Adult type anomalous origin of the left coronary artery from the pulmonary artery: When should we be aware?

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Letter to the Editor

Adult type anomalous origin of the left coronary artery from the pulmonary artery: When should we be aware?

Abdenasser Drighil^⁎, Said Chraibi, Ahmed Bennis

Ibn Rochd Hospital, Department of Cardiology, Casablanca, Morocco Received 15 February 2006; accepted 8 July 2006;

Available online 12 October 2006

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a clinical entity characterized by myocardial ischemia which becomes symptomatic shortly after birth leading to left heart failure and death. It is rare for an ALCAPA patient to survive to adulthood. In this case, the diagnosis is sometimes missed because of atypical clinical presentation or of the unawareness of doctors. Here, we report a case of an 11-year-old boy with ALCAPA syndrome whose diagnosis took many years to be set-up.

Keywords:Anomalous origin of the left coronary artery from the pulmonary artery; Adult type; Coronary anomalies

1. Introduction

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly occurring in approximately 1 in 300,000 live births[1]. This anomaly can cause myocardial ischemia leading to left ventricular dysfunction, mitral insufficiency, congestive heart failure, and death. Without surgical intervention, about 90%

of patient with ALCAPA die within the first year of life[2].

The diagnosis of ALCAPA is sometimes difficult even in childhood when the clinical expression is misleading as a dilated cardiomyopathy or an isolated mitral regurgitation. We report the case of an 11-year-old boy with ALCAPA syndrome for whom the diagnosis took many years to be set-up.

2. Case report

An 11-year-old boy was admitted recently in our hospital for fatigue. As personal antecedents, he reported several consultations since year 2000 due to fatigue on great exertion. He had several echocardiographies and was

thought to have moderate mitral regurgitation due to prolapse of the mitral valve.

The physical examination revealed a grade 2 systolic murmur in the front intercostals space. The baseline electrocardiogram (ECG) was normal. The chest X-ray revealed a slight enlargement in the cardiac area due to the left ventricle, and normal pulmonary vessels. The echocardiography revealed a dilated left ventricle (end diastolic diameter = 50 mm) with normal function, a slight prolapse of the mitral valve, and a large coronary artery arising from the right aortic sinus (Fig. 1). Color flow mapping and pulsed Doppler investigation demonstrated mild mitral regurgitation, abnormal flow pattern in the pulmonary artery (Fig. 2), and turbulent diastolic flow within the inter-ventricular septum and right ventricular apex.

The exercise test was effective with no precordialgia, but revealed a 4 mm straightened depression of the ST segment at exterional peak, indicating myocardial ischemia.

The patient underwent cardiac catheterization. The manometry of the right and left chamber was normal. The right coronary was of a high caliber, tortuous (Fig. 3A), and through collateral vessels, it retrogradely filled the left coronary artery, which was well developed and connected to the pulmonary trunk (Fig. 3B). Contrast injection in the left valsalva's sinus showed no artery originating from this site.

International Journal of Cardiology 113 (2006) e119–e121

www.elsevier.com/locate/ijcard

⁎Corresponding author. Tel.: +212 64237566.

E-mail address:sdrighil@gmail.com(A. Drighil).

doi:10.1016/j.ijcard.2006.07.039

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The patient currently is awaiting surgical repair to establish 2-coronary myocardial perfusion.

3. Discussion

Anomalous origin of the left coronary artery from the pulmonary artery is a rare anomaly accounting for 0.24% of congenital heart disease.

Wesselheoft et. al.[3]classified the clinical spectrum and the presentation mode of ALCAPA as follows : 1—infantile syndrome: patients develop acute episodes of respiratory insufficiency and profuse sweating; 2—mitral regurgitation:

characterized by mitral regurgitation murmur, congestive heart failure, cardiomagaly in children, adolescents, or adults; 3—Syndrome of the continuous murmur: occurs in

asymptomatic adults or adults with angina pectoris; 4—

Sudden death in adolescents or adults.

On electrocardiography, ALCAPA has characteristic alterations compatible with infarction of the anterolateral wall, frequent deviation of the electric axis to the left, and left ventricular hypertrophy [4]. However a normal baseline ECG can't eliminate the diagnosis of ALCAPA as seen in the present case.

Two-dimensional Doppler echocardiography is consid- ered the major support method for diagnosing ALCAPA[5].

This examination, however, has limitations[6]. In the case of young children, the right coronary artery may not be dilated enough to lead to the suspicion of ALCAPA. In the present case, the first echocardiographies performed did not show the anomaly. This may be due to the slight dilatation of the right coronary artery or to an incomplete echocardiography examination due to the lack of awareness of doctors of this syndrome. The clinical diagnosis of ALCAPA is possible in about 50% of patients without catheterization. They are sometimes mistaken to have cardiomyopathy[7]or organic

Fig. 2. Doppler color imaging reveals abnormal flow pattern in the pulmonary artery on transthoracic echocardiographic short axis view (PA, pulmonary artery; RVOT, right ventricle outflow tract).

Fig. 1. Echocardiography with parasternal long-axis view shows a prominent large vessel (arrow) arsing from the right aortic sinus. Ao indicates aorta; LA, left atrium; LV, left ventricle; RCA, right coronary artery.

Fig. 3. A: Selective right coronary angiography. Early phase of angiography reveals a tortuous widely dilated right coronary artery (RCA). B: Late phase of the same angiography reveals extensive collaterals and left coronary artery (LCA) filling the pulmonary artery (PA).

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mitral regurgitation as seen in the case of our patient. This will result in misdiagnosis and delay in appropriate management with a risk of sudden death or of worsening of the ventricular dysfunction.

ALCAPA has a high mortality rate without surgical repair [3]. Long-term clinical outcome and left ventricular function are reported to be favourable after operation, despite severe left ventricular dysfunction at presentations [8,9]. The disappearance of mitral regurgitation after operation is also possible if ALCAPA is diagnosed before severe left ventricular dysfunction [10]. An aggressive approach to early repair in all children with ALCAPA is warranted, regardless of the degree of the left ventricular dysfunction.

Corrective coronary artery surgery appears to be the most reasonable choice for patients with ALCAPA. Although the patients with adult type are more asymptomatic, surgical correction should be undertaken as soon as the diagnosis is established.

In conclusion, although ALCAPA is rare and usually an isolated anomaly, a high index of suspicion is required for diagnosis in children with left ventricular dilatation or mitral regurgitation of unknown etiology. A complete 2-dimensional Doppler echocardiogram should show the origin of both coronary arteries. Early recognition and treatment of ALCAPA before the occurrence of myocardial damage provides the patient with an excellent long-term prognosis.

References

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[2] Wollenek G, Domanig E, Salzer-Muar U, Havel M, Wimmer M, Wolner E. Anomalous origin of the left coronary artery: a review of surgical management in 13 patients. J Cardiovasc Surg 1993;34:399–405.

[3] Wesselheoft H, Fawcelt JS, Johson AL. Anomalous origin of the left coronary from 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.

[4] Takimura CK, Hotta VT, Campos MF, Malamo M, Otsubo R.

Anomalous origin of the left coronary artery from the pulmonary artery: report of an adult case. Arq Bras Cardiol 2002;78(3):312–4.

[5] Sanjay T, Apurba KS, Kurur SV. Lessons from a case of anomalous the left coronary artery from the pulmonary artery. IJTCVS 2005;21:171–2.

[6] Ortiz VE, De Leval M, Somerville J. Ductus arteriosus associated with an anomalous origin of the left coronary artery from the pulmonary artery: catastrophe after duct ligation. Br Heart J 1996;55:415–7.

[7] Berdjis F, Takahashi M, Wells WJ, Stiles GR, Lindesmith GG.

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[8] Schwartz ML, Jonas RA, Colan SD. Anomalous origin of left coronary artery: recovery of left ventricular function after dual coronary repair. J Am Coll Cardiol 1997;30:547–53.

[9] Cochrane AD, Coleman DM, Davis AM, Brizard CP, Wolfe R, Karl TR.

Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1999;117:332–42.

[10] Tutar E, Kysalel A, Nacar N, et al. Anomalous origin of the left coronary artery from the main pulmonary artery detected on echocardiographic screening study of newborns. Int J Cardiol 2004;97:561–2.

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