RESUMEN
Kawasaki disease (KD) is a self-limited vasculitis with significant morbidity and even mortality if not treated early. The diagnosis and timely treatment in children younger than 3 months is challenging as most of them have an incomplete or atypical presentation. Coronary artery abnormalities are frequent in this type of patients. We present a 6-week-old female infant with KD who developed a giant coronary aneurysm. An early diagnosis and promptly treatment, as well as the echocardiographic and multimodality follow-up allowed us to improve our clinical approach and management.
Asunto(s)
Aneurisma Coronario , Enfermedad de la Arteria Coronaria , Síndrome Mucocutáneo Linfonodular , Niño , Aneurisma Coronario/diagnóstico por imagen , Ecocardiografía , Femenino , Humanos , Lactante , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/diagnóstico por imagenRESUMEN
BACKGROUND: Congenital heart defects such as coarctation or valvular aortic stenosis are followed by changes in left ventricular myocardial deformation mechanics induced by pressure overload. METHODS: Echocardiography was performed in 37 patients (aged 0-27 years, 15 female) with coarctation (27) or valvular aortic stenosis (10) before and after interventional catheterization and compared with 37 body surface area/age-matched healthy children. Deformation was calculated by 2-dimensional strain speckle tracking. RESULTS: Stress gradients under provocation with orciprenaline in coarctation decreased from 51.8 +/- 20.0 mm Hg to 6.0 +/- 12.0 mm Hg (P < .0001), and resting gradients in aortic stenoses decreased from 57.5 +/- 18.8 mm Hg to 25.5 +/- 14.0 mm Hg (P < .0001) after intervention. Patients had an increased maximal torsion (tor(max): 16.7 +/- 6.7 deg vs 11.0 +/- 4.7 deg (controls; P < .0001), which decreased significantly after therapy (11.8 +/- 4.9 deg, P < .0001). CONCLUSION: Compensatory elevation of left ventricular tor(max) in children with moderate left ventricular pressure load changes rapidly after successful interventional treatment. tor(max) may be a valuable tool to assess cardiac unloading or indicate the need for interventional treatment.