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Ann Thorac Surg 2000;69:1591-1592
© 2000 The Society of Thoracic Surgeons

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Case reports

Emergency ligation of anomalous left coronary artery arising from the pulmonary artery

^a Division of Cardiovascular Surgery, Ricardo Gutierrez Children’s Hospital, Buenos Aires, Argentina
^b Ricardo Gutierrez Children’s Hospital, Buenos Aires, Argentina

Address reprint requests to Dr Christian Kreutzer, Ricardo Gutierrez Children’s Hospital, Gallo 1330 (1425) Buenos Aires, Argentina

	Abstract

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We report two cases of successful emergency ligation of anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in patients with previous cardiac arrest. Both patients had regained marginal cardiac output after cardiopulmonary resuscitation and had maximal doses of inotropic support. The ALCAPA ligation was then performed as a life-saving procedure in the absence of any kind of mechanical circulatory support.

	Introduction

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Anomalous left coronary artery arising from the pulmonary artery (ALCAPA) is a rare anomaly, with an estimated incidence of 1 in 300,000 live births. This anomaly produces a coronary steal phenomenon, left-to-right shunt, and commonly, significant left ventricular dysfunction with mitral regurgitation from abnormal perfusion of the left ventricle. Different surgical approaches have been reported including ligation of the anomalous left coronary artery in its origin [1], subclavian-left coronary anastomosis [2], transpulmonary baffling or Takeuchi procedure [3], and direct reimplantation of the coronary artery in the aorta [4]. The use of left ventricular assist device (LVAD) in this anomaly had a tremendous impact on surgical mortality. In recent series postoperative LVAD was used with significant reduction in mortality [5, 6]. We describe two cases in which emergency ligation of the anomalous coronary artery was performed in the intensive care unit (ICU) in 2 patients in cardiac arrest or severe low output and hypotension when extracorporeal circulatory support (extracorporeal membrane oxygenation [ECMO] or LVAD) was unavailable.

	Case reports

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Patient 1
In June 1979, a 5-month-old girl was admitted because of severe congestive heart failure with dilated cardiomyopathy. The diagnosis of ALCAPA was suspected with the electrocardiographic (ECG) changes and confirmed by cardiac catheterization in which large intercoronary vessels were visualized and left-to-right shunt was detected. One day after admission, the baby experienced worsening cardiac failure, severe low output state despite inotropic support, and arrested heart. In that scenario, immediate cardiopulmonary resuscitation was established, which successfully achieved minimal ventricular function with a very poor ejection fraction. It was decided to perform a left thoracotomy in the ICU and perform ALCAPA ligation as an ultimate measure to increase cardiac output.

Patient 2
An 8-month-old girl was referred in June 1998 for severe congestive heart failure and dilated cardiomyopathy with a history of cardiac arrest 1 day before admission. The baby was intubated in profound acidosis. The diagnosis of ALCAPA was suspected with the ECG changes and confirmed by echocardiography. The patient was scheduled for reimplantation of the anomalous coronary artery the next morning but her condition worsened requiring increasing doses of inotropic agents and bicarbonate. After a second cardiac arrest and after unsatisfactory cardiac output was regained, the decision to perform ligation of the ALCAPA was made.

	Comment

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The pleural cavity was entered through a posterolateral thoracotomy. The lung was retracted and the pericardium was opened in a longitudinal fashion. The anomalous left coronary artery was identified and doubly ligated proximal to its origin. The chest was closed in the usual fashion.

In patient 1, after retracting the lung, the heart arrested and internal cardiac massage was instituted. The coronary artery was ligated in an arrested heart. Both patients markedly improved left ventricular function immediately after ligation of ALCAPA and had an uneventful recovery. Patient 1 regained a normal ventricular function in the late follow-up but died suddenly while dancing at the age of 13 years. There was no authorization for autopsy. Patient 2 is alive and well with a nearly normal ECG 9 months after the operation. A left internal mammary artery or subclavian type of myocardial revascularization is scheduled at 2 years of age to achieve a dual coronary system.

The complete benefit of achieving a dual coronary system ALCAPA has become clear. The success of a ligation procedure is related to the amount of intercoronary system anastomosis. Patients with coronary steal phenomena benefit from a simple ligation of the anomalous left coronary artery. The results of ligation in infants have been disappointing and the absence of large intercoronary vessels was thought to be a contraindication for this procedure. The late results of ligation have shown a significant late mortality [8]. Furthermore, the late results of the two-coronary system repairs show excellent survival and good recovery of ventricular function [5, 7].

It is our policy to repair all patients with ALCAPA with a two-coronary system either by a direct reimplantation or by a Takeuchi procedure, and the current mortality in our institution is 30% for the last 10 patients operated since 1990. If there is limited availability of ECMO or LVAD because of the inherent financial restrictions in an underdeveloped country, the indication for a dual coronary repair in an acidotic patient in cardiac arrest with severely limited myocardial reserve seems inappropriate. A procedure with a certain time of myocardial ischemia by aortic cross clamping can pay the final insult to an already injured left ventricle without myocardial reserve. In this specific scenario, ligation of the ALCAPA arises as a life-saving possibility.

The late outcome of patient 1 has taught us the lesson that even though the ventricular function seems normal and the patient is asymptomatic there is an indication of achieving a two-coronary system by a left internal mammary artery revascularization in this subset of patients. Wilson and colleagues [8] demonstrated a higher incidence of sudden death in the long-term follow-up of patients with ligation.

In conclusion, ligation of an ALCAPA can be performed easily in the ICU in the presence of intractable severe low cardiac output or cardiac arrest as a life-saving procedure. This procedure should be performed only in an emergency in the absence of any form of circulatory support and a procedure to achieve a dual coronary system should be added later to avoid ischemic complications and sudden death.

	References

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1. Sabiston D.C., Neill C.A., Taussig H.B. The direction of flow in anomalous left coronary artery arising from the pulmonary artery. Circulation 1960;22:591-597.[Abstract/Free Full Text]
2. Meyer B.W., Stefanik G., Stiles Q.R., Lindesmith C.G., Jones J.C. A method of definitive surgical treatment of anomalous origin of left coronary artery. A case report. J Thorac Cardiovasc Surg 1968;56:104-107.[Medline]
3. Takeuchi S., Imamura H., Katsumoto K., et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979;78:7-11.[Abstract]
4. Vouhé P.R., Baillot-Vernant F., Trinquet F., et al. Anomalous left coronary artery from the pulmonary artery in infants. J Thorac Cardiovasc Surg 1987;78:192-199.
5. Cochrane A.D., Coleman D.M., Davis A.M., Brizard C.P., Wolfe R., Karl T.R. Excellent long-term functional outcome after an operation for anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1999;117:332-342.[Abstract/Free Full Text]
6. Del Nido P.J., Duncan B.W., Mayer J.E., Jr, Wessel D.L., LaPierre R.A., Jonas R.A. 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. Ann Thorac Surg 1999;67:169-172.[Abstract/Free Full Text]
7. Schwartz M.L., Jonas R.A., Colan S.D. Anomalous origin of left coronary artery from pulmonary artery. Am Coll Cardiol 1997;30:547-553.[Abstract]
8. Wilson C.L., Dlabal P.W., McGuire S.A. Surgical treatment of anomalous left coronary artery from pulmonary artery. Follow up in teenagers and adults. Am Heart J 1979;98:440-446.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Christian Kreutzer Andres J. Schlichter Guillermo O. Kreutzer Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kreutzer, C. Articles by Kreutzer, G. O. Search for Related Content PubMed PubMed Citation Articles by Kreutzer, C. Articles by Kreutzer, G. O.