Diagnosis of ALCAPA syndrome in a asymptomatic 70-years-old man by Computed Tomography: Imaging Findings

Congress:

ESCR 2017

Poster Number:

P-0075

Type:

Scientific Poster

Keywords:

Cardiac, Cardiovascular system, Arteries / Aorta, CT-Angiography, MR, Catheter arteriography, Computer Applications-3D, Comparative studies, Congenital, Haemodynamics / Flow dynamics, Ischaemia / Infarction

Authors:

P. Cucchi¹, M. Zerbini¹, R. Righi¹, A. Vizzuso¹, M. Macchia¹, M. Giganti¹, G. Benea²; ¹Ferrara/IT, ²Lagosanto/IT

DOI:

DOI-Link:

https://dx.doi.org/

Fig. 3: 3D volume-rendered CT angiograms show the ALCAPA (arrows). Ao: Aorta, TP:...

Fig. 4: Dilated intercoronary arteries can be seen (★) along the epicardial surface...

Fig. 9: Angiographic views. The following angiogram shows the contrast agent opacifying...

Purpose

We present a rare case of Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) syndrome in an asymptomatic elderly man and we describe cardiac CT angiography (CCTA) imaging findings to improve diagnostic confidence for evaluation of this uncommon coronary artery anomaly. We also showthat CCTA is the tool of choice in coronary artery anomaly detection.

Methods and Materials

A 70-years-old male came to our Hospital with slight limitation of physical activity (NYHA II), asymptomatic for angina, faintings and palpitations. After an echocardiography , a cardiac MRI was performed because an hypertrophic cardiomyopathy was suspected and, as a collateral evidence, the exam showed a plausible coronary artery anomaly (Fig 1-2). Cardiac CT angiography was performed using Philips Healthcare iCT 256-row. A preliminary scan for the detection of coronary calcification was performed and Agatston score was 0. Iopamidol contrast media with an iodine concentration of...

Results

CCTA showed the anomalous origin of the left main coronary artery (LMCA) from the right side of main pulmonary artery which was characterized by a circumferential dilation (maximum caliber 20 mm); the diagonal vessels had a normal origin from LMCA and a regular course. Distally to the aneurysmatic portion the wall of LCA appeared hypertrophic with an intramyocardial course of 20 mm length and 3 mm depth. The LCA was bifurcated into the left anterior descending (LAD) artery and the left circumflex (LCx) artery. The...

Conclusion

ALCAPA (or Bland - White – Garland syndrome) is a rare hemodynamically significant congenital coronary artery anomaly affecting 1 / 300000 live birth and comprises between 0.25 – 0.50 % of all congenital cardiac diseases. It is very rare to make a diagnosis in adults because this malformation usually leads to death in childhood. In the prenatal period the blood pressure in systemic circulation and in pulmonary arterial circulation are equalized by the patent ductus arteriosus , so the flow results antegrade. After the birth...

Personal Information

References

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