Clinical, ECG and echocardiographic clues to the diagnosis of TTR-related cardiomyopathy.
Clinical, ECG and echocardiographic clues to the diagnosis of TTR-related cardiomyopathy.
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DOI：
10.1136/openhrt-2015-000289
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发表时间：
2016
期刊：
影响因子：
2.7
通讯作者：
Kristen AV
中科院分区：
文献类型：
--
作者：
Damy T;Maurer MS;Rapezzi C;Planté-Bordeneuve V;Karayal ON;Mundayat R;Suhr OB;Kristen AV
心脏甲状腺素运载蛋白（TTR）淀粉样变性（ATTR）在超声心动图室间隔厚度（IVST）增加的患者中缺乏体征。确定与ATTR中IVST增加相关的临床和ECG/超声心动图体征。甲状腺素运载蛋白淀粉样变性结局调查（THAOS）登记研究中基线超声心动图患者的分析（N=1682）。根据美国超声心动图学会的分类，将患者分为IVST类别（即正常、轻度、中度、重度）;然后分为两个合并的IVST类别（正常-轻度和中度-重度）。425例患者包括：336例TTR突变（m-TTR）和89例野生型TTR（WT-TTR）。72%为男性。中位（第25、75百分位数）年龄为62（45、72）岁。Non-Val 30 Met和WT-TTR在中度（41%和35%）和重度（50%和33%）IVST类别中常见。中位IVST为15 mm（14，16）（中度）和20 mm（18，22）（重度）。 在合并的中重度分类中，85%的患者年龄≥55岁; 81%为男性; 86%的患者血压<140 mm Hg; 77%的患者右心室厚度增加（≥7 mm）。 高达66%的患者有心脏闪烁。收缩功能不全（左心室射血分数<50%）、限制型和低电压发生率较低，分别为49%、18%和33%。IVST增加，特别是在收缩压正常、右心室游离壁和瓣膜厚度增加以及闪烁的≥55岁男性中，应提醒从业者注意ATTR的可能性。 缺乏限制性模式和低电压不应排除怀疑。NCT 00628745（clinicaltrials.gov）。
Signs of cardiac transthyretin (TTR) amyloidosis (ATTR) in patients with echocardiographic increase in interventricular septal thickness (IVST) are lacking. To identify clinical and ECG/echocardiographic signs associated with increased IVST in ATTR. Analysis of patients with baseline echocardiography in the Transthyretin Amyloidosis Outcomes Survey (THAOS) registry (N=1682). Patients were categorised into IVST classes according to the American Society of Echocardiography classification adapted to gender (ie, normal, mild, moderate, severe); then into two combined IVST classes (normal-mild and moderate-severe). 425 patients were included: 336 with a TTR mutation (m-TTR) and 89 with wild-type TTR (WT-TTR). 72% were men. Median (25th, 75th centile) age was 62 (45, 72) years. Non-Val30Met and WT-TTR were frequent in moderate (41% and 35%) and severe (50% and 33%) IVST classes. Median IVST was 15 mm (14, 16) (moderate) and 20 mm (18, 22) (severe). In the combined moderate-severe class, 85% of patients were ≥55 years of age; 81% were men; 86% had blood pressure <140 mm Hg; and 77% had increased right ventricle thickness (≥7 mm). Up to 66% of patients had cardiac sparkling. Systolic dysfunction (left ventricular ejection fraction <50%), restrictive pattern and low voltage were less frequent, and observed in 49%, 18% and 33% of patients, respectively. Increased IVST, especially in men ≥55 years with normal systolic blood pressure, increase in right ventricle free wall and valve thicknesses, and sparkling, should alert practitioners to the possibility of ATTR. Absence of restrictive pattern and low voltage should not rule out the suspicion. NCT00628745 (clinicaltrials.gov).