Congenital heart disease with hypereosinophilic syndrome

The idiopathic hypereosinophilic syndrome, common in adults, is a leukoproliferative disorder marked by a predilection to damage specific organs. This report describes a unique case of an infant with tricuspid stenosis, a hypoplastic right ventricle, severe pulmonary stenosis, patent ductus arteriosus, and a patent foramen ovale associated with hypereosinophilic syndrome. Although treatment with high-dose oral steroids failed to decrease the leukocyte counts, a right classic Blalock-Taussig shunt through a right thoracotomy resolved her cyanosis. Idiopathic hypereosinophilic syndrome (HES) is a multisystem disease with peripheral eosinophilia of 1.5 eosinophils x 10(9)/L for at least 6 months' duration, multiple organ system involvement, and no evidence for other known causes of eosinophilia. Cardiovascular manifestations of the syndrome are the major source of morbidity and mortality and include progressive subendocardial fibrosis with thrombus formation leading to peripheral emboli, restrictive cardiomyopathy, and valvular dysfunction. HES is typically seen in men in their fourth decade who live in temperate climates, and it is very rare in neonates. This report describes the surgical treatment of a patient with tricuspid stenosis, hypoplastic right ventricle, severe pulmonary stenosis, patent ductus arteriosus, and patent foramen ovale associated with HES.