Severe mitral regurgitation due to coronary arteritis of mucocutaneous lymph node syndrome. A new surgical entity
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Mucocutaneous lymph node syndrome (MCLS) can cause severe mitral regurgitation in children, often alongside coronary artery issues. Surgical intervention for this mitral regurgitation is feasible and should be recognized as a distinct clinical entity.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
- Rheumatology
Background:
- Mucocutaneous lymph node syndrome (MCLS), a pediatric vasculitis, can lead to significant cardiac sequelae.
- Coronary artery lesions, including aneurysms and obstructions, are known complications of MCLS.
Observation:
- This report details three pediatric cases (ages 1-4) with severe mitral regurgitation secondary to MCLS.
- These patients presented with associated coronary artery abnormalities, such as aneurysms and occlusions.
- One patient with extensive coronary aneurysms and impaired left ventricular function died from congestive heart failure.
Findings:
- Mitral regurgitation in MCLS is frequently complicated by coronary artery disease and impaired left ventricular function.
- One patient with myocardial infarction underwent successful mitral valve replacement.
- Another patient with coronary aneurysms and mitral regurgitation is under observation due to the challenges of early surgical intervention.
Implications:
- Mitral regurgitation resulting from MCLS presents a complex clinical challenge due to concurrent coronary artery disease.
- Despite complexities, mitral regurgitation secondary to MCLS is a treatable condition.
- MCLS-associated mitral regurgitation should be considered a new surgical entity in pediatric cardiovascular care.
Abstract:
Case histories are reported of three patients, 1 to 4 years of age, with severe mitral regurgitation that occurred as a sequela of mucocutaneous lymph node syndrome (MCLS). These patients were found among 12 patients with coronary artery lesions such as aneurysms, narrowings, and obstructions. One of them with multiple coronary aneurysms and also severely impaired left ventricular function died of congestive heart failure in the 2 years and 7 months' follow-up period after the acute illness. The second patient, 1 year of age, had multiple coronary aneurysms and mitral regurgitation. Because of the difficulty in treating coronary artery lesions at this patient's age and also because of medically controllable mitral regurgitation at present, this patient is now under careful observation. The third patient, whose condition was complicated by inferior wall myocardial infarction due to occlusion of the right coronary artery, underwent mitral valve replacement for intractable heart failure and cardiac cachexia. This patient had slow but definite improvement postoperatively. Mitral regurgitation secondary to MCLS usually is complicated by the associated difficult coronary artery lesions and impaired left ventricular function. Although this fact causes an extra-difficult situation, mitral regurgitation consequent to MCLS is amenable to surgical treatment and this disease should now be considered as a new surgical entity.