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J Thorac Cardiovasc Surg 2005;130:897-899
© 2005 The American Association for Thoracic Surgery


Brief Communication

Partial left ventriculectomy for infantile ischemic cardiomyopathy caused by anomalous origin of the left coronary artery from the pulmonary artery

Masaaki Yamagishi, MD, PhD a , * , Keisuke Shuntoh, MD a , Takeshi Shinkawa, MD a , Takahiro Hisaoka, MD a , Mitsugu Ogawa, MD a , Keitarou Kohshi, MD a , Hitoshi Yaku, MD, PhD a , You Kajiyama, MD b , Kenji Hamaoka, MD, PhD b

a Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
b Department of Pediatrics Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan

Received for publication March 21, 2005; accepted for publication April 1, 2005.

* Address for reprints: Masaaki Yamagishi, MD, PhD, Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kawaramachi, Hirokoji, Kamikyo-ku, Kyoto, 602-8566 Japan (Email: myama{at}koto.kpu-m.ac.jp).


Figure 1
Dr Yamagishi


Broad ischemia of the left ventricle and subsequent severe ventricular failure are unavoidable in infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Here we report the first case of successful partial left ventriculectomy in a 3-month-old infant with severe ischemic cardiomyopathy caused by ALCAPA and vanishingly scarce collateral vessels.

Clinical Summary

A 3-month-old female infant weighing 5300 g with congestive heart failure was referred to our hospital. Abnormal Q waves were detected in leads I and aVL and precordial leads V4 through V6 on electrocardiography. Echocardiography demonstrated marked left ventricular (LV) dilatation with significant reduction in ventricular wall thickness (Figure 1, left). The LV end-diastolic diameter was 48 mm. Wall motion showed marked diffuse deterioration. Shortening fraction was reduced to 7%. Posterolateral wall thickness faded to 2 mm. The papillary muscles and endocardium showed markedly increased echogenicity. Severe mitral regurgitation was also observed. The left coronary artery originated from the left posterior sinus of the pulmonary artery. 201Thallium myocardial scintigraphy demonstrated broad ischemia and the absence of myocardial viability at the posterolateral wall of the left ventricle.


Figure 1
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Figure 1. Left, Preoperative echocardiography; right, postoperative echocardiography.

 
The infant underwent reimplantation of the left coronary artery and partial left ventriculectomy. After institution of moderately hypothermic cardiopulmonary bypass and subsequent chemical cardiac arrest, the pulmonary artery was transected at the bifurcation. The pulmonary cuff, including the left coronary orifice, was harvested and implanted directly into the left lateral aortic wall. This was followed by mitral annuloplasty at the posterocommissural line.

The LV wall showed dark red discoloration and was markedly reduced in thickness. Island-shaped scar formations were also observed. The lateral LV wall measuring 25 x 40 mm was resected in an elliptical shape along the left ascending coronary artery (Figure 2). Histologic examination showed significant transmural myocardial infarction. The ventriculotomy was closed longitudinally with reinforcing polytetrafluoroethylene felt strips. Coercive weaning from the cardiopulmonary bypass was not attempted because the patient had a high left atrial pressure of 23 mm Hg. The infant was placed on an extracorporeal circulatory support system and was weaned easily from this circulatory support system on the third postoperative day.


Figure 2
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Figure 2. Macroscopic findings of the resected LV wall showing transmural, large, island-shaped scar formations and significant subendocardial fibrosis. The wall thickness was reduced to 2 mm. A, Epicardial aspect; B, endocardial aspect.

 
Postoperative echocardiography (Figure 1, right) showed marked improvement in LV function and a significant decrease in cavity volume. The LV shortening fraction was improved to 26%. The LV end-diastolic diameter was decreased to 29 mm, LV wall thickness returned to 6.5 mm, and no mitral regurgitation was detected. Postoperative 201thallium myocardial scintigraphy showed considerable improvement of myocardial viability in all segments of the LV wall.

Discussion

In infants with ALCAPA, the myocardium of the left ventricle is exposed to severe ischemia because of initial depletion of the coronary flow and subsequent coronary-pulmonary steal. However, LV function frequently recovers after surgical repair in most infants 1 Go because potential recoverable myocardial contractility is preserved by immediate development of the collateral blood supply to varying degrees. On the other hand, if broad transmural infarction, ensuing fibrosis or fibroelastosis, and ischemic cardiomyopathy go to completion because of underdevelopment of the collateral vessels, sufficient restoration of LV function is not expected despite 2-coronary revascularization. In addition, refractory low cardiac output early after the operation cannot be overcome in most cases. In such cases, prompt reduction of LV wall stress and immediate improvement of LV muscle mass–cavity volume mismatch are indispensable for amelioration of mortality and morbidity.

Partial left ventriculectomy is an established procedure for treatment of nonischemic dilated cardiomyopathy in adult patients to decrease wall stress and improve contractility. 2,3 Go Encouraging results of partial left ventriculectomy combined with mitral valve repair for infantile dilated cardiomyopathy have also been reported. 4,5 Go These observations suggested that partial left ventriculectomy is applicable even in infantile ischemic cardiomyopathy. In infants with ischemic cardiomyopathy caused by ALCAPA, partial resection of the LV wall, in which viability of the myocardium was severely reduced by severe ischemia, decreases wall stress and increases the potential of recoverable myocardial contractility of the residual LV wall. Nevertheless, it must be noted that indications of partial left ventriculectomy for ALCAPA should be confined to transmural infarction and irreversible fibrosis at the posterolateral wall, along with marked LV dilatation and reduced ventricular wall thickness, because LV function in infantile ALCAPA still has the potential for recovery after 2-coronary revascularization. 1 Go

In conclusion, we present the first report of successful partial left ventriculectomy in an infant with severe ischemic cardiomyopathy caused by ALCAPA. Partial left ventriculectomy has a beneficial effect on restoration of ventricular function for infantile ALCAPA with incurable ischemic cardiomyopathy.

References

  1. Michielon G, Di Carlo D, Brancaccio G, Guccione P, Mazzera E, Toscano A, et al. Anomalous coronary artery origin from the pulmonary artery. correlation between surgical timing and left ventricular function recovery. Ann Thorac Surg 2003;76:581-588.[Abstract/Free Full Text]
  2. Batista RJV, Santos JLV, Takeshita N, Bocchino L, Lima PN, Cunha MA. Partial left ventriculectomy to improve left ventricular function in end-stage heart disease. J Card Surg 1996;11:96-98.[Medline]
  3. Suma H, Isomura T, Horii T, Sato T, Kikuchi N, Iwahashi K, Hosokawa J. Nontransplant cardiac surgery for end-stage cardiomyopathy. J Thorac Cardiovasc Surg 2000;119:1233-1245.[Abstract/Free Full Text]
  4. Yoshii S, Hosaka S, Takahashi W, Amano H, Abraham SJK, Kaga S, et al. Partial left ventriculectomy in an infant with dilated cardiomyopathy. J Thorac Cardiovasc Surg 1999;117:616-618.[Free Full Text]
  5. Berger S, Tweddell JS, Frommelt PC, Weinhaus L. Partial left ventriculectomy for dilated cardiomyopathy in a newborn. J Thorac Cardiovasc Surg 1999;117:1017-1018.[Free Full Text]



This article has been cited by other articles:


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S. Westaby, N. Archer, and S. G. Myerson
Long-term cardiac remodeling after salvage partial left ventriculectomy in an infant with anomalous left coronary artery from the pulmonary artery.
J. Thorac. Cardiovasc. Surg., March 1, 2009; 137(3): 757 - 759.
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