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Ann Thorac Surg 1999;67:169-172
© 1999 The Society of Thoracic Surgeons

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Original Articles

Left ventricular assist device improves survival in children with left ventricular dysfunction after repair of anomalous origin of the left coronary artery from the pulmonary artery

^a Department of Cardiac Surgery, Children’s Hospital-Boston, Harvard Medical School, Boston, Massachusetts, USA
^b Department of Cardiology, Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA

Accepted for publication October 26, 1998.

Address reprint requests to Dr del Nido, Department of Cardiac Surgery, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115
e-mail: delnido{at}a1.tch.harvard.edu

Presented at the Thirty-third Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Feb 3–5, 1997.

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
Background. Repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants carries a high operative risk, particularly in infants with myocardial infarction and poor left ventricular function. The marked recovery of left ventricular function reported late after repair, however, suggests that an aggressive approach to repair should be undertaken.

Methods. Of 31 children undergoing primary repair of ALCAPA at our institution from 1987 to 1996, 26 were infants (6 weeks to 9 months old). All but 2 had severe left ventricular dysfunction, and 8 had moderate to severe mitral regurgitation. Seven children were unable to be weaned from cardiopulmonary bypass because of poor left ventricular function and elevated left atrial pressure. These 7 children were placed on mechanical left ventricular support using a centrifugal pump, with support ranging from 2.2 to 70.6 hours.

Results. One child died shortly after the start of left ventricular assist (2.2 hours), and another died of arrhythmia within 24 hours after successful decannulation. All 5 survivors had significant improvement in left ventricular function, with 2 requiring late mitral valve repair.

Conclusions. Infants with ALCAPA who have severe left ventricular dysfunction represent a higher risk group for repair. However, with use of mechanical circulatory support in those unable to be weaned from cardiopulmonary bypass, a high survival rate can be achieved with good long-term recovery. We conclude that an aggressive approach to early repair in all children with ALCAPA is warranted, regardless of the degree of left ventricular dysfunction.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
Successful repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) usually results in a marked recovery of contractile function on late follow-up, with left ventricular function returning to normal or nearly normal levels within 1 to 2 years after repair [1]. Postoperative low cardiac output requiring high-dose inotropic support, however, is seen frequently, particularly in younger infants and infants with preoperative evidence of acute myocardial infarction [2]. Mechanical support of the left ventricle may be required in some children who are unable to be weaned from cardiopulmonary bypass or who require very high dose inotropic support to avoid other organ injury.

The present report describes our experience with a mechanical left ventricular assist device (LVAD) using a centrifugal pump in 7 children who were unable to be weaned from cardiopulmonary bypass after operative repair of ALCAPA.

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
From 1987 to December 1996, 31 children have undergone primary repair of ALCAPA at Children’s Hospital in Boston. Seven of these 31 were unable to be weaned from cardiopulmonary bypass after corrective surgical intervention and constitute our report. We obtained institutional approval for a retrospective chart review of these 7 patients. Charts were available for review in all 7 patients, and data were collected and analyzed using a statistical program (JMP Software, SAS Institute, Cary, NC) for the Macintosh Power-PC (Apple Corporation, Inc, Cupertino, CA).

Components of left ventricular assist device circuit
Our entire experience with LVAD has been with a centrifugal pump system (Bio-Pump; Bio-Medics, Minneapolis, MN). For infants less than 10 kg, a 50-mL pump was used. Since September 1994 we have used Carmeda-coated cannulas and Carmeda-coated polyvinyl chloride tubing (Medtronic Co, Minneapolis, MN) in all patients, including 2 in the present study, both survivors. Standard polyvinyl chloride tubing without Carmeda bonding (Olsen Med Sales, Ashland, MA) had been used in the 5 patients supported before September 1994. The priming volume for the 50-mL Bio-Pump and 0.25-in. tubing to complete the circuit was approximately 180 mL.

Sites of cannulation for left ventricular assist device
In the 7 patients with LVAD support, venous drainage was established by cannulation below the interatrial groove above the pulmonary veins in 4 or through the left atrial appendage in 3. Arterial cannulation was performed through the ascending aorta.

Anticoagulation
Hematologic variables were normalized by transfusion of blood components to maintain the following: hematocrit greater than 38%, platelets greater than 100,000/mm³, fibrinogen greater than 195 mg/dL, and prothrombin time less than 17 seconds. The activated clotting time was determined every 1 to 2 hours and maintained at 180 to 200 seconds. Lower activated clotting time levels (160 to 180 seconds) were tolerated when Carmeda-coated tubing was used. One patient was treated with -aminocaproic acid (AMICAR; Lederle Parenterals, Carolina, PR) using a 100-mg/kg bolus and then 30 mg · kg^-1 · h^-1 as a continuous infusion.

	Results

Top Abstract Introduction Patients and methods Results Comment References

 
There were 5 female and 2 male patients. The median age at operation was 89 days (range, 48 to 243 days), and the median weight was 4.8 kg (range, 4.1 to 6.2 kg). There were 5 survivors and 2 nonsurvivors. Six patients underwent translocation of the left coronary artery from the pulmonary artery to the aorta, and 1 patient underwent an intrapulmonary tunnel, or Takeuchi procedure. The median duration of cardiopulmonary bypass was 210 minutes (range, 85 to 286 minutes), with a median aortic cross-clamp time of 60 minutes (range, 35 to 84 minutes). Crystalloid cardioplegia (St. Thomas Soln, Abbot Labs, Chicago, IL) was used in 5 patients and blood cardioplegia in 2. Circulatory arrest was used in 2 patients for 48 and 60 minutes (surgeon preference). All patients had mechanical circulatory support instituted in the operating room because of inability to be weaned from cardiopulmonary bypass despite high-dose multidrug inotropic support. All patients received LVAD support without requiring biventricular support.

Support with LVAD was required for a median duration of 43.5 hours (range, 2.4 to 80.0 hours). Return of cardiac ejection occurred in 4 of the 5 survivors within 24 hours and within 48 hours of support in the other surviving patient (Table 1). Incisions were covered with plastic membranes for a median of 3 days (range, 1 to 7 days), and intubation was required for a median duration of 7 days postoperatively (range, 1 to 28 days).

Two of the surviving patients have subsequently required reoperation for severe mitral regurgitation. One patient had significant mitral regurgitation requiring repair within 6 weeks of left coronary transfer. At the time of operation there was prolapse of the anterior leaflet of the mitral valve caused by elongated chordae. Calcification of the tips of the papillary muscles was noted at the time of this operation. The patient underwent a successful chordal shortening procedure along with commissuroplasty. Although initially well after this procedure, the patient developed significant mitral regurgitation requiring repair at 15 months after the original operation. Intraoperative findings at the second valve operation revealed a flail segment of the anterior leaflet caused by rupture of chords to the anterolateral papillary muscle at the areas previously noted to be calcified. Repair at this operation was performed by transfer of chords from the posterior leaflet to the anterior leaflet and a commissuroplasty.

The second patient had progressive mitral regurgitation over 2 years postoperatively. At the time of reoperation, there was significant prolapse of the anterior leaflet of the mitral valve as well as annular dilatation. In addition, there were congenital defects of both the anterior and posterior leaflets. Repair was performed by chordal shortening, primary repair of the leaflet defects, and commissuroplasty. Both patients recovered uneventfully and are currently doing well. Despite the severely depressed contractile function at the time of original presentation, all survivors have demonstrated recovery of cardiac function to normal or nearly normal levels.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

 
Severely depressed left ventricular function with dilatation and mitral regurgitation are ominous findings in an infant with ALCAPA. Younger age and evidence of acute myocardial infarction have been reported to be important risk factors for operative death after repair [2]. For this reason, some centers in the past have advocated delaying operation or offering only medical therapy [3, 4]; in isolated cases, heart transplantation has been performed [5]. However, despite the extent of apparent damage to the left ventricle in these children, long-term follow-up indicates that the majority of infants will have substantial recovery of left ventricular functions [5–8]. Furthermore, establishment of a two-coronary system, which can be achieved in virtually all patients, offers lower perioperative mortality and better long-term outcome [6, 9, 10–12]. With improved techniques for protecting the myocardium and delivery of cardioplegia to both coronary arteries through the aortic and pulmonary roots, postoperative recovery of contractile function may be sufficient to obviate the need for high-dose inotropic support.

Some children, however, have persistent, severe contractile dysfunction despite cardioprotective measures and are either unable to be weaned from cardiopulmonary bypass or require inotropic doses sufficiently high to cause injury to other organs. In our experience, the problem was seen exclusively in younger infants, possibly because of poor collateralization of coronary blood flow and acute myocardial infarction. In this group of patients, our results with mechanical circulatory support indicate that recovery of contractile function is still very likely and therefore justifies the use of LVAD during the immediate postoperative period. This finding is similar to the reported experience with mechanical support after repair of ALCAPA at other centers [7, 8, 10, 11].

Our results with LVAD support in patients with repair of ALCAPA compare favorably with previous experience with either LVAD or other forms of mechanical support for all cardiac indications [13, 14] where survival rates of 40% to 50% have been reported. The choice of LVAD versus total extracorporeal circulatory support was made on the basis of the isolated nature of the injury in our patients. The simplicity of the circuit and avoidance of an oxygenator makes setup of the circuit and transport of these children less complicated. Arrhythmia, however, is a potential source of danger with LVAD support. Patients with poorly controlled or frequent episodes of supraventricular or ventricular arrhythmia should be considered for total circulatory support.

As with other methods of mechanical support, the need for anticoagulation and resultant bleeding remain the most common source of morbidity. Because of the short duration of support required by most infants, however, the need for large-volume transfusion and mediastinal exploration is limited. Most important, the excellent long-term outcome seen in these patients warrants an aggressive approach to surgical repair, regardless of the level of ventricular dysfunction, and if necessary a short period of mechanical circulatory support in the immediate postoperative period.

	References

Top Abstract Introduction Patients and methods Results Comment References

 

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