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Ann Thorac Surg 1996;62:151-154
© 1996 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Partial Atrioventricular Canal With Congestive Heart Failure in the First Year of Life: Surgical Options

Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Hospital, Rome, Italy

Accepted for publication February 27, 1996.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure.

Methods. From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter.

Results. There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10^-2 m/m² died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy.

Conclusions. Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.

	Introduction

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See also page 154.

The natural history of partial atrioventricular canal (pAVC) is similar, in most cases, to that of large ostium secundum atrial septal defect [1–3]; surgical series usually are concerned with children older than 4 years [4, 5]. However, in the setting of this anomaly, there is a subgroup of patients who require surgical treatment in the first year of life [6, 7] because of refractory congestive heart failure. The clinical features, associated heart malformations, surgical options, and results were only recently reported in literature [7]. The aim of this study is to describe our experience in the treatment of 35 infants with pAVC operated on at less than 1 year of age.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Patients
From June 1982 to April 1995, 128 children with pAVC were operated on in our department. The records of 35 patients (27%) who underwent operation in the first year of life were reviewed in the present study. All patients had situs solitus of the atria, ostium primum atrial septal defect, two separate atrioventricular orifices, cleft of the anterior leaflet of the mitral valve, and intact ventricular septum [8]. All infants presented with severe congestive heart failure unresponsive to medical treatment. The mean age at operation was 5.0 ± 3.1 months (range, 0.1 to 11 months) and the mean body weight was 4.1 ± 1.6 kg (range, 2 to 7.1 kg). There were 17 male and 18 female patients. Seven patients (20%) had Down's syndrome. All patients underwent complete preoperative two-dimensional echocardiographic study. In 25 patients (71%) cardiac catheterization was added: mean pulmonary artery pressure ranged from 18 to 55 mm Hg (mean, 31 mm Hg) and pulmonary-to-systemic flow ratio from 2.4 to 6.8 (mean, 3.8). Mean right ventricle-to-left ventricle pressure ratio was 0.64 (range, 0.37 to 1). Associated cardiac anomalies were present in 22 patients (63%): 5 patients had severe left ventricular hypoplasia (with aortic coarctation in 4). Eleven patients had left-sided obstructive lesions with normal left ventricle: 9 patients had aortic coarctation isolated in 6, (with subaortic stenosis in 2, and with multiple mitral cleft in 1), 1 had isolated subaortic fibrous membrane, and 1 had subaortic stenosis plus multiple mitral cleft and double-orifice mitral valve. Additional anomalies of the mitral valve occurred in 4 patients (double-outlet mitral valve in 3 and multiple mitral cleft in 1) and common atrium in 2.

Operation
In 5 patients the left ventricle was considered severely hypoplastic [9, 10] and not suitable for biventricular repair. These patients underwent aortic coarctectomy in 2 cases, aortic coarctectomy followed by orthotopic heart transplantation in 1 case, and Norwood procedure in 2 cases. The other 30 patients, considered suitable for biventricular repair, underwent anatomic repair in 24 cases (with relief of fibrous subaortic membrane in 1) and aortic coarctectomy in 6 cases. The aortic coarctectomy was performed in all patients by a subclavian flap plasty. The anatomic repair was performed with complete closure of the mitral cleft in 19 cases and with partial closure of the mitral cleft in 5 cases. The ostium primum atrial septal defect was closed by a Dacron fabric patch in 21 patients (until 1993) and by an autologous pericardial patch in 3 patients. In all cases the coronary sinus was left in the right atrium.

Retrospective Echocardiographic Measures
Because the precise evaluation of the left ventricular size in patients with atrioventricular canal and moderate left ventricular hypoplasia is very difficult for cardiac geometry in the presence of severely enlarged right ventricle [7,9–11], we retrospectively reviewed the preoperative echocardiography to measure the mitral valve annulus [12, 13]. In all 24 patients submitted to anatomic repair the review of the echocardiographic tapes showed pictures eligible for the study. The mitral valve annulus was measured by viewing the tapes on frame-by-frame basis without knowledge of surgical outcome. Three measurements were taken on the parasternal long-axis view and three for the four-chamber apical view in each case by two observers, and a mean value was calculated [12, 13]. The largest dimension occurring during the cardiac cycle was chosen for the analysis. The final values have been normalized against the body-surface area (m/m²). Statistical analysis was performed by analysis of variance. The measures of the mitral valve of these patients were correlated to the surgical outcome.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Anatomic Repair
There were seven in-hospital deaths (7/24 = 25%). Three patients died in the operating room and 4 in the postoperative period: 3 of low cardiac output and 1, with Down's syndrome, of pulmonary hypertensive crisis. In a mean follow-up of 105 months (range, 22 to 153 months) there were no deaths. Four patients required reoperation for secondary mitral valvuloplasty 1 and 4 months and 5 and 6 years after the first operation (Fig 1): 2 of these patients had partial closure of the mitral cleft at the first operation. The mean diameter of the mitral valve (Fig 2) among the patients who survived after the anatomic repair was of 4.28 x 10^-2 ± 0.89 x 10^-2 m/m² (range, 2.94 x 10^-2 to 6.00 x 10^-2 m/m²), whereas in the patients who died it was 2.23 x 10^-2 ± 0.85 x 10^-2 m/m² (range, 1.05 x 10^-2 to 3.00 x 10^-2 m/m²) (p = 0.003). It was impossible to find a boundary value separating the two groups because of the lack of statistical power of the test due to the relatively small number of cases. As shown by Figure 2 there is an "undefined zone" around the values from 2.5 x 10^-2 to 3.0 x 10^-2 m/m². Above this range of values, the surgical results were good, whereas in the case of a lower value we had no survivors.

View larger version (13K): [in this window] [in a new window]   Fig 1. . Actuarial freedom from reoperation for patients submitted to anatomic repair.

View larger version (19K): [in this window] [in a new window]   Fig 2. . Correlation between echocardiographic mitral valve diameter and surgical outcome for patients submitted to anatomic repair.

Norwood Operation
Both patients who underwent Norwood operation died in the operating room.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Partial atrioventricular canal is a cardiac defect usually well tolerated in infancy and successfully repaired during childhood [1–5]. However, a subgroup of patients with early signs of congestive heart failure exist and require operation in the first year of life [6, 7]. In these patients left-sided anomalies are frequent and Down's syndrome is rare [6, 7,14–18]. Twenty-two of our patients (63%) showed associated left-sided cardiac anomalies including left ventricular hypoplasia, left-sided obstructive lesions, and additional malformation of the mitral valve. Such left-sided anomalies are prevalent in children with partial and complete atrioventricular canal without Down's syndrome [6, 7,14–18]. Complete repair of partial or complete atrioventricular canal is contraindicated in the presence of severe left ventricular hypoplasia [1, 6, 7,9–11 17, 18]. When the left ventricle is only moderately hypoplastic, biventricular repair may be hazardous but is possible [6, 7], keeping the Norwood-type palliation only when the left ventricle is severely hypoplastic. However, in general these patients present "unique features and prognosis" [7] and the surgical results are problematic if compared with the "classic" form of pAVC [1–5]. In the time period of this study, surgical repair of pAVC was undertaken in 93 patients in our unit with a mean age of 5.5 years (range, 1 to 20 years). There were no hospital deaths and only one late death.

Because a precise evaluation of the left ventricular dimension is very difficult in these patients due to the geometry of the ventricles [7,9–11] and because of our surgical results, we attempted to identify which patients to send to anatomic repair and which to a Norwood operation. We retrospectively measured, in our patients submitted to anatomic repair, the echocardiographic mitral valve diameter [12, 13]. Because our cases do not reach a sufficient number to give statistical power to search for a definitive boundary between the two groups, we decided to examine only if such a boundary could exist. The relationship between valvular dimension growth and body surface area is not linear, as it follows a logarithmic regression curve [19]. However, because the range of body surface areas in our patients is relatively limited, a linear relationship can be assumed to portray the variation of mitral valve dimension through the different patients, thus simplifying the calculations. The mean values for the mitral valve diameter in patients who died were significantly lower than those in the survived patients (see Fig 2). In our series all infants with a mitral valve diameter less than 2.5 x 10^-2 m/m² died at operation. In contrast, a mitral valve diameter greater than 3.2 x 10^-2 m/m² seemed, in our experience, to be associated with good surgical results (see Fig 2). For infants with a mitral valve diameter between 2.5 x 10^-2 and 3.2 x 10^-2 m/m² a partial intracardiac correction with an adjustable atrial septal defect [20] may be considered. If a Norwood procedure is considered, an important prerequisite is that the right ventricle has remained hypertensive beyond the neonatal period to be capable to sustain the systemic circulation. For this reason, for some patients with pAVC and severe left ventricular hypoplasia, heart transplantation is the only possible surgical option after the first months of life. In the presence of a left ventricle of adequate size, an additional malformation of the mitral valve will not preclude anatomic repair primary or secondary to aortic coarctectomy if necessary. In our experience, in patients with normal left ventricle, the primary treatment of aortic coarctation seemed to change the natural history of these patients compared with those of simple pAVC. Six patients of our series underwent successful primary aortic coarctectomy in the first year of life. Three of them underwent secondary repair at a mean time of 23 months after the first operation. The other 3 patients await surgical repair in good clinical condition 2, 4, and 60 months after aortic coarctectomy.

Another interesting aspect of this subgroup of patients is the unusually high reoperation rate (see Fig 1). Different from the Boston group [7], the cause of reoperation in our series is not the progression of subaortic stenosis but the mitral valve incompetence after anatomic repair (5/24 = 24%), probably secondary to more complex anatomy of the left-sided atrioventricular valve [14–16, 21]. As previously reported, this high incidence of reoperation for correction of severe postoperative regurgitation of the left atrioventricular valve is more frequent in patients who do not have Down's syndrome [21–23].

Although detailed diagnosis can be usually obtained in infants with partial and complete atrioventricular canal by echocardiography [24, 25], preoperative cardiac catheterization and angiography is often needed in this subgroup of patients to further clarify anatomic and functional aspects of the malformation. In our series 71% of patients underwent preoperative cardiac catheterization, whereas in the group of 93 patients more than 1 year of age, hemodynamic study was performed in 37 patients (40%) and in 6/40 (15%) after 1989.

In summary, an important subgroup of patients with pAVC, in large majority without Down's syndrome, requires operation in the first year of life. This is likely due to associated left-sided anomalies and complex left atrioventricular valve anomaly. The echocardiographic mitral valve diameter seems to be useful for determining the correct surgical indication to anatomic repair. In the extreme forms of the spectrum, severe left ventricular hypoplasia precludes conventional repair, and Norwood operation or heart transplantation should be considered. In the presence of aortic coarctation, the patients with normal left ventricle benefit from aortic coarctectomy and may undergo delayed repair. However, children submitted to complete repair are at substantial risk of reoperation due to left atrioventricular valve insufficiency, which is usually difficult to treat at the first operation.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Address reprint requests to Dr Giamberti, Dipartimento Medico Chirurgico, di Cardiologia Pediatrica, Ospedale Bambino Gesù, P.zza S. Onofrio 4, 00165 Rome, Italy.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

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