A Surgical Case of Severe Mitral Regurgitation with Idiopathic Hypereosinophilic Syndrome / 日本心臓血管外科学会雑誌

ABSTRACT

Idiopathic hypereosinophilic syndrome (IHES) is a rare systemic disease, but frequently associated with the eosinophil-infiltrated end organ (cardiac, hematologic, cutaneous, neurologic, pulmonary, splenic and thromboembolic) failure. Mechanical valve replacement for valvular heart disease in cases of IHES has the potential of thromboembolic accidents in the early post-operative period, even though the strict anti-coagulant therapy was performed. We reported a younger IHES case with congestive heart failure due to severe mitral regurgitation who underwent mitral valve repair instead of replacement. In this case, only the lateral scallop of the posterior mitral leaflet was involved with endocarditis due to the eosinophyl infiltration, with no other symptoms, i.e., initial hyperplasia and thrombus in the left ventricle that were detected in the most cases with IHES endocarditis. Mitral annuloplasty with the complete ring was simply performed because of the presence of sufficient intact anterior leaflet. The eosinophil count had been strictly controlled with imatinib mesilate in addition to the anti-coagulant therapy, and any thromboembolic event had been detected post operatively. In case of valvular heart disease due to IHES endocarditis, valve repair should be firstly attempt and imatinib mesilate is very useful and effective for preventing from post-operative thromboembolic accidents in FIP1L1-PDGFRα gene positive cases.