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Ann Thorac Surg 1995;60:530-537
© 1995 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Repair of Complete Atrioventricular Canal Defects: Results With the Two-Patch Technique

Divisions of Cardiovascular-Thoracic Surgery and Cardiology, The Children's Memorial Hospital, and Departments of Surgery and Pediatrics and the Feinberg Cardiovascular Research Institute, Northwestern University Medical School, Chicago, Illinois

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. Between 1983 and 1994, 115 infants and children underwent repair of a complete atrioventricular canal defect with the two-patch technique and routine mitral valve ``cleft'' closure.

Methods. A retrospective review of these 115 patients was performed. Age at the time of repair ranged from 1 month to 108 months (mean age, 14.2 ± 16.5 months; median age, 8 months). Preoperative cardiac catheterization in 113 patients revealed a mean pulmonary to systemic flow ratio of 3.37 ± 1.8, a mean pulmonary artery systolic pressure of 71.1 ± 15.7 mm Hg, and a mean pulmonary vascular resistance of 4.9 ± 3.3 units. Associated anomalies included Down's syndrome (99 patients), patent ductus arteriosus (47), and coarctation of the aorta (4). Rastelli classification was A (76 patients), B (10), C (24), and unknown (5). Twenty-four patients had intraoperative epicardial or transesophageal echocardiography.

Results. Although there was a trend toward increasing mean preoperative pulmonary vascular resistance with age from 2.1 ± 0.9 units (0 to 3 months) to 4.0 ± 2.6 units (4 to 6 months) to 5.7 ± 3.0 units (7 to 12 months), the mean pulmonary vascular resistance of each age group was not significantly different from that of the main group. The operative survival rate was 94% (seven early deaths) and the overall survival rate, 91% (three late deaths). Intraoperative echocardiography altered the surgical therapy for 1 patient. No patient has required reoperation for a residual ventricular septal defect. Four patients (3.5%) had heart block requiring permanent pacemakers. Eight patients (7%) required reoperation for mitral insufficiency; 6 of whom had successful repair of a residual cleft.

Conclusions. For infants with complete atrioventricular canal defect, repair using the two-patch technique with routine mitral valve cleft closure at 4 to 6 months of age results in a low operative mortality, a low incidence of permanent heart block, and a low reoperation rate for mitral insufficiency.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
The first successful repair of a complete atrioventricular canal defect (AVC) was reported by Lillehei and colleagues [1] in 1955. They used cross circulation and direct suture of the atrial rim of the septal defect to the crest of the ventricular septum. Since that time, further understanding of the pathophysiology and anatomy of AVCs has improved the surgical results. Lev [2] described the position of the atrioventricular node and bundle of His. Rastelli and associates [3] classified the morphology of the common atrioventricular valve leaflets. Mair and McGoon [4] demonstrated that complete correction is possible in infants less than 1 year of age. The single-patch technique was described by Maloney and associates [5] in 1962 and was recently reviewed by Hanley and coauthors [6]. In 1976, Trusler [7] introduced the two-patch technique with a prosthetic patch for the ventricular septal defect, a pericardial patch for the atrial septal defect, and suture closure of the mitral ``cleft.'' The two-patch technique was reviewed in 1990 [8].

Current areas of controversy in the surgical management of the child with complete AVC include the following: (1) preoperative evaluation with echocardiogram versus cardiac catheterization; (2) optimal age for repair and utility of pulmonary artery banding; (3) one-patch versus two-patch technique; (4) surgical management of the left atrioventricular valve; and (5) management of associated coarctation of the aorta. New therapeutic modalities undergoing evaluation include intraoperative evaluation with transesophageal echocardiography (TEE) and the management of pulmonary hypertension with newer agents such as nitric oxide (NO). The purpose of this analysis is to review our experience with the two-patch technique including closure of the mitral valve cleft for the repair of complete AVC. We examined our patient characteristics, preoperative evaluation, operative technique, and incidence of reoperation for residual shunts, pacemaker, left atrioventricular valve regurgitation, and left ventricular outflow tract obstruction. From this review, we attempt to describe surgical guidelines that may optimize patient outcome.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Patient Population
This is a retrospective review of all 115 patients who underwent intracardiac repair of a complete AVC at The Children's Memorial Hospital in Chicago between 1983 and 1994. Patients with associated tetralogy of Fallot, patients with transitional or intermediate AVC, and patients with unbalanced defects leading to a single-ventricle type of repair were excluded. For purposes of definition, a complete AVC was defined as absence of the atrioventricular septum with a single common atrioventricular valve. Thirty-four patients were boys and 81, girls. Age at the time of repair ranged from 1 month to 108 months (mean age, 14.2 ± 16.5 months; median age, 8 months). Weight ranged from 3.0 to 17.2 kg (mean weight, 6.49 ± 3.11 kg). Ninety-nine (86%) of these 115 infants had associated Down's syndrome.

Prior palliation in the form of a pulmonary artery band had been performed in 15 patients at a mean age of 6.2 ± 3.5 months. Ten patients had been banded at our institution; the last patient banded, in 1989, was a child with associated multiple muscular VSDs. In those patients with a prior pulmonary artery band, complete repair (with band removal) was at a mean age of 37.1 ± 20.8 months. Four patients had had a modified Blalock-Taussig shunt after pulmonary artery banding prior to complete repair. Nine patients were intubated and on a ventilator preoperatively for various diagnoses including severe congestive heart failure, malnourishment, failure to thrive, and pneumonia.

Preoperative evaluation was by cardiac catheterization in 113 patients and echocardiogram in all patients. Mean pulmonary artery pressure was 71.1 ± 15.7/27.3 ± 10.6 mm Hg. The mean pulmonary to systemic flow ratio was 3.37 ± 1.80:1.0. Mean pulmonary vascular resistance (PVR) was 4.98 ± 3.3 units in room air and 3.15 ± 1.94 units in oxygen. Associated anomalies noted at the time of preoperative evaluation included patent ductus arteriosus (47 patients), ostium secundum atrial septal defect (30), common atrium (6), left superior vena cava (5), subaortic stenosis (4), coarctation of the aorta (4), aberrant origin of the right subclavian artery from the descending aorta (3), muscular VSD (2), cor triatriatum (1), and sinus venosus atrial septal defect (1). Rastelli classification at the time of operation was Rastelli A for 76 patients, Rastelli B for 10 patients, Rastelli C for 24 patients, and unknown for 5 patients. Six patients had a double-orifice mitral valve, and 2 patients had a parachute mitral valve.

Surgical Technique
All procedures were performed through a median sternotomy with bicaval cannulation for cardiopulmonary bypass. No patient underwent circulatory arrest. All procedures were done with moderate hypothermia from 22° to 28°C. A pericardial patch was harvested at the beginning of the procedure for the atrial closure. A vent was placed in the right superior pulmonary vein. This was pulled back into the atrium during the time of the left-sided atrioventricular valve repair. Myocardial protection was with cold blood cardioplegia, which was administered every 20 minutes during the aortic cross-clamp time. In addition, topical cooling was performed with ice slush.

In the early part of the series, an oblique incision was made from the tip of the right atrium in the direction of the pulmonary veins. Later in the series, a longitudinal incision was made in the atrium from the tip of the right atrial appendage parallel to the right coronary artery and extending between the right ventricle and the inferior vena cava (Fig 1). If the atrial component of the defect was small, the atrial septum was incised between the ostium primum opening and the patent foramen ovale to improve exposure. The VSD was closed with a Gore-Tex patch (106 patients), Impra patch (6), or Dacron patch (3) (Fig 2).

View larger version (60K): [in this window] [in a new window]   Fig 1. . Exposure and assessment of anatomy. (A) The atrial incision (broken line) is parallel to the right atrioventricular groove. (B) The right atrium has been opened after administration of cardioplegia. Note the coronary sinus and the location of the atrioventricular (AV) node. (C) Relationship of ventricular septal defect (VSD), atrial septal defect (ASD), and common atrioventricular valve. The valve leaflets are identified as follows: left inferior leaflet (LIL); left lateral leaflet (LLL); left superior leaflet (LSL); right inferior leaflet (RIL); right lateral leaflet (RLL); and right superior leaflet (RSL). (IVC = inferior vena cava; LV = left ventricle; PFO = patent foramen ovale; RV = right ventricle; SVC = superior vena cava.)

View larger version (74K): [in this window] [in a new window]   Fig 2. . Closure of ventricular septal defect (VSD). (A) The ventricular component is closed with a polytetrafluoroethylene patch fashioned as shown and anchored with interrupted pledget-supported sutures. (B) The sutures are placed on the right ventricular side of the septum to avoid the left bundle branch.

View larger version (47K): [in this window] [in a new window]   Fig 3. . Mitral valve reconstruction. (A) The left superior and inferior leaflets are approximated centrally. (B) The mitral leaflets are suspended from the ventricular septal defect (VSD) patch, and the same sutures are carried through the pericardial patch, which will close the atrial component. (C) The mitral valve is sandwiched between the VSD patch and the pericardial patch.

View larger version (32K): [in this window] [in a new window]   Fig 4. . Mitral valve ``cleft'' closure. (A) The superior and inferior leaflets are approximated to the point where the chordae insert on the leading edge of these leaflets. (B) The mitral valve is irrigated with cold saline solution to assess mitral competence.

View larger version (46K): [in this window] [in a new window]   Fig 5. . Atrial septal defect (ASD) closure and tricuspid valve reconstruction. (A) The pericardial patch is tailored and sutured to the free edge of the ASD. (B) The central portions of the right superior and inferior leaflets are approximated, thereby creating a tricuspid valve. (C) The tricuspid valve is anchored to the ventricular septal defect patch, and several more sutures are placed between the leaflets to provide tricuspid competence.

In the latter portion of the series, either epicardial echocardiography or TEE was used to assess the adequacy of the repair (Fig 6). Concomitant pulsed and color Doppler interrogation were used to assess the presence and severity of valvar regurgitation or stenosis along with residual shunting at the atrial or ventricular level.

View larger version (89K): [in this window] [in a new window]   Fig 6. . Transesophageal echocardiography in the four-chamber plane with the probe in the midesophageal position. (A) Preoperative study demonstrating the atrial and ventricular components of complete atrioventricular canal. (B) Postoperative study demonstrating the atrial septal defect (ASD) and ventricular septal defect (VSD) patches (arrows). (CAVV = common atrioventricular valve; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.)

In 5 patients (previously reported), inhaled NO was administered intraoperatively after repair of the defect and immediately after separation from cardiopulmonary bypass [10]. Hemodynamic monitoring of right atrial, left atrial, aortic, and pulmonary artery pressures was performed along with measurement of cardiac output. Nitric oxide was administered at 0, 20, 40, and 80 ppm, and the hemodynamic effects were assessed. Five different patients were administered NO postoperatively in the intensive care unit for persistent refractory pulmonary hypertension.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
All patients were weaned from cardiopulmonary bypass. There were seven early deaths for an operative survival rate of 94% and three late deaths (mean time, 7.3 months postoperatively) for an overall survival of 91%. The characteristics of the patients who died are shown in Table 1. Of the seven early deaths, three were due to pulmonary hypertension, two were due to pneumonia, one was secondary to an intracranial hemorrhage, and one was secondary to low cardiac output. Of the three late deaths, two were due to pneumonia, and one was due to pulmonary hypertension.

Hospital stay ranged from 6 to 365 days with a mean hospital stay of 23 ± 43 days. Of the 9 patients who were on a ventilator preoperatively, only 1 died postoperatively. In this subgroup, postoperative hospitalization ranged from 11 to 360 days (mean hospital stay, 107 ± 111 days).

Postoperative echocardiographic follow-up is available for 73 patients. Currently, 16 patients have trivial mitral regurgitation, 41 have mild mitral regurgitation, 10 have moderate mitral regurgitation, and 2 have severe mitral regurgitation. Of the 8 patients requiring reoperation for severe left atrioventricular valve insufficiency, 6 had a residual cleft and 1, patch repair of a valve perforated by subacute bacterial endocarditis. One patient had placement of an Omniscience 23-mm mitral valve 8 years after the initial operation.

Four patients required reoperation for left ventricular outflow tract obstruction. Two of them had transaortic resection of a fibromuscular ring, 1 also requiring augmentation of the mitral valve [11], and the other 2 underwent a modified Konno procedure with preservation of the native aortic valve [12]. One patient required reoperation for tricuspid valve insufficiency. Other postoperative complications included tracheostomy for subglottic stenosis or granulomas (6 patients), pericardial effusions requiring drainage (2), cerebrovascular accidents (2), and reoperation for bleeding (1 patient). Three patients have tiny residual VSDs by color-flow Doppler echocardiography.

We evaluated the preoperative PVR to see if there was a significant increase in PVR with age. The patients were divided by age into three groups: less than or equal to 3 months, 4 to 6 months, and 7 to 12 months (Table 2). Although there was a trend toward increasing PVR with age, multiple comparisons using Tukey's HSD failed to identify a difference between the age groups, and comparison of average PVR between our AVC population and each age group also failed to identify any significant difference, possibly because of the small sample size in the age group 0 to 3 months.

The patients who had intraoperative or postoperative administration of NO have previously been reported [10]. In the 5 patients who had an intraoperative trial of NO, mean pulmonary artery pressure immediately after repair dropped from 20 ± 2.2 mm Hg to 18.0 ± 2.8 mm Hg after administration of NO. There was a corresponding decrease in mean PVR from 4.3 ± 0.9 units to 3.8 ± 0.7 units with NO. The response to inhaled NO in this group appeared limited because of the already substantial decrease in the pulmonary artery pressure with the elimination of the left-to-right shunt after intracardiac repair and because of the conventional therapy administered on discontinuation of cardiopulmonary bypass. Five additional patients received inhaled NO for persistent pulmonary hypertension for 4 to 20 days (mean duration, 10 days) postoperatively; 4 of the 5 patients survived.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
The operative repair of complete AVC in children has undergone major advances during the past 40 years. Improvements in cardiopulmonary bypass and myocardial protection have essentially eliminated low cardiac output as a cause of postoperative mortality. Attention has now been directed toward the establishment of a competent left atrioventricular valve and the elimination of residual intracardiac shunting. Our results demonstrate that the two-patch repair using a Gore-Tex patch for the ventricular component and a pericardial patch for the atrial component with closure of the mitral valve cleft results in a low incidence of residual shunting and competent left atrioventricular valves.

The single-patch technique involves dividing the common valve leaflets and suspending them from a single patch used to close the atrial and ventricular defects. We think that the use of separate atrial and ventricular patches creates less distortion of the valve tissue, thereby allowing a more accurate reconstruction of the mitral and tricuspid valves. In particular, when common leaflets are divided and then sewn back onto a single patch, 3 to 4 mm of leaflet tissue is used up [13]. This situation is avoided in the two-patch technique and may be important in smaller infants in whom the sacrificed valve tissue comprises a greater proportion of the whole. The technique of sandwiching the valves between pericardium and Gore-Tex decreases the chance of dehiscence [9]. The single-patch technique has been associated with patch dehiscence and residual VSDs [6, 14]. Our series with the two-patch technique using pledget-supported sutures and a Gore-Tex patch for the ventricular component of the defect had no reoperations for residual VSDs. Several other surgeons [8, 15] have reported similarly excellent results with the two-patch technique.

Much attention has been given to the technique of mitral valve repair. Some surgeons [16] emphasize leaving the mitral valve as a trifoliate valve; others [14, 17] emphasize cleft closure. In our experience, reoperation for mitral valve insufficiency was most frequently associated with a cleft that was incompletely closed (6/8 patients requiring reoperation). This experience has been corroborated by several others [18, 19]. In particular, Bando and colleagues [20] recently reported routine cleft closure in 93% of 200 patients, with only a 4% reoperation rate for left atrioventricular valve regurgitation.

Pulmonary hypertension may be minimized as a risk factor by performing repair prior to the age of 6 months [21, 22]. In our patients, there was a steady increase in PVR with age: 2.1 at 3 months, 4.0 at 6 months, and 5.7 at 12 months. Nitric oxide does not appear to provide much benefit for the patient who has a dramatic fall in pulmonary artery pressure with elimination of the left-to-right shunt and conventional therapy for pulmonary hypertension. However, an occasional patient with refractory pulmonary hypertension may experience a favorable hemodynamic response to NO. Increasing experience with complete repair of complete AVC has led us to abandon pulmonary artery banding in almost all instances. Notable exceptions include patients with ``Swiss-cheese'' VSDs. Banding is known to be unsuccessful in patients with severe mitral insufficiency [23].

Preoperative need of assisted ventilation because of respiratory distress, congestive heart failure, or pneumonia can result in major morbidity. In our series, 9 patients were on a ventilator preoperatively, and only 1 did not survive AVC repair, but the mean postoperative hospitalization in this subgroup was quite long (107 days). In the series of Weintraub and colleagues [8], the only two early deaths were in patients who had preoperative assisted ventilation for congestive heart failure complicated by viral bronchiolitis.

For patients with AVC and coarctation of the aorta, our 4 patients reflect the evolution of surgical management over time. The first 2 patients had a two-stage approach with initial subclavian flap aortoplasty and pulmonary artery banding followed by remote AVC repair. The third patient had left thoracotomy with subclavian flap aortoplasty and same-day median sternotomy with AVC repair. The most recent patient had one-stage total repair through a median sternotomy with resection of the coarctation, extended end-to-end anastomosis, and AVC repair.

Preoperative two-dimensional and Doppler echocardiography allow visualization of the common atrioventricular valve morphology, chordal attachments, and degree of commitment of the leaflets [24]. A recent report [25] demonstrated that echocardiography alone can adequately define preoperative anatomy and hemodynamic status before repair of complete atrioventricular septal defect in infants less than 1 year of age. At our institution currently, cardiac catheterization is not performed if the anatomic features are completely identified by echocardiography, the child is less than 6 months of age, and there are no critical associated anomalies. The use of TEE has greatly improved the ability to assess the repair in the operating room [26]. The left atrioventricular valve can be assessed for stenosis and insufficiency, as can the tricuspid valve. Intracardiac shunting at the atrial or ventricular level can be detected. If any of these are noted, resumption of cardiopulmonary bypass can be accomplished to repair any residual defects. We believe that TEE should be used in all patients undergoing AVC repair unless contraindicated by patient size.

Infants with complete AVC should undergo preoperative assessment with two-dimensional and color Doppler echocardiography and have complete repair before 6 months of age. The two-patch technique of repair with routine cleft closure as evaluated by intraoperative TEE results in a low operative mortality, a low incidence of permanent heart block, and competent atrioventricular valves.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Presented at the Poster Session of the Thirty-first Annual Meeting of The Society of Thoracic Surgeons, Palm Springs, CA, Jan 30–Feb 1, 1995.

Address reprint requests to Dr Backer, Division of Cardiovascular-Thoracic Surgery, The Children's Memorial Hospital, 2300 Children's Plaza, Mail Code 22, Chicago, IL 60614.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

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