European Journal of Echocardiography 2008 9(2):309-310; doi:10.1016/j.euje.2006.11.012
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Echocardiographic diagnosis of anomalous origin of the left coronary artery from the pulmonary artery
Nik
a Drinkovi
,
Eduard Margetic*,
Anton 
malcelj and
Vojtjeh Brida
Clinical Hospital Center Zagreb, University Clinic of Cardiovascular Diseases, Kipatieva 12, 10000 Zagreb, Croatia
Received 17 March 2006; accepted after revision 26 November 2006.
* Corresponding author. Tel: +385 12388482; fax: +385 12312247. E-mail address: eduard_margetic{at}yahoo.com
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Abstract
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We found increased systolic coronary flow in transthoracic pulsed
wave (PW) Doppler in a 42-year-old patient with anomalous origin
of left main coronary artery from the pulmonary artery. This
is a characteristic echocardiographic finding in this anomaly
in the presence of collateral circulation and coronary L–R
shunt. In comparison with so far used echocardiographic criteria
this parameter when present allows quick recognition of anomalous
origin of left coronary artery from the pulmonary artery, and
its differentiation from other potentially lethal coronary anomalies.
Keywords: Coronary artery anomalies; Anomalous origin of left coronary artery; Echocardiography; Pulsed wave Doppler
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Introduction
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We report on a young woman in whom a potentially lethal anomalous
origin of left coronary artery from pulmonary artery was recognized
by presence of characteristically increased systolic coronary
flow in transthoracic PW Doppler.
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Case report
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A 42-year-old woman was referred for further evaluation of a
ventricular septal defect diagnosed on an outpatient echocardiogram.
We performed pre-catheterization transthoracic echocardiography
and in the 4-chamber view multiple regions of colour flow turbulence
in the ventricular septum were seen, which at first glance appeared
as multiple ventricular septal defects (
Figure 1A). However,
in the LV short axis view it became immediately obvious that
colour turbulences resulted from flow in a huge tortuous coronary
artery (
Figure 1B).
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Figure 1 (A) Two-dimensional colour Doppler echocardiogram. Multiple flow turbulences are seen in the interventricular septum. LV = left ventricle; RV = right ventricle. (B) Turbulent coronary flow in a huge coronary artery depicted by colour Doppler. LV = left ventricle; VS = ventricular septum.
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Coronary ostia were then interrogated, and only one large coronary
artery arising from the right sinus Valsalva was visualized
(
Figure 2). PW Doppler examination with the sample volume
placed just distal of the coronary ostium revealed higher coronary
flow velocities in systole than in diastole. The same finding
was present when the coronary artery was interrogated by PW
Doppler in septal and apical regions suggesting the existence
of L–R shunt in coronary circulation (
Figure 3A and B).
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Figure 3 (A) Increased systolic coronary flow with PW Doppler sample volume placed in the turbulence in proximal septal region. (B) Increased systolic coronary flow in PW Doppler recorded in the apical portion of the ventricular septum.
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The patient underwent cardiac catheterization. Coronary angiography
revealed an enlarged tortuous right coronary artery (
Figure 4A).
Contrast injection in the pulmonary artery confirmed the origin
of left main coronary artery from the pulmonary trunk (
Figure 4B).
Subsequently, a 1.67 L/min L–R shunt was calculated.
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Figure 4 (A) Single and enlarged tortuous right coronary artery on aortic root contrast injection. (B) Origin of left main coronary artery from the pulmonary trunk demonstrated by pulmonary angiography. LMCA = left main coronary artery.
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The patient was referred to cardiac operation. The orifice of
the LCA was closed by a direct suture and a saphenous venous
graft to the LCA was performed. The postoperative course was
uneventful.
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Discussion
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An anomalous origin of one or more coronary arteries from the
pulmonary artery is usually an isolated abnormality, occurring
in 0.4% of all patients with congenital cardiac abnormalities.
1 The most common defect of this type is origin of left coronary
artery from the pulmonary artery, known as Bland-White-Garland
syndrome.
2
Echocardiography may suggest or demonstrate the abnormal coronary artery.3,4 However, differentiation between various coronary anomalies, particularly those with increased risk of sudden death (origin of coronary artery from pulmonary trunk, ectopic origin of coronary artery and single coronary artery) is rather time consuming and uncertain with this method.5 In our patient, a characteristic finding of increased systolic coronary flow in transthoracic PW Doppler was diagnostic for the origin of left coronary artery from the pulmonary artery. This is a unique echocardiographic diagnostic parameter of this anomaly in the presence of collateral circulation, and a coronary L–R shunt. In other coronary anomalies and in normals coronary blood flow is always substantially higher in diastole. Therefore, interrogation of coronary system at any site with PW Doppler for an increased systolic flow allows to differentiate the Bland-White-Garland syndrome from other coronary anomalies.
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References
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- Frescura C, Basso C, Thiene G, Corrado D, Pennelli T, Angelini A, et al. Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol (1998) 29:689–95.[CrossRef][Web of Science][Medline]
- Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of unusual case associated with cardiac hypertrophy. Am Heart J (1933) 8:787.[CrossRef]
- Pelliccia A, Spataro A, Maron BJ. Prospective echocardiographic screening for coronary artery anomalies in 1,360 elite competitive athletes. Am J Cardiol (1993) 72:978–9.[CrossRef][Web of Science][Medline]
- Pisacane C, Pinto SC, De Gregorio P, Pisacane F, Monda V, Caso P, et al. ‘Steal’ collaterals: an echocardiographic diagnostic marker for anomalous origin of the left main coronary artery from the pulmonary artery in the adult. J Am Soc Echocardiogr (2006) 19:107. e3–6.
- Stefanelli CB, Stevenson JG, Jones TK, Lester JR, Cecchin F. A case for routine screening of coronary artery origins during echocardiography: fortuitous discovery of a life-threatening coronary anomaly. J Am Soc Echocardiogr (1999) 12:769–72.[CrossRef][Web of Science][Medline]
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Abstract
Introduction