Acquired Left Ventricular Submitral Aneurysms in the Course of
Takayasu Arteritis in a Child
Alban-Elouen Baruteau, MD,*
†‡§¶
Raphaël Pedro Martins, MD,*
†‡§
Dominique Boulmier, MD,*
†‡§
Adeline Basquin, MD,*
†‡§
David Briard, MD,
#
Virginie Gandemer, MD,
#
and
Jean-Marc Schleich, MD, PhD*
†‡§
*CHU Rennes, Cardiology Department, Rennes, France;
†
Rennes 1 University, LTSI, Rennes, France;
‡
INSERM, CIC-IT
804, Rennes, France;
§
INSERM, U642, Rennes, France;
¶
INSERM, U915, Nantes, France;
#
CHU Rennes, Pediatric
Department, Rennes 1 University, Rennes, France
ABSTRACT
A 9-year-old black African boy was hospitalized for heart failure revealing a severe left ventricular dysfunction
associated with dilated cardiomyopathy, two submitral aneurysms, occlusion of the circumflex artery and a giant
coronary artery aneurysm on the proximal left anterior descending artery. The boy was coinfected with human
immunodeficiency virus and Mycobacterium tuberculosis. Though rare, association of Takayasu arteritis and submitral
aneurysm leads to rethinking the pathogenesis of submitral aneurysm and suggests that some of them may be
acquired. In our case, a common inflammatory process, possibly triggered by tuberculosis or HIV, may underlie
Takayasu and submitral aneurysms.
Key Words. Pediatrics; Inflammation; AIDS; Aneurysm; Coronary Disease
O
ne month after arriving in France, an
11-year-old black Congolese boy presented
fever and breathlessness. His medical history
included an episode of malaria necessitating a
blood transfusion 7 years prior. Clinical examina-
tion revealed a 3/6 apex systolic murmur, blood
pressure asymmetry in the upper limbs without
arterial hypertension (95/50 mm Hg in the right
arm and 55/30 mm Hg in the left arm), the
absence of the left radial pulse and physical signs
of left heart failure with tachycardia, tachypnea
and crackles in the two-lung fields. All other pulses
were found without asymmetry.
A 12-lead electrocardiogram showed normal
sinus rhythm with complete right bundle branch
block, left atrial hypertrophy, and an abnormal Q
wave in lateral leads. Echocardiography revealed
a depressed left ventricular ejection fraction
at 25% with postero- and latero-basal akinesia.
Aneurysmal deformations of the ventricular
wall, situated beneath the posterior leaflet of the
mitral valve, were associated with massive mitral
regurgitation (Figure 1, Panels A and B). Vascular
Doppler revealed vasculitis lesions of the verte-
bral, subclavian, and femoral arteries (Figure 1,
Panels C and D). The celiac trunk was occluded.
A 64-slice cardiac computed tomography scan
diagnosed two large postero- and latero-basal
submitral aneurysms communicating with the
dilated left ventricular cavity (Figure 2, Panel A).
The circumflex artery, in contact with an aneu-
rysm, was occluded. A giant coronary aneurysm
was situated on the proximal left anterior
descending artery (Figure 2, Panels B, C, and D).
The boy was coinfected with human im-
munodeficiency virus (HIV) and Mycobacterium
tuberculosis. Investigations ruled out viral, bacterial,
and parasitic HIV-associated vasculitis. Takayasu
arteritis was diagnosed with 90.5% sensitivity
and 97.8% specificity, as more than three of
the six American College of Rheumatology
criteria were met.
1
The boy was placed on a
regimen of vitamin K antagonist, beta-adrenergic
blocker, angiotensin-converting enzyme inhibitor,
anti-aldosterone agent, corticosteroid therapy,
antitubercular therapy, and highly active antiret-
roviral therapy, and was doing well 3 years later.
The incidence of HIV-associated vasculitis,
including Takayasu’s disease, is less than 1%.
Takayasu’s arteritis, which carries a 40% mortality
rate in the absence of treatment, is the third most
common vasculitis in children. This rare chronic
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© 2011 Wiley Periodicals, Inc. Congenit Heart Dis. 2012;7:76–79