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Ann Thorac Surg 2004;78:666-672
© 2004 The Society of Thoracic Surgeons

Original article: cardiovascular

Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome

Roberto Formigari, MD, FACCa*, Roberto M. Di Donato, MDb, Gaetano Gargiulo, MDa, Duccio Di Carlo, MDb, Cristiana Feltri, MDb, Fernando M. Picchio, MD, FESCa, Bruno Marino, MDb

a Pediatric Cardiology and Cardiac Surgery, University of Bologna, Bologna, Italy
b Pediatric Cardiology and Cardiac Surgery, "Bambino Gesù" Hospital, Rome, Italy

Accepted for publication December 2, 2003.

* Address reprint requests to Dr Formigari, Cardiologia e Cardiochirurgia Pediatrica, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy.
e-mail: r.formigari1{at}tin.it


   Abstract
Top
 Abstract
Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 References
 
BACKGROUND: Several studies have shown that Down's syndrome is not a risk factor for biventricular repair of complete atrioventricular septal defects. However, few data are available about the comprehensive outcome of all the cardiac surgical procedures in patients with trisomy 21, including palliative surgery.

METHODS: This is a retrospective study of 206 consecutive patients who underwent cardiac surgery from January 1992 to January 2002. Data about mortality and morbidity were analyzed and the impact of Down's syndrome was evaluated.

RESULTS: Overall mortality was 7.7%. Actuarial survival was 94% among patients with Down's syndrome versus 86% of the group with normal karyotype (p = 0.12). The presence of unbalanced ventricles was the only independent risk factor affecting survival at multivariate analysis (p < 0.0001). The need for a Norwood type surgery was more frequent among non-Down patients (12.0% vs 1.5%, p = 0.02) as was the prevalence of pulmonary artery banding operations (22.9% vs 9.3%, p = 0.04). Cumulative mortality after palliation was higher in non-Down patients (44% vs 2.9%, p = 0.0001). Freedom from reoperation was lower in the group with normal chromosomes in respect to patients with Down's syndrome (81.4% vs 94.6%, p = 0.04), due to the higher prevalence of anomalies of the mitral valve (4.9% vs 1.8%, p = 0.03) or left ventricular outflow tract (7.3% vs 0%, p = 0.01).

CONCLUSIONS: Down patients showed a decreased risk for biventricular repair and lower mortality and morbidity in cases of complex cardiac malformations requiring complex palliative operations.


   Introduction
Top
 Abstract
 Introduction
Material and methods
 Results
 Comment
 Acknowledgments
 References
 
Complete atrioventricular septal defect (cAVSD) is a congenital heart malformation frequently associated with Down's syndrome [1]. While cardiac surgery in Down children has been questioned in the past on the basis of surgical results [2–4] or long-term natural history of these individuals [5], other studies demonstrated that complete biventricular repair yields substantial benefits with no regard to genetic status. While higher pulmonary vascular resistance, abnormal airways, and lung parenchyma of Down patients may require special care, these factors do not increase the operative risk over that observed in patients with normal chromosomes [6–10]. However, already published series are based on selected cases with balanced ventricles and optimal candidates for complete biventricular repair, thus excluding a significant group of patients with associated cardiac malformations or not suitable for anatomic repair. Therefore, the impact of Down's syndrome has been hitherto analyzed only in relation to complete biventricular repair of cAVSD, and the outcome of the overall surgical treatment including palliative surgery has not been presented, so far, in the literature. The aim of this study was to determine the impact of Down's syndrome on the comprehensive outcome of all cardiac surgical procedures for cAVSD in a series of consecutive and unselected patients.


   Material and methods
Top
 Abstract
 Introduction
 Material and methods
Results
 Comment
 Acknowledgments
 References
 
Patient population
A review was undertaken of all patients with cAVSD who have been operated from January 1992 to January 2002 at the two institutions involved in the study. Two hundred and six patients form the study population. Median age at first intervention (primary complete repair, pulmonary artery banding, systemic-to-pulmonary shunt, or a first stage Norwood type operation) was 5.9 months with a median weight of 5.0 kgs. Down's syndrome, confirmed by cytogenetic analysis, was found in 131 cases (63.6%). All patients had complete preoperative echocardiographic assessment and 33 (16%) underwent cardiac catheterization due to associated complex heart defects, history of previous palliative procedures, or to assess pulmonary vascular resistance [11].

All patients showed situs solitus of the atria, ventricular D loop, and concordant normally related great arteries. Associated heart malformations were found in 53 patients: tetralogy of Fallot (14 patients), subaortic stenosis without left ventricular hypoplasia (2 patients), aortic coarctation (14 patients), additional muscular ventricular septal defect (4 patients), double orifice mitral valve (5 patients), single papillary muscle of the left ventricle (10 patients), total anomalous pulmonary venous return (2 patients), or double aortic arch (1 patient). Twenty-three patients (11.1%) had either right or left ventricular dominance causing concerns in respect to primary repair surgery. Extracardiac anomalies were recognized in 4 patients (duodenal stenosis in 2, diaphragmatic hernia in 1, and tracheal stenosis in 1). The associated cardiac anomalies in the Down and non-Down groups are shown in Table 1. Systemic-to-pulmonary shunts were performed by interposition of a 3.5 or 4.0 mm polytetrafluoroethylene (PTFE) or Gore-tex tube between one subclavian artery and the left, right, or central pulmonary arteries.


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  		Table 1. Prevalence of Associated Cardiac Malformations

 
The technical approach for achieving a Norwood type palliation varied among the two institutions and over time. Some patients have been treated by a classic Norwood operation, whereas in most recent years the modification proposed by Fraser and Mee [12] was frequently used in association with other special techniques for reconstruction of the ascending aorta [13]. Similarly, palliative procedures in patients with complex anatomy of the mitral valve or associated tetralogy were more frequent during the early phase of our experience. Associated aortic coarctation is still frequently repaired by a two-stage strategy. The flow chart in Figure 1 shows the surgical management and outcome of the study population.



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  		Fig 1. Flow-chart of surgical management and outcomes in all 206 patients with Down and non-Down syndrome. (AVSD = atrioventricular septal defects; BCPA = bidirectional cavopulmonary anastomosis; D = Down; DKS = Damus-Kaye-Stansel; ND = non-Down; SP = systemic-to-pulmonary.)

 
Primary complete repair
Primary biventricular repair was performed in 71.4% of cases, at a mean age of 7.0 ± 3.1 months (range, 3 to 11) and a mean weight of 9.3 ± 3.1 kgs (range, 2.1 to 9.0 kgs) using a standard cardiopulmonary bypass technique with moderate hypothermia. Two patients, aged 5.3 years and 12 months, respectively, underwent primary repair of cAVSD with associated tetralogy by interposition of a homograft conduit between the right ventricle and the pulmonary artery or a transannular patch repair.

Palliative operations
Palliative procedures were performed in 59 patients (28.6%) at a mean age of 2.5 ± 1.2 months (range, 0.3 to 16 months) and a mean weight of 4.5 ± 5.1 kgs (range, 2.4 to 13.3 kgs). A pulmonary artery banding was performed in 36 patients because of hypoplasia of the left (12 patients) or right (7 patients) ventricle, single left ventricular papillary muscle (4 patients), double orifice mitral valve (2 patients), or associated aortic coarctation (11 patients) which was repaired by subclavian flap plasty (8 patients), or end-to-end anastomosis (3 patients). One patient with aortic coarctation had associated total anomalous venous connections which underwent complete repair during the pulmonary banding-coarctectomy procedure.

A systemic-to-pulmonary shunt was performed in 12 patients who had associated tetralogy (one non-Down and 11 Down patients). Severe hypoplasia of the left ventricle and ascending aorta suggested a Norwood operation in 10 patients and a Damus-Kaye-Stansel (DKS) procedure with a modified Blalock shunt in another one.

Surgical course after a first palliative procedure
Complete biventricular repair was performed in 33 cases, after a median time of 21.3 months. Repair of associated tetralogy after palliation was achieved by an infundibular patch in 5 patients, a transannular patch in 6, and a right ventricle to pulmonary artery conduit in 1. One patient with borderline left ventricular hypoplasia had pulmonary artery banding at 5 months of age followed by bidirectional cavopulmonary anastomosis at 14 months of age and later takedown with complete repair at 5 years of age.

Among the survivors of palliative surgery not eligible for definitive repair, 6 with previous pulmonary artery banding underwent bidirectional cavopulmonary shunt (mean age, 10.2 ± 2.3 months) with associated DKS procedure. Of these, 2 had completion of the Fontan principle at 3.2 and 4.1 years of age, respectively. Three survivors of a stage I Norwood operation (2 patients) or a DKS and shunt operation (1 patient) underwent a bidirectional cavopulmonary shunt procedure with one operative death. Four patients with dominant left ventricle underwent a one and one-half ventricle procedure with bidirectional cavopulmonary shunt at a mean age of 16.0 ± 4.3 months (range, 9.8 to 19.8 months).

Reoperations
Criteria for censorship in the Kaplan-Meier estimate for freedom from reoperation was the occurrence of cardiac surgery after either definitive biventricular repair or definitive monoventricular palliation. Thus, a bidirectional cavopulmonary anastomosis and(or) completion of the Fontan principle were considered as predictable events within the roadmap of palliation of patients with only one functional ventricle. Final repair in a two-stage strategy was also not regarded as an unplanned reintervention.

Overall, 12 patients needed reoperation, all after biventricular repair, after a mean time of 2.5 ± 1.3 years. Mitral valve redo surgery was needed in 5 cases, with one operative death. Resection of residual or recurrent subaortic obstruction was performed in 5 cases while closure of a residual significant ventricular septal defect was carried out in 2 cases. One non-Down patient died late after biventricular repair during mitral valve redo surgery. There were no reoperations, so far, due to residual defects after repair of associated tetralogy.

Indications for Norwood type operation
Because of the relatively high mortality and complication rate of Norwood-DKS palliations, a critical review of the preoperative echocardiograms of the patients with unbalanced ventricles was performed in order to validate the indications to monoventricular palliation in the light of some of the currently proposed criteria.

By selection of end-diastolic frames on echocardiographic recordings, the left ventricular volume (LVV) indexed for body surface area, using the bullet or the simplified Simpson rule [14, 15], and the atrioventricular valve index (AVVI) [16] were calculated by one of the authors (RF). The anatomy of the cardiac apex was classified as being formed mainly by the left or the right ventricle [17]. Eight patients had reliable recordings which enabled accurate retrospective evaluation of the left ventricle. Predictors of poor outcome of biventricular repair were a LVV less than 20 mm3/m2, an AVVI higher than 0.67, or the left ventricle not reaching the cardiac apex.

Follow-up
The median follow-up time is 7.1 years (range, 1.5 to 11.3 years).

Statistical analysis
The time-related factors were analyzed by the modified Cox multivariate regression method. Univariate analysis was assessed by the two-tailed Fischer's exact test. Survival and freedom from reoperation curves were analyzed by the log-rank test. A p value less than 0.05 was considered as significant. The size of the study population allowed for a maximum of five cofactors to be included in the hazard model for survival. Thus, only the variables which are usually believed to be the most important in respect to survival and reoperation rate were considered: Down's syndrome (y/n), left ventricular hypoplasia (y/n), right ventricular outflow tract obstruction (y/n), major mitral valve abnormalities (single papillary muscle or a double orifice mitral valve) (y/n), and isolated left-sided obstructive anomalies (like subaortic stenosis or coarctation of the aorta with a normally developed left ventricle) (y/n). The proportional hazard assumption was tested and confirmed after comparison from the Kaplan-Meier (product-limit) survival estimates of each single variable. Descriptive statistics are summarized as the mean and standard deviation (SD) or as a median for continuous variables and frequencies and percentages for categorical variables.


   Results
Top
 Abstract
 Introduction
 Material and methods
 Results
Comment
 Acknowledgments
 References
 
Overall mortality
Overall mortality was 7.7% (15 in-hospital and 1 late deaths). There was a higher in-hospital mortality for non-Down patients, but this difference may be due to chance (4.6% vs 12.5%, p = 0.13). Actuarial survival after 12 years of follow-up was 94% among patients with Down's syndrome versus 86% of the group with normal karyotype (p = 0.12, hazard ratio 2.44, 95% confidence intervals [CI] 0.74 to 11.22 [Fig 2 ]). Mortality was highest among the non-Down subjects with unbalanced cAVSD who underwent a Norwood procedure (Fig 3), but not significantly different in respect to Down patients treated by the same type of procedure (p = 0.14; hazard ratio [HR] = 0.23, 95%; CI 0.06 to 1.51). Overall, patients with unbalanced ventricles were significantly more frequent among the group without Down's syndrome (Table 1). One Down patient died after complete repair of the cAVSD associated with tetralogy of Fallot.



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  		Fig 2. Kaplan-Meier plot of survival after biventricular repair or definitive monoventricular palliation. The numbers at risk for the whole population are shown. Down (triangles); non-Down (squares).

 


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  		Fig 3. Kaplan-Meier plot of survival after biventricular repair or definitive monoventricular palliation among the different subgroups of patients. The numbers at risk for the whole population are shown. (cAVSD = complete atrioventricular septal defect; DKS = Damus-Kaye-Stansel operation.)

 
The presence of unbalanced ventricles was the only independent risk factor affecting survival at multivariate analysis (p < 0.0001; 95% CI 0.12 to 0.17). Forcing the exclusion of the "Down's syndrome" covariate from the multivariate model did not change the outcome.

Palliative operations
A neonatal Norwood or DKS operation was less frequent among patients with trisomy 21 in respect to patients with normal karyotype (1.5% vs 12.0%, p = 0.02). We found no significant difference, regarding the overall need for other types of palliations, between normal and Down patients (23.1% vs 12.0%, p = 0.12). However, non-Down patients showed a significantly higher prevalence of pulmonary artery banding operations (25.3% vs 12.9%, p = 0.03). While, a systemic-to-pulmonary shunt was needed in 8.4% of the patients with Down's syndrome versus 1.3% among those with normal chromosomes, this difference was not significant at univariate analysis (p = 0.06). Eight patients died early after monoventricular palliation by Norwood stage I or neonatal DKS operations (6 non-Down and 2 Down patients). Three non-Down patients with borderline left ventricle died after palliation by pulmonary artery banding and repair of coarctation. One non-Down patient died late after a Norwood stage II procedure. Cumulative mortality of non-Down patients after surgical palliation was higher in non-Down patients (2.9% vs 44%, p = 0.0001).

Reoperations
Freedom from reoperation (Fig 4), after successful biventricular repair or a definitive univentricular palliation, was lower in the group with normal chromosomes in respect to the patients with Down's syndrome (81.4% vs 94.6%, p = 0.04, HR 0.28, 95% CI 0.08 to 0.80). Mitral valve plasty was the most frequent cause for reintervention, especially in non-Down patients (5.3% vs 1.5%, p = 0.03). Similarly, there was a higher prevalence of recurrent-residual subaortic stenosis associated with normal karyotype (7.3% vs 0%, p = 0.01). Two patients in the non-Down and 1 in the Down group have been reoperated for residual ventricular septal defect (2.5% vs 0.9%, p = 0.45). Overall, syndromic patients showed a lower prevalence of reoperation.



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  		Fig 4. Kaplan-Meier plot of freedom from reoperation after biventricular repair or definitive monoventricular palliation. The numbers at risk for the whole population are shown. Down (squares); non-Down (triangles).

 
Evaluation of hearts with right ventricular dominance
Among the 8 patients with available echocardiographic recordings, a positive correlation between the final surgical decision and the preoperative findings was found in 7 (Table 2). One patient (No. 4) was submitted to a pulmonary artery banding and a bidirectional cavopulmonary anastomosis before being reconsidered for anatomic repair 3.5 years later.


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  		Table 2. Preoperative Echocardiographic Predictors for Biventricular Repair in Patients with Right Ventricular Dominance

 

   Comment
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
Acknowledgments
 References
 
The role of chromosome 21 in the normal development of the embryonic heart is supported by the high prevalence of defects of the atrioventricular junction in patients with Down's syndrome, with anomalies of the ventricular loop or the transposition complex being exceedingly rare [18, 19]. Even within the same type of cardiac defect, as in the case of ventricular septal defects [20] and partial atrioventricular canal [21], clinically relevant differences have been found between trisomy 21 patients and children with normal karyotype. For what concerns cAVSD, peculiar features showed by the trisomic population have been described in terms of a lower prevalence of left-sided obstructions [22, 23], right ventricular dominance, and muscular ventricular septal defects [20]. All the previously published surgical series were concerned with biventricular repair of uncomplicated types of cAVSD [7–9, 24], eventually with associated tetralogy or aortic coarctation [24]. Therefore, due to the systematic exclusion of a small, but clinically important, number of patients not suited for biventricular repair or with major left-sided associated anomalies, the "true" genetic impact of Down's syndrome has been underestimated. Only two previous studies addressed the issue of cAVSD in nonsyndromic patients [25], or with right ventricular dominance needing monoventricular palliation [26], but without comparing the results with a control group of trisomic patients. Even the paper by Al-Hay and colleagues [27], while confirming Down's syndrome not a risk factor for cardiac surgery, was limited to biventricular repair ignoring the higher prevalence of left-sided malformations associated with normal chromosomes. Interestingly, in a recent study by Cleves and colleagues [28] about the survival probability of infants born with congenital heart defect, Down patients with atrioventricular septal defect were much more likely to survive than those without Down's syndrome. This epidemiologic study, which did not rely on any kind of surgical data, gives further strength to our observations.

We provide a comprehensive insight on the clinical impact of Down's syndrome on mortality and morbidity after cardiac surgery for cAVSD by a comparative evaluation of an unselected series of patients, including palliative operations. Due to the high prognostic value of the associated malformations in patients with heterotaxic syndromes [29–32], this type of cAVSD has been excluded from our study. Similarly, specific technical issues are beyond the scope of this paper.

Biventricular repair
There are no significant differences in survival between Down and normal chromosome patients. After an initial toll of in-hospital mortality, due to respiratory infections and pulmonary hypertension, trisomic patients featured a stable survival curve, which may be expected to improve by better preoperative and postoperative care. The presence of tetralogy was not associated with increased overall mortality in our group. The mortality of non-Down patients who underwent biventricular repair was lower in respect to the 38% reported by Lacour-Gayet and colleagues [26], but 3 out of the 11 deaths from the French group were related to patients with a small left ventricle and who might have survived if submitted to monoventricular palliation.

Palliative surgery
Non-Down patients featured a significantly higher prevalence of pulmonary artery banding procedures, reflecting the problem of biventricular repair in the presence of unbalanced ventricles or left-sided associated malformations. Indeed, palliative procedures were more frequent among our series in respect to the 7.0% reported by Redmond and colleagues [25], with a higher risk for non-Down patients.

Mortality for Norwood-type operations among the nonsyndromic cases was 62.5%, Interestingly, Down patients showed a higher survival rate, in respect to nonsyndromic infants, even after first-stage monoventricular palliation with further bidirectional Glenn anastomosis, despite the known issues about the increased pulmonary vascular reactivity of these subjects. However, the small size of the Down group with right ventricular dominance prevented a reliable statistical evaluation. The long time between a first palliative procedure and further complete repair is due to the presence of patients with complex anatomy (left or right ventricular dominance or associated tetralogy) who have been considered as a high risk group during the early decades of our experience, thus prompting for delayed anatomic repair.

Reoperations
Our study confirms that Down patients have a lower probability of reintervention, especially for mitral valve plasty or prosthetic replacement, perhaps due to the different amount of tissue from the atrioventricular valve available for the creation of competent valves without progressive annular dilatation [8, 32]. The higher prevalence of reoperation in non-Down cAVSD for recurrence of subaortic stenosis or the progressive development of a fibrous ridge within the left ventricular outflow tract is not surprising [22, 30].

Limits of the study
As in most similar reports, the main limitation lies in the retrospective analysis of the data. The presence of a learning curve in performing complex operations may jeopardize the reliability of a proportional hazard method, but the surgical technique for cAVSD repair did not change significantly across time.

The retrospective evaluation of predictors for suitability to biventricular repair was not blinded and only 8 patients were considered. Moreover, there is still no consensus about the morphologic and functional factors to be used as reliable tools to predict survival in patients with a small left ventricle, especially if different types of cardiac defects are considered [33–36]. Even if a fair correlation was found between the echocardiographic evaluation and the final surgical decision, we cannot rule out the impact of this difficult decision making process on our study, especially for what concerns mortality. Estimation of the left ventricular volume and the cardiac apex anatomy are the indicators we are currently using for indication toward monoventricular versus biventricular repair. The AVVI is not used, even if the mitral valve dimensions are always considered in the surgical decision making algorithm.

In conclusion, not only does Down's syndrome not increase the risk for biventricular repair, as suggested by past literature, but it is a protective factor in respect to all the cardiac surgical procedures, even if complex monoventricular palliative operations are considered.


   Acknowledgments
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
References
 
We are grateful to Alessandro Giamberti, MD, and Carlo Pace Napoleone, MD, for assisting in collecting the data and to Maria C. Digilio, MD, for the revision of the paper.


   References
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 References
 

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