Objective Analysis of the clinical characteristics and prognosis of patients with transthyretin cardiac amyloidosis(ATTR-CA).Methods This was a single-center,retrospective study.A total of 24 patients diagnosed with ATTR-CA in Tongji Hospital,Tongji Medical College of Huazhong University of Science&Technology from August 2018 to June 2023 were enrolled.The clinical,echocardiographic,and cardiac magnetic resonance(CMR)imaging features were comprehensively analyzed,and the prognosis was evaluated through follow-up.Results The mean age of the 24 patients was 61.7±8.8 years,of whom 18(75.0%)were male,and the median time from symptom onset to definitive diagnosis was 13(9,33)months.Among the first symptoms,cardiac symptoms such as chest tightness and shortness of breath were present in 11 cases(45.8%),while the remaining manifestations were dizziness and/or syncope in 6 cases(25.0%),diarrhea in 3 cases(12.5%),limb disorder in 2 cases(8.3%),low back pain in 1 case(4.2%),and blurred vision in 1 case(4.2%).In 8 cases(33.3%),the initial department was not cardiovascular medicine.Electrocardiographic manifestations were diverse,with different types of conduction block(54.2%)being prevalent.Echocardiography examination revealed the mean left ventricular ejection fraction(LVEF)was 47.9%±12.4%,13 cases(54.2%)LVEF of<50%;21 cases(87.5%)had thickened left ventricular wall thickness,with a mean interventricular septum thickness of 17.3±5.1 mm and a mean posterior wall thickness of 15.5±3.9 mm;Right ventricular thickening,biatrial enlargement and pericardial effusion were observed in 19 cases(79.2%);Restrictive diastolic dysfunction in 17 cases(70.8%);Left ventricular global longitudinal strain(GLS)decreased with a median value of-9.1%(-12.0%,-7.2%),and 21 cases(87.5%)had the relative apical sparing.CMR was characterized by biventricular late gadolinium enhancement,elevated T1 values and extracellular volume.As of 1 November 2023,with a median follow-up time of 24(15,37)months,8(33.3%)patients died,and the median survival time was 38 months.According to the NAC staging system,10(41.7%)were in stage I,12(50.0%)were in stage Ⅱ,and 2(8.3%)were in stage Ⅲ.Survival time was longer for patients with NAC stage I than that of NAC stage Ⅱ-Ⅲ[log-rank(Mantel-Cox),χ2=4.051,P=0.044].Conclusions ATTR-CA patients have high clinical heterogeneity and poor overall prognosis,with the worst prognosis for patients with NAC stage Ⅱ-Ⅲ.
关键词
转甲状腺素蛋白/心肌淀粉样变性/射血分数保留的心力衰竭/预后
Key words
Transthyretin/Cardiac amyloidosis/Heart failure with preserved ejection fraction/Prognosis
维普
万方数据