Multiple Coronary Stenosis in Infantile Moyamoya Disease
A 3-year-old girl was referred to our institution for evaluation of mitral valve regurgitation. A heart murmur was observed when she visited a pediatrician for treatment of a respiratory infection, and it was diagnosed as mitral valve regurgitation. The regurgitation gradually became worse and she was referred to the hospital. An echocardiographic examination showed moderate mitral valve regurgitation. Because her ECG demonstrated a slight ST depression on the left chest leads, dipyridamole stress thallium imaging was performed. However, it was interrupted when an aminophylline injection was required because of chest pain with ST depression on leads II, III, aVf, and V4 through V6. This strongly suggested ischemic mitral valve regurgitation, and coronary angiography was performed. A coronary angiogram demonstrated multiple stenoses in both coronary arteries (Figure 1). Because her 16-year-old sister was suffering from moyamoya disease, intracranial magnetic resonance angiography was performed.1 The image showed severe stenosis of the bilateral internal carotid arteries with small collateral vessels (Figure 2), and she was therefore with the early stages of moyamoya disease.
Although she had been suffering from chest pain, the administration of aspirin, an angiotensin-converting enzyme inhibitor, a β-blocker, nicorandil, and a calcium channel antagonist relieved her symptoms.
Moyamoya disease is characterized by progressive steno-occlusive changes at the terminal portions of the bilateral internal carotid arteries with arterial collateral vessels, called moyamoya vessels, at the base of the brain. Familial occurrence is reported in about 15% of patients with moyamoya disease. Therefore, the patient’s family history led to the diagnosis, although she had had no neurological symptoms.2 The scant collateral arteries probably indicate an early stage of the disorder, which is consistent with the fact that she demonstrated no neurological symptoms.
Cases of moyamoya disease associated with coronary artery disease, coronary stenosis, and spastic angina have only rarely been reported.3 The present report demonstrated the first case of multiple coronary artery stenosis in a young patient with moyamoya disease. A coronary artery disease is rare in small children, and it is important to consider moyamoya disease as a potential cause of ischemic heart disease even in children.