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Ann Thorac Surg 1999;67:1412-1414
© 1999 The Society of Thoracic Surgeons


Original Articles

Repair of partial atrioventricular septal defect in children less than five years of age: late results

Manoj Agny, MDa, Adnan Cobanoglu, MDa

a Division of Cardiopulmonary Surgery, Oregon Health Sciences University, Portland, Oregon, USA

Accepted for publication November 17, 1998.

Address reprints requests to Dr Cobanoglu, Division of Cardiopulmonary Surgery, Heart and Lung Transplantation Programs, Oregon Health Sciences University, 3181 SW Sam Jackson Park Rd, L353, Portland, OR 97201-3098
e-mail: cobanogl{at}ohsu.edu


    Abstract
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Background. Presently, surgical correction of partial atrioventricular septal defects is an extremely viable option giving good results. An aggressive approach toward operating on these patients at an early age may be warranted given the otherwise unfavorable natural history.

Methods. A retrospective study was done in 38 consecutive patients from 3 to 58 months of age, who underwent correction between 1981 and 1997. Preoperatively, moderate to severe mitral regurgitation was present in 45% of the patients. Congestive cardiac failure was noted in 41% of the cases. Closure of the left atrioventricular valve cleft was performed in 92% of the cases. A need for mitral annuloplasty was felt in 28% of the cases. Majority of the ostium primum defects in our series were closed by a pericardial patch.

Results. The early, 30 day mortality was 7.9%. A significantly low incidence of late mitral regurgitation (0.9%) was noted on a follow-up extending up to 14 years. There was only one reoperation during late follow-up. On their last follow-up, 87% of the patients are asymptomatic.

Conclusion. An aggressive approach toward operating at an early age on children with this malformation is safe, effective, and yields excellent long term results.


    Introduction
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
The surgery for partial atrioventricular septal defect has, in most surgical series, usually been described in children older than 4 years of age [13]. It has also been done for infants with refractory congestive cardiac failure [4]. But an elective repair at an early age for all cases, asymptomatic or in cardiac failure, has been infrequently reported. The aim of our study was to evaluate our experience in the treatment of partial atrioventricular septal defect in children less than 5 years of age.


    Material and methods
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
An elective repair of partial atrioventricular septal defect was carried out in 51 patients in our institute, from July 1981 to July 1997, by a single surgeon using the same surgical technique. The present study is a retrospective analysis of the repairs done for this malformation in 38 children less than 5 years of age. We defined partial atrioventricular septal defect as a malformation comprised of an ostium primum defect along with a cleft in either the left or right atrioventricular valve, or in both valves. Intermediate forms of the lesion were not included in the present study. Cases with a ventricular septal defect that required a patch closure of the interventricular septum were also excluded, as they were considered to belong to the category of complete atrioventricular septal defect. There were 21 males and 17 females in the study. Their ages at the time of operation ranged from 3 months to 58 months (mean = 22 months). The weight range was from 3.2 kg to 22.5 kg (mean = 4.1 kg). Down’s syndrome was encountered in one third of our patients (35%). Congestive cardiac failure was present in 41% of the cases. Left atrioventricular valve regurgitation was assessed by clinical evaluation, roentgenography, and M-mode and Doppler echocardiography in all the cases. In 45% of the cases, the regurgitation was found to be moderate to severe in extent. In 55% of the patients, the regurgitation was either mild or absent. Cardiac catheterization was carried out in 16 cases (42%). It revealed pulmonary hypertension in 7 patients (18%). The mean pulmonary to systemic flow ratio (Qp : Qs) was noted to be 2.7 (range 1.2–4.1). The mean pulmonary vascular resistance was 1.48 (range 0.3–3.7). Associated cardiac anomalies were present in 5 patients: 2 cases of pulmonic stenosis; and 1 each of a common atrium; a patent ductus arteriosus; and a small left ventricular inflow tract. An ostium secundum defect was present in 5 cases (13%). Structural anomalies of the mitral valve apparatus were found in 5 patients: 2 cases with a double orifice mitral valve; 2 cases with a small posterior leaflet; and 1 case of an extra scalloped anterior leaflet.

Surgical technique
Standard cardiopulmonary bypass with moderate hypothermia was used for repair in the majority (27) of the cases. A combination of cardiopulmonary bypass and brief periods of circulatory arrest was used in 11 cases. The ostium primum defect was closed with a treated autologous pericardial patch. Sutures in the vicinity of the coronary sinus were taken superficially to avoid the conduction system. The coronary sinus was always left on the right side of the patch. Closure of the left atrioventricular cleft was done in 35 patients (92%). This cleft closure was complete in 34 patients. A partial closure of the cleft was done in 1 patient with a small posterior leaflet. Cleft closure was not done in 3 patients due to the absence of the cleft in 1 and a small left ventricular annulus in the other 2, in whom closure of the cleft would have caused orifice stenosis. Cleft closure was performed with 6-0 ethibond suture. An intraoperative assessment of the status of regurgitation was done with a saline distention of the left ventricle, thereby floating the leaflets and doing a careful anatomic and functional evaluation. A need for annuloplasty was felt in 13 cases and a Wooler annuloplasty [5] was carried out in all of these patients. The cardiopulmonary bypass time ranged from 58 to 354 minutes, with a mean of 89 minutes. The mean aortic clamp time was 56 minutes (range = 23–104 minutes). On final intraoperative assessment, 31 patients (83%) were free of regurgitation. A mild regurgitation, which we felt would not interfere with hemodynamics, was seen in 7 cases (17%) (Table 1 ). Closure of the ostium primum defect was done with a dacron patch until 1985. Thereafter, we started closing all ostium primum defects with a treated autologous pericardial patch. A need to repair the right atrioventricular valve was found in 4 cases. The ostium secundum defect was closed primarily in 3 patients, and with a patch in the other 2. For 1 patient with associated valvular and annular pulmonic stenosis, valvotomy along with transannular patching was done. For the other patient with pulmonic stenosis, only valvotomy sufficed. In the patient with the common atrium, a large pericardial patch was used to partition the two atria. Ligation of the ductus was done in the child with the patent ductus arteriosus.


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Table 1. Status of Left Atrioventricular Valve Regurgitation: Preoperative and Postoperative

 

    Results
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 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Early
There were three in-hospital deaths. One patient died due to biventricular dysfunction 8 hours after the surgery and was noted to have had a large left atrioventricular valve cleft, which had been closed during surgery. He also had severe pulmonary hypertension preoperatively. The other death due to severe low output syndrome was in a child who had a small hypoplastic left ventricle and a malformed right atrioventricular valve. The third death was in a patient whose parents were Jehovah’s witness and had declined the use of any blood products. These 3 deaths contributed to an early 30 day mortality of 7.9%. Postoperatively, the mean time spent in the intensive care unit was 3.7 days (range = 1–10 days). The mean hospital stay was 7.5 days (range 4–29 days). Two patients required to be reexplored; one for bleeding; and the other for sternal instability. One patient required a permanent pacemaker implantation for complete heart block.

Late
One late death was encountered in a patient who had been reoperated for left atrioventricular valve stenosis, 8 months after the primary repair. A partial cleft closure had been done in this patient in the first instance due to a small posterior leaflet. At the second operation, severe papillary and chordal fusion was encountered and had been relieved. He died 1 year after the second operation due to progressive left ventricular failure. On a follow-up of 32 patients extending up to 14 years, no patient has required reoperation for left atrioventricular valve regurgitation. A residual atrial shunt has not been encountered in any patient. Only 1 patient has a mild left ventricular outflow tract obstruction. But, on an echocardiogram done postoperatively, peak velocities across the aortic valve demonstrated an insignificant gradient. On their last follow-up, 29 patients had either none or trace mitral regurgitation. Mild to moderate regurgitation was seen in 3 patients. Of these 3 patients, 1 is totally asymptomatic, and the other 2 are well controlled medically with only digoxin. There has been no change in either the left ventricular dimensions or function on follow-up echocardiography in any of these 3 patients.


    Comment
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Surgery is mandatory for partial atrioventricular septal defects. This is due to the progressive left atrioventricular valve regurgitation, development of arrhythmias, and the likelihood of pulmonary vascular disease [6]. Correction for this malformation has been done at all ages. In view of the aforementioned undesirable natural history, repair at an earlier age may be called for in all the patients, irrespective of clinical status.

Baufreton and coauthors [7] have elaborated on the risk factors in the early postoperative period after repair of partial atrioventricular septal defect. Their results emphasized the importance of residual left atrioventricular valve regurgitation as a risk factor in the early postoperative period. The association of preoperative left atrioventricular valve regurgitation with residual regurgitation after correction, early reoperation, and with the long-term outcome is well known. Operative repair at an earlier age may prevent the progression of this regurgitation and aid in achieving a better long-term result. Our experience reveals that, though most patients in our study were having either moderate or severe regurgitation preoperatively, very few have any regurgitation postoperatively during last follow-up. In our series, the relatively high incidence of Down’s syndrome may account for the rarity of left sided obstructive lesions [8]. This may also have helped us in achieving good results, since these lesions have also been incriminated as risk factors in Baufreton’s study.

As regards the cleft in the left atrioventricular valve, our policy has always been to close it. This is due to our belief that leaving the left atrioventricular valve as a trileaflet structure will, in due course of time, result in significant regurgitation, which could be detrimental. The only exception to this would be in cases with either a very small posterior leaflet or in valves with a small annulus. Our results in this study reinforce this stance, since 87% of our patients on their last follow-up are asymptomatic and have either none or trivial left atrioventricular valve regurgitation.

None of our patients have required reoperation for left atrioventricular valve regurgitation. This, we believe, is due to careful and meticulous anatomic and functional evaluation of the left atrioventricular valve before and after cleft closure. Also, the commissural annuloplasty done in 11 cases must have contributed toward achieving an optimal result. Added to this is the relative infrequency of left sided obstructive lesions in our series, since these have been known to be risk factors for reoperation. Since 1985, we have been routinely closing ostium primum defects with an autologous pericardial patch. This has been to minimize trauma to blood elements from a regurgitant jet, if any, hitting a dacron patch.

In conclusion, it can be safely stated that, given the excellent long-term results as evidenced in our series, early elective repair of partial atrioventricular septal defects is a safe and effective approach in dealing with these malformations.


    References
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 

  1. 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/Free Full Text]
  2. Permut L.C., Mehta V. Late results and reoperation after repair of complete and partial atrioventricular canal defect. Semin Thorac Cardiovasc Surg 1997;9:44-54.[Medline]
  3. King R.M., Puga F.J., Danielson G.K., Schaff H.V., Julsrud R.R., Feldt R.H. Prognostic factors and surgical treatment of partial atrioventricular canal. Circulation 1986;74(Suppl 1):42-46.
  4. 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]
  5. Wooler G.H., Nixon P.G., Grimshaw V.A., Watson D.A. Experiences with the repair of mitral valve in mitral incompetence. Thorax 1962;17:49-57.
  6. Sommerville J. Ostium primum defects: factors causing deterioration in natural history. Br Heart J 1965;27:413-423.
  7. Baufreton C., Leca F., Khoury W., Tamisier D., Vouhé 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]
  8. Marino B., Vairo U., Corno A., et al. Atrioventricular canal in Down syndrome. Prevalance of associated cardiac malformations compared with patients without Down syndrome. Am J Dis Child 1990;144:1120-1122.[Medline]



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