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Ann Thorac Surg 1998;65:1737-1740
© 1998 The Society of Thoracic Surgeons

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Original articles: cardiovascular

Truncal Valve Repair: Initial Experience in Neonates

^a Division of Cardiovascular Surgery, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada
^b Division of Cardiology, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada

Accepted for publication November 19, 1997.

Address reprint requests to Dr Black, Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave, Toronto, Ont, Canada M5G 1X8
e-mail: (michael.black{at}mailhub.sickkids.on.ca)

	Abstract

Top Abstract Introduction Material and methods Results Comment References

 
Background. The identification of moderate to severe preoperative truncal valve regurgitation has been synonymous with significant postoperative mortality after neonatal repair of truncus arteriosus. Spurned by the deficiencies of current truncal valve substitutes surgeons are once again reexamining the option of truncal valve reparative techniques.

Methods. From May 1996 until June 1997, 8 children underwent correction of truncus arteriosus. A retrospective analysis was conducted.

Results. There was one in-hospital death secondary to a postoperative massive coronary air embolism. Moderate-to-severe truncal valve regurgitation was identified clinically and confirmed with cardiac ultrasound in 3 neonates with a mean age of 7 days (range, 4 to 12 days) all with quadracusp truncal valves. Successful truncal valve repair was accomplished in 2 infants, with a third neonate requiring homograft replacement with coronary reimplantation for failure in achieving valvular competence after attempted valvuloplasty. Postoperative echocardiograms in those neonates who underwent truncal valve repair confirmed a functional "tricuspid" valve with only mild to mild-plus regurgitation.

Conclusions. We give further credence to the hypothesis that primary neonatal truncal valve repair is feasible and may be successful in the avoidance and delay of serial truncal valve replacements using either mechanical or allograft substitutes.

	Introduction

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Truncus arteriosus communis is an uncommon congenital heart defect occurring in approximately 0.04 of 1,000 live births [1]. The initial presentation remains variable but mild cyanosis and tachypnea remain common signs and symptoms due to the obligate pulmonary blood flow through the common arterial trunk and ventricular septal defect. Severe congestive heart failure develops in time, simultaneous with the inevitable decrease in pulmonary vascular resistance.

Truncal valve regurgitation is estimated to occur in 25% to 50% of those afflicted with this condition [1, 2]. In those neonates with moderate-to-severe truncal valve regurgitation, urgent resuscitation and surgical stabilization are to be expected. A double-homograft repair with coronary artery reimplantation may be required [3], although as an alternative, mechanical valve implantation has been implemented successfully. Both of these latter options condemn the neonate to multiple further operations with the additional burden of the risks of reoperation, eventual valve failure and anticoagulation, and prosthetic valve endocarditis [4]. Moderate-to-severe truncal valve regurgitation places a tremendous burden on the fragile myocardium and neonates must be managed aggressively with intubation, ventilation, paralysis, and urgent surgical consultation and intervention. The mortality in this subgroup usually exceeds rates found in those with "simple" truncus due to the extreme but usually underestimated persistent preoperative cardiac ischemia (combination of low diastolic pressures and increased ventricular wall tension secondary to the large volume loading) placed on the myocardium.

	Material and methods

Top Abstract Introduction Material and methods Results Comment References

 
Between May 1996 and June 1997, 8 children underwent repair of truncus arteriosus communis at the Hospital for Sick Children, Toronto. Three neonates were identified as having significant preoperative truncal valve regurgitation, suitable for truncal valve repair (Table 1). Only 1 of the 3 neonates required replacement of the truncal valve because of persistent postrepair truncal valve regurgitation. Two neonates underwent successful repair by conversion of their quadracuspid truncal valves into a functional trileaflet structure without the need for replacement. All truncal valvuloplasties were performed simultaneously with the complete repair using the techniques described below.

The quadracuspid valves commonly appear extremely dysplastic but retain two well-developed commissures. The posterior leaflet in both neonates that underwent successful repair was identified as the nidus for the significant regurgitation. Approximation of the incomplete commissure (approximately 30% commissural fusion) with resuspension of the true commissure successfully converted the quadracusp regurgitant valve into a functional trileaflet valve (Figs 1, 2). The reparative techniques used are similar to those described by Elami and colleagues [5].

View larger version (102K): [in this window] [in a new window]   Fig 1. Preoperative echocardiogram demonstrating quadracuspid truncal valve. Each sinus is numbered (1 to 4). Chambers are as labeled.

View larger version (84K): [in this window] [in a new window]   Fig 2. Postoperative echocardiogram demonstrating "tricuspid" truncal valve. Each sinus is numbered (1 to 3).

After direct intraventricular valve inspection further evaluation of aortic valve (truncal valve) may be accomplished by (1) the estimation of the amount of left ventricular vent return (by maintaining a steady level within the cardiotomy reservoir) as a percentage of the total cardiopulmonary bypass flow; (2) continuous monitoring of left atrial pressures with the direct observation for subsequent ventricular distention; and (3) intraoperative transesophageal echocardiography.

Echocardiography: intraoperative transesophageal echocardiography
A comparison of intraoperative transesophageal echocardiography with preoperative transthoracic views has been found helpful in the determination of the degree of truncal valve competence.

We evaluate intraoperative truncal valve function by transesophageal echocardiography imaging with color pulsed and continuous wave Doppler echocardiography. Short- and long-axis views provide excellent visualization of leaflet mobility, coaptation, and morphology. Five- and two-chamber views allow semiquantification of truncal valve regurgitation based on the jet width at the valve (truncal) level. The pressure half-time of regurgitation jets and truncal valve gradients are obtained from transgastric views.

	Results

Top Abstract Introduction Material and methods Results Comment References

 
All 3 neonates survived their surgical repair and were discharged home. Postoperative echocardiograms were extensively reviewed to assess postoperative valve function. Mild postoperative regurgitation was identified in all children. Importantly patients 2 and 3 had only a small central jet of regurgitation through the newly created trileaflet structure. There did not appear to be significant truncal valve stenosis after truncal valve repair.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
It has become apparent that long-term survival after truncal valve replacement as a neonate remains poor [2]. Truncal valve regurgitation is not infrequent to some degree in those who present for operation [1,2]. The previous lack of enthusiasm intertwined with recent statements of extreme discretion has perhaps delayed the inevitable refinement of neonatal truncal valve reparative techniques [5,6]. Alternatives for significant truncal valve regurgitation (1) delayed truncal valve operation (medical therapy), (2) mechanical replacement, and (3) allograft replacement, remain suboptimal [2,3].

The implantation of mechanical valves or allografts carries a high rate of failure within the first 5 years. Data from both our past institutional experience and recent literature demonstrates poor long-term results after truncal valve replacement with either mechanical or allograft substitutes. McElhinney and colleagues [4] report freedom from intervention ranging from 48% to 17% at 5 years, depending on the severity of initial truncal valve regurgitation. During the 15 years before 1996 at the Hospital for Sick all children operated on with severe truncal valve regurgitation (n = 5) died in the early postoperative period. Only 2 children underwent truncal valve replacement during their initial operation. A third child initially repaired at 4 months of age had her dysfunctional truncal valve replaced during the same admission (5 months) because of persistent valve regurgitation. She continues to do well after her second prosthetic valve replacement and is currently 9 years of age.

Of equal and perhaps more contemporary concern, remains the dismal allograft root replacement results with only 25% surviving to a follow-up period of 13 months [4,5]. Our 1 survivor with allograft root replacement remains at high risk for future valve failure and death and will be followed closely.

Spurned by the deficiencies of the current truncal valve substitutes and motivated by improved techniques of semilunar valvuloplasty surgeons are once again reexamining the options of truncal valve reparative techniques [5]. The extrapolation of successful aortic valve repair from the adult population has launched preliminary successful results in the repair of the pediatric truncal valve.

The probability of successful truncal valvuloplasty is likely intimately related to the number of valve leaflets (68% to 69% tricuspid, 22% to 25% quadricuspid, and 7% to 9% bicuspid) and to the presence of incomplete division of valve cusps (trileaflet valves, 2.5%; quadricuspid valves, 20%) [7–11]. It is not uncommon for bicuspid and tricuspid valves to represent a form fruste quadricuspid valves because of the presence of raphes [8]. Bicuspid and quadricuspid valves are more likely to be thickened and nodular than their tricuspid counterparts [9]. Thickening and unequal size of valve cusps are recurrent characteristics of incompetent truncal valves. Histologically there is irregular expansion of the spongiosa and fibrosa layers, with focal fibroelastic thickening of the ventricularis and arterialis layers [8]. There remains no correlation between the extent of valve thickening and age of the child [9].

Truncal valvuloplasty is likely most productive with quadracuspid valves because (1) successful conversion to a trileaflet valve is possible without significant postoperative stenosis, unlike the reciprocal conversion of a trileaflet to a bicuspid valve; and (2) quadracuspid valves are more inclined to be dysplastic initially and thus have the greatest incidence of significant preoperative regurgitation (ie, they are the valves that require repair). An oxymoron perhaps, a repair that initially appears most futile is most likely to succeed! Fortunately only 7% of truncal valves are bicuspid [9]. It is suspected that valvuloplasty in the latter group would lead to significant postrepair stenosis. Tricuspid truncal valves are least likely dysplastic and as such are least likely to require surgical repair. Exceptions to the incidence of actual trileaflet valve dysfunction, however, have been described [8].

We hope that our preliminary results will encourage surgeons to at least attempt neonatal truncal valve repair. The reparative techniques can be instituted quickly without significant prolongation of myocardial ischemia and immediately checked (through the ventriculotomy).

The 2 neonates who had conversion of their quadracuspid valves to trileaflet structures are currently managed on afterload reducing agents with only mild to mild-plus truncal valve regurgitation. The nodular valvular thickening and fibrosis are believed to be the result, rather than the cause of chronic valvular regurgitation [8]. It may, therefore, be possible that over time, progressive remodeling of the dysmorphic valve may take place with the eventual delay in truncal valve replacement.

	References

Top Abstract Introduction Material and methods Results Comment References

 

1. Freedom R.M. Anomalies of aortopulmonary septation: persistent truncus arteriosus, aortopulmonary septal defect, and hemitruncus arteriosus. In: Freedom R.M., Benson L.N., Smallhorn J.F., eds. Neonatal heart disease. London: Springer-Verlag, 1992:428-452.
2. De Leval M.R., McGoon D.C., Wallace R.B., Danielson G.K., Mair D.D. Management of truncal valvular regurgitation. Ann Surg 1974;180:427-432.[Medline]
3. Elkins R.C., Steinberg J.B., Razook J.D., Ward K.E., Overholt E.D., Thompson W.E., Jr Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts. Ann Thorac Surg 1990;50:728-733.[Abstract/Free Full Text]
4. McElhinney D.B., Reddy V.M., Rajasinghe H.A., Mora B.N., Silverman N.H., Hanley F.L. Trends in the management of truncal valve insufficiency. Ann Thorac Surg 1998;65:517-524.[Abstract/Free Full Text]
5. Elami A., Laks H., Pearl J.M. Truncal valve repair: initial experience with infants and children. Ann Thorac Surg 1994;57:397-402.[Abstract/Free Full Text]
6. Bove E.L., Beekman R.H., Snider R., et al. Repair of truncus arteriosus in the neonate and young infant. Ann Thorac Surg 1989;47:499-506.[Abstract/Free Full Text]
7. Becker A.E., Becker M.J., Edwards J.E. Pathology of the semilunar valve in persistent truncus arteriosus. J Thorac Cardiovasc Surg 1971;62:16-26.[Medline]
8. Fuglestad S.J., Puga F.J., Danielson G.K., Edwards E.D. Surgical pathology of the truncal valve: a study of 12 cases. Am J Cardiovasc Pathol 1988;2:39-47.[Medline]
9. Bharati S., McAllister H.A., Jr, Rosenquist G.C., Miller R.A., Tatooles C.J., Lev M. The surgical anatomy of truncus arteriosus communis. J Thorac Cardiovasc Surg 1974;67:501-510.[Medline]
10. Gelband H., Van Meter S., Gersony W.M. Truncal valve abnormalities in infants with persistent truncus arteriosus: a clinicopathologic study. Circulation 1972;45:397-403.[Abstract/Free Full Text]
11. Deely W.J., Hagstom J.M., Engle M.A. Truncus insufficiency. Common truncus arteriosus with regurgitant truncal valves. Report of 4 cases. Am Heart J 1963;65:542-548.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Michael D. Black Ian Adatia Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Black, M. D. Articles by Freedom, R. M. Search for Related Content PubMed PubMed Citation Articles by Black, M. D. Articles by Freedom, R. M.