International Journal of Cardiology 172 (2014) e66–e68

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International Journal of Cardiology

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Letter to the Editor

A rare case of anomalous origin of the left anterior descending arteryfrom the pulmonary artery

Hironori Hara a, Hiroshi Yamashita a,⁎, Atsuko Nakayama a, Yumiko Hosoya a, Jiro Ando a, Katsuya Iijima b,c,Yasunobu Hirata a, Issei Komuro a

a Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyob Department of Geriatric Medicine, Graduate School of Medicine, The University of Tokyoc Institute of Gerontology, The University of Tokyo

⁎ Corresponding author at: Department of Cardiovascof Medicine, The University of Tokyo, 7-3-1 Hongo, BunkTel.: +81 3 3815 5411x37153; fax: +81 3 3814 0021.

E-mail address: heartmuscle-tky@umin.ac.jp (H. Yama

0167-5273/$ – see front matter © 2013 Elsevier Ireland Lhttp://dx.doi.org/10.1016/j.ijcard.2013.12.050

a r t i c l e i n f o

Article history:

Received 24 September 2013Accepted 21 December 2013Available online 27 December 2013

Keywords:Coronary anomalyALCAPACollateral circulationMultislice computed tomographic coronaryangiographyScintigraphy

cusp (Fig. 1A). No stenotic lesions were observed in the coronary arter-ies. After improvement of the congestive heart failure, invasive coronaryangiography was performed in order to delineate the anatomy andcoronary circulation. Collaterals from the ectatic LCx and hypoplasticRCA flowed into the LAD and then retrogradely into the main PA(Fig. 1B). In the right heart catheterization, a step-up in oxygen satura-tion was noted, from 56.9% in the right ventricle to 62% in the PA. Thecalculated pulmonary to systemic blood flow ratio (Qp:Qs ratio) was1.06 and insignificant. Exercise stressmyocardial perfusion scintigraphydemonstrated reversible perfusion defect in the anteroseptal regioncorresponding to the distribution territory of the LAD (Fig. 2). Althoughsurgical repair was recommended, he refused the operation and instead

A 71-year-old man with a history of hypertension, aortic regurgita-tion, atrial fibrillation and chronic heart failure presented to our emer-gency department with dyspnea and chest pain. Chest X-rays showedsevere pulmonary edema. His electrocardiogram revealed atrial fibrilla-tion and ST-segment depression in leads I, aVL andV3–6. In transthorac-ic echocardiography, wall motion was decreased in the anteroseptalregion. After urgent hospitalization, his acute pulmonary edema wasimproved by non-invasive positive pressure ventilation and intrave-nous administration of diuretics and nitrates. The ST-segment changewas also relieved. His creatine phosphokinase (CK) and CK-MB levelsincreased marginally to a peak value of 279 units/L (reference range:55–210 units/L) and 37 units/L, respectively, indicating non-ST seg-ment elevation myocardial infarction.

Four months before the admission, he had undergone coronarycomputed-tomographic angiography due to diffusely decreased leftventricular systolic function detected by echocardiography. Multi-detector computed-tomography showed anomalous origin of the leftanterior descending artery (LAD) from the pulmonary artery (PA).The ectatic left circumflex artery (LCx) arose from the left coronarycusp and supplied dilated and tortuous collaterals to the LAD. The

ular Medicine, Graduate Schoolyo-ku, Tokyo 113-8655, Japan.

shita).

td. All rights reserved.

hypoplastic right coronary artery (RCA) arose from the right coronary

opted for medical treatment only. His symptoms were relieved withmedical treatment that consisted of calcium channel blocker, angioten-sin II receptor blocker and diuretic administration. He was followed-upas an outpatient and was asymptomatic.

Anomalous origin of the left coronary artery from the pulmonary ar-tery (ALCAPA) is a rare congenital anomaly [1]. Approximately 90% ofpatients with ALCAPA die in the first year of life (infant type) [2]. In pa-tients with ALCAPA, adequate collateral circulation from the RCA to theLCA is mandatory for survival beyond infancy (adult type). The presentcase is different from typical adult type ALCAPA in that only the LADoriginated from the PA and that the LCx as well as the RCA arisingfrom the aorta supplied fair collaterals to the LAD. Therefore, the extentof ischemia might have been less severe than that in patients with thetypical type of adult ALCAPA, and thus, he was able to survive for aslong as 71 years old. Due to the low pulmonary vascular resistance, col-lateral flow is preferentially directed into the pulmonary vascular bedaway from the left ventricular myocardium. This left-to-right shunt isknown as the coronary steal phenomenon andmay causemyocardial is-chemia. In the present case, non-transmural myocardial ischemia wasdemonstrated in exercise stress scintigraphy andmay have played a sig-nificant role in the development of congestive heart failure and non-STsegment elevation myocardial infarction.

The optimal treatment of cases with only LAD originating from thePA is not established, but may be surgical intervention as in cases withtypical ALCAPA; construction of a two-coronary system and ligation ofthe origin of the anomalous LAD to abolish the coronary steal under cer-tain stress conditions [3–5]. In our case, surgerymay have been the besttreatment, given the stress-induced myocardial ischemia in the broad

Fig. 1. A: Axial images and volume rendering of coronary computed tomographic angiography showed that the LAD originated from themain PA. The LCx and RCA arose from the left andright coronary cusps of the aorta, respectively. B: Selective right coronary angiography showed the right coronary artery giving off collaterals to the LAD (left panel). In selective leftcoronary angiography, early phase revealed the dilated LCx (middle panel), while late phase revealed dilated and tortuous collaterals flowing into the LAD and then the PA (rightpanel). Ao, aorta; LA, left atrium; LV, left ventricle; RCA, right coronary artery; LAD, left anterior descending artery; LCx, left circumflex artery; PA, pulmonary artery; CB, conus branch.

e67H. Hara et al. / International Journal of Cardiology 172 (2014) e66–e68

LAD distribution territory. However, the patient refused to undergo theoperation. Careful follow-up as well as sufficientmedical treatmentwasessential for secondary prevention of myocardial ischemic events.

References

[1] Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary ar-tery from the pulmonary artery: collective review of surgical therapy. Ann ThoracSurg 2002;74:946–55.

[2] Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary arteryfrom the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology,based on a review of 140 cases with seven further cases. Circulation 1968;38:403–25.

[3] Liu PI, Hsieh PL, Ohta I, Wu DK, Hsu JS, Chuang MT. Anomalous origin of the left an-terior descending artery from the main pulmonary artery with multi-detector rowcomputed tomography coronary angiography. Int J Cardiol 2010;139:e8-10.

[4] Vavouranakis I, Hamilos MI, Kochiadakis GE, Vardas PE. Acute coronary syndrome inan adult with anomalous origin of the left anterior descending coronary artery fromthe pulmonary trunk. Int J Cardiol 2007;121:323–5.

[5] Peña E, Nguyen ET, Merchant N, Dennie G. ALCAPA syndrome: not just a pediatric dis-ease. Radiographics 2009;29:553–65.

Fig. 2. Exercise stress myocardial perfusion scintigraphy demonstrated non-transmural and reversible perfusion defect in the anteroseptal region. Bull's eye plots created from the stressand rest data clearly showed reversible perfusion defect in the left anterior descending artery territory.

e68 H. Hara et al. / International Journal of Cardiology 172 (2014) e66–e68