Background While transthyretin (TTR) amyloidosis is usually characterized by peripheral and autonomic neuropathy, a majority of patients also have evidence of restrictive cardiomyopathy. In addition, amyloid cardiomyopathy may occur in the elderly without the presence of a mutant form of TTR. Both the hereditary and wild-type TTR amyloidosis are characterized by progressive restrictive cardiomy...

Cardiac morbidity, early mortality, and sudden death are the major consequences of sickle cell disease (SCD) in patients surviving into adulthood. Pulmonary hypertension (PH), elevated tricuspid regurgitant jet velocity (TRV), and diastolic dysfunction have all been identified to correlate with early mortality in adults with SCD. However, the unifying pathophysiology behind these abnormalities ...

OBJECTIVES The purpose of this study was to determine the prevalence, clinical significance, and genetic basis of hypertrophic cardiomyopathy (HCM) with "restrictive phenotype" characterized by restrictive filling and minimal or no left ventricular hypertrophy. BACKGROUND Hypertrophic cardiomyopathy is a heterogeneous myocardial disorder with a broad spectrum of clinical presentation and morp...

概 要 拘 束型心 筋症は ア ミロイ ドーシ スな どに伴 うものが多 く,特 発性 の もの は まれ であ る. 今回我 々は特発性 の2例 を経 験 した.心 臓 カテ ーテル検 査な どでは収縮性 心膜炎 に類似 した病 態がみ られたた め心膜切除 術 を施 行 した.症 例1. 31才,女 性.17才 よ り息 切れ が出現 し,21才 時MSと して交連切 開術 を受けて いる,心 エ コー図上,心 膜の肥厚 を認め た.症 例2. 46才,女 性.1年 前 よ り心 膜液貯留 と右心不 全症状 が出現 した.心 臓 カテ ーテ ル検 査で は両例 ともに右 心室拡張 寒期圧 の上昇 を認 め,左 右 の心室圧 は拡張期 はほぼ 等 しく,圧 波形 はdip and plateau を呈 した.術 中所 見で は2例 とも心膜 の肥厚,癒 着は軽度 であつ た.術 後...

Chloroquine is frequently used to treat systemic autoimmune diseases, such as systemic lupus erythematosus (SLE). With long term treatment, associated toxicity is well known, with retinopathy been the most common complication. Other chronic complications include skin disorders (hyperpigmentation), blood dyscrasias, corneal deposits, encephalopathy, neuropathy, myopathy, and impairment of audito...

Chloroquine is frequently used to treat systemic autoimmune diseases, such as systemic lupus erythematosus (SLE). With long term treatment, associated toxicity is well known, with retinopathy been the most common complication. Other chronic complications include skin disorders (hyperpigmentation), blood dyscrasias, corneal deposits, encephalopathy, neuropathy, myopathy, and impairment of audito...

Five patients with only mildly dilated ventricles but other features typical of congestive cardiomyopathy underwent cardiac transplantation for class IV NYHA heart failure. The findings of clinical studies, cardiac catheterization, endomyocardial biopsy, and pathologic examination of the removed hearts in this group with mildly dilated congestive cardiomyopathy (MDCM) were compared with similar...