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J Thorac Cardiovasc Surg 1998;115:244-246
© 1998 Mosby, Inc.

BRIEF COMMUNICATIONS

Aortic coarctation, multiple ventricular septal defects, andanomalous coronary artery arising from the right pulmonary artery

Eduardo da Cruz, MD, Daniel Carbognani, MD, François Laborde, MD, Joëlle Bougaran, MD, Alain Dibie, MD, Jerôme Le Bidois, MD, Alain Batisse, MD, Laurent Fermont, MD


Paris, France

From the Department of Pediatric Cardiac Surgery, Centre Médico-Chirurgicalde la Porte de Choisy,a and the Department of Pediatric Cardiology,Institut de Puériculture de Paris,b Paris, France.

Received for publication June 13, 1997 Accepted for publication June 25, 1997. Address for reprints: Eduardo da Cruz, MD, Service of PediatricCardiac Surgery, H.C.I International Medical Centre, Beardmore Street,Clydebank, G81 4HX, Scotland.

The literature reveals no reports of a left coronary artery arising fromthe right pulmonary artery in association with aortic coarctation and multipleventricular septal defects. The patient we describe here died of left myocardialinfarction after palliative operation because this defect was unanticipated.

Case report. The patient was admitted atthe age of 12 days with a diagnosis of aortic coarctation and multipleventricular septal defects. She had classic signs of aortic coarctation andpredominantly left-sided heart failure, which responded partially toprostaglandin E1 and diuretics. Chest radiography revealed severecardiomegaly and symmetrically plethoric lungs. The electrocardiogram showedsinus rhythm at 140 beats/min, right ventricular hypertrophy, and no signs ofmyocardial ischemia. Echocardiography demonstrated an aortic coarctation withlong, severe hypoplasia of the arch, a large ductus arteriosus, and multipleventricular septal defects. The left ventricle was neither hypokinetic norhypoplastic. No coexisting mitral regurgitation was documented. There was also aleft superior caval vein draining into the coronary sinus and a large ostiumsecundum–type atrial septal defect.

The patient underwent pulmonary artery banding, division of the ductusarteriosus, and aortic coartectomy. Within 2 hours of the patient's return tothe intensive care unit, her condition deteriorated rapidly and dramatically,with a global but predominantly left-sided intractable heart failure, completeatrioventricular block, and cardiogenic shock. The postoperativeelectrocardiogram revealed signs of severe myocardial anterolateral ischemia andnecrosis and a third-degree atrioventricular block (Fig. 1). Creatine kinase had increased to 530 U/L(normal range 15 to 100 U/L) with an MB fraction of 365 U/L (68%),representing a significant acute myocardial necrotic phenomenon.



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  		Fig. 1. Postoperativeelectrocardiogram shows a third-degree atrioventricular block and evidence ofmyocardial ischemia and necrosis.

 
Postoperative cross-sectional echocardiography visualized a mildlydilated and severely hypokinetic left ventricle (less than 10% ofshortening fraction) with severe impairment of diastolic function. The pulmonaryartery banding was adequate, and there was no residual aortic coarctation. Theright coronary artery was predominant, and the left coronary artery's origin wasnot seen from the corresponding aortic Valsalva sinus.

This raised suspicion of an anomalous left coronary artery arising fromthe pulmonary artery, which would provide an explanation for the myocardialinfarction. Unfortunately, the child's deteriorating condition did not allowperformance of any further investigations. Despite maximum hemodynamic supportand intravascular cardiac pacing with a 5F bipolar balloon pacing electrode(USCI International, BARD, C.R. Bard Ireland Ltd., Galway, Ireland), she died onthe third postoperative day.

Postmortem examination. The heart weighed30 gm. There was, as expected, an abnormal left superior caval vein draininginto the coronary sinus, a large ostium secundum–type atrial septaldefect, and one large peri­membranous and multiple muscular ventricularseptal defects. The right ventricle was predominant, and the left one was smallbut not hypoplastic. Necrotic patches, confirmed by histologic analysis, wereevident in the left anterolateral wall. The endocardium did not exhibitfibroelastosis. The aortic coarctation was well repaired, and the pulmonarybanding was found to be in place.

A tortuous, dilated right predominant coronary artery originated from theright aortic Valsalva sinus. The left coronary artery arose from the posteriorright pulmonary artery wall immediately after the pulmonary bifurcation andfollowed a tortuous course over the anterior interventricular groove (Fig. 2).



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  		Fig. 2. Postmortem examinationreveals the presence of a left coronary artery (arrow)arising from the posterior wall of the right pulmonary artery.

 
Discussion. Within the wide variety ofleft coronary anomalous origin, left coronary artery arising from the rightpulmonary artery is extremely uncommon. The chief reason for this report,however, is the association of this origin with lesions producing high pulmonaryflow, with consequent lack of clinical data suggesting such a diagnosis. Manyfactors led to misdiagnosis. First, this finding is not classically anticipatedin cases of ventricular septal defects and aortic coarctation. Second, theleft-to-right shunt provided normal perfusion to the aberrant coronary arterybefore the operation. Until palliation, this child's left coronary blood flowwas normal, and so were the left ventricular myocardial perfusion andoxygenation, because the pulmonary and aortic pressures and saturations weresimilar. The fall in pulmonary pressure to a low level after the pulmonaryartery banding was therefore accompanied by a decline in the left coronary flow,with drops in coronary perfusion pressure and oxygen saturation leading to leftventricular ischemia and myocardial infarction. A similar physiopathologiccondition related to closure of patent ductus arteriosus has been described inthe literature.Go Go 1-4 Third, cross-sectionalechocardiography may not be reliable for accurate diagnosis of such amalformation.Go 5

The main point of this report, other than describing a unique anatomicassociation, is to highlight the fact that an anomalous left coronary arteryarising from the pulmonary artery still remains a difficult diagnosticchallenge. This is especially true if it is associated with a left-to-rightshunt producing pulmonary overload and hypertension, maintaining an almostnormal coronary blood flow.

References

  1. Vlodaver Z, Neufeld HN, Edwards JE. Coronaryarterial variations in the normal heart and in congenital heart disease. NewYork: Academic Press; 1975. p. 84.
  2. Ortiz E, de Leval M, Somerville J. Ductusarteriosus associated with an anomalous left coronary artery arising from thepulmonary artery: catastrophe after duct ligation. Br Heart J 1986;55:415-7.
  3. Sreeram N, Hunter S, Wren C. Acute myocardialinfarction in infancy: unmasking of anomalous origin of the left coronary arteryfrom the pulmonary artery by ligation of an arterial duct. Br Heart J 1989;61:307-8.
  4. Nehgme RA, Dewar ML, Lutin WA, Talner NS,Hellenbrand WE. Anomalous left coronary artery from the main pulmonary trunk:physiologic and clinical importance of its association with persistent ductusarteriosus. Pediatr Cardiol 1992;13:98-9.
  5. Robinson PJ, Sullivan ID, Kumpeng V, AndersonRH, Macartney FJ. Anomalous origin of the left coronary artery from thepulmonary trunk: potential for false negative diagnosis with cross-sectionalechocardiography. Br Heart J 1984;52:272-7.




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