstable
Stable
Unstable
Stable
Intraventicular Baffle
Intraventicular Baffle
Intraventicular Baffle + RVOT reconstruction (Rastelli operation)
Coarctation repair, resection of subaortic stenosis
Atrioventricular valve repair
Intraventicular Baffle + RVOT reconstruction (RVOT enlargement) Atrioventricular valve repair
Delay of death
Intra-operative
Cause of death
Low output cardiac failure with multiple organ failure
Four days after surgery Low output cardiac failure
Location of the VSD Additional anatomical detail Age at repair (days) Weight at repair (kg) Hemodynamic situation before surgery Surgical strategy
Concomitant surgical procedure
Intra-operative Left heart failure after CPB
Atrioventricular valve repair + plasty of pulmonary arteries Two hours after surgery Obstruction under RV to PA conduit
ASVD: Atrioventricular septal defect; CPB: Cardiopulmonary bypass; RVOT: Right ventricle outflow tract; PA: Pulmonary artery
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Table 4. Late Risks Factors of reoperation for Anatomical repair (univariate analysis) P-value (risk of reoperation) Anatomical characteristics Location of VSD Two or multiple VSD Restrictive VSD RVOTO Left Outflow Tract Obstruction AVSD Straddling Coronary Artery Anomalies Surgical characteristics Previous palliative procedure Age at repair Weight at repair Type of surgical strategy CPB duration Duration of intubation
0.26 0.57 0.13 1 1 <0.01 0.62 1 0.34 0.18 0.82 0.17 0.23 0.11
VSD: Ventricle septal defect; ASVD: Atrioventricular septal defect; RVOT: Right ventricle outflow tract; CPB: Cardiopulmonary bypass;
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DISCUSSION The classification of the conotruncal anomalies under the term of DORV according to the location of the VSD had been commonly accepted and used in clinical practice. Despite the relative precision regarding subaortic, subpulmonary and doubly-committed location of the VSD, confusion still exists to define the ‘non-committed’ location of the VSD, the latter being often accorded to the subjective impression of the investigator and the surgeon5,7. In the past, we analyzed a series of patients presenting with DORV and proposed a definition according to the length of the sub-arterial conus, compared the indexed diameter of the aortic valve annulus6. This definition had the weakness of not being related to the specific elements of the infundibular anatomy. In the present study, in light of our recent work on the anatomical classification/definition of the VSD, we reviewed the investigations and reports in order to establish the selection criteria according to the specific anatomy of the VSD and its relation to the outlet of the right ventricle and the septal band8. Thus, many patients were excluded from the study despite their VSD being at a notable distance to the aorta and the pulmonary artery but still between the 2 limbs of the septal band. Using this anatomical criterion reduces the proportion of the ncDORV patients in our total cohort of DORV10 but has the advantage to define a more precise and more challenging sub-group with regard to the ambition for anatomical repair. The study group consisted in 36 consecutive patients presenting with 2 ventricles appropriately sized for anatomical repair. Anatomical repair was performed only in 24 and was abandoned in 12, sometimes intra-operatively. The conventional anatomical data did unfortunately not allow determining the feasibility of the anatomical repair in this subgroup of patients: this is often unpredictable and constitutes an additional clinical challenge. The study was also designed also to emphasize this particular character. The unique identified significant risk factor for hospital mortality was the presence of associated AVSD. Repair of AVSD and DORV has been published with encouraging results11. However, in presence of a ncVSD anatomy, the construction of a baffle to tunnel the left ventricle to the aorta is always technically challenging: enlargement options are limited because the absence of 15
interventricular septum between the VSD and the Aortic valve, and, the common AV valve anatomy can also preclude repair. On the other hand, in presence of RVOTO, the intracardiac baffle can amputate a significant amount of the right ventricular cavity and diminish functional right ventricular size. Although the common AV valve dysfunction can be concerning for late outcome, one can suggest that in the absence of any outlet component of the VSD, patients with AVSD and DORV with pure inlet VSD could be considered for univentricular palliation despite the presence of two functional ventricles12,13. In late 90’s there was an enthusiasm to push the limits for anatomical repair in cases with small ventricles, complex anatomy as well as very early ages. On the other hand, the patients stated in table 3 were unstable because of common AV valve dysfunction. The need for “open heart” surgery seemed to push to an early repair strategy. Nowadays, alternative management strategies are also accepted in similar challenging conditions. The VSD had to be enlarged in 50% of the patients. The VSD constitutes the outflow of the left ventricle and had to be non-restrictive to avoid immediate and prevent late subaortic obstruction. The anterior or antero-apical enlargement of the VSD was performed without any related complication. The palliative enlargement of the VSD either to decompress the left ventricle or to prepare to a subsequent anatomical repair was not performed in this cohort except in one case after neonatal PAB. In this patient, the planned anatomical repair was abandoned intraoperatively because of the length of the tunnel and the presence of a restrictive subaortic area: this patient benefited one year later from a Yasui type repair including baffle from the VSD to both great arteries, a modified Damus procedure and RV to PA conduit. For the remaining patients from group l, the early palliative enlargement of the VSD was not necessary or not performed because of earlier experiences resulting in ventricular dysfunction. One patient in the group II also benefited from LV decompression by surgical concomitant VSD enlargement. In five patients (example Figure 3) without initial pulmonary stenosis and presenting with ‘more or less’ normal or side-by-side great arteries, the inlet restrictive muscular VSD was enlarged and then tunneled to the subpulmonary infundibulum, the repair was then
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completed by an ASO14. All patients had uneventful outcome and follow-up. This complex type of repair for DORV ncVSD could only be performed in the presence of a well identified and developed conal (or infundibular) septum between the two great arteries and allowed to avoid univentricular strategy in these rare cases. If anatomically suitable, this alternative, although technically demanding, appeared to be the procedure of choice for DORV ncVSD without associated pulmonary stenosis and also AVSD. After anatomical repair, the development of subaortic stenosis is frequent. In this cohort, the anatomical repair was performed in the first year of life in the majority of patients. Despite statistically not significant, early anatomical repair seemed to be associated with higher risk for the development of subaortic stenosis in DORV patients15. However, the surgical management of subaortic stenosis in repaired DORV has became a standardized procedure and the performance of an extended septoplasty by double approach through aorta and right ventriculotomy seems to allow adequate release of obstruction and avoids subsequent reoperations for the same cause16.
CONCLUSION The term DORV includes a large spectrum of conotruncal anomalies and the presence of a ncVSD constitutes a rare but challenging subgroup. Surgical management options are various and the feasibility of anatomical repair often challenging despite the presence of two DORV adequate ventricles. The limited size of the cohort does not permit a non disputable statistical result. Associated AVSD seemed to be the principal pejorative lesion that influences outcome. In patients without pulmonary stenosis, rerouting the VSD to the PA associated with ASO gave encouraging results. In this challenging group of patients, anatomical repair should be considered with extreme caution. Further investigations are mandatory to improve outcome.
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Belli E, Serraf a, Lacour-Gayet F, et al. Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle. J Thorac Cardiovasc Surg. 1996;112(6):1570-78; discussion 1578-80.
Legends to the figures:
Figure 1: Double outlet right ventricle with non-committed muscular VSD. The probe passes through a mid-muscular VSD, just behind the septal band. The blood flow coming from the left ventricle is perpendicular to the ventricular septum and is not directed towards either great artery. The other probe passes through the aorta, which is right-sided and posterior. Ao, aorta; PA, pulmonary artery; SB, septal band; TV, tricuspid valve; VSD, ventricular septal defect.
Figure 2: Overall survival (Figure 2A) and freedom of reoperation (Figure 2B) according to surgical strategy.
Figure 3: Double outlet right ventricle with non-committed inlet restrictive VSD ()٭, Figure 3A. This patient underwent an intraventricular baffle with enlargement VSD and an arterial switch operation, Figure 3B. Evaluation after reparation AO: Aorta; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle.
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Double Outlet right Ventricle with Non-Committed Ventricular Septal Defect and two Adequate Ventricles: is Anatomical Repair Advantageous? Olivier Villemain MD, Damien Bonnet MD, PhD, Lucile Houyel MD, Mathieu Vergnat MD, Magalie Ladouceur MD, Virginie Lambert MD, Zakaria Jalal MD, PhD, Pascal Vouhé MD, PhD, Emre Belli MD www.elsevier.com/locate/buildenv
PII: DOI: Reference:
S1043-0679(16)00024-1 http://dx.doi.org/10.1053/j.semtcvs.2016.01.007 YSTCS823
To appear in:
Semin Thoracic Surg
Cite this article as: Olivier Villemain MD, Damien Bonnet MD, PhD, Lucile Houyel MD, Mathieu Vergnat MD, Magalie Ladouceur MD, Virginie Lambert MD, Zakaria Jalal MD, PhD, Pascal Vouhé MD, PhD, Emre Belli MD, Double Outlet right Ventricle with NonCommitted Ventricular Septal Defect and two Adequate Ventricles: is Anatomical Repair Advantageous?, Semin Thoracic Surg, http://dx.doi.org/10.1053/j.semtcvs.2016.01.007 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Double Outlet Right Ventricle with Non-Committed Ventricular Septal Defect and Two Adequate Ventricles: is Anatomical Repair Advantageous?
Authors : Olivier Villemain, MD1; Damien Bonnet, MD, PhD1; Lucile Houyel, MD2; Mathieu Vergnat, MD2; Magalie Ladouceur, MD1; Virginie Lambert, MD2 ; Zakaria Jalal, MD, PhD1; Pascal Vouhé, MD, PhD1; and Emre Belli, MD3.
1
M3C-Necker Enfants malades, Pediatric and Congenital Cardiology and Cardiac Surgery, Université Paris Descartes, Sorbonne Paris Cité, Paris, France 2 Centre Chirurgical Marie Lannelongue, M3C, Pediatric Cardiac Surgery, Université Paris Sud, Le Plessis Robinson, France 3 Institut Hospitalier Jacques Cartier. Pediatric and Congenital Cardiac Surgery, Massy, France
Corresponding Author : Emre Belli, MD Institut Hospitalier Jacques Cartier. Pediatric Cardiac Surgery, Massy, France Telephone: 00.33.1.60 13 46 56 Fax: 00.33.1.60 13 46 07
No potential conflicts of interest exist, for all authors No funding sources
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Abbreviations list
ASO: Arterial switch operation AV: Atrioventricular AVSD: Atrioventricular septal defect CPB: Cardiopulmonary bypass DORV: Double outlet right ventricle IVR: Intraventricular baffle repair ncVSD: non committed ventricular septal defect RVOT: Right ventricle outflow tract VSD: Ventricular septal defect
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Abstract Objective: The management of Double Outlet Right Ventricle (DORV) associated with anatomically non-committed Ventricular Septal Defect (NCVSD) constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical versus univentricular repair still remain to be established. Methods: Between 1993 and 2011, 36 consecutive patients presenting with DORV/NC-VSD (21 inlet, 10 muscular and 5 central perimembranous) and two adequately sized ventricles underwent surgical repair at two centers. Right ventricular outflow tract (RVOT) obstruction was present in 18/36 (50%). Twenty-one patients had undergone previous palliative procedures. Results: Anatomical repair (Group l) by means of intraventricular baffle construction was performed in 24 (associated RVOT reconstruction in 12 and Arterial Switch in 5) at a median age of 10.5 months. VSD was surgically enlarged in 12 (50%). The remaining 12 patients underwent univentricular repair (Group ll). There were 4 hospital deaths (11%), all in Group l (p=.30 versus group II). 8/20 group l survivors underwent 13 reoperations after a median delay of 24 months: subaortic stenosis was the main cause for reoperation in 6/8. There was one late death in group I and two late deaths in group II. The median follow-up was 5.6 years (CI95%; 0.2-9.8). Ten years actuarial survival rate and freedom from reoperation were respectively 74.7 ± 5% and 58 ± 5 % in Group l, and, 71 ± 7% and 70 ± 7% in Group ll. At last visit, all survivors were in NYHA class l-ll. Univariate analysis showed that AVSD and/or isolated mitral cleft were associated with death (p=.04) and need for reoperation (p=.038). Conclusion: Anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution. Associated AVSD and/or isolated mitral cleft were the only risk factors for mortality and reoperation.
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Perspective Statement: The surgical management of DORV with ncVSD constitutes a challenge. Between 1993 and 2011, 36 consecutive patients presenting with DORV/NC DORV/NC-VSD and two adequately sized ventricles underwent surgical repair at two centers. Anatomical repair in DORV with NCVSD should be considered with caution. caution Associated AVSD and/or isolated mitral cleft were the only risk factors for mortality and reoperation.
Central Picture: Overall survival (2A) and freedom of reoperation (2B) according to surgical strategy
Central Message: Anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution.
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INTRODUCTION The term Double Outlet Right Ventricle (DORV) is a type of ventriculo-arterial connection (or alignment) of the great arteries in which both great arteries arise entirely or predominantly from the right ventricle1,2. The classification according to the location of the ventricular septal defect (VSD) introduced by Lev and Bharati contributed significantly to the understanding of this malformation and helped the evolution of management strategies3. The term “noncommitted” (nc) was generally used to define the intra-cardiac anatomy where the VSD was anatomically far from both great arteries precluding the difficulties to properly redirect the blood flow of the left ventricle to the systemic or pulmonary circulation. The term “noncommitted” had been subject to debates and different definitions. On the other hand, DORV with subarterial VSD are nowadays routinely managed by anatomical repair strategy using surgical intra-cardiac baffle construction, however, the feasibility and the justification of anatomical repair in this rare subgroup of ncDORV is still under debate: the preference for univentricular palliation despite the presence of two adequate ventricles is not uncommon4. The purpose of the present study was, in light of recent anatomical description of the VSD, to precisely define the anatomy of ncDORV, to assess surgical strategies and outcomes in this specific subgroup of DORV.
METHODS Patients We reviewed all records of 498 consecutive patients presenting with DORV and considered for anatomical repair in two Parisian centers between 1993 and 2011. DORVs with ncVSD associated with univentricular heart or severely imbalanced ventricles precluding biventricular repair were excluded. The diagnosis of DORV with two adequate ventricles (or mild asymmetry not precluding biventricular consideration) was confirmed after review of echocardiography, MRI and/or CT-scan, angiography and surgical reports. If the exact type of DORV could not be defined with enough accuracy from the preoperative data, the anatomical description of the surgical report was considered as the final diagnosis. 5
Demographic data included gender, prenatal or postnatal diagnosis, center, and associated genetic anomalies. Three anatomical groups were defined according to the location of the VSD: 1- Inlet VSD, 2- Muscular VSD, 3- Central perimembranous VSD. Associated cardiac anomalies were noted: VSD [number, restriction], right and left outflow tracts obstruction, atrioventricular septal defect (ASVD), mitral valve cleft and other mitral valve anomalies, straddling of the mitral or of the tricuspid valve, and coronary artery anomalies. Two surgical strategies were used: 1- Anatomical repair by means of intraventricular baffle construction (group I), including: -
Intraventricular baffle
-
Intraventricular baffle + arterial switch operation (ASO)
-
Intraventricular baffle + right ventricular outflow tract (RVOT) reconstruction (patch or conduit) and the Yasui procedure.
2- Univentricular palliative repair (group II), performed when biventricular anatomy was considered unattainable in light of both preoperative investigations and/or intraoperative evaluation. The univentricular palliative repair was acted at the end of the surgical program (with planned staged reoperation). Concomitant surgical procedures during anatomical repair were noted: VSD enlargement, atrioventricular valve repair, coarctation repair, subvalvular apparatus mobilization to divide or displace subvalvular structures precluding baffle construction, sub-aortic stenosis release and pulmonary artery branch stenosis repair. Surgical data included prior palliative intervention, age and weight at time of repair, surgical strategy, duration of cardiopulmonary bypass (CPB), duration of mechanical ventilation, reoperation and delay between repair and reoperation. Anatomical description
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Double outlet right ventricle was defined as a type of ventriculo-arterial connection in which both great arteries arise entirely or predominantly from the right ventricle2. According to Lev, the VSD in DORV can be defined as committed, when the blood coming from the left ventricle goes directly or preferentially in one or both great vessels, or non committed3. The definition of non-committed VSD has been the subject of many controversies. Some authors have advocated an anatomic distinction between “truly non committed VSD” (inlet or muscular, Figure 1) and “not directly committed VSD” (VSD opening in the outlet of the right ventricle, located between the two limbs of the septal band, but “remote” from the aorta and the pulmonary artery)5,6. To solve these ambiguities, we chose to include in this category all the VSD which are not located in the outlet of the right ventricle, between the two limbs of the septal band7. The blood flow through a non-committed VSD is therefore not directed towards the outflow tract, but directly towards the right ventricular cavity, running perpendicular to the ventricular septum. These VSD include inlet and muscular VSD, and also central perimembranous VSD, located below the posterior limb of the septal band, behind the septal leaflet of the tricuspid valve8. Statistical Analysis Continuous variables were presented as mean ± standard deviation or median with minimum and maximum range, and categorical variables were presented as percentage. Comparisons of categorical variables were made using chi-square test, or Fisher exact test when appropriate. Time to death and time to reoperation are shown in Kaplan–Meier curves. Univariate analyses of continuous variables were performed with the Student’s t-test (normal distribution) or Wilcoxon rank test (abnormal distribution). Univariate comparisons for categorical variables were performed with the two-tailed χ2 test or, when necessary (one or
more of the cells have an expected frequency of five or less), the Fisher’s exact test. Cox proportional hazard regression analysis, with the date of repair used as start date was performed as univariate analysis to investigate risk factors for early death, late death, early reoperation and late reoperation, when 5 or more events were associated with the variable tested, otherwise a logistic regression was used to determine the predicted value of the
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variable. All entered variables were selected on the basis of clinical experience, univariate analysis, and previously published data. The level of significance was set at an alpha level of 0.05 or less. Analysis was conducted using Medcalc (MedCalc Software, Mariakerke, Belgium). Finally, we collected as early mortality and reoperation that occurring less than 30 days after repair, and, as late mortality and reoperation occurring beyond that period.
RESULTS Population and Anatomic Characteristics Overall, 42 patients with DORV and ncVSD have been identified (42/498; 8,4%). Four patients with notable hypoplasia of the left ventricle were excluded. One patient died just after a previous palliative procedure (PA banding) and one patient died before surgery (associated genetic disorder). Finally, 36 DORV with ncVSD with two balanced ventricles constituted the study group. Their demographic and anatomical characteristics are summarized in Table 1. This study had been approved by our ethics board (Comité de Protection des Personnes – Ile-de-France VI) Surgical procedures The number and the type of prior palliative interventions according to the anatomical groups are shown in Table 2. The decision making process often included intraoperative evaluation of the cardiac anatomy in order to privilege the anatomical repair option as much as possible. Anatomical repair was performed in 24 patients (Group I) at a median age of 0.9 years (CI95%, 0.1-8.6). Seven patients underwent an intraventricular baffle, five patients underwent an intraventricular baffle + ASO, twelve patients underwent a intraventricular baffle + RVOT reconstruction (six RVOT enlargements, five Rastelli operations and one Yasui procedure9). For group I, 28 concomitant surgical procedures during anatomical repair were done: 7 enlargements of VSD, 6 atrioventricular valve repairs (2 atrioventricular valve repairs, 4 mitral cleft closures), 2 coarctation repairs, 6 chordae mobilizations, 5 resections of sub-aortic stenosis, and 2 pulmonary artery branch stenosis repairs. 8
Table 1. Patient characteristics Total cohort n=36
Group I (Anatomical) n=24
Group II (Univentricular) n=12
P-value
Demographic Characteristics Male/Female Center (Necker/Marie Lannelongue) Prenatal diagnosis Chromosomal/genetic abnormalities
19/17 12/24 10 (28%) 4 (11%)
13/11 6/18 8 (33%) 3 (13%)
6/6 6/6 2 (17%) 1 (8%)
1 0.26 0.43 1
Location of the VSD Inlet Muscular Perimembranous
21 (58%) 10 (28%) 5 (14%)
12 (50%) 8 (33%) 4 (17%)
9 (75%) 2 (17%) 1 (8%)
0.45 0.44 0.64
28 (77%) 3 (8%) 5 (14%) 9 (23%)
21 (87%) 2 (9%) 1 (4%) 7 (29%)
7 (58%) 1 (8%) 4 (33%) 2 (17%)
0.09 1 0.10 0.68
23 (64%) 3 (8%) 10 (28%) 18 (50%) 7 (19%) 12 (33%) 9 (25%) 3 (8%) 0 6 (17%) 8 (22%) 4 (11%) 4 (11%) 8 (23%) 1 (3%) 3 (9%) 5 (14%) 5 (14%)
18 (75%) 2 (8%) 4 (17%) 9 (38%) 3 (13%) 6 (25%) 7 (29%) 2 (8%) 0 5 (21%) 6 (25%) 2 (9%) 4 (17%) 7 (30%) 1 (4%) 2 (9%) 4 (17%) 5 (28%)
5 (42%) 1 (8%) 6 (50%) 9 (75%) 4 (33%) 5 (42%) 2 (17%) 1 (8%) 0 1 (8%) 2 (17%) 2 (17%) 0 1 (8%) 0 1 (8%) 1 (8%) 0
0.08 1 0.06 0.07 0.20 0.45 0.69 1 0.64 0.69 0.59 0.27 0.21 1 1 0.64 0.14
Variable
Anatomical characteristics VSD Number of VSD One Two Multiple Restrictive VSD Ventriculo-arterial spatial relationship D-Malposition L-Malposition Undetermined # RVOTO Pulmonary atresia Stenosis (Infundibular/Valvular/Supravulvular) Left Outflow Tract Obstruction Coarctation of the aorta Interrupted aortic arch Valvular or sub-valvular aortic stenosis Atrio-ventricular septal defect (ASVD) Complete Isolated mitral cleft Straddling Mitral Tricuspid Bivalvulvar Coronary artery anomalies
VSD: Ventricle septal defect; RVOTO: Right ventricle outflow tract obstruction; ASVD: Atrioseptal ventricular defect
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Table 2. Surgical Strategy and early outcomes Total cohort n=36 21 9 10 1 1
Group I (Anatomical) n=24 12 8 4 1 0
Group II (Univentricular) n=12 8 1 6 0 1
Median age at repair (years, [CI95%]) Median weight at repair (kg)
1.6 [0.1-11] 7.1 [2.6-21]
0.9 [0.1-8.6] 5 [2.6-21]
7.4 [0.5-16]† 15 [4.2-43]†
0.01 0.01
Major repair Duration of CPB (minutes, mean) Aortic clamping time (minutes, mean) Inotropic support (days, median [CI95%]) Duration of intubation (days, median [CI95%]) % Reoperation (< 30 days) % Mortality (< 30 days)
151±68 92±89 4 [0-13] 3.5 [0-20] 3% (1/36) 11% (4/36)
172±59 101±32 6 [1-13] 6.5 [2-20] 4% (1/24) 17% (4/24)
105±97 * 55±33 * 2 [0-4] 0 [0-5] 0 0
0.17 0.08 0.27 0.03 1 0.28
Previous palliative procedure PA banding Modified Blalock-Taussig shunt Atrial septectomy pAPVR repair
P-value 0.71 0.20 0.06 1 1
CPB: cardiopulmonary bypass; PA: pulmonary artery; pAPVR: partial anomalous pulmonary venous return † Concerning the Univentricular Group, the median age of the first stage of the repair was at 2.6 [0.5-7] years and the median weight was at 10 [4.2-19] kg. * 55% of univentricular repair were performed without aortic clamp and 21% without CPB
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Univentricular repair was performed in 12 patients (Group II), with eleven total cavopulmonary connections with extracardiac conduit (ten in two stages, one in one stage) and one with Puga’s operation. For group II, 8 concomitant surgical procedures during anatomical repair were done: 1 enlargement of VSD, 2 atrioventricular valve repairs, 1 coarctation repair, 1 sub-aortic stenosis release and 3 pulmonary artery branch stenosis repairs. The surgical data are summarized in Table 2. Overall survival and reoperation The median follow-up was 5.6 years (CI95%; 0.2-9.8). The overall early survival and freedom of reoperation rate were respectively 89% and 97%. The overall survival and freedom from reoperation rate were respectively 81.2% and 95% at five year, 73.1% and 63.9% at 10 years. Ten years actuarial survival rate and freedom from reoperation were respectively 74.7 ± 5% and 58 ± 5 % in Group l, and, 71 ± 7% and 70 ± 7% in Group ll. No significant statistical difference was found in overall mortality (p=0.57, Figure 2A) according to the surgical strategies (anatomical or univentricular repair). At ten years, reoperation was more frequent (Hazard Ratio [HR] with 95% Confidence Interval [CI95] = 5.62 [1.57-20.1], with p < 0.01 on the Logrank Test) in anatomical repair than in univentricular repair (Figure 2B). At last followup, 22/29 (76%) survivors were in NYHA functional class I, and 7/29 (24%) in NYHA functional class II. Univariate analysis showed that AVSD and/or isolated mitral cleft were associated with death (p=.04) and need for reoperation (p=.038). Anatomical repair (Group I) Early mortality or reoperation. 4/24 patients (16.6%) died within 30 days after repair. These four cases of early death are summarized in Table 3. The only risks factors associated with early mortality, in univariate regression logistic analysis, were the presence of an AVSD (p=0.035). One patient (1/24, 4%) needed a reoperation, for a residual VSD, ten days after surgery. It was an inlet VSD with mitral cleft and with RVOTO (pulmonary stenosis). This patient underwent initially an interventricular baffle + RVOT reconstruction (RVOT enlargement).
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Late mortality or reoperation. One patient died, 51 days after surgery. The VSD was perimembranous. This patient underwent initially an interventricular baffle + ASO. The coronary artery pattern was usual. The cause of his death was a sudden cardiac arrest. Eight/19 survivors (42%) underwent 13 reoperations at a median time interval of 2 years after repair (CI95 0.1-8.8 years). Five patients underwent only one reoperation, one patient underwent two reoperations and two patients underwent three reoperations. 6/8 patients (75%) have been reoperated for subaortic stenosis. The second main indication for reoperation was RVOTO in 3/8 patients (37%). Risk factors associated with reoperation in univariate analysis are shown in Table 4. The presence of associated ASVD was the only significant risk factor (p<0.01). Univentricular repair (Group II) Early mortality or reoperation. No early event was observed in this group of patients. Late mortality or reoperation. Two patients (2/12, 17%) died during follow-up. The first patient had an inlet VSD with RVOTO (infundibular and valvar pulmonary stenosis) in the setting of a complete ASVD, operated on at 3 months of life and had bidirectional cavo-pulmonary connection (associated with a TAPVC reparation). He died 68 days after surgery from low cardiac output. The second patient had an inlet restrictive VSD with pulmonary atresia and a Kartagener syndrome. He underwent a previous palliative procedure (a modified BlalockTaussig-Thomas shunt) and a total cavopulmonary connection at the age of 11 years. He was reoperated on 7.9 years later for severe aortic valve regurgitation (mechanical aortic valve replacement). He died 31 days after his reoperation. This patient was the only one late reoperation in group II.
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Table 3. Early death after anatomical repair (n=4) Case 1 Perimembranous
Case 2 Inlet
Case 3 Inlet
Case 4 Inlet
Coarctation of aorta
Complete ASVD
13
28
Complete ASVD Pulmonary stenosis 92
Partial ASVD Pulmonary atresia 1351
3.1
2.9
3.4
13
Unstable
Stable
Unstable
Stable
Intraventicular Baffle
Intraventicular Baffle
Intraventicular Baffle + RVOT reconstruction (Rastelli operation)
Coarctation repair, resection of subaortic stenosis
Atrioventricular valve repair
Intraventicular Baffle + RVOT reconstruction (RVOT enlargement) Atrioventricular valve repair
Delay of death
Intra-operative
Cause of death
Low output cardiac failure with multiple organ failure
Four days after surgery Low output cardiac failure
Location of the VSD Additional anatomical detail Age at repair (days) Weight at repair (kg) Hemodynamic situation before surgery Surgical strategy
Concomitant surgical procedure
Intra-operative Left heart failure after CPB
Atrioventricular valve repair + plasty of pulmonary arteries Two hours after surgery Obstruction under RV to PA conduit
ASVD: Atrioventricular septal defect; CPB: Cardiopulmonary bypass; RVOT: Right ventricle outflow tract; PA: Pulmonary artery
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Table 4. Late Risks Factors of reoperation for Anatomical repair (univariate analysis) P-value (risk of reoperation) Anatomical characteristics Location of VSD Two or multiple VSD Restrictive VSD RVOTO Left Outflow Tract Obstruction AVSD Straddling Coronary Artery Anomalies Surgical characteristics Previous palliative procedure Age at repair Weight at repair Type of surgical strategy CPB duration Duration of intubation
0.26 0.57 0.13 1 1 <0.01 0.62 1 0.34 0.18 0.82 0.17 0.23 0.11
VSD: Ventricle septal defect; ASVD: Atrioventricular septal defect; RVOT: Right ventricle outflow tract; CPB: Cardiopulmonary bypass;
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DISCUSSION The classification of the conotruncal anomalies under the term of DORV according to the location of the VSD had been commonly accepted and used in clinical practice. Despite the relative precision regarding subaortic, subpulmonary and doubly-committed location of the VSD, confusion still exists to define the ‘non-committed’ location of the VSD, the latter being often accorded to the subjective impression of the investigator and the surgeon5,7. In the past, we analyzed a series of patients presenting with DORV and proposed a definition according to the length of the sub-arterial conus, compared the indexed diameter of the aortic valve annulus6. This definition had the weakness of not being related to the specific elements of the infundibular anatomy. In the present study, in light of our recent work on the anatomical classification/definition of the VSD, we reviewed the investigations and reports in order to establish the selection criteria according to the specific anatomy of the VSD and its relation to the outlet of the right ventricle and the septal band8. Thus, many patients were excluded from the study despite their VSD being at a notable distance to the aorta and the pulmonary artery but still between the 2 limbs of the septal band. Using this anatomical criterion reduces the proportion of the ncDORV patients in our total cohort of DORV10 but has the advantage to define a more precise and more challenging sub-group with regard to the ambition for anatomical repair. The study group consisted in 36 consecutive patients presenting with 2 ventricles appropriately sized for anatomical repair. Anatomical repair was performed only in 24 and was abandoned in 12, sometimes intra-operatively. The conventional anatomical data did unfortunately not allow determining the feasibility of the anatomical repair in this subgroup of patients: this is often unpredictable and constitutes an additional clinical challenge. The study was also designed also to emphasize this particular character. The unique identified significant risk factor for hospital mortality was the presence of associated AVSD. Repair of AVSD and DORV has been published with encouraging results11. However, in presence of a ncVSD anatomy, the construction of a baffle to tunnel the left ventricle to the aorta is always technically challenging: enlargement options are limited because the absence of 15
interventricular septum between the VSD and the Aortic valve, and, the common AV valve anatomy can also preclude repair. On the other hand, in presence of RVOTO, the intracardiac baffle can amputate a significant amount of the right ventricular cavity and diminish functional right ventricular size. Although the common AV valve dysfunction can be concerning for late outcome, one can suggest that in the absence of any outlet component of the VSD, patients with AVSD and DORV with pure inlet VSD could be considered for univentricular palliation despite the presence of two functional ventricles12,13. In late 90’s there was an enthusiasm to push the limits for anatomical repair in cases with small ventricles, complex anatomy as well as very early ages. On the other hand, the patients stated in table 3 were unstable because of common AV valve dysfunction. The need for “open heart” surgery seemed to push to an early repair strategy. Nowadays, alternative management strategies are also accepted in similar challenging conditions. The VSD had to be enlarged in 50% of the patients. The VSD constitutes the outflow of the left ventricle and had to be non-restrictive to avoid immediate and prevent late subaortic obstruction. The anterior or antero-apical enlargement of the VSD was performed without any related complication. The palliative enlargement of the VSD either to decompress the left ventricle or to prepare to a subsequent anatomical repair was not performed in this cohort except in one case after neonatal PAB. In this patient, the planned anatomical repair was abandoned intraoperatively because of the length of the tunnel and the presence of a restrictive subaortic area: this patient benefited one year later from a Yasui type repair including baffle from the VSD to both great arteries, a modified Damus procedure and RV to PA conduit. For the remaining patients from group l, the early palliative enlargement of the VSD was not necessary or not performed because of earlier experiences resulting in ventricular dysfunction. One patient in the group II also benefited from LV decompression by surgical concomitant VSD enlargement. In five patients (example Figure 3) without initial pulmonary stenosis and presenting with ‘more or less’ normal or side-by-side great arteries, the inlet restrictive muscular VSD was enlarged and then tunneled to the subpulmonary infundibulum, the repair was then
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completed by an ASO14. All patients had uneventful outcome and follow-up. This complex type of repair for DORV ncVSD could only be performed in the presence of a well identified and developed conal (or infundibular) septum between the two great arteries and allowed to avoid univentricular strategy in these rare cases. If anatomically suitable, this alternative, although technically demanding, appeared to be the procedure of choice for DORV ncVSD without associated pulmonary stenosis and also AVSD. After anatomical repair, the development of subaortic stenosis is frequent. In this cohort, the anatomical repair was performed in the first year of life in the majority of patients. Despite statistically not significant, early anatomical repair seemed to be associated with higher risk for the development of subaortic stenosis in DORV patients15. However, the surgical management of subaortic stenosis in repaired DORV has became a standardized procedure and the performance of an extended septoplasty by double approach through aorta and right ventriculotomy seems to allow adequate release of obstruction and avoids subsequent reoperations for the same cause16.
CONCLUSION The term DORV includes a large spectrum of conotruncal anomalies and the presence of a ncVSD constitutes a rare but challenging subgroup. Surgical management options are various and the feasibility of anatomical repair often challenging despite the presence of two DORV adequate ventricles. The limited size of the cohort does not permit a non disputable statistical result. Associated AVSD seemed to be the principal pejorative lesion that influences outcome. In patients without pulmonary stenosis, rerouting the VSD to the PA associated with ASO gave encouraging results. In this challenging group of patients, anatomical repair should be considered with extreme caution. Further investigations are mandatory to improve outcome.
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Lacour-Gayet F, Haun C, Ntalakoura K, et al. Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch. Eur J Cardiothorac Surg. 2002;21(6):1042-8.
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15.
Belli E, Serraf A, Lacour-Gayet F, et al. Biventricular repair for double-outlet right ventricle. Results and long-term follow-up. Circulation. 1998;98(19 Suppl):II360-5; discussion II365-7.
16.
Belli E, Serraf a, Lacour-Gayet F, et al. Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle. J Thorac Cardiovasc Surg. 1996;112(6):1570-78; discussion 1578-80.
Legends to the figures:
Figure 1: Double outlet right ventricle with non-committed muscular VSD. The probe passes through a mid-muscular VSD, just behind the septal band. The blood flow coming from the left ventricle is perpendicular to the ventricular septum and is not directed towards either great artery. The other probe passes through the aorta, which is right-sided and posterior. Ao, aorta; PA, pulmonary artery; SB, septal band; TV, tricuspid valve; VSD, ventricular septal defect.
Figure 2: Overall survival (Figure 2A) and freedom of reoperation (Figure 2B) according to surgical strategy.
Figure 3: Double outlet right ventricle with non-committed inlet restrictive VSD ()٭, Figure 3A. This patient underwent an intraventricular baffle with enlargement VSD and an arterial switch operation, Figure 3B. Evaluation after reparation AO: Aorta; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle.
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