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Ann Thorac Surg 2002;73:69-75
© 2002 The Society of Thoracic Surgeons

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Original article: cardiovascular

Surgical results of double-orifice left atrioventricular valve associated with atrioventricular septal defects

^a Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, Fukuoka, Japan

Accepted for publication August 31, 2001.

* Address reprint requests to Dr Kado, Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, 2-5-1, Tojin-machi, chu-ou ku, Fukuoka 810-0063 Japan
e-mail: f-kodomo{at}aurora.dti.ne.jp

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
Background. Double-orifice left atrioventricular valve (LAVV) is a rare but surgically important anomaly, which is regarded as a risk factor for surgical correction of atrioventricular septal defects (AVSDs).

Methods. Of 209 consecutive patients with AVSDs, double-orifice LAVV was identified in 19 patients (9.1%, including 7 infants). Preoperative LAVV function, surgical procedures and results, incidence of postoperative LAVV dysfunction and reoperations were reviewed and compared between patients with this valve malformation (group I, n = 19) and those without it (group II, n = 190).

Results. There were no operative or late deaths in group I. Preoperative LAVV function was similar in both groups. The cleft was totally closed in 77.2% of group II and 47.1% of group I (p < 0.01). In partial AVSDs, freedom from postoperative LAVV insufficiency was 77.0% in group II versus 30.5% in group I at 5 years (p = 0.009) and freedom from reoperation was 89.9% in group II versus 58.3% in group I at 5 years (p = 0.012); however, there was no difference in complete AVSDs. None of the infants in group I underwent total cleft closure and 4 of them showed more than moderate LAVV insufficiency postoperatively.

Conclusions. Double-orifice LAVV is a significant predictor for postoperative LAVV incompetence and reoperation in partial AVSDs, but not in complete AVSDs. Surgical procedures for the cleft should be individualized with careful intraoperative evaluation of the structure and function of this abnormal valve, especially in partial AVSDs and infants.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
Results of surgical correction for patients with atrioventricular septal defects (AVSDs) have been improving; however, left atrioventricular valve (LAVV) dysfunction after correction has remained a major factor predicting operative death and reoperation [1–7]. In patients with AVSDs, a wide spectrum of abnormalities of the valve and subvalvular apparatus has been reported [4, 7, 8–10]. The presence of these additional valve abnormalities may increase the complexity of the procedure, necessitate technical modifications, and affect surgical outcome [2, 4, 7, 11, 12]. Double-orifice LAVV is an uncommon valve anomaly, which is most often associated with AVSDs and has been regarded as a risk factor for operative mortality and morbidity [1, 13–15]. The aims of this retrospective analysis were to evaluate the impacts of double-orifice LAVV on the outcome of surgical treatment for AVSDs, and to consider the most appropriate surgical approach to this complex valve lesion.

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
From February 1981 to December 1999, 209 consecutive patients (134 with complete AVSDs and 75 with partial AVSDs) underwent definitive surgical correction for AVSDs at the Department of Cardiovascular Surgery of Fukuoka Children’s Hospital (Fukuoka, Japan). Complete AVSDs were defined as a large interventricular communication with common atrioventricular orifice. Partial AVSDs were defined as being without major interventricular communications with dual atrioventricular orifices. Six patients with minor interventricular communications with dual atrioventricular orifices were involved in partial AVSDs. Double-orifice LAVV was defined when there were two separate valve orifices in the LAVV, and each orifice was supported by a distinct subvalvular apparatus. Among these patients, 19 patients (9 with complete AVSDs and 10 with partial AVSDs) had double-orifice LAVV (9.1%). Seven of the 19 patients were infants. The profile of the patients in this study are shown in Table 1. Preoperative evaluations were made on all patients, including two-dimensional echocardiography or cardiac angiography, or both. Color Doppler echocardiography became available in our institution in 1987 and was routinely used thereafter. Preoperative and postoperative clinical data were collected from the patients’ records, and intracardiac findings and surgical procedures were reviewed from the operative records. The severity of the LAVV insufficiency was divided into four groups (none or trivial, mild, moderate, and severe) according to the findings of echocardiography and cardiac angiography.

Surgical methods
All patients were approached through median sternotomy. Cardiopulmonary bypass with moderate hypothermia was established by bicaval and aortic cannulation. Cardiac arrest was obtained with crystalloid cardioplegic solution with an initial dose of 10 to 15 mL/kg followed by a half dose infusion every 20 to 30 minutes. Intracardiac repair was performed through a right atriotomy. The double patch method was used in all patients with complete AVSDs. We used a single semilunar shaped velour patch or polytetrafluoroethylene patch for ventricular septal defect closure and an autologous pericardial patch for atrial septal defect closure in all patients. In partial AVSDs, a single autologous pericardial patch was used to close the ostium primum defect.

Procedure for LAVV
We basically leave the accessory orifice in the LAVV intact. When a cleft is present in the true orifice, it is our policy to close it until the edge where leaflet tissue is supported by chordae tendinea (total cleft closure). In patients with a small mural leaflet portion, and when total cleft closure seems to significantly reduce the true orifice area, we close the cleft partially. In these patients, we secure a true orifice area of at least 80% normal size according to Rowlatt Standard [16]. We routinely assess the anatomy and function of the valvular apparatus by cold saline injection into the ventricle, and valvular competence is checked after repair by repeated saline injection tests.

Follow-up
Clinical follow-up data from 16 to 228 months (median, 79 months) were available in all patients in group I. Follow-up data were available in 179 of 190 patients (94.2%) from 13 to 244 months (median, 98 months) in group II. Clinical evaluations and two-dimensional color Doppler echocardiograms were performed on all survivors regularly in the outpatient clinic.

Statistical analysis
The degree and frequency of LAVV insufficiency and the frequency of the type of cleft closure performed (total, partial, or left intact) between the two groups were compared by Fischer’s exact test. Freedom from reoperation and freedom from postoperative LAVV insufficiency for the two groups were calculated according to the Kaplan-Meier method and log rank test.

	Results

Top Abstract Introduction Patients and methods Results Comment References

 
There were no operative or late deaths in patients with double-orifice LAVV during the median follow-up of 79 months (16–228 months). The profile of the patients in group I is shown in Tables 2 and 3.

Intraoperative findings
The location of the accessory orifice is shown in Tables 2 and 3. In partial AVSDs, 1 patient with an accessory orifice in the posterior leaflet had three papillary muscles for the LAVV, 1 patient with an accessory orifice in the anterior leaflet had an accessory orifice in the right atrioventricular valve as well, and 1 patient with an accessory orifice in the anterior leaflet and 1 in the posterior leaflet had no obvious cleft in the true orifice. The mural leaflet portion was less developed in 2 patients with posterior accessory orifice. One of them had supravalvular fibrous ring. With an intraoperative regurgitation test, valvular regurgitation was mainly seen from the true orifice and trivial regurgitation was detected from the accessory orifice only in a few patients in both partial and complete AVSDs. All the accessory orifices were supported by their own tensor apparatus.

Cleft closure
In group I, the cleft in the true orifice was totally closed in 8 patients (47.1%), partially closed in 8 patients (47.1%), and the cleft was left intact (because no regurgitation was detected intraoperatively) in 1 patient (5.9%) (Tables 2 and 3). On the other hand, the cleft was totally closed in 142 patients (77.2%), partially closed in 26 patients (14.1%), and left intact in 16 patients (8.7%) in group II. The proportion of total cleft closure was significantly lower in group I (p = 0.005), particularly in patients less than 1 year of age. No infant except 1 (whose cleft was left intact) in group I underwent total cleft closure, whereas 59 of 86 infants (68.6%) in group II underwent total cleft closure (p = 0.0001). In contrast, the proportion of total cleft closure in patients over 1 year of age was 8 of 10 (80.0%) in group I and 83 of 98 (84.7%) in group II (p = 0.262). In a 6-year-old girl with partial AVSDs, an accessory orifice was found in the anterior leaflet and cleft was absent. She had moderate preoperative LAVV insufficiency. However, the origin of the regurgitation was not clear. She underwent direct closure of the accessory orifice and no regurgitation has been detected after 177 months of follow-up.

Postoperative LAVV function
Freedom from postoperative LAVV insufficiency ( moderate) in the two groups is shown in Figure 1. It was not statistically different between the two groups in complete AVSD; however, in partial AVSDs patients with double-orifice LAVV had a significantly lower rate of freedom from postoperative LAVV insufficiency (84.0% in group II vs 45.7% in group I in 3 years, and 77.0% in group II vs 30.5% in group I in 5 years; p = 0.009). In 1 patient with a complete AVSD who had an accessory orifice in the posterior commissure, mild LAVV stenosis (inflow velocity of 160 cm per second) was detected by follow-up echocardiography 110 months later as well as mild regurgitation. The correlation of age at operation, cleft closure, and postoperative LAVV insufficiency is shown in Figure 2.

View larger version (14K): [in this window] [in a new window]   Fig 1. Kaplan-Meier estimates of freedom from postoperative left atrioventricular valve (LAVV) insufficiency in partial atrioventricular septal defects (AVSDs) (A) and complete AVSDs (B). In partial AVSDs, freedom from LAVV insufficiency was significantly lower in group I compared with group II (freedom from LAVV insufficiency at 3, 5, and 10 years was 45.7%, 30.5%, and 30.5% in group I, respectively, and 84.0%, 77.0%, and 74.3% in group II, respectively (p = 0.009). Vertical bars enclose a 95% confidence interval. Numbers indicate patients at risk.

View larger version (9K): [in this window] [in a new window]   Fig 2. Correlation between age at operation, cleft closure, and late left atrioventricular valve (LAVV) insufficiency in patients with double-orifice LAVV. Five of 8 patients with partial atrioventricular septal defects (AVSDs) showed moderate or more LAVV insufficiency. All but 1 patient less than 1 year of age underwent partial cleft closure. Six of 8 patients with total cleft closure showed mild or less than mild LAVV insufficiency. (• = partial AVSDs/partial cleft closure; = partial AVSDs/total cleft closure; = complete AVSDs/partial cleft closure; = complete AVSDs/total cleft closure; = complete AVSDs/cleft left intact; Lt. AVV = left atrioventricular valve; R = patients who underwent reoperation.)

View larger version (12K): [in this window] [in a new window]   Fig 3. Kaplan-Meier estimate of freedom from reoperation in partial atrioventricular septal defects (AVSDs) (A) and complete AVSDs (B). In partial AVSDs, freedom from reoperation was significantly lower in group I compared with group II (freedom from reoperation at 5 and 10 years was 58.3% and 58.3% in group I, and 89.9% and 86.8% in group II, p = 0.012, respectively). Vertical bars enclose a 95% confidence interval. Numbers indicate patients at risk.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

 
Double-orifice LAVV is a rare but important anomaly and is most commonly reported in association with AVSDs [10, 18–20]. In our experience, the incidence of double-orifice LAVV was 9.1% and other large series have reported incidences of 2% to 8.6% [1, 4–7, 11, 13–15] among AVSD patients. Clinically, this anomalous valve has been considered a significant risk factor for operative mortality and reoperation in AVSD repair [1, 13–15]. In the report of 8 surgical cases by Ilbawi and colleagues [18], operative mortality was 50%. Warnes and Somerville [19] reported their 11 surgical experiences with 2 early deaths and 1 late death. Najim and colleagues [15] reported that among 21 patients with double-orifice LAVV, 6 patients (29%) suffered early death and another 6 (29%) required reoperation. On the other hand, Lee and colleagues [20] showed fairly good surgical results in 25 patients with only 1 operative death and 2 late deaths after reoperation for valvular insufficiency. In our experience of 19 patients, there were no operative or late deaths. However, although the presence of double-orifice LAVV did not affect the surgical results of complete AVSDs, it was a significant predictor for development of late LAVV insufficiency and reoperation for the valvular incompetence in partial AVSD patients.

There are several possible explanations for our results, which showed that double-orifice LAVV was a predictor for operative morbidity in patients with partial AVSDs, but not in those with complete AVSDs. Piccoli and colleagues [8] reported that leaflet abnormalities, which could cause inlet obstruction, were associated more frequently with partial AVSDs. In a pathologic study, subvalvular apparatus malformation was more likely to be found in partial AVSD hearts [9]. Furthermore, Abbruzzese and colleagues [7] reported that 29.8% of partial AVSDs had additional malformations in the LAVV apparatus. Thus the more frequent anatomical valvular abnormalities may contribute to worse surgical results in partial AVSDs. Indeed, Michielon and colleagues [13, 21] have shown in their large series that the incidence of postoperative moderate to severe LAVV insufficiency was larger in partial AVSD patients than in complete AVSD patients. Also it should be considered that patients with partial AVSDs who require surgical correction in their first year of life have a more significant LAVV anomaly that could adversely affect the outcome [11, 12]. Manning and colleagues [12] reported that 7 of 11 infants with partial AVSDs had an abnormal LAVV structure, and Ilbawi and colleagues [18] reported that all of their 4 infants with double-orifice LAVV were associated with intermediate AVSDs and died. One of our 2 infants with partial AVSDs with double-orifice LAVV had a hypoplastic mural leaflet and moderate LAVV regurgitation, and the other showed mild LAVV regurgitation, an abnormal supravalvular fibrous ring, and moderate right atrioventricular valve regurgitation. Both infants underwent partial cleft closure that resulted in severe LAVV regurgitation soon after the operation.

Double-orifice LAVV is often associated with various kinds of anatomical malformations of the tensor apparatus, and more than one additional malformation can coexist in one valve [10]. This complex valvular structure is supposed to make effective valve repair difficult and contribute to postoperative valve dysfunction. In most of our patients, the accessory orifice was supported by firm chordae tendinea and, in some patients it obviously had a separate papillary muscle. We did not detect more than trivial regurgitation from the accessory orifice by intraoperative evaluation. This finding is consistent with that of other reports [19, 20]; therefore, most of the accessory orifice seems to be competent and nothing should be done to it unless significant regurgitation is detected. Accessory orifice is not within a leaflet but rather between the leaflets. The bridging tissue between the two orifices is not a simple fusion of the anterior and posterior leaflets but a leaflet tissue supported by firm chordae tendinea [10]. Surgical division of the bridging tissue caused massive regurgitation that resulted in valve replacement or operative death [10, 19, 20].

Considering the fairly competent features of the accessory orifice, surgical procedures to create a competent LAVV should be focused on the cleft in the true orifice. Surgical closure of the cleft has been controversial; however, many surgeons admit that surgical closure of the cleft reduces the incidence of operative mortality, residual LAVV insufficiency and reoperation, and that most postoperative LAVV regurgitation is from a residual cleft [3, 5, 6, 14, 15]. As for patients with double-orifice LAVV, careful treatment of the cleft is crucial because of its tendency to narrow the valve orifice [18–20]. It is our policy to close the cleft totally whenever regurgitation is detected, even in patients with double-orifice LAVV. However, the proportion of total cleft closure in patients with double-orifice LAVV was significantly lower than that seen in patients without this anomaly (47.1% vs 77.2%). This is largely because we tried to avoid creating valvular stenosis by cleft closure in this abnormal valve, based on our policy to accept mild regurgitation rather than make significant stenosis during valve repair.

Early surgical intervention and cleft closure is recommended for patients with complete AVSDs [3, 13, 14]. In our series of complete AVSDs, 65.3% of infants without double-orifice LAVV underwent total cleft closure and 76.1% of them showed mild or less than mild valvular regurgitation during follow-up. However, none of our 5 infants with double-orifice LAVV underwent total cleft closure. As a result, although 2 of them showed competent LAVV, 2 infants developed moderate LAVV insufficiency and the remaining 1 developed mild LAVV insufficiency and slight stenosis postoperatively (Fig 2). Although our results with complete AVSDs showed no correlation of the presence of double-orifice LAVV with postoperative LAVV insufficiency or incidence of reoperation, the appropriate surgical procedure to obtain a competent LAVV in this subset of patients is very difficult and the procedure must be individualized as emphasized by Carpentier [22].

In conclusion, the surgical results of double-orifice LAVV associated with complete AVSDs were acceptable in our series; however, this valvular malformation was a significant predictor of postoperative LAVV insufficiency and need for reoperation in partial AVSD patients. Most of the accessory orifices seem to be competent and should be left untouched. Left atrioventricular valve cleft closure should be individualized according to a precise evaluation of the structure of the valvular and the subvalvular apparatus, and careful manipulation must be done to insure a competent valve without creating valvular stenosis, especially in partial AVSDs and in infants.

	References

Top Abstract Introduction Patients and methods Results Comment References

 

1. Hanley F.L., Fenton K.N., Jonas R.A., et al. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg 1993;106:387-394.[Abstract]
2. Gunther T., Mazzitelli D., Haehnel C.J., Holper K., Sebening F., Meisner H. Long-term results after repair of complete atrioventricular septal defects: analysis of risk factors. Ann Thorac Surg 1998;65:754-759.[Abstract/Free Full Text]
3. Capouya E.R., Laks H., Drinkwater D.C., Jr, Pearl J.M., Milgalter E. Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects. J Thorac Cardiovasc Surg 1992;104:196-201.[Abstract]
4. Baufreton C., Journois D., Leca F., Khoury W., Tamisier D., Vouhe P. Ten-year experience with surgical treatment of partial atrioventricular septal defect: risk factors in the early postoperative period. J Thorac Cardiovasc Surg 1996;112:14-20.[Abstract/Free Full Text]
5. Najm H.K., Caldarone C.A., Smallhorn J., Coles J.G. A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg 1998;115:468-470.[Free Full Text]
6. El-Najdawi E.K., Driscoll D.J., Puga F.J., et al. Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg 2000;119:880-889.[Abstract/Free Full Text]
7. Abbruzzese P.A., Napoleone A., Bini R.M., Annecchino F.P., Merlo M., Parenzan L. Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants. Ann Thorac Surg 1990;49:111-114.[Abstract]
8. Piccoli G.P., Ho S.Y., Wilkinson J.L., Macartney F.J., Gerlis L.M., Anderson R.H. Left-sided obstructive lesions in atrioventricular septal defects: an anatomic study. J Thorac Cardiovasc Surg 1982;83:453-460.[Abstract]
9. Penkoske P.A., Neches W.H., Anderson R.H., Zuberbuhler J.R. Further observations on the morphology of atrioventricular septal defects. J Thorac Cardiovasc Surg 1985;90:611-622.[Abstract]
10. Bano-Rodrigo A., Van Praagh S., Trowitzsch E., Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988;61:152-160.[Medline]
11. Giamberti A., Marino B., di Carlo D., et al. Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options. Ann Thorac Surg 1996;62:151-154.[Abstract/Free Full Text]
12. Manning P.B., Mayer J.E., Jr, Sanders S.P., et al. Unique features and prognosis of primum ASD presenting in the first year of life. Circulation 1994;90(5 Pt 2):II30-II35.
13. Michielon G., Stellin G., Rizzoli G., Casarotto D.C. Repair of complete common atrioventricular canal defects in patients younger than four months of age. Circulation 1997;96(Suppl 9):II316-II322.
14. Najm H.K., Coles J.G., Endo M., et al. Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation 1997;96(Suppl 9):II311-II315.
15. Bando K., Turrentine M.W., Sun K., et al. Surgical management of complete atrioventricular septal defects. A twenty-year experience. J Thorac Cardiovasc Surg 1995;110:1543-1552.[Abstract/Free Full Text]
16. Rowlatt U.F., Rimoldi H.J.A., Lev M. The quantitative anatomy of the normal child’s heart. Pediatr Clin North Am 1963;10:499-588.
17. Kadoba K., Jonas R.A., Castaneda A.R., et al. Mitral valve replacement in the first year of life. J Thorac Cardiovasc Surg 1990;100:762-768.[Abstract]
18. Ilbawi M.N., Idriss F.S., DeLeon S.Y., et al. Unusual mitral valve abnormalities complicating surgical repair of endocardial cushion defects. J Thorac Cardiovasc Surg 1983;85:697-704.[Abstract]
19. Warnes C., Somerville J. Double mitral valve orifice in atrioventricular defects. Br Heart J 1983;49:59-64.[Abstract/Free Full Text]
20. Lee C.N., Danielson G.K., Schaff H.V., Puga F.J., Mair D.D. Surgical treatment of double-orifice mitral valve in atrioventricular canal defects. Experience in 25 patients. J Thorac Cardiovasc Surg 1985;90:700-705.[Abstract]
21. Michielon G., Stellin G., Rizzoli G., et al. Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg 1995;60:S604-S609.
22. Carpentier A. Surgical anatomy and management of the mitral component of atrioventricular canal defects. In: Anderson R.H., Shinebourne E.A., eds. Pediatric cardiology. London: Churchhill Livingstone, 1978:477-490.

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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): Hideaki Kado Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Nakano, T. Articles by Fukae, K. Search for Related Content PubMed PubMed Citation Articles by Nakano, T. Articles by Fukae, K.