Mattheyses M, Hespel J P, Brissot P, Daubert J C, Hita de Nercy Y, Lancien G, Le Treut A, Pony J C, Simon M, Ferrand B, Gouffault J, Bourel M
Arch Mal Coeur Vaiss. 1978 Apr;71(4):371-9.
A retrospective study of the case histories of 216 patients with idiopathic haemochromatosis has highlighted the frequency of cardiac involvement in this condition (53%). Two forms can be distinguished: a latent one (65%), in which the changes are predominantly electrocardiographic, and a clinical form (35%) with the features of congestive cardiomyopathy, notable for the rapidity of onset after right heart failure, the degree of cardiomegaly, the constant finding of abnormalities of ventricular repolarisation, the relative frequency of latent disorders of supra-His atrio-ventricular conduction, and the finding of elongation of the isovolumic contraction time on the phonomechanocardiogram. A haemodynamic profile is the same as for non-obstructive hypotonic cardiomyopathies, and is usually associated with a slow rise in left ventricular pressure. The cardiomyopathy, which is the most frequent cause of death, determines the prognosis in this condition. It may be found in association with diabetes and gonad failure. The finding of cardiomyopathy indicates basic treatment by veresection, which may be the only means of establishing a favourable outcome.
一项对216例特发性血色素沉着症患者病历的回顾性研究突出显示了该病中心脏受累的频率（53%）。可区分出两种形式：一种是隐匿型（65%），其变化主要是心电图改变；另一种是临床型（35%），具有充血性心肌病的特征，其特点是右心衰竭后发病迅速、心脏扩大程度、心室复极异常持续存在、希氏束以上房室传导潜在障碍的相对频率以及心音图上等容收缩时间延长。血流动力学特征与非梗阻性低渗性心肌病相同，通常与左心室压力缓慢上升有关。心肌病是最常见的死亡原因，决定了该病的预后。它可能与糖尿病和性腺功能衰竭有关。发现心肌病表明需进行放血治疗，这可能是取得良好预后的唯一方法。
