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Ann Thorac Surg 2001;72:831-835
© 2001 The Society of Thoracic Surgeons

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Original article: cardiovascular

Atrioventricular valve regurgitation in patients with single ventricle: impact of the bidirectional cavopulmonary anastomosis

^a Divisions of Cardiology and Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^b Departments of Pediatrics and Surgery at the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA

Accepted for publication May 17, 2001.

Address reprint requests to Dr Mahle, Sibley Heart Center, Egleston Children’s Hospital, 1405 Clifton Rd, NE, Atlanta, GA 30322
e-mail: mahlew{at}kidsheart.com

	Abstract

Top Abstract Introduction Material and methods Results Comment References

 
Background. It is not known whether atrioventricular valve regurgitation (AVVR) can improve in some patients after the bidirectional cavopulmonary anastomosis (BCPA) without undertaking concomitant valvuloplasty.

Methods. We retrospectively reviewed our experience with patients who underwent the BCPA between June 1989 and June 2000 with specific attention to those patients with moderate or severe AVVR. The degree of AVVR was determined by color Doppler echocardiography. During the study period there were 576 patients who underwent BCPA at a median age of 6.8 months (range, 1.3 months to 4.8 years). The majority of subjects (66%) had a tricuspid valve as the systemic atrioventricular valve. Significant (moderate or severe) AVVR before BCPA was noted in 36 of 576 patients (6%) and was significantly more prevalent (8 of 52 patients, 15%; p = 0.01) in patients with a common atrioventricular canal.

Results. Of the 36 subjects with moderate or severe AVVR, 8 had interventions (6 valvuloplasty, 2 aortic arch dilation) at BCPA. There was 1 hospital death (2.8%). Of the remaining 27 survivors who did not undergo additional interventions at BCPA, improvement in AVVR (mild or none) was noted in 6 (22%) at intermediate follow-up (median, 2 years; range, 0.5 to 3.1 years). The presence of significant AVVR before BCPA was not significantly associated with hospital survival or intermediate-term freedom from death or transplantation.

Conclusions. We conclude that AVVR improves in some patients after BCPA and that valvuloplasty is not justified in all patients with moderate preoperative AVVR.

	Introduction

Top Abstract Introduction Material and methods Results Comment References

 
Significant atrioventricular valve regurgitation (AVVR) has been associated with increased mortality in patients with a single ventricle after cavopulmonary operations in some series [1, 2]. As such, some investigators have proposed that early surgical intervention with valvuloplasty may improve outcome in this patient population [1–3]. Because many centers use a strategy of intermediate staging with a bidirectional cavopulmonary anastomosis (BCPA), it has been suggested that concomitant valvuloplasty is best performed at this time [3]. However, studies have shown that ventricular volume decreases after cavopulmonary operation [4, 5]. The decreased ventricular volume is accompanied by a reduction in the atrioventricular annulus dimension [6] and that may result in improved atrioventricular valve function—especially those patients that have significant annular dilation and leaflet noncoaptation. Several small case series have suggested that such resolution is uncommon [3, 7]. However, this question has not been addressed in a systematic fashion. The goals of the present study were to review our experience with the management of AVVR in the single ventricle population and to determine whether significant AVVR improves in some patients after BCPA without concomitant valvuloplasty.

	Material and methods

Top Abstract Introduction Material and methods Results Comment References

 
Patient population
Beginning in June 1989, BCPA was performed routinely at our institution as an intermediate stage before Fontan operation. There were 576 subjects who underwent a staging BCPA at less than 5 years of age between June 1, 1989 and June 1, 2000. Heterotaxy was present in 33 (6%). We retrospectively reviewed the clinical and echocardiographic data for all 576 subjects. Information regarding the current clinical status of the study subjects was obtained from the referring cardiologist or medical records. Atrioventricular valve morphology of the systemic ventricle was classified as tricuspid, mitral, common atrioventricular canal, or two atrioventricular valves.

Echocardiographic examination
The degree of AVVR was determined by review of echocardiographic database at our institution. A complete two-dimensional, Doppler, and color Doppler echocardiographic examination was routinely performed before BCPA. The grade of AVVR was based on the ratio of the color Doppler jet to the area of the atrium as previously described [8]. The AVVR was graded as none, mild (jet < 30% of atrial area), moderate (jet = 30% to 50% of atrial area), or severe (jet = > 50% of atrial area). In addition, the identification of significant AVVR by echocardiography was compared to that determined at cardiac catheterization. Angiographic grades 3+ or more were considered moderate or severe. To identify the primary mechanism of AVVR preoperative echocardiographic studies as well as operative reports were examined. We attempted to ascribe a primary mechanism of AVVR to each of the subjects with moderate or severe preoperative AVVR.

Statistical analysis
Data are expressed as mean ± standard deviation or median and range, where appropriate. Statistical analysis was performed by Fisher’s exact test, ² test, Wilcoxon rank sum test, Kaplan Meier survival curve estimates, log-rank tests to compare survival curves, and Cox proportional hazards model for assessing multivariate associations between risk factors and freedom from death or transplantation. Analysis was performed with STATA 6.0 (College Station, TX). Significance was determined at p value of less than 0.05. All p values are two-sided and confidence intervals are 95%.

	Results

Top Abstract Introduction Material and methods Results Comment References

 
Preoperative AVVR
Significant (moderate or severe) AVVR as determined by color Doppler interrogation was present in 36 patients (6%) before BCPA (Table 1). When compared to angiography, color Doppler echocardiography had a sensitivity of 90% and specificity of 98.5% for detecting significant AVVR in the 520 patients who had preoperative ventriculograms. Moderate or severe AVVR was significantly more common in patients with common atrioventricular valve morphology, p = 0.01. There was no significant difference in the prevalence of significant AVVR among patients with mitral, tricuspid, or two atrioventricular valves. Abnormalities in valve leaflet architecture were noted in 18 of the 36 patients with significant AVVR. Abnormalities included dysplastic leaflets in 8 (22%), restricted movement of one or more leaflets in 4 (11%) and prolapse in 6 patients (17%). Sixteen subjects (44%) had no obvious leaflet abnormalities and AVVR appeared to result from central noncoaptation. In 2 subjects leaflet anatomy was incompletely visualized. Two of the 36 patients with significant AVVR had undergone valvuloplasty before BCPA.

AVVR after BCPA
The follow-up echocardiographic data for subjects with significant preoperative AVVR is shown in Figure 1. There were 6 patients who underwent valve operation at the time of BCPA. Three of these 6 patients had two atrioventricular valves and valve operation consisted of oversewing of the regurgitant valve. Two additional subjects were thought to have significant AVVR on the basis of residual arch obstruction after the Norwood procedure. Both of these patients demonstrated improvement in atrioventricular valve function after the interventions were undertaken to relieve arch obstruction at the time of BCPA. Therefore, there were 27 subjects with significant AVVR who underwent BCPA without additional interventions. Six subjects (22%), all of whom had moderate AVVR before BCPA, demonstrated significant improvement in atrioventricular valve function such that at most recent follow-up they had mild or no AVVR. There were 18 patients (67%) with intermediate-term follow-up (> 6 months after BCPA) who continued to demonstrate significant AVVR after BCPA. Ten of these subjects went on to require later valvuloplasty. Intermediate follow-up echocardiographic studies were unavailable for 3 (11%) patients.

View larger version (22K): [in this window] [in a new window]   Fig 1. Outcome of 36 patients with moderate or severe atrioventricular valve regurgitation (AVVR) before bidirectional cavopulmonary anastomosis (BCPA).

Valvuloplasty and valve replacement
Of the 36 patients with significant AVVR before BCPA, 16 underwent valvuloplasty (6 at BCPA, 10 later). In the 3 subjects with two atrioventricular valves repair consisted of oversewing of the regurgitant valve. For the remaining 13 subjects reparative techniques included circular annuloplasty, suturing of a cleft, and leaflet resection. Intermediate-term (> 6 months after valvuloplasty) follow-up echocardiograms were available for 12 of 16 patients. Sustained improvement in AVVR (mild or none) was noted in 6 (50%) patients. The remaining 6 subjects had at least moderate AVVR at intermediate follow-up. Two patients required valve replacement at a later date.

Overall survival and freedom from transplantation
The 1-year and 5-year freedom from death or transplantation was 86% and 76%, respectively. The survival curve for the entire cohort of 576 study patients stratified by the degree of preoperative AVVR is shown in Figure 2. Preoperative AVVR and need for valvuloplasty were not significantly associated with survival (Table 2). Later era of BCPA procedure was the only factor significantly associated with greater freedom from death or transplantation.

View larger version (16K): [in this window] [in a new window]   Fig 2. Kaplan-Meier plot of freedom from death or transplantation after bidirectional cavopulmonary anastomosis for entire cohort (n = 576) stratified by presence/absence of moderate or severe atrioventricular valve regurgitation. Time 0 = time of bidirectional cavopulmonary anastomosis.

	Comment

Top Abstract Introduction Material and methods Results Comment References

The mechanism of AVVR may be an important factor in determining which patients are likely to demonstrate improve atrioventricular valve function after BCPA. Although the small sample size limits statistical analysis, it would appear that significant improvement occurs most commonly in patients with normal atrioventricular valve structure in which the jet of AVVR is central. Patients with structural abnormalities of the valve rarely demonstrate improvement after BCPA. None of the subjects with dysplastic valve leaflets or restricted movement of valve leaflets demonstrated meaningful improvement in AVVR after BCPA if valvuloplasty was not undertaken concomitantly.

One of the factors that has lead investigators to advocate aggressive treatment of AVVR is the potential for increased mortality after cavopulmonary operation. A number of reports have identified AVVR as a risk factor for mortality, both at the time of cavopulmonary operation and later [1, 2]. Imai and colleagues [1] reported a 12-year mortality of 27% for patients with significant AVVR versus 9% for patients with no or trivial AVVR. In our series, there was no increased risk for mortality or need for transplantation in patients with moderate or severe AVVR at the time of BCPA. This would agree with several more recent reports that have failed to identify AVVR as a risk factor for patients after cavopulmonary operation [9–11]. The increased volume load, as occurs in the setting of significant AVVR, has been considered particularly deleterious in patients with single ventricle. Whether other factors such as routine use of after-load reducing agents both in the postoperative period and for chronic management mitigate the effect of AVVR remain a matter of speculation.

The prevalence of significant AVVR in our study population was 6%. In previous series the reported prevalence of significant AVVR in patients with single ventricle has varied considerably from 5% to 41% [1–3, 12]. The degree of AVVR may be influenced by factors such as the amount of pulmonary blood flow and the length of time the ventricle has been volume loaded. In general, it has been our policy to use smaller systemic–pulmonary artery shunts where possible, and perform the BCPA at a relatively young age. Atrioventricular valve morphology is also associated with the risk of developing AVVR. As in previous series, we found that significant AVVR was more common in patients with a common atrioventricular canal [1, 13]. This patient group comprised only 8.8% of study population. In addition, heterotaxy was relatively uncommon. Together, these factors may account for the relatively low prevalence of AVVR in our series. Interestingly, we found no difference in the prevalence of significant AVVR in patients with a systemic tricuspid valve compared to those subjects with a systemic mitral valve.

There have been several published reports examining the use of valvuloplasty in the single ventricle population [1, 3, 14]. Imai and colleagues [1] used a circular annuloplasty in 157 patients with single ventricle. The degree of AVVR improved by one grade on average in the hospital survivors. However, it is difficult to extrapolate these data to our own patient population, as these investigators used valvuloplasty even in patients with mild AVVR. Mosca and colleagues reported improved atrioventricular valve function in 8 patients with hypoplastic left heart syndrome who were evaluated in the perioperative period, although at least 1 patient went on to require valve replacement at a later date [15]. In this present series significant longer-term improvement in atrioventricular valve function was noted in one-half the patients after valvuloplasty. Because of the small number of patients undergoing valvuloplasty and the various surgical techniques used it is not possible to determine whether performing a valvuloplasty at a later age (ie, after BCPA) is more or less likely to achieve long-term success. Valve operation may be technically easier in a larger child.

Study limitations
In spite of the large number of patients in this series, the number of patients with significant AVVR was relatively small. This may have limited the risk factor analysis, especially with regard to relationship between the mechanism of AVVR and the potential for improvement after BCPA. In addition, the assessment of AVVR by color Doppler echocardiography has known limitations and the retrospective study design relied on various readers. Nonetheless, there was a fairly good correlation with angiography in this series. Last, the study population was limited to patients who underwent BCPA. As such, the potential impact of AVVR on early mortality—in the first months of life—was not addressed in this study.

Conclusion
The current study suggests that significant AVVR can improve in some patients undergoing the BCPA without concomitant valvuloplasty. In addition, significant AVVR does not appear to be a major determinant of early or intermediate-term mortality after BCPA. As such, an approach of performing valvuloplasty in all patients with moderate AVVR at the time of the BCPA does not appear to be justified. At our own institution, we would consider valvuloplasty at the time of BCPA in those patients with structural valve abnormalities amenable to surgical intervention.

	References

Top Abstract Introduction Material and methods Results Comment References

 

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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): Thomas L. Spray Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mahle, W. T. Articles by Rychik, J. Search for Related Content PubMed PubMed Citation Articles by Mahle, W. T. Articles by Rychik, J. Related Collections Congenital - cyanotic