CASE 10445 Published on 07.11.2012

A case of coronary artery lesions with giant aneurysms in a patient with suspected Kawasaki Syndrome

Section

Cardiovascular

Case Type

Clinical Cases

Authors

Romagnoli Andrea, Sperandio Massimiliano, Coco Irene, Antonicoli Marco, Girardi Valentina, Simonetti Giovanni.

Department of Diagnostic Imaging,
Molecular Imaging,
Interventional Radiology and Radiotherapy,
Tor Vergata University Hospital,
University of Rome Tor Vergata, Italy.
Viale Oxford 81;
Email:ire.coco@hotmail.it
Patient

53 years, male

Categories
Area of Interest Cardiovascular system ; Imaging Technique CT-High Resolution, CT-Angiography, Echocardiography
Clinical History
The patient was admitted with dyspnoea and history of chest pain. Laboratory results revealed the presence of an increase of transaminase, bilirubin and neutrophils values. A careful medical history reported an acute illness in paediatric ages, onset with prolonged fever, bilateral conjunctivitis, gingivitis, maculo-erythematous rash and cervical lymphadenopathy of unknown aetiology.
Imaging Findings
The electrocardiogram performed in the emergency department showed a sinus tachycardia and blood tests demonstrated an increase of transaminase, bilirubin and neutrophils values.
Electrocardiography showed a prolonged PR interval. Cardiac enzyme levels, cardiac troponin and lactate dehydrogenates were normal.
Chest radiograph was obtained to exclude cardiomegaly or subclinical pneumonitis.
A chest CT examination showed the presence of multiple giant coronary aneurysm, one at the left coronary artery in the proximal third of the circumflex branch, 30 x 28 mm wide and 45 mm long and multiple smaller dilatations in the most distal part of the artery. Coronary CT showed the presence of two calcific aneurysms at the anterior descending coronary artery located in the proximal third (35 x 35 mm wide and 49 mm long) and in the middle third (23 x 20 mm wide and 35 mm long).
Echocardiography documented the presence of left ventricle dilatation.
Discussion
Kawasaki disease is a relative common acute febrile illness in infant or young children that mainly affects small and medium-sized arteries, with a predilection for the coronary arteries, first described by Tomisaku Kawasaki in Japan in 1967 [1, 2]. About 80% of the patients are younger than 5 years with the peak of incidence between 6-11 months.
The aetiology remains unknown, and there are no specific laboratory tests to identify affected children reliably.
Diagnosis is a demanding challenge because it relies essentially on clinical criteria and its presentation varies over time. Classic cases demonstrate persistent fever with at least 4-6 major clinical features [3].
The illness was initially thought to be self-limited, however, untreated children develop cardiovascular sequelae with coronary artery lesions [4], ranging from asymptomatic coronary artery ecstasy, premature atherosclerosis, coronary artery aneurysm (CAA) - that occur in about 10 to 20% of patients - myocardial infarction and sudden death in children and young adults with a mortality of 1-2%. CAA show a strong tendency to regression and smaller tendency to resolve on their own (60% of cases) for marked proliferation of intima without massive thrombus formation; although larger CAA may expand and myocardial infarction may occur [5]. The risk factors to develop ischaemic heart disease are: diameter more than 8 mm, large or saccular-type aneurysms, prolonged fever for more than 21 days and age at onset over two years.
Chest pain, angina, myocardial infarction, heart failure, the presence of a mediastinal mass, superior vena cava obstruction and even haemoptysis have been described as presenting problems, however, no reliable clinical features to identify a CAA have been described, and in fact most patients are asymptomatic [6, 7, 8, 9].
Untreated or undiagnosed CAA can be complicated by rupture, thrombo-embolic phenomenon, fistulization into cardiac chambers, tamponade, heart failure or even sudden death [10]. Prognosis is controversial, however, overall five-year survival is in the region of 71% [11].
Management may be medical, with anticoagulation or anti-platelet agents, endovascular intervention with positioning of a covered stent to exclude aneurysmal flow, or surgical management with cardiopulmonary bypass and coronary artery bypass grafting in conjunction with exclusion of the aneurysm [10].
Today, thanks to the remarkable technological advances a non-invasive diagnostic imaging for coronary screening in patients is possible, especially if asymptomatic, with clinical-anamnestic data suspected for KD, so this clinical entity must be considered in the differential diagnosis because the early KD children are now adults.
Differential Diagnosis List
Coronary giant aneurysms in an asymptomatic patient with Kawasaki syndrome
Atherosclerotic coronary artery aneurysm
Autoimmune disease coronary involvement
Congenital malformation
Post-traumatic coronary aneurysm
Final Diagnosis
Coronary giant aneurysms in an asymptomatic patient with Kawasaki syndrome
Case information
URL: https://www.eurorad.org/case/10445
DOI: 10.1594/EURORAD/CASE.10445
ISSN: 1563-4086