Importance of pleural findings in patients with amyloid cardiomyopathy complicated with refractory pleural effusion.

A 76-year-old male was admitted to our hospital for progressive bilateral pleural effusion. Because of typical echocardiographic findings such as left ventricular (LV) hypertrophy, thickness of the mitral valve, and a granular sparkling appearance of the LV wall, amyloid cardiomyopathy was suspected. Regardless of up-titration of several diuretic agents, the bilateral pleural effusion did not improve. Because the histological findings of the right ventricular septum (direct-fast-scarlet staining) obtained by biopsy that demonstrated amyloid deposits in perivascular and pericellular lesions, amyloid cardiomyopathy was diagnosed. However, cardiac catheterization revealed normal right and left atrial pressure and normal right and left ventricular end-diastolic pressure. Therefore, hemodynamic deterioration was less likely to be the cause of persistent pleural effusion. Amyloid deposits were also detected in the pleural biopsy specimen, so pleural amyloidosis was diagnosed and may have played an important role in the refractoriness of the pleural effusion. <Learning objective: Systolic and diastolic dysfunction of various degrees can occur in patients with amyloid cardiomyopathy, which is usually progressive and induces heart failure. In these patients, diuretics are key drugs for resolving fluid retention issues such as pleural effusion. In cases of refractory pleural effusion associated with amyloid cardiomyopathy despite aggressive diuretic therapy, these may be induced by pleural amyloidosis.>.