Images in Cardiovascular Pathology
Congenital left main coronary artery aneurysm
Roberto Andrés Guerri-Guttenberg, Gabriela Celeste Francos, José Milei
Instituto de Investigaciones Cardiológicas “Prof. Dr. Alberto C. Taquini” (ININCA) UBA-CONICET, Buenos Aires, Argentina
Received 29 April 2011; accepted 31 May 2011
We report a rare case of a congenital left main coronary artery aneurysm in a 2-year-old patient with concomitant intimal hyperplasia and
strong intimal apolipoprotein B expression. © 2012 Elsevier Inc. All rights reserved.
Keywords: Coronary artery aneurysm; Apolipoprotein B; Intimal hyperplasia; Coronary artery ectasia; Congenital
1. Case report
The following image shows the heart of 2-year-old
Caucasian male patient with a surgically repaired tetralogy of
Fallot. The autopsy revealed a focal and fusiform left main
coronary artery aneurysm (Fig. 1, panel A). The dilatation
represents a 2.5-fold increase in normal size from the
adjacent arterial segment. The patient had no known history
of Kawasaki disease or any other inflammatory or
connective tissue disorder. Histological analysis exposed
diffuse intimal thickening with an intima:media ratio of 1.
The artery, as appreciated in panel C, presented moderate to
extensive media degeneration with smooth muscles partially
replaced by hyalinized collagen. Immunohistochemistry
revealed a strongly positive stain for apolipoprotein B
(apoB; cross-reactive with B-48 and B-100) in the intima
layer (Fig. 1, panel B).
Coronary artery aneurysms are defined as arterial di-
latations, which can be saccular or fusiform, and exceed the
diameter of the normal adjacent segment or the diameter of
Cardiovascular Pathology 21 (2012) e39 – e40
Funding: received from the National Scientific and Technical Research
Council (CONICET), Argentina.
We declare no conflict of interest.
Corresponding author. ININCA-UBA-CONICET, Marcelo T de
Alvear 2270-C1122AAJ-Ciudad Autónoma de Buenos Aires, Argentina.
Tel.: +54 11 45083836; fax: +54 11 45083836.
E-mail address: firstname.lastname@example.org (J. Milei).
Fig. 1. (A): RVOT, right ventricular outflow tract repair; LM, congenital left
main coronary aneurysm; LAD, left anterior descending coronary artery;
Ao, aorta; PA, pulmonary artery. (B) Immunostaining for apoB in the
coronary aneurysm. Note how the stain is distributed predominantly in the
intimal layer. ApoB 50×. (C) Intimal hyperplasia with moderate collagen
infiltrate in the media layer. Masson's trichrome stain 100×.
1054-8807/11/$ – see front matter © 2012 Elsevier Inc. All rights reserved.