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Interact CardioVasc Thorac Surg 2009;9:814-818. doi:10.1510/icvts.2009.208215
© 2009 European Association of Cardio-Thoracic Surgery
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Institutional report - Congenital

Anomalous left coronary artery from the pulmonary artery: intermediate results of coronary elongation{star}

William M. Novicka,g*, Xiao F. Lib, Darko Anicc,g, Alexander Baskevitchd, Nestor Sandovale,g, Christian L. Gilbertg and Thomas G. Di Sessaf,g

a Departments of Surgery and Pediatrics, University of Tennessee Health Sciences Center, Memphis, TN, USA
b Department of Cardiac Surgery, Beijing Children's Hospital, Beijing, China
c Department of Cardiac Surgery, KBC-Rijeka, Rijeka, Croatia
d Department of Neonatal and Infant Cardiac Surgery, National Center for Children's Cardiac Surgery, Minsk, Belarus
e Department of Cardiac Surgery, Cardio-Infantile, Bogotá, Colombia
f Department of Pediatrics, University of Kentucky, Lexington, KY, USA
g International Children's Heart Foundation, Memphis, TN, USA

Received 31 March 2009; received in revised form 23 July 2009; accepted 24 July 2009

{star} Funding sources for this study were provided by the Paul Nemir Endowment Fund and Professorship of the University of Tennessee Health Sciences Center and the International Children's Heart Foundation. The authors have no other financial disclosures.

*Corresponding author. 1750 Madison Ave., Suite 500, Memphis, TN 38104, USA. Tel.: +1 901-8694243; fax: +1 901-4324243.

E-mail address: ichfno{at}aol.com (W.M. Novick).


   Abstract
Top
 Abstract
1. Introduction
 2. Methods
 3. Results
 4. Discussion
 References
 
A two coronary system is preferred for correcting anomalous left coronary artery from the pulmonary artery (ALCAPA); however, translocation is not always possible. In countries where neonatal arterial switch operations have not been perfected coronary transfer can be difficult. The purpose of this report is to describe the intermediate results using the coronary elongation and translocation technique in developing countries. Records of patients undergoing operation by the International Children's Heart Foundation team were reviewed (April 1993–October 2008) for those undergoing ALCAPA repair. All patients received a 2-D echocardiographic–color Doppler examination prior to discharge and at follow-up. A total of 13 patients were identified, age ranged from 9 days to 41 years. All but one patient were operated upon at one of our affiliate hospitals in Croatia, Belarus, China and Colombia. All patients presented with moderate to severe mitral regurgitation and cardiac failure. Follow-up ranged from six months to 9.5 years postoperatively. Color Doppler showed a patent left coronary artery; echocardiography estimated a normal left ventricular ejection fraction and improved mitral regurgitation in all patients. The technique provides an alternative approach to translocation for ALCAPA in countries where routine neonatal coronary transfer techniques may not be perfected. Intermediate results are comparable to translocation.

Key Words: Anomalous left coronary artery; Surgical correction; Congenital


   1. Introduction
Top
 Abstract
 1. Introduction
2. Methods
 3. Results
 4. Discussion
 References
 
Anomalous origin of the left coronary artery from the pulmonary artery was first described in 1866 [1]. Patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) usually present in infancy with symptoms of congestive heart failure. However, a few patients survive into childhood, adolescence and adulthood relatively asymptomatic. A variety of surgical approaches have been devised to correct this anomaly. The preferred method is coronary translocation [2]. However, in many parts of the world surgeons are not familiar with or have not perfected the technique of coronary transfer associated with the arterial switch operation. Moreover, coronary transfer is not possible in all patients with ALCAPA and repairs that do not result in a two coronary system carry a high mortality [2]. In an effort to provide an easier operation we introduced the technique of coronary elongation to several of our affiliate hospitals. Thus, we report our initial and intermediate results in developing countries utilizing the technique of left coronary elongation and translocation.


   2. Methods
Top
 Abstract
 1. Introduction
 2. Methods
3. Results
 4. Discussion
 References
 
2.1. Patients and evaluation

Records of patients undergoing operation by the International Children's Heart Foundation team were reviewed (April 1993–October 2008) to determine the patients undergoing operation for ALCAPA. The records of subject's undergoing left coronary elongation were extracted (n=13). Follow-up with their local cardiologists was conducted from 1 November to 15 December 2008. All patients received a 2-D echocardiographic evaluation with color Doppler to assess left ventricular ejection fraction (LVEF), degree of mitral regurgitation (MR) and patency of the left coronary elongation.

Six patients were female, age ranged from nine days to 41 years. Mean weight was 8.9 kg, range 2.8–55 kg. There were three neonates 9, 17 and 21 days; five infants 2, 3, 6, 8 and 11 months; four children 1.3, 3.9, 6.9, and 10 years, and one adult 41 years. All 13 patients presented with MR and cardiac failure. Preoperative evaluation included electrocardiogram (ECG), chest X-ray, two-dimensional echocardiogram with color Doppler, cardiac catheterization and in one case three-dimensional reconstruction of a computerized tomography study. Preoperatively MR was determined to be severe in nine and moderate to severe in four; left ventricular ejection fraction 20–30% in six, 30–40% in five, 50% in one and in one neonate <20%.

2.2. Sites of operation and conditions

All patients, except one child, were operated on at our affiliate sites in Belarus, China, Colombia and Croatia. No site had extra-corporeal membrane oxygenation (ECMO) support at the time of operation [3]. Several patients had been previously misdiagnosed as congenital MR (n=7) and one neonate had been sent home for supportive care with the diagnosis of cardiomyopathy.

The International Children's Heart Foundation is a group of volunteer pediatric cardiovascular specialists and biomedical engineers who volunteer for humanitarian cardiovascular surgical missions to underdeveloped countries. Our 14 years experience performing surgical procedures on a variety of congenital anomalies, as well as upgrading the surgical performance at multiple sites has been reported [4]. The foundation volunteer team members are dedicated to the care of children with congenital/acquired heart disease. This common goal allows team members from multiple centers to integrate their skill to provide excellent outcomes in what would be considered a less than optimal environment in the Western world.

2.3. Surgical management

All surgical procedures were performed by one of the authors (W.M.N., X.F.L.). Cardiopulmonary bypass with routine bi-caval cannulation and moderate hypothermia (28 °C) was employed. Antegrade aortic root and left coronary ostial cardioplegia was repeated every 15 min. The mean cardiopulmonary bypass time was 92±16 min and mean ischemic time was 39±9 min. The heart was exposed and the course of the left coronary evaluated (Fig. 1). The main pulmonary artery was transected above and below the origin of the left coronary artery. The width of the pulmonary artery flaps approximated 1/2 the circumference of the coronary artery (Fig. 2). The ring of pulmonary artery was cut along its diameter opposite the origin of the coronary artery and the two flaps were sutured together along their margins to elongate the left coronary artery using the appropriate Hegar dilator for sizing (Fig. 3). When necessary the flaps were curved up and over commissural posts to leave them intact. A button was removed from the aorta above the aortic valve and the elongated segment sutured into place (Fig. 4). The pulmonary artery was mobilized to the upper lobe branches and the proximal and distal ends were then approximated after the cross-clamp was removed. A milrinone loading dose was given with re-warming. Modified ultra-filtration was performed on all patients. Residual MR was assessed by transesophageal echocardiogram (available at two sites) and/or left atrial line wave form following bypass.


Figure 1
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  		Fig. 1. This figure demonstrates the dilated tortuous left main coronary artery originating from the pulmonary trunk.

 

Figure 2
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  		Fig. 2. Ring of pulmonary artery excised from the pulmonary trunk.

 

Figure 3
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  		Fig. 3. Creation of the elongated segment of the coronary artery from the two flaps of pulmonary artery tissue.

 

Figure 4
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  		Fig. 4. Anastamosis of the elongated segment of the coronary artery into the aorta.

 
2.4. Postoperative management

Postoperatively patients were extubated if they fulfilled criteria previously published by this group [5]. Early extubation was aggressively pursued in all patients who met criteria. Discontinuation of inotropic drugs and intravenous vasodilators was accomplished as quickly as the clinical status allowed as determined by bedside transthoracic echocardiographic assessment of ventricular function and degree of MR. Discharge echocardiograms were performed on all patients. All patients were discharged on aspirin, 5 mg/kg/day for three months.

2.5. Follow-up and data analysis

Patients were re-evaluated at three and six months and yearly thereafter. Echocardiographic assessment included evaluation of LVEF, left ventricular end diastolic dimension (LVEDD), degree of MR, patency of the LCA elongation and any supra-valvar pulmonary artery stenosis. All values are presented as the mean±S.D. Comparison of preoperative and postoperative variables was performed using the Student t-test; P values were considered significant if <0.05.


   3. Results
Top
 Abstract
 1. Introduction
 2. Methods
 3. Results
4. Discussion
 References
 
3.1. Mortality

Operative mortality was 7.7% (1/13). One neonate died following operation. The child was intubated, receiving intravenous inotropic and furosemide infusions preoperatively. The child had been sent home previously with the diagnosis of cardiomyopathy. Preoperative ejection fraction was estimated to be <20%, MR was severe and ECMO support was not available. There was 100% survival of the 12 discharged patients at follow-up from three months to 9.5 years postoperatively.

3.2. Mitral valve function

Four patients had severe MR upon cessation of bypass. Two of these patients required return to bypass and mitral valve reconstruction for severe MR with hemodynamic instability. Residual MR at discharge was graded as 0 (n=6), I–II (n=5), II–III (n=1) and III–IV (n=0). One patient required mitral valve re-repair at 16 months postoperatively. The original post-mitral repair echocardiogram performed in the operating room revealed grade I–II MR. The discharge echo showed grade II residual MR. Subsequent follow-up echocardiograms displayed worsening MR. At reoperation a complex repair was performed. Discharge echo showed no residual MR and at follow-up three months postoperatively MR remains absent.

MR at three months, six months and at most recent follow-up is shown in Table 1. The patient with grade III at three months is detailed above.


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  		Table 1 Mitral regurgitation pre- and postoperatively

 
3.3. LVEF and right ventricular outflow tract obstruction

The average LVEF preoperatively was 31%±15% (range: 15–55%). Discharge LVEF was improved in all patients (mean 43%±9%) and was significantly improved (P<0.05). No patient had residual right ventricular outflow tract obstruction by Doppler echo upon discharge. At the three months follow-up, LVEF was normalized in all patients (62%±7%) and has remained so on subsequent follow-up exams. One patient was determined to have a 25-mmHg gradient across the pulmonary anastomosis at three months follow-up, which has remained stable over the last four years.

3.4. Left coronary patency and LVEDD

Left main elongation patency was documented in all 12 patients by echocardiogram at discharge. LVEDD was not assessed in all patients preoperatively, but was documented at discharge as increased and the average Z score indexed to BSA was +1.9±0.5. Left main elongation patency was documented at subsequent follow-up visits by echocardiogram except in two patients who required cardiac catheterization. In both patients non-obstructed flow from the elongation into the left main were demonstrated. One patient was the adult who developed atypical chest pain three months after operation and the other was the child who required mitral valve re-repair 16 months after the original operation. The LVEDD was normalized in all patients, except the one requiring re-operation for MR, at six months (Z=1.15±0.12) and was significantly improved compared to discharge (P<0.01).

3.5. Postoperative care, thrombo-embolic events and functional status

All surviving patients were extubated within 24 h and inotropes were discontinued within 48 h except for one patient (neonate), who was extubated on day 3 and inotropes discontinued on day 4. The average length of stay in the intensive care unit (ICU) was 3.2±1.1 days.

All patient were in New York Heart Association (NYHA) class I (11); II (1) at most recent follow-up. No thrombo-embolic events have occurred.


   4. Discussion
Top
 Abstract
 1. Introduction
 2. Methods
 3. Results
 4. Discussion
References
 
Anomalous origin of the left main coronary artery from the pulmonary artery is a rare form of congenital heart disease making-up about 0.25% of children with congenital heart disease [2]. Most infants develop signs and symptoms in the first few months of life and the vast majority die within the third- to sixth-month of life. Attempts to resolve the ischemia from ALCAPA have evolved from palliative procedures, to providing bypasses using multiple types of conduits, to intra-pulmonary tunneling, to direct coronary translocation [6–9]. The combined operative mortality of these more commonly used techniques ranges from 0% to 23% and repairs that do not result in a two coronary system carry a high mortality and continued poor ventricular function [2]. Our experience using the pulmonary artery double flap elongation technique confirms these previous reports which provide a dual coronary system.

Bypass procedures not infrequently lead to significant vessel diameter mismatch and the complication rate from intrapulmonary tunnel requiring re-intervention may be as high as 30% [2]. Coronary translocation may require a substantial length to the left main coronary artery for mobilization so that simple translocation may not always be possible.

A number of other techniques involving the use of pulmonary arterial wall flaps to extend the left coronary, in cases where direct translocation is not possible, have been reported since Turley's original description [10–12]. We prefer circumferential flaps and primary pulmonary artery reconstruction as opposed to reconstruction of a partial pulmonary arterial defect, which may lead to late supravalvar stensosis [13]. We would agree that there are cases where simple excision of a button around the anomalous left coronary and translocation to the aorta is easily performed without mobilization of the left main coronary from its bed. However, unless the anomalous coronary is located posteriorly in the facing sinus then mobilization becomes necessary for simple translocation. Thus, those surgeons who are not familiar with coronary transfer may face a difficult task in judging the appropriate length of coronary to mobilize. The technique of creating flaps, whether they are dual based from both aorta and pulmonary artery or as we have shown, based upon a circumferential ring of pulmonary artery, provides a widely patent left main coronary with no mobilization of the left coronary artery and, therefore, no danger of kinking or excessively stretching the left main coronary artery [14].

MR in the setting of ALCAPA usually results from left ventricular dysfunction and dilatation. Thus, MR frequently decreases as left ventricular function improves and left ventricular size decreases after repair. Therefore, MR is primarily addressed with ALCAPA repair during the operation. However, in some cases annular dilatation is so severe that even after repair MR remains severe and requires repair. We agree with Huddleston et al. [15] that with recurrence of late MR or persistence of significant MR, cardiac catheterization to determine coronary patency is necessary prior to mitral valve repair. In such cases coronary stenosis may be problematic.

We conclude that successful repair of ALCAPA can be accomplished in underdeveloped countries provided the surgical team is well trained. Moreover, the method of coronary artery elongation described herein provides in-experienced surgeons an excellent outcome in this environment. Intermediate results show good ventricular function, patent left coronary elongations and improved MR.


   References
Top
 Abstract
 1. Introduction
 2. Methods
 3. Results
 4. Discussion
 References
 

  1. Brooks SJ. Two cases of abnormal coronary artery of the heart arising from the pulmonary artery: with some remarks upon the effect of this anomaly producing cirsoid dilatation of the vessels. J Anat Physiol 1866;20:26–32.
  2. Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thor Surg 2002;74:946–955.[Abstract/Free Full Text]
  3. Del Nido PJ, Duncan BW, Meyer JE, Wessel DL, LaPierre RA, Jonas RA. Left ventricular assist device improves survival in children with left ventricular dysfunction after repair of anomalous left coronary artery from the pulmonary artery. Ann Thor Surg 1999;67:169–172.[Abstract/Free Full Text]
  4. Novick WM, Stidham GL, Karl TR, Arnold RW, Anic D, Rao S, Baum VC, Fenton KE, Di Sessa TG. Pediatric cardiac assistance in developing and transitional countries: the impact of a fourteen year effort. Cardiology in the Young 2008;18:316–323.[Medline]
  5. Novick WM, Sandoval N, Lazorhysynets VV, Castillo V, Baskevitch A, Mo X, Ried RW, Marinovic B, Di Sessa TG. Flap valve double patch closure of ventricular septal defects with increased pulmonary vascular resistance. Ann Thorac Surg 2005;79:21–28.[Abstract/Free Full Text]
  6. Potts WJ, quoted by Kittle CF, Diehl AM, Heilbrunn A. Anomalous left coronary arising from the pulmonary artery; a report of a case and surgical consideration. J Pediatr 1955;47:198–206.[CrossRef][Medline]
  7. Cooley DA, Hallman GL, Bloodwell RD. Definitive surgical treatment of anomalous origin of the left coronary artery from pulmonary artery: indications and results. J Thorac Cardiovasc Surg 1966;52:798–808.[Medline]
  8. Takeuchi S, Imamura H, Katsumoto K, Hayashi I, Katohgi T, Yozu R, Ohkura M, Inoue T. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979;78:7–11.[Abstract]
  9. Neches WH, Mathews RA, Park SC, Lenox CC, Zuberbulher JR, Siewers RD, Bahnson HT. Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation 1974;50:582–587.[Abstract/Free Full Text]
  10. Turley K, Szarnicki RJ, Flachsbart KD, Richter RC, Popper RW, Tarnoff H. Aortic translocation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 1995;60:84–89.[Abstract/Free Full Text]
  11. Sese A, Imoto Y. New technique in the transfer of an anomalously originated left coronary artery to the aorta. Ann Thorac Surg 1992;53:527–529.[Abstract]
  12. Ando Y, Kado H, Masuda M, Toshihide N, Hinokiyama K, Shiose A, Kajimoto M. ‘Spiral-Cuff’ Technique for repair of anomalous left coronary from the pulmonary artery. Ann Thorac Surg 2008;86:667–668.[Abstract/Free Full Text]
  13. Barth MJ, Allen BJ, Culecyuz M, Chieimmongkoltip P, Cuneo B, Ilbawi MN. Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery. Ann Thor Surg 2003;76:1429–1434.[Abstract/Free Full Text]
  14. Alsoufi B, Sallehuddin A, Bulbul Z, Joufan M, Khouqeer F, Canver CC, Kalloghlian A, Al-Halees A. Surgical strategy to establish a dual-coronary system for the management of anomalous left coronary artery origin from the pulmonary artery. Ann Thorac Surg 2008;86:170–176.[Abstract/Free Full Text]
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Interactive CardioVascular and Thoracic Surgery 2009 9: 818. [Full Text] [PDF]



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eComment: Management of mitral regurgitation associated with anomalous left coronary artery from the pulmonary artery
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