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Fate of the Truncal Valve in Truncus Arteriosus
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PEDIATRIC CARDIAC SURGERY: The Annals of Thoracic Surgery CME Program is located online at http://cme.ctsnetjournals.org. To take the CME activity related to this article, you must have either an STS member or an individual non-member subscription to the journal.
Fate of the Truncal Valve in Truncus Arteriosus Roland Henaine, MD, Kasra Azarnoush, MD, Emre Belli, MD, André Capderou, MD, Régine Roussin, MD, Claude Planché, MD, and Alain Serraf, MD, PhD Department of Cardiac Surgery C, Cardiologic Hospital Louis Pradel, Lyon, and Departments of Congenital Heart Surgery and Physiology and Biostatistics, Marie-Lannelongue Hospital, Le Plessis-Robinson, France
Background. The fate of the truncal valve (TV) after truncus arteriosus repair remains poorly defined. The purpose of this report was to analyze how the TV influences outcome of truncus arteriosus repair. Methods. From January 1986 to December 2003, 153 patients underwent complete repair of the truncus arteriosus. Median age was 35 days. Preoperative TV insufficiency was absent or trivial in 59 patients (39%), mild in 72 patients (47%), moderate in 13 patients (8%), and severe in 9 patients (6%). The TV was quadricuspid in 36 patients (24 %), bicuspid in 10 patients (7%), and tricuspid in the remaining patients. At surgery, TV plasty (n ⴝ 6) or replacement (n ⴝ 3) was associated with truncus arteriosus repair. Truncal valve–aorta continuity was restored by patch interposition in 27 patients (17%), by conduit in 7 patients (5%), and directly in the remaining patients. Results. A 97-month mean follow-up was achieved in all survivors. The actuarial survival rates were 81.7% ⴞ
3.1% and 79.1% ⴞ 3.3% at 6 months and 18 years, respectively. Among 85 patients who underwent 113 reoperations, 19 underwent 24 TV reoperations: 5 isolated TV reinterventions and 19 associated with right ventricular–pulmonary artery conduit replacement. Freedom from TV reoperation was 96%, 82.3%, and 62.7% at 1, 10, and 18 years, respectively. Multivariate logistic regression showed that moderate or severe TV insufficiency at initial presentation was a risk factor for late TV reoperation but not for early mortality. Initial TV replacement was associated with lower survival. Conclusions. Initial TV insufficiency is associated with higher reoperation rate. This condition requires more refined techniques of TV plasty. The overall long-term freedom of TV reintervention rate remains within reasonable ranges. Truncal valve reintervention presents a low risk for mortality. (Ann Thorac Surg 2008;85:172– 8) © 2008 by The Society of Thoracic Surgeons
T
immediate-term and long-term evolution after TA repair remains poorly defined. The purpose of this report was to analyze how TV anatomy and dysfunction influence the management of TA and its outcome.
runcus arteriosus (TA) is a rare cardiovascular malformation that represents 2% to 4% of all congenital heart defects [1]. The common arterial trunk supplies systemic, coronary, and pulmonary blood flow and is thought to result from failure of proper conotruncal septation. Since the first successful TA repair in 1967 [2], major advances have been made in the management of TA, transforming it from a fatal condition early in life for more than 50% of patients to one in which neonatal repair is increasingly common and successful [3]. Associated lesions such as interrupted aortic arch (IAA), truncal valve insufficiency (TVI), and coronary artery anomalies remain major risk factors for short-term and long-term survival [4, 5]. Truncal valve (TV) dysfunction is frequently associated with TA. The impact of TV anatomy and function such as insufficiency or stenosis on Accepted for publication July 12, 2007. Address correspondence to Dr Henaine, Department of Cardiac Surgery C, Cardiologic Hospital Louis Pradel, Avenue du Doyen Lépine, Lyon, 69394, France; e-mail: [email protected].
© 2008 by The Society of Thoracic Surgeons Published by Elsevier Inc
Patients and Methods Patients Records from 153 patients who presented at MarieLannelongue Hospital between January 1986 and December 2003 with the diagnosis of TA were reviewed. The study was approved by the hospital investigation review board, and all the patients or their parents were informed of such an ongoing study. All patients underwent complete primary repair. There were 73 boys and 80 girls. Median age at intervention was 35 days (range, 3 to 495 days). Nineteen patients (12%) were operated on 4 months after birth or later as a result of late referral. Median weight at intervention was 3 kg (range, 1.86 to 12 kg). Before repair, all patients underwent complete 0003-4975/08/$34.00 doi:10.1016/j.athoracsur.2007.07.039
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Table 1. Indications, Operation, and Outcome of 9 Patients Who Underwent Initial Truncal Valve Procedure at Truncus Arteriosus Correction Indication Severe TVI Severe TVI Severe TVI ⫹ Stenosis TV grade III Severe TVI Severe TVI Severe TVI Severe TVI Severe TVI Severe TVI
Type of TV
TV Operation
Outcome
Quadricuspid Tricuspid Quadricuspid
AVR (pulmonary homograft) AVR (bioprothesis tube) AVR (pulmonary homograft)
Early death Early death Late AVR, late Bentall, late deatha
Quadricuspid Bicuspid Quadricuspid Tricuspid Quadricuspid Tricuspid
Valvuloplasty Commissuroplasty Valvuloplasty Commissuroplasty Valvuloplasty Commissuroplasty
Early AVR Early death Early AVR Actual moderate TVIb Actual trivial TVIc Early death
a 7 months after primary truncus arteriosus repair. repair.
AVR ⫽ aortic valve replacement;
b
10 years after primary truncus arteriosus repair.
TV ⫽ truncal valve;
c
4 years after primary truncus arteriosus
TVI ⫽ truncal valve insufficiency.
two-dimensional and Doppler echocardiography. Cardiac catheterization was performed in only 15 patients. Catheterization was generally used to quantify pulmonary vascular resistances in older patients, to evaluate nonconfluent pulmonary arteries when they were not well assessed by echocardiography, and to further quantify the degree of TVI in selected cases. Using the Van Praagh classification [6], 87 patients (57%) were A1, 38 patients (25%) were A2, 10 patients (6%) were A3, and 18 patients (12%) were A4. The most frequently associated cardiovascular lesions are IAA (n ⫽ 14), coronary anomalies (n ⫽ 13), multiple ventricular septal defect (n ⫽ 7), and aortic isthmic coarctation (n ⫽ 4). Truncal valve stenosis was graded from I to III according to the determined peak-to-peak gradient (I: ⱕ15 mm Hg; II: ⱕ30 mm Hg; III: ⬎30 mm Hg); according to these criteria, 84 patients (55%) presented with grade I TV stenosis, 45 patients (29%) with grade II, and 24 patients (16%) with grade III. Presence and quantification of TVI was evaluated by color Doppler imaging and graded as none or trivial, mild, moderate, and severe, depending on the ratio of the width of the regurgitant jet to the diameter of the high left outflow tract [7].This method was validated later in children after using it in this study [8]. Preoperative TVI was absent or trivial in 59 patients (39%), mild in 72 patients (47%), moderate in 13 patients (8%), and severe in 9 patients (6%). From an anatomic point of view, TV was quadricuspid in 36 patients (24 %), bicuspid in 10 patients (7%), and tricuspid in the remaining. In 91 patients (59%), the TV seemed macroscopically dysplastic, and was normal in the remaining patients.
Surgical Techniques Until 2000, operative techniques included aortobicaval cardiopulmonary bypass with systemic cooling to 28°C, left ventricular venting (through the right superior pulmonary vein), and antegrade cold-crystalloid cardioplegia. From 2000, surgery was conducted at normothermia with blood-potassium cardioplegia. Rein-
terventions for TV were performed as indicated by the hemodynamic data and the results of echocardiography and angiography. At the first procedure, after harvesting both pulmonary arteries from the aorta, a TV–aorta continuity was reestablished by direct anastomosis in 120 patients (78%), by patch interposition in 27 patients (17%), or a conduit in 7 patients (5%). Polytetrafluoroethylene, pulmonary homograft, and xenogenic or autologous pericardium were the patch materials used when direct suture was judged impossible. Among the 7 patients who required aortic conduit interposition, 4 had isolated conduit without a TV procedure (three polyethylene terephthalate fiber [Dacron] and one polytetrafluoroethylene tubes), and 3 had truncal root replacement (two pulmonary homografts and one valvular bioprosthesis conduit). The TV procedures included six valvoplasties and three truncal root replacements (the above 3 patients). As shown in Table 1, among 9 patients who underwent the TV procedure, all presented with severe TVI. In patients with moderate TVI, the TV was not addressed at surgery because it was thought that restoring a pulmonary blood flow to systemic blood flow ratio of 1 should reduce the degree of the TVI. Surgical techniques for valvuloplasties when the TV was quadricuspid consisted of attaching the edges of the prolapsed leaflet to adjacent leaflets using fine sutures, thereby creating a functional tricuspid semilunar valve. Commissuroplasty by traction of the TV commissure was applied in two cases. Indications for truncal root replacements were severe TVI with preoperative severe left heart failure when valvuloplasty was felt impossible because of very dysplastic valve in two cases, and associated severe grade III stenosis in on case.
Statistical Analysis Follow-up was obtained by direct patient contact or discussion with the referring cardiologist and was 100% complete. StatView software (SAS Institute Inc, Cary, NC) was used by our statistician (AC). Survival and
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value of less than 0.05 in univariate analysis were introduced in the logistic regression.
Results A median follow-up of 97 months (range, 13 to 246 months) was achieved in all survivors. The actuarial survival rates were 81.7% ⫾ 3.1%, 79.7% ⫾ 3.2%, and 79.1% ⫾ 3.3% at 6 months and 1 and 18 years, respectively (Fig 1). Global death involved 32 patients (odds ratio, 21%; 70% confidence limits, 17.3% to 24.8%), whereas hospital mortality was 26 patients (odds ratio, 17%; 70% confidence limits, 13.7% to 20.7%). The TV-related mortality involved 4 patients among the 26 hospital deaths and 2 patients among the 6 late deaths.
Early Truncal Valve Outcomes Fig 1. Actuarial cumulative survival curve. The actuarial survival rates were 81.7% ⫾ 3.1%, 79.7% ⫾ 3.2%, and 79.1% ⫾ 3.3% at 6 months and 1 and 18 years, respectively.
freedom from TV reintervention were analyzed according to the standard actuarial Kaplan–Meier method. The following variables were examined as potential risk factors for survival and late TV reintervention or actual severe TVI by means of both univariate and multivariate logistic regression: age at repair, sex, initial ascending aorta closure by patch or by conduit, TV dysplasia, initial TVI, initial TV stenosis, the number of TV cusps, initial TV plasty, initial TV replacement, and IAA. Analysis regarding potential risk factors for late TV reintervention or actual severe TVI was performed after excluding patients with early death after initial TA repair. When analysis showed statistical differences with a probability value of less than 0.05, odds ratio (parameter estimate) and confidence limits 70% or 95% were noted. 2 analysis and Fisher’s exact test were used to determine differences when variables were expressed by dichotomous values. Median values are reported with their minimal and maximal values. All the factors with a probability Fig 2. Flow chart showing the fate of the 24 late truncal valve (TV) reinterventions. (AVR ⫽ aortic valve replacement.)
Among the 9 patients who underwent TV plasty or replacement at initial procedure, there were 4 early deaths (Table 1). One death was attributable to accidental extubation in the intensive care unit; the 3 other deaths were subsequent to low cardiac output.
Late Truncal Valve Outcomes and Reinterventions Among the 85 patients who underwent 113 cardiac reoperations, 24 had TV reoperations with 2 early deaths (odds ratio, 8.3%; 70% confidence limits, 12.8% to 18.6%; Fig 2). The latter were associated with right ventricular– pulmonary artery conduit replacement for stenotic conduit in 19 cases and were isolated in 5 cases. The median delay between the first and second surgeries was 77 months (range, 1.6 to 169 months). Indications for TV operations in the presence of TVI were based on clinical tolerance and echocardiographic demonstration of left ventricular dilatation above normal ranges matched with age or left ventricular dysfunction [9]. In 19 patients, TV reoperation consisted of valve replacements; 3 patients received a valvuloplasty and 2 had Bentall procedures. The early death occurred in a patient who experienced severe TVI after TA repair and was associated with an early right ventricular–pulmonary artery conduit ob-
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(Table 1); valve replacement occurred 50 and 80 days after the first operation. The mean age at reoperation was 7.4 years (range, 3 months to 14 years). The median size of the inserted prosthesis was 23 mm diameter (range, 16 to 29). Causes for TV reinterventions were severe regurgitation in 21 patients (88%) and severe stenosis in 2 patients (8%), and 1 patient (4%) had severe TV disease. One-, 5-, 10-, and 18-year freedom rates from TV reoperation were 96%, 94.2%, 82.3%, and 62.7%, respectively (Fig 3). At last visit, 3 patients presented with severe TVI.
Risk Factors for Truncal Valve Reintervention or Late Severe Truncal Valve Insufficiency
Fig 3. Freedom from truncal valve (TV) reintervention after correction of truncus arteriosus. Freedom from truncal valve reoperation rates at 1, 5, 10, and 18 years were 96%, 94.2%, 82.3%, and 62.7%, respectively.
struction and severe left ventricular failure. Reintervention was performed 3 months later and included aortic valve replacement with a mechanical prosthesis, right ventricular–pulmonary artery conduit replacement, and left pulmonary artery patch enlargement, but the global heart failure led to death soon after surgery. The other death occurred after a Bentall procedure. This neonate underwent TA repair associated with truncal root replacement with a pulmonary homograft. Early failure of the homograft led to homograft replacement 3 months later. Endocarditis then developed on the second homograft, and he was reoperated on 6 weeks later and underwent a Bentall procedure. Unfortunately, sudden death occurred 7 months later, and postmortem examination revealed a valve thrombosis. Among the 19 valve replacements at reintervention, 2 patients previously had TV plasty for correction of TA
Table 2 summarizes statistical analysis with univariate and multivariate models. Multivariate logistic regression analysis showed that only initial moderate (p ⫽ 0.015) or severe TV (p ⫽ 0.017) was a risk factor for TV reoperation or late development of severe TVI. Age at TA surgery, sex, stenotic or dysplastic TV valve, and association with IAA, as well as initial TV replacement and aortic surgical technique (patch or conduit), did not influence TV reintervention or late severe TVI.
Truncal Valve and Mortality Statistical analysis revealed only initial TV replacement (p ⫽ 0.016) was risk a factor for global mortality in a multivariate logistic regression (Table 2). Conversely, age at TA surgery, sex, dysplasia, stenosis, quadricuspid form, initial mild or moderate or severe regurgitation, and IAA as well as initial repair of TV and initial aortic closure by patch did not influence mortality.
Comment This retrospective study encompasses a 20-year period with 6 different surgeons and a true evolution in the understanding of the pathophysiology of this defect.
Table 2. Univariate and Multivariate Logistic Regression for the Outcome Event of Late Truncal Valve Reoperation or Severe Regurgitation and for the Outcome Event of Global Death Late TV Reoperation or Severe TVI
Factors Analyzed
Events/Alive Patients at Risk
Initial moderate TVI
9/16
Initial severe TVI
5/8
Quadricuspidy Initial TV plasty
11/32 3/4
Initial TV replacement
Multivariate Odds Ratio (95% CL)
p ⫽ 0.017 4.8 (1.32–17.6) p ⫽ 0.0016 21.3 (3.2–142.3) p ⫽ 0.0124 3.3 (1.3–8.7) p ⫽ 0.0182 16.2 (1.6–164.7) p ⫽ 0.82
p ⫽ 0.015 6.7 (1.4–31.1) p ⫽ 0.017 14.7 (1.6–134) p ⫽ 0.96 p ⫽ 0.064
p ⫽ 0.71
Initial aortic conduit
CL ⫽ confidence limits;
Univariate Odds Ratio (95% CL)
TV ⫽ truncal valve;
Mortality Death/Patients at Risk
p ⫽ 0.98
5/10
p ⫽ 0.83
4/7
TVI ⫽ truncal valve insufficiency.
Univariate Odds Ratio (95% CL)
Multivariate Odds Ratio (95% CL)
p ⫽ 0.75
p ⫽ 0.82
p ⫽ 0.092
p ⫽ 0.097
p ⫽ 0.06
p ⫽ 0.07
p ⫽ 0.092
p ⫽ 0.192
p ⫽ 0.004 3.8 (1–14.5) p ⫽ 0.0186 6.6 (1.3–31.8)
p ⫽ 0.016 6.2 (1.4–31.1) p ⫽ 0.0692
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Initially, only patients with severe TVI were addressed for associated TV repair. All other patients with a lower degree of TVI were believed to have accommodated to this condition. Moreover, TVI is well tolerated in early infancy, and we often observed that it diminished after TA repair without valve intervention; therefore, our policy is to wait as long as possible without any TV procedure.
Initial Repair of Truncal Valve Although congenital aortic stenosis remains a troublesome problem, a number of interventions have been designed to provide relief. Unfortunately, procedures designed to provide a more competent systemic valve in the neonate are limited. The reproducibility of reparative techniques might be unpredictable, and prostheses are limited to small-caliber homografts. Some authors [10] suggest that TV repair should be the first option in case of significant TVI because it is effective and durable. Pigula and colleagues [11] described an external annuloplasty technique without the need for cardiopulmonary bypass, but their long-term outcome is unknown. A selected literature review of TV repair [12–16] reveals that a variety of techniques have been used, among them suture valvuloplasty, leaflet excision, commissural suspension, and remodeling techniques. The annulovalvuloplasty technique [14, 17] downsizes and remodels the annular configuration of the new TV without placing sutures in the valve leaflets; the resultant valve accepts the forward flow during systole and supports the aortic pressure during diastole without undue stress on the valvular suture lines, which are prone to disruption and other unwanted complications. The techniques described by Imamura and associates [14] and Mavroudis and Backer [17] seem interesting, but we do not have any experience with that procedure. The lack of consensus and reliable surgical technique as well as improvement of the valve function after repair and tolerance of TVI prompted us not to intervene in patients with moderate TVI at the time of repair. Although after analysis of our data, it appears that moderate TVI is a risk factor for late TV reintervention, we wonder whether delaying such an operation is not a safer option rather than an early unpredictable TV repair. This observation was also made by Watanabe and colleagues [18]. Indeed, preoperatively the TV is the single egress for systemic and pulmonary outputs, which may enhance a previous mild or moderate TVI. By normalization of the ratio of pulmonary blood flow to systemic blood flow after repair, one might expect reduction of the TVI. In addition, age was not an increased risk factor for reoperation after TV replacement. Although we are aware that small-size prostheses were inserted and most of these patients will have to undergo aortic root enlargement, by now none of them was scheduled for such a procedure.
Initial Truncal Valve Replacement Studies have reported good results with the aortic allograft in TV replacement [4, 19], but others have been
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disappointed with allografts in children, primarily with their intermediate-term function, and prefer to use an oversized mechanical valve along with annular enlargement [5]. McElhinney and coworkers [20] reported that severe allograft insufficiency developed less than a year after operation in 2 of their 3 early survivors with allograft TV replacement and the other survivor died suddenly 2 months after operation; other authors reported unfavorable short-term and long-term results with aortic homograft replacement [4, 13, 19]. In our series, 3 patients underwent initial TV replacement, with pulmonary homograft in 2 and a bioprosthetic conduit in 1, after failure to implant a mechanical bileaflet prosthesis. Two of them died early, and the reminder, 7 months after a third reoperation.
Freedom From Truncal Valve Reinterventions One-, 5-, 10-, and 18-year freedom rates from TV reoperation were 96%, 94.2%, 82.3%, and 62.7%, respectively (Fig 3). The main indication for TV reoperation was severe aortic regurgitation (88%); this observation was also found by others [21]. McElhinney and associates [20], in a similar study with 159 patients, present a freedom from TV reintervention of 83% and 75% at 10 and 18 years, respectively; they reported 22 reinterventions in 19 patients, similar to our report.
Risk Factors for Truncal Valve Reintervention or Late Severe Truncal Valve Insufficiency In our study, TVI, whether moderate or severe, quadricuspid TV, and initial TV plasty were risk factors for TV reoperation or late severe TVI in an univariate logistic regression analysis; however, in a multivariate logistic regression analysis, only initial moderate or severe TV was a risk factor for TV reoperation or late severe TVI. These results may be owing to the small population size of initial TV repair (n ⫽ 9) and because of the fact that quadricuspid TV and initial TV procedure are dependent variables whereas TVI is an independent variable. McElhinney and coworkers [20] found that the only independent variable to be associated significantly with shorter duration to TV reintervention was severe TVI at the time of the initial repair. Interrupted aortic arch, which accounts for 9% of associated lesions, as in other series [4, 22], was not a risk factor for late TVI as it was for other complex heart defects [23].
Risk Factor for Mortality Related to Truncal Valve In our study, significantly poorer survival was associated with initial TV replacement and initial aortic conduit interposition. Conversely, dysplasia, stenosis, quadricuspid form, initial TVI, and initial plasty of TV, as well as initial aortic closure by patch and IAA did not influence mortality. Multivariate stepwise logistic regression modeling revealed that initial TV replacement was the single risk factor for mortality. For many years, TVI was known as the most important factor contributing to early or late death [4, 10, 17, 20, 22] whether they underwent early repair or not [24]. Rajasinghe and associates [25] found that any degree of TVI before repair (mild, moderate, and
severe) was found to be a significant predictor of subsequent TV replacement; furthermore they found that moderate to severe TVI was an independent risk factor for poorer long-term survival. In our study, initial moderate and severe TVI was associated with late TV reinterventions but did not appear as a risk factor for mortality: this finding suggests that increasing experience with the surgical and perioperative management of patients with moderate and severe TVI has effectively improved their outcome. All patients with initial TV replacement died; the poor outcome of this group was similarly reported by others [5, 26]. Our current recommendation is that TV replacement and initial aortic conduit interposition must be avoided at repair if possible. Truncal valve replacement must be performed only when patients are not able to be weaned from cardiopulmonary bypass.
Weaknesses of the Study One of the shortcomings in the present study is that data regarding the morphology of the TV in our patients are limited to the perioperative surgical impression, and the definition of dysplasia was sometimes contradictory between preoperative echocardiography and operative findings. Another shortcoming is that our data regarding TV function early after repair are limited. This inadequacy is unfortunate, given the fact that preoperative TVI may not reflect accurately the postoperative situation. In unrepaired TA, pulmonary runoff leads to reduced diastolic pressure, which consequently may minimize the degree of TVI [1, 27]. Alternatively, a reduction in flow volume through the TV repair is likely to alter the distention of the truncal root, which can lead to either improved or worsened valve function. It has been observed that TVI often develops anew or progresses after TA repair without valve intervention [18, 27, 28], and that TVI can improve in the same circumstances [18]. Both these patterns were found to occur in our report. Finally, because the study started in 1986 with 6 surgeons performing interventions during the study period, the technique for TV initial repair was based on personal attitude and was time related; since 1998 we have been adopting a more consensual attitude: TV repair procedures are applied in patients with severe preoperative TVI and concordant anatomic findings. In other cases, the TV is left untouched even if anatomically abnormal and left to its natural history unless the patient is not able to be weaned from cardiopulmonary bypass because of TV insufficiency.
Conclusions Initial moderate or severe TVI was a risk factor for late TV reoperation or late severe TVI in a multivariate logistic regression analysis but not for early mortality. Initial TV replacement was associated with lower survival. More refined techniques of TV repair may be developed. The long-term freedom of TV reintervention is good. Truncal valve reintervention procedures can be performed with good early-term and mid-term results.
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We would like to acknowledge the assistance of Sophie Magat in the preparation of this manuscript.
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Silverman NH, Hanley FL. Trends in the management of truncal valve insufficiency. Ann Thorac Surg 1998;65:517– 24. Rajasinghe HA, McElhinney DB, Reddy VM, Mora BN, Hanley FL. Long-term follow-up of truncus arteriosus repaired in infancy: a twenty-year experience. J Thorac Cardiovasc Surg 1997;113:869 –78. Thompson LD, McElhinney DB, Reddy M, Petrossian E, Silverman NH, Hanley FL. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg 2001;72:391–5. Kirklin JW, Barratt-Boyes BG. Truncus arteriosus: In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery. New York: John Wiley & Sons, 1992:1131–51. Applebaum A, Bargeron LM, Pacifico AD, Kirklin JW. Surgical treatment of truncus arteriosus, with emphasis on infants and small children. J Thorac Cardiovasc Surg 1976;71:436 – 40.
INVITED COMMENTARY The management of truncus arteriosus has evolved, and results are now more consistent [1]. As has been well described, however, the primary approach to truncus arteriosus and to significant associated lesions, such as interrupted aortic arch and truncal valve insufficiency, remain the major risk factors for reoperation and mid to late survival rates. The initial management of truncal valve is a good example of the problems that arise when pediatric cardiac surgeons try to standardize therapy. The lack of consensus on timing and surgical techniques for treatment of moderate or severe truncal valve insufficiency impacts mid- and long-term outcomes. Nowadays, rational analysis of specific lesions and possible solutions is recommended. The following are influential lesions that may impact results: 1. Truncal valve morphology that includes dysplastic, dysfunctional, and an abnormal number of leaflets; conal septal hypoplasia; and valve prolapse due to the ventricle septal defect (VSD) should be considered. 2. Aortic arch obstruction and anomalous coronary anatomy are important associated lesions. 3. Morphology of the pulmonary arteries and their relationship to the aorta should be inspected. The following are some influential solutions that have contributed to improved results:
1. For moderate to severe truncal valve insufficiency, truncal valve repair is the most desirable and first choice. 2. Multiple leaflets (more than three) require careful attention. 3. Carefully consider the efficacy of different valvuloplasty alternatives. 4. Avoid valve replacement as a first option. 5. Prevent injury to truncal valve commissures during dissection of pulmonary arteries. 6. Try to construct an ascending aorta of uniform diameter, usually by using a direct anastomosis to reestablish truncal valve–aortic continuity. 7. Limit the incision distance of your right ventriculotomy through the truncal valve orifice. 8. Close the VSD with a large patch that is a larger size than the truncal valve orifice. 9. Assess the function of the truncal valve using an infusion of cardioplegia through the ascending aorta; view the valve from the right ventricle before finishing the VSD closure. 10. Take advantage of a perioperative transesophageal echocardiographic evaluation, and remember that truncus arteriosus is a challenging disease so repair. Juan V. Comas, MD, PhD
1. Use of direct anastomosis, patch interposition, or conduit to produce truncal valve/aorta continuity. 2. Improve truncal valve function by truncal valve replacement, commissuroplasty, external or internal annuloplasty, truncal valve reduction, creating a functional tricuspid semilunar valve, or attachment of edges of prolapsed leaflets. 3. VSD closure using appropriate right ventriculotomy and patch size.
Reference
I have found the following procedures and guidelines to be helpful:
1. Henaine R, Azarnoush K, Belli E, et al. Fate of the truncal valve in truncus arteriosus. Ann Thorac Surg 2008;85:172– 8.
© 2008 by The Society of Thoracic Surgeons Published by Elsevier Inc
Paediatric Heart Institute Hospital Universitario “12 de Octubre” Carretera de Andalucía km 5, 400, Edificio Materno-Infantil 28041 Madrid, Spain e-mail: [email protected]
0003-4975/08/$34.00 doi:10.1016/j.athoracsur.2007.10.086
PEDIATRIC CARDIAC SURGERY: The Annals of Thoracic Surgery CME Program is located online at http://cme.ctsnetjournals.org. To take the CME activity related to this article, you must have either an STS member or an individual non-member subscription to the journal.
Fate of the Truncal Valve in Truncus Arteriosus Roland Henaine, MD, Kasra Azarnoush, MD, Emre Belli, MD, André Capderou, MD, Régine Roussin, MD, Claude Planché, MD, and Alain Serraf, MD, PhD Department of Cardiac Surgery C, Cardiologic Hospital Louis Pradel, Lyon, and Departments of Congenital Heart Surgery and Physiology and Biostatistics, Marie-Lannelongue Hospital, Le Plessis-Robinson, France
Background. The fate of the truncal valve (TV) after truncus arteriosus repair remains poorly defined. The purpose of this report was to analyze how the TV influences outcome of truncus arteriosus repair. Methods. From January 1986 to December 2003, 153 patients underwent complete repair of the truncus arteriosus. Median age was 35 days. Preoperative TV insufficiency was absent or trivial in 59 patients (39%), mild in 72 patients (47%), moderate in 13 patients (8%), and severe in 9 patients (6%). The TV was quadricuspid in 36 patients (24 %), bicuspid in 10 patients (7%), and tricuspid in the remaining patients. At surgery, TV plasty (n ⴝ 6) or replacement (n ⴝ 3) was associated with truncus arteriosus repair. Truncal valve–aorta continuity was restored by patch interposition in 27 patients (17%), by conduit in 7 patients (5%), and directly in the remaining patients. Results. A 97-month mean follow-up was achieved in all survivors. The actuarial survival rates were 81.7% ⴞ
3.1% and 79.1% ⴞ 3.3% at 6 months and 18 years, respectively. Among 85 patients who underwent 113 reoperations, 19 underwent 24 TV reoperations: 5 isolated TV reinterventions and 19 associated with right ventricular–pulmonary artery conduit replacement. Freedom from TV reoperation was 96%, 82.3%, and 62.7% at 1, 10, and 18 years, respectively. Multivariate logistic regression showed that moderate or severe TV insufficiency at initial presentation was a risk factor for late TV reoperation but not for early mortality. Initial TV replacement was associated with lower survival. Conclusions. Initial TV insufficiency is associated with higher reoperation rate. This condition requires more refined techniques of TV plasty. The overall long-term freedom of TV reintervention rate remains within reasonable ranges. Truncal valve reintervention presents a low risk for mortality. (Ann Thorac Surg 2008;85:172– 8) © 2008 by The Society of Thoracic Surgeons
T
immediate-term and long-term evolution after TA repair remains poorly defined. The purpose of this report was to analyze how TV anatomy and dysfunction influence the management of TA and its outcome.
runcus arteriosus (TA) is a rare cardiovascular malformation that represents 2% to 4% of all congenital heart defects [1]. The common arterial trunk supplies systemic, coronary, and pulmonary blood flow and is thought to result from failure of proper conotruncal septation. Since the first successful TA repair in 1967 [2], major advances have been made in the management of TA, transforming it from a fatal condition early in life for more than 50% of patients to one in which neonatal repair is increasingly common and successful [3]. Associated lesions such as interrupted aortic arch (IAA), truncal valve insufficiency (TVI), and coronary artery anomalies remain major risk factors for short-term and long-term survival [4, 5]. Truncal valve (TV) dysfunction is frequently associated with TA. The impact of TV anatomy and function such as insufficiency or stenosis on Accepted for publication July 12, 2007. Address correspondence to Dr Henaine, Department of Cardiac Surgery C, Cardiologic Hospital Louis Pradel, Avenue du Doyen Lépine, Lyon, 69394, France; e-mail: [email protected].
© 2008 by The Society of Thoracic Surgeons Published by Elsevier Inc
Patients and Methods Patients Records from 153 patients who presented at MarieLannelongue Hospital between January 1986 and December 2003 with the diagnosis of TA were reviewed. The study was approved by the hospital investigation review board, and all the patients or their parents were informed of such an ongoing study. All patients underwent complete primary repair. There were 73 boys and 80 girls. Median age at intervention was 35 days (range, 3 to 495 days). Nineteen patients (12%) were operated on 4 months after birth or later as a result of late referral. Median weight at intervention was 3 kg (range, 1.86 to 12 kg). Before repair, all patients underwent complete 0003-4975/08/$34.00 doi:10.1016/j.athoracsur.2007.07.039
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Table 1. Indications, Operation, and Outcome of 9 Patients Who Underwent Initial Truncal Valve Procedure at Truncus Arteriosus Correction Indication Severe TVI Severe TVI Severe TVI ⫹ Stenosis TV grade III Severe TVI Severe TVI Severe TVI Severe TVI Severe TVI Severe TVI
Type of TV
TV Operation
Outcome
Quadricuspid Tricuspid Quadricuspid
AVR (pulmonary homograft) AVR (bioprothesis tube) AVR (pulmonary homograft)
Early death Early death Late AVR, late Bentall, late deatha
Quadricuspid Bicuspid Quadricuspid Tricuspid Quadricuspid Tricuspid
Valvuloplasty Commissuroplasty Valvuloplasty Commissuroplasty Valvuloplasty Commissuroplasty
Early AVR Early death Early AVR Actual moderate TVIb Actual trivial TVIc Early death
a 7 months after primary truncus arteriosus repair. repair.
AVR ⫽ aortic valve replacement;
b
10 years after primary truncus arteriosus repair.
TV ⫽ truncal valve;
c
4 years after primary truncus arteriosus
TVI ⫽ truncal valve insufficiency.
two-dimensional and Doppler echocardiography. Cardiac catheterization was performed in only 15 patients. Catheterization was generally used to quantify pulmonary vascular resistances in older patients, to evaluate nonconfluent pulmonary arteries when they were not well assessed by echocardiography, and to further quantify the degree of TVI in selected cases. Using the Van Praagh classification [6], 87 patients (57%) were A1, 38 patients (25%) were A2, 10 patients (6%) were A3, and 18 patients (12%) were A4. The most frequently associated cardiovascular lesions are IAA (n ⫽ 14), coronary anomalies (n ⫽ 13), multiple ventricular septal defect (n ⫽ 7), and aortic isthmic coarctation (n ⫽ 4). Truncal valve stenosis was graded from I to III according to the determined peak-to-peak gradient (I: ⱕ15 mm Hg; II: ⱕ30 mm Hg; III: ⬎30 mm Hg); according to these criteria, 84 patients (55%) presented with grade I TV stenosis, 45 patients (29%) with grade II, and 24 patients (16%) with grade III. Presence and quantification of TVI was evaluated by color Doppler imaging and graded as none or trivial, mild, moderate, and severe, depending on the ratio of the width of the regurgitant jet to the diameter of the high left outflow tract [7].This method was validated later in children after using it in this study [8]. Preoperative TVI was absent or trivial in 59 patients (39%), mild in 72 patients (47%), moderate in 13 patients (8%), and severe in 9 patients (6%). From an anatomic point of view, TV was quadricuspid in 36 patients (24 %), bicuspid in 10 patients (7%), and tricuspid in the remaining. In 91 patients (59%), the TV seemed macroscopically dysplastic, and was normal in the remaining patients.
Surgical Techniques Until 2000, operative techniques included aortobicaval cardiopulmonary bypass with systemic cooling to 28°C, left ventricular venting (through the right superior pulmonary vein), and antegrade cold-crystalloid cardioplegia. From 2000, surgery was conducted at normothermia with blood-potassium cardioplegia. Rein-
terventions for TV were performed as indicated by the hemodynamic data and the results of echocardiography and angiography. At the first procedure, after harvesting both pulmonary arteries from the aorta, a TV–aorta continuity was reestablished by direct anastomosis in 120 patients (78%), by patch interposition in 27 patients (17%), or a conduit in 7 patients (5%). Polytetrafluoroethylene, pulmonary homograft, and xenogenic or autologous pericardium were the patch materials used when direct suture was judged impossible. Among the 7 patients who required aortic conduit interposition, 4 had isolated conduit without a TV procedure (three polyethylene terephthalate fiber [Dacron] and one polytetrafluoroethylene tubes), and 3 had truncal root replacement (two pulmonary homografts and one valvular bioprosthesis conduit). The TV procedures included six valvoplasties and three truncal root replacements (the above 3 patients). As shown in Table 1, among 9 patients who underwent the TV procedure, all presented with severe TVI. In patients with moderate TVI, the TV was not addressed at surgery because it was thought that restoring a pulmonary blood flow to systemic blood flow ratio of 1 should reduce the degree of the TVI. Surgical techniques for valvuloplasties when the TV was quadricuspid consisted of attaching the edges of the prolapsed leaflet to adjacent leaflets using fine sutures, thereby creating a functional tricuspid semilunar valve. Commissuroplasty by traction of the TV commissure was applied in two cases. Indications for truncal root replacements were severe TVI with preoperative severe left heart failure when valvuloplasty was felt impossible because of very dysplastic valve in two cases, and associated severe grade III stenosis in on case.
Statistical Analysis Follow-up was obtained by direct patient contact or discussion with the referring cardiologist and was 100% complete. StatView software (SAS Institute Inc, Cary, NC) was used by our statistician (AC). Survival and
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value of less than 0.05 in univariate analysis were introduced in the logistic regression.
Results A median follow-up of 97 months (range, 13 to 246 months) was achieved in all survivors. The actuarial survival rates were 81.7% ⫾ 3.1%, 79.7% ⫾ 3.2%, and 79.1% ⫾ 3.3% at 6 months and 1 and 18 years, respectively (Fig 1). Global death involved 32 patients (odds ratio, 21%; 70% confidence limits, 17.3% to 24.8%), whereas hospital mortality was 26 patients (odds ratio, 17%; 70% confidence limits, 13.7% to 20.7%). The TV-related mortality involved 4 patients among the 26 hospital deaths and 2 patients among the 6 late deaths.
Early Truncal Valve Outcomes Fig 1. Actuarial cumulative survival curve. The actuarial survival rates were 81.7% ⫾ 3.1%, 79.7% ⫾ 3.2%, and 79.1% ⫾ 3.3% at 6 months and 1 and 18 years, respectively.
freedom from TV reintervention were analyzed according to the standard actuarial Kaplan–Meier method. The following variables were examined as potential risk factors for survival and late TV reintervention or actual severe TVI by means of both univariate and multivariate logistic regression: age at repair, sex, initial ascending aorta closure by patch or by conduit, TV dysplasia, initial TVI, initial TV stenosis, the number of TV cusps, initial TV plasty, initial TV replacement, and IAA. Analysis regarding potential risk factors for late TV reintervention or actual severe TVI was performed after excluding patients with early death after initial TA repair. When analysis showed statistical differences with a probability value of less than 0.05, odds ratio (parameter estimate) and confidence limits 70% or 95% were noted. 2 analysis and Fisher’s exact test were used to determine differences when variables were expressed by dichotomous values. Median values are reported with their minimal and maximal values. All the factors with a probability Fig 2. Flow chart showing the fate of the 24 late truncal valve (TV) reinterventions. (AVR ⫽ aortic valve replacement.)
Among the 9 patients who underwent TV plasty or replacement at initial procedure, there were 4 early deaths (Table 1). One death was attributable to accidental extubation in the intensive care unit; the 3 other deaths were subsequent to low cardiac output.
Late Truncal Valve Outcomes and Reinterventions Among the 85 patients who underwent 113 cardiac reoperations, 24 had TV reoperations with 2 early deaths (odds ratio, 8.3%; 70% confidence limits, 12.8% to 18.6%; Fig 2). The latter were associated with right ventricular– pulmonary artery conduit replacement for stenotic conduit in 19 cases and were isolated in 5 cases. The median delay between the first and second surgeries was 77 months (range, 1.6 to 169 months). Indications for TV operations in the presence of TVI were based on clinical tolerance and echocardiographic demonstration of left ventricular dilatation above normal ranges matched with age or left ventricular dysfunction [9]. In 19 patients, TV reoperation consisted of valve replacements; 3 patients received a valvuloplasty and 2 had Bentall procedures. The early death occurred in a patient who experienced severe TVI after TA repair and was associated with an early right ventricular–pulmonary artery conduit ob-
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(Table 1); valve replacement occurred 50 and 80 days after the first operation. The mean age at reoperation was 7.4 years (range, 3 months to 14 years). The median size of the inserted prosthesis was 23 mm diameter (range, 16 to 29). Causes for TV reinterventions were severe regurgitation in 21 patients (88%) and severe stenosis in 2 patients (8%), and 1 patient (4%) had severe TV disease. One-, 5-, 10-, and 18-year freedom rates from TV reoperation were 96%, 94.2%, 82.3%, and 62.7%, respectively (Fig 3). At last visit, 3 patients presented with severe TVI.
Risk Factors for Truncal Valve Reintervention or Late Severe Truncal Valve Insufficiency
Fig 3. Freedom from truncal valve (TV) reintervention after correction of truncus arteriosus. Freedom from truncal valve reoperation rates at 1, 5, 10, and 18 years were 96%, 94.2%, 82.3%, and 62.7%, respectively.
struction and severe left ventricular failure. Reintervention was performed 3 months later and included aortic valve replacement with a mechanical prosthesis, right ventricular–pulmonary artery conduit replacement, and left pulmonary artery patch enlargement, but the global heart failure led to death soon after surgery. The other death occurred after a Bentall procedure. This neonate underwent TA repair associated with truncal root replacement with a pulmonary homograft. Early failure of the homograft led to homograft replacement 3 months later. Endocarditis then developed on the second homograft, and he was reoperated on 6 weeks later and underwent a Bentall procedure. Unfortunately, sudden death occurred 7 months later, and postmortem examination revealed a valve thrombosis. Among the 19 valve replacements at reintervention, 2 patients previously had TV plasty for correction of TA
Table 2 summarizes statistical analysis with univariate and multivariate models. Multivariate logistic regression analysis showed that only initial moderate (p ⫽ 0.015) or severe TV (p ⫽ 0.017) was a risk factor for TV reoperation or late development of severe TVI. Age at TA surgery, sex, stenotic or dysplastic TV valve, and association with IAA, as well as initial TV replacement and aortic surgical technique (patch or conduit), did not influence TV reintervention or late severe TVI.
Truncal Valve and Mortality Statistical analysis revealed only initial TV replacement (p ⫽ 0.016) was risk a factor for global mortality in a multivariate logistic regression (Table 2). Conversely, age at TA surgery, sex, dysplasia, stenosis, quadricuspid form, initial mild or moderate or severe regurgitation, and IAA as well as initial repair of TV and initial aortic closure by patch did not influence mortality.
Comment This retrospective study encompasses a 20-year period with 6 different surgeons and a true evolution in the understanding of the pathophysiology of this defect.
Table 2. Univariate and Multivariate Logistic Regression for the Outcome Event of Late Truncal Valve Reoperation or Severe Regurgitation and for the Outcome Event of Global Death Late TV Reoperation or Severe TVI
Factors Analyzed
Events/Alive Patients at Risk
Initial moderate TVI
9/16
Initial severe TVI
5/8
Quadricuspidy Initial TV plasty
11/32 3/4
Initial TV replacement
Multivariate Odds Ratio (95% CL)
p ⫽ 0.017 4.8 (1.32–17.6) p ⫽ 0.0016 21.3 (3.2–142.3) p ⫽ 0.0124 3.3 (1.3–8.7) p ⫽ 0.0182 16.2 (1.6–164.7) p ⫽ 0.82
p ⫽ 0.015 6.7 (1.4–31.1) p ⫽ 0.017 14.7 (1.6–134) p ⫽ 0.96 p ⫽ 0.064
p ⫽ 0.71
Initial aortic conduit
CL ⫽ confidence limits;
Univariate Odds Ratio (95% CL)
TV ⫽ truncal valve;
Mortality Death/Patients at Risk
p ⫽ 0.98
5/10
p ⫽ 0.83
4/7
TVI ⫽ truncal valve insufficiency.
Univariate Odds Ratio (95% CL)
Multivariate Odds Ratio (95% CL)
p ⫽ 0.75
p ⫽ 0.82
p ⫽ 0.092
p ⫽ 0.097
p ⫽ 0.06
p ⫽ 0.07
p ⫽ 0.092
p ⫽ 0.192
p ⫽ 0.004 3.8 (1–14.5) p ⫽ 0.0186 6.6 (1.3–31.8)
p ⫽ 0.016 6.2 (1.4–31.1) p ⫽ 0.0692
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Initially, only patients with severe TVI were addressed for associated TV repair. All other patients with a lower degree of TVI were believed to have accommodated to this condition. Moreover, TVI is well tolerated in early infancy, and we often observed that it diminished after TA repair without valve intervention; therefore, our policy is to wait as long as possible without any TV procedure.
Initial Repair of Truncal Valve Although congenital aortic stenosis remains a troublesome problem, a number of interventions have been designed to provide relief. Unfortunately, procedures designed to provide a more competent systemic valve in the neonate are limited. The reproducibility of reparative techniques might be unpredictable, and prostheses are limited to small-caliber homografts. Some authors [10] suggest that TV repair should be the first option in case of significant TVI because it is effective and durable. Pigula and colleagues [11] described an external annuloplasty technique without the need for cardiopulmonary bypass, but their long-term outcome is unknown. A selected literature review of TV repair [12–16] reveals that a variety of techniques have been used, among them suture valvuloplasty, leaflet excision, commissural suspension, and remodeling techniques. The annulovalvuloplasty technique [14, 17] downsizes and remodels the annular configuration of the new TV without placing sutures in the valve leaflets; the resultant valve accepts the forward flow during systole and supports the aortic pressure during diastole without undue stress on the valvular suture lines, which are prone to disruption and other unwanted complications. The techniques described by Imamura and associates [14] and Mavroudis and Backer [17] seem interesting, but we do not have any experience with that procedure. The lack of consensus and reliable surgical technique as well as improvement of the valve function after repair and tolerance of TVI prompted us not to intervene in patients with moderate TVI at the time of repair. Although after analysis of our data, it appears that moderate TVI is a risk factor for late TV reintervention, we wonder whether delaying such an operation is not a safer option rather than an early unpredictable TV repair. This observation was also made by Watanabe and colleagues [18]. Indeed, preoperatively the TV is the single egress for systemic and pulmonary outputs, which may enhance a previous mild or moderate TVI. By normalization of the ratio of pulmonary blood flow to systemic blood flow after repair, one might expect reduction of the TVI. In addition, age was not an increased risk factor for reoperation after TV replacement. Although we are aware that small-size prostheses were inserted and most of these patients will have to undergo aortic root enlargement, by now none of them was scheduled for such a procedure.
Initial Truncal Valve Replacement Studies have reported good results with the aortic allograft in TV replacement [4, 19], but others have been
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disappointed with allografts in children, primarily with their intermediate-term function, and prefer to use an oversized mechanical valve along with annular enlargement [5]. McElhinney and coworkers [20] reported that severe allograft insufficiency developed less than a year after operation in 2 of their 3 early survivors with allograft TV replacement and the other survivor died suddenly 2 months after operation; other authors reported unfavorable short-term and long-term results with aortic homograft replacement [4, 13, 19]. In our series, 3 patients underwent initial TV replacement, with pulmonary homograft in 2 and a bioprosthetic conduit in 1, after failure to implant a mechanical bileaflet prosthesis. Two of them died early, and the reminder, 7 months after a third reoperation.
Freedom From Truncal Valve Reinterventions One-, 5-, 10-, and 18-year freedom rates from TV reoperation were 96%, 94.2%, 82.3%, and 62.7%, respectively (Fig 3). The main indication for TV reoperation was severe aortic regurgitation (88%); this observation was also found by others [21]. McElhinney and associates [20], in a similar study with 159 patients, present a freedom from TV reintervention of 83% and 75% at 10 and 18 years, respectively; they reported 22 reinterventions in 19 patients, similar to our report.
Risk Factors for Truncal Valve Reintervention or Late Severe Truncal Valve Insufficiency In our study, TVI, whether moderate or severe, quadricuspid TV, and initial TV plasty were risk factors for TV reoperation or late severe TVI in an univariate logistic regression analysis; however, in a multivariate logistic regression analysis, only initial moderate or severe TV was a risk factor for TV reoperation or late severe TVI. These results may be owing to the small population size of initial TV repair (n ⫽ 9) and because of the fact that quadricuspid TV and initial TV procedure are dependent variables whereas TVI is an independent variable. McElhinney and coworkers [20] found that the only independent variable to be associated significantly with shorter duration to TV reintervention was severe TVI at the time of the initial repair. Interrupted aortic arch, which accounts for 9% of associated lesions, as in other series [4, 22], was not a risk factor for late TVI as it was for other complex heart defects [23].
Risk Factor for Mortality Related to Truncal Valve In our study, significantly poorer survival was associated with initial TV replacement and initial aortic conduit interposition. Conversely, dysplasia, stenosis, quadricuspid form, initial TVI, and initial plasty of TV, as well as initial aortic closure by patch and IAA did not influence mortality. Multivariate stepwise logistic regression modeling revealed that initial TV replacement was the single risk factor for mortality. For many years, TVI was known as the most important factor contributing to early or late death [4, 10, 17, 20, 22] whether they underwent early repair or not [24]. Rajasinghe and associates [25] found that any degree of TVI before repair (mild, moderate, and
severe) was found to be a significant predictor of subsequent TV replacement; furthermore they found that moderate to severe TVI was an independent risk factor for poorer long-term survival. In our study, initial moderate and severe TVI was associated with late TV reinterventions but did not appear as a risk factor for mortality: this finding suggests that increasing experience with the surgical and perioperative management of patients with moderate and severe TVI has effectively improved their outcome. All patients with initial TV replacement died; the poor outcome of this group was similarly reported by others [5, 26]. Our current recommendation is that TV replacement and initial aortic conduit interposition must be avoided at repair if possible. Truncal valve replacement must be performed only when patients are not able to be weaned from cardiopulmonary bypass.
Weaknesses of the Study One of the shortcomings in the present study is that data regarding the morphology of the TV in our patients are limited to the perioperative surgical impression, and the definition of dysplasia was sometimes contradictory between preoperative echocardiography and operative findings. Another shortcoming is that our data regarding TV function early after repair are limited. This inadequacy is unfortunate, given the fact that preoperative TVI may not reflect accurately the postoperative situation. In unrepaired TA, pulmonary runoff leads to reduced diastolic pressure, which consequently may minimize the degree of TVI [1, 27]. Alternatively, a reduction in flow volume through the TV repair is likely to alter the distention of the truncal root, which can lead to either improved or worsened valve function. It has been observed that TVI often develops anew or progresses after TA repair without valve intervention [18, 27, 28], and that TVI can improve in the same circumstances [18]. Both these patterns were found to occur in our report. Finally, because the study started in 1986 with 6 surgeons performing interventions during the study period, the technique for TV initial repair was based on personal attitude and was time related; since 1998 we have been adopting a more consensual attitude: TV repair procedures are applied in patients with severe preoperative TVI and concordant anatomic findings. In other cases, the TV is left untouched even if anatomically abnormal and left to its natural history unless the patient is not able to be weaned from cardiopulmonary bypass because of TV insufficiency.
Conclusions Initial moderate or severe TVI was a risk factor for late TV reoperation or late severe TVI in a multivariate logistic regression analysis but not for early mortality. Initial TV replacement was associated with lower survival. More refined techniques of TV repair may be developed. The long-term freedom of TV reintervention is good. Truncal valve reintervention procedures can be performed with good early-term and mid-term results.
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We would like to acknowledge the assistance of Sophie Magat in the preparation of this manuscript.
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INVITED COMMENTARY The management of truncus arteriosus has evolved, and results are now more consistent [1]. As has been well described, however, the primary approach to truncus arteriosus and to significant associated lesions, such as interrupted aortic arch and truncal valve insufficiency, remain the major risk factors for reoperation and mid to late survival rates. The initial management of truncal valve is a good example of the problems that arise when pediatric cardiac surgeons try to standardize therapy. The lack of consensus on timing and surgical techniques for treatment of moderate or severe truncal valve insufficiency impacts mid- and long-term outcomes. Nowadays, rational analysis of specific lesions and possible solutions is recommended. The following are influential lesions that may impact results: 1. Truncal valve morphology that includes dysplastic, dysfunctional, and an abnormal number of leaflets; conal septal hypoplasia; and valve prolapse due to the ventricle septal defect (VSD) should be considered. 2. Aortic arch obstruction and anomalous coronary anatomy are important associated lesions. 3. Morphology of the pulmonary arteries and their relationship to the aorta should be inspected. The following are some influential solutions that have contributed to improved results:
1. For moderate to severe truncal valve insufficiency, truncal valve repair is the most desirable and first choice. 2. Multiple leaflets (more than three) require careful attention. 3. Carefully consider the efficacy of different valvuloplasty alternatives. 4. Avoid valve replacement as a first option. 5. Prevent injury to truncal valve commissures during dissection of pulmonary arteries. 6. Try to construct an ascending aorta of uniform diameter, usually by using a direct anastomosis to reestablish truncal valve–aortic continuity. 7. Limit the incision distance of your right ventriculotomy through the truncal valve orifice. 8. Close the VSD with a large patch that is a larger size than the truncal valve orifice. 9. Assess the function of the truncal valve using an infusion of cardioplegia through the ascending aorta; view the valve from the right ventricle before finishing the VSD closure. 10. Take advantage of a perioperative transesophageal echocardiographic evaluation, and remember that truncus arteriosus is a challenging disease so repair. Juan V. Comas, MD, PhD
1. Use of direct anastomosis, patch interposition, or conduit to produce truncal valve/aorta continuity. 2. Improve truncal valve function by truncal valve replacement, commissuroplasty, external or internal annuloplasty, truncal valve reduction, creating a functional tricuspid semilunar valve, or attachment of edges of prolapsed leaflets. 3. VSD closure using appropriate right ventriculotomy and patch size.
Reference
I have found the following procedures and guidelines to be helpful:
1. Henaine R, Azarnoush K, Belli E, et al. Fate of the truncal valve in truncus arteriosus. Ann Thorac Surg 2008;85:172– 8.
© 2008 by The Society of Thoracic Surgeons Published by Elsevier Inc
Paediatric Heart Institute Hospital Universitario “12 de Octubre” Carretera de Andalucía km 5, 400, Edificio Materno-Infantil 28041 Madrid, Spain e-mail: [email protected]
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