Soustek Z
Pathol Annu. 1977;12 Pt 2:111-30.
Cardiac hypertrophy of obscure origin, with or without features of congestive heart failure, is a relatively common finding at autopsy. A primary abnormality of cardiac muscle has usually been considered the basis of this condition. This paper proposes that the basic pathologic process does not affect the myocardium, but rather the ascending aorta. This "aortic dystrophy" is characterized grossly by diffuse ectasia of the ascending aorta, and microscopically by typical histologic features. The hypertrophy of the left heart appears to be work hypertrophy secondary to increased volume of the ascending aorta and reduced elastic recoil of the aortic wall following ventricular systole. The disorder in "idopathic" cardiac hypertrophy with aortic dystrophy is heritable (mendelian dominant), also affects the skeleton, and can be demonstrated in relatives. Dissecting aneurysm of the aorta is an alternative manifestation of aortic dystrophy, and may occur in families or in patients with "idiopathic" cardiac hypertrophy.
病因不明的心脏肥大，伴或不伴有充血性心力衰竭的特征，是尸检中相对常见的发现。心肌的原发性异常通常被认为是这种情况的基础。本文提出，基本病理过程并非影响心肌，而是影响升主动脉。这种“主动脉营养不良”在大体上表现为升主动脉弥漫性扩张，在显微镜下表现为典型的组织学特征。左心室肥大似乎是继升主动脉容量增加和心室收缩后主动脉壁弹性回缩降低所致的工作性肥大。伴有主动脉营养不良的“特发性”心脏肥大疾病是可遗传的（孟德尔显性遗传），也会影响骨骼，并且在亲属中也可表现出来。主动脉夹层动脉瘤是主动脉营养不良的另一种表现形式，可能发生在家族中或患有“特发性”心脏肥大的患者身上。
