Ann Thorac Surg 2008;86:176. doi:10.1016/j.athoracsur.2008.03.076
© 2008 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Invited Commentary
William M. DeCampli, MD, PhD
Pediatric Cardiothoracic Surgery, The Congenital Heart Institute, Arnold Palmer Hospital for Children and Women, Orlando, FL 32806-2036
(Email: wdecampli{at}orhs.org).
Perhaps because anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is so rare an anomaly, presents so dramatically, and is so effectively treatable, it appears regularly in our surgical literature. Alsoufi and coworkers [1] have added to this knowledge with a cogent analysis of their experience with 30 patients with this disorder with encouraging results.
There remain a few dilemmas with this lesion. The first dilemma is the mortality, which remains about 10% in recent reports. Most of the mortality occurs in patients presenting with shock or significant end-organ injury. Unfortunately, there is no "signature" of the lesion either in fetal life or in the neonatal period. The mortality may decrease coincidently with the increasing use of echocardiography to investigate "transitional" murmurs in the first month of life, as long as cardiologists check the coronary anatomy. An astute pediatrician might detect a murmur of mitral regurgitation before symptoms develop. The challenge is to increase the awareness, at the "front line," of the possibility of this abnormality.
The second dilemma is the quality of the coronary reconstruction. As mentioned by the authors, reimplantation techniques (most of them showcased in numerous reports in The Annals of Thoracic Surgery during the past 15 years) have high success rate in the hands of experienced surgeons who routinely perform the arterial switch operation. On the other hand, long-term follow-up of patients who underwent the latter operation show a significant incidence of coronary ostial or proximal coronary artery stenosis. One might expect the incidence to be similar in repaired ALCAPA. A recent report demonstrates that myocardial flow reserve, evaluated with adenosine challenge and 13NH4 dynamic positron emission tomographic imaging, is impaired after ALCAPA repair. Thus, these patients need judicious lifelong follow-up.
The final dilemma is the non-zero incidence of persistent mitral regurgitation (MR) after ALCAPA repair. In some reports in which the mitral valve is not repaired at the time of ALCAPA repair, the incidence of persistent significant MR is 25% to 35%. In the present report, the authors repaired the mitral valve at initial operation in all 5 patients with severe MR, and on late follow-up only 1 of 25 patients had even moderate MR. (Two other patients with significant MR also had important coronary stenosis or occlusion and ventricular dysfunction and underwent reoperation). These results would suggest that severe mitral regurgitation should be addressed at the time of ALCAPA repair. There is certainly not uniform agreement on this approach. Therefore, the challenge is to come up with better predictors of the need for initial mitral valve repair other than the initial presence of severe MR. Careful analysis of the mitral valve using three-dimensional echocardiography and future techniques to differentiate ischemic papillary muscle dysfunction from permanent injury may help guide the decision.
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References
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- Alsoufi B, Sallehuddin A, Bulbul Z, et al. 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]
Related Article
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Surgical Strategy to Establish a Dual-Coronary System for the Management of Anomalous Left Coronary Artery Origin From the Pulmonary Artery
- Bahaaldin Alsoufi, Ahmed Sallehuddin, Ziad Bulbul, Mansour Joufan, Fareed Khouqeer, Charles C. Canver, Avedis Kalloghlian, and Zohair Al-Halees
Ann. Thorac. Surg. 2008 86: 170-176.
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