Key words: Coronary Artery Disease, Children
A simple PubMed search on coronary artery disease AND children
returns nearly six thousand citations. Needless to say,
majority of them are on Kawasaki disease. It is now well
established that Kawasaki disease is an important cause of
heart disease in children, to the extend that it is the
commonest cause in the developed world [1]. Coronary anomalies
as a cause of myocardial infarction in infancy with the
classical presentation of anomalous origin of left coronary
artery from pulmonary artery (ALCAPA) as a cause of
greying
spells on crying has been known for a long time.
Coronary artery calcification in children with chronic kidney
disease is another important cause [2]. Coronary artery
dilatation has been noted in children with sickle cell
disease, though the significance is unknown. In one study,
coronary artery dilatation could not predict morbidity in
children with sickle cell disease [3]. Other reasons for
coronary artery disease in children are progeria, post
surgical coronary lesions and cardiac allograft vasculopathy
[4]. Coronary artery dilatation has been documented in
children with paediatric onset systemic lupus erythematosus
[5].
Coronary artery involvement in Kawasaki disease
Kawasaki disease is the most important cause of acquired
coronary artery abnormalities in children. It can lead to
coronary aneurysms in about one fourth of the untreated cases
[1]. Though early initiation of treatment with intravenous
immunoglobulin (IVIG) is the sheet anchor of treatment in
Kawasaki disease, 10 – 20% may not respond to the initial IVIG
treatment and additional treatment may be required. Timely
treatment with IVIG can reduce the chance of coronary aneurysm
formation from 25% to 4%. Children who develop coronary
aneurysms need life long cardiology follow up.
Though very mildly dilated and inflamed coronary arteries may
return to normal, this does not occur in large saccular
aneurysms. The coronary artery in this region has lost intima,
media and elastica and cannot be regenerated. In giant
aneurysms, only a thin layer of adventitia is remaining. Psudo
resolution of aneurysms may occur with decrease in lumen size
by formation of layered mural thrombi. Large aneurysms can
rarely rupture leading to pericardial tamponade. Rupture
usually occurs in the first two to three weeks and seldom
after that. Myocardial infarction can occur due to thrombosis
in affected coronary arteries or due to stenosis of the
vessels.
Coronary artery anomalies
Though various types of coronary artery anomalies involving
different coronary branches have been described, the one which
is most familiar to all is the ALCAPA. It may present as
cardiac failure in infancy or an anginal equivalent with
pallor on crying or feeding (greying spells). ALCAPA is one
condition to be kept in mind while evaluating isolated mitral
regurgitation in infants. A close look at coronary origins is
a must during echocardiographic evaluation. The pressure in
pulmonary artery being lower, ALCAPA acts a left to right
shunt, though usually small in volume, with flow from
collaterals retrogradely into the left anterior descending
artery and then into the pulmonary artery.
Other important coronary anomalies are those in tetralogy of
Fallot which cross the right ventricular outflow tract and one
in which a malignant anomalous origin of right coronary artery
from left sinus, passes between the aorta and pulmonary
artery. Later can even cause sudden death during exercise,
especially in young asymptomatic athletes [6].
Coronary artery fistula and associated ectasia
Coronary artery fistula can be seen occasionally in
children. Vinograd CA and associates found that small
fistulae mostly arose from left anterior descending coronary
artery, drained into pulmonary artery and only 3 out of 92 had
associated ectasia. Large fistulae was more often seen in
females and most originated form the right coronary artery and
drained into right atrium. Of the 12 patients who underwent
procedural closure, all had ectatic feeding coronary arteries
at a mean follow up period of around 4 years [7].
Transplant coronary artery disease
Cardiac allograft vasculopathy or transplant coronary artery
disease is one of the important problems which limit long term
survival in pediatric cardiac allograft recepients. Routine
surveillance coronary angiography is an established method to
detect cardiac allograft vasculopathy. Contrast enhanced
cardiac magnetic resonance imaging could detect significant
difference in coronary enhancement intensity between those
with and without angiographically detectable transplant
coronary artery disease in one study [8].
Conclusion
Though coronary artery disease is extremely uncommon in
children, one should be on the look out for the rare causes,
which can occasionally manifest. Kawasaki disease is the most
important cause of acquired coronary artery disease in
children and manifest mostly as coronary aneurysms. Coronary
involvement in Kawasaki disease can be prevented to a large
extend by early IVIG treatment. Other acquired causes are
coronary calcification in CKD, cardiac allograft vasculopathy
and coronary dilatation in SLE. Coronary anomalies are the
major group of congenital coronary lesions, which usually
manifest in early infancy.
References
1. McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF,
Gewitz M, Baker AL, Jackson MA, Takahashi M, Shah PB,
Kobayashi T, Wu MH, Saji TT, Pahl E; American Heart
Association Rheumatic Fever, Endocarditis, and Kawasaki
Disease Committee of the Council on Cardiovascular Disease in
the Young; Council on Cardiovascular and Stroke Nursing;
Council on Cardiovascular Surgery and Anesthesia; and Council
on Epidemiology and Prevention. Diagnosis, Treatment, and
Long-Term Management of Kawasaki Disease: A Scientific
Statement for Health Professionals From the American Heart
Association. Circulation. 2017 Apr 25;135(17):e927-e999.
2. Paoli S, Mitsnefes MM. Coronary artery calcification
and cardiovascular disease in children with chronic kidney
disease. Curr Opin Pediatr. 2014 Apr;26(2):193-7.
3. Johnson MC, Johnikin MJ, Euteneuer JC, DeBaun MR, Hildebolt
C. Coronary artery dilation and left ventricular hypertrophy
do not predict morbidity in children with sickle cell disease.
Pediatr Blood Cancer. 2015 Jan;62(1):115-9.
4. Ou P, Kutty S, Khraiche D, Sidi D, Bonnet D. Acquired
coronary disease in children: the role of multimodality
imaging. Pediatr Radiol. 2013 Apr;43(4):444-53.
5. Shen CC, Chung HT, Huang YL, Yeh KW, Huang JL. Coronary
artery dilation among patients with paediatric-onset systemic
lupus erythematosus. Scand J Rheumatol. 2012;41(6):458-65.
6. Satija B, Sanyal K, Katyayni K. Malignant anomalous right
coronary artery detected by multidetector row computed
tomography coronary angiography. J Cardiovasc Dis Res. 2012
Jan;3(1):40-2.
7. Vinograd CA, Ostermayer S, Lytrivi ID, Ko
HH, Parness I, Geiger M, Panesar LE, Love
B, Srivastava S. Prevalence and outcomes
of coronary artery ectasia associated with
isolated congenital coronary artery fistula. Am
J Cardiol. 2014 Jul 1;114(1):111-6.
8. Madani MH, Canter CE, Balzer DT, Watkins MP, Wickline SA.
Noninvasive detection of transplant coronary artery disease
with contrast-enhanced cardiac MRI in pediatric cardiac
transplants. J Heart Lung Transplant. 2012 Nov;31(11):1234-5.