Clinical characteristics and prognosis of Chinese patients with hereditary transthyretin amyloid cardiomyopathy.
Shan HeZhuang TianHongzhi GuanJian LiQuan FangShu-Yang ZhangPublished in: Orphanet journal of rare diseases (2019)
The clinical characteristics of ATTR are heterogeneous: men are more likely to be affected and onset symptoms are not obvious in the heart and mainly include peripheral neuropathy and autonomic neuropathy; however, LV hypertrophy, especially a thick ventricular septum and posterior wall with preserved LVEF, are often detected on echocardiography. Abnormal ECG manifestations are common. The prognosis is poor, and patients with EF >ā50% have better survival. Clinicians should be more aware of the complex clinical profile of ATTR amyloidosis to avoid misdiagnosis in practice.