Suhr O B, Anan I, Backman C, Karlsson A, Lindqvist P, Mörner S, Waldenström A
Department of Public Health and Clinical Medicine, Umeå University Hospital, Umeå, Sweden.
J Intern Med. 2008 Mar;263(3):294-301. doi: 10.1111/j.1365-2796.2007.01888.x. Epub 2007 Dec 5.
Cardiomyopathy is a well known complication in familial amyloidotic polyneuropathy (FAP). Troponin T and B-natriuretic peptide (BNP) have been shown to be excellent markers for heart complications in AL-amyloidosis. The aim of the study was to investigate troponin T, troponin I and BNP as markers for myocardial damage and failure in FAP.
Retrospective investigation of patients with FAP.
Tertiary referral centre.
Twenty-nine patients who had been submitted for evaluation of FAP.
Two-dimensional M-mode and Doppler echocardiography and strain echocardiographic examination. Measurement of Troponin T, troponin I and BNP.
Troponin T was detectable in only three patients who all had abnormal interventricular septal (IVS) thickness. Troponin I was abnormal in six patients (21%), of which only two had an increased IVS thickness. The heart function was generally well preserved in the patients in spite of hypertrophy of the IVS in 14 patients. BNP was elevated in 22 patients (76%), and it correlated significantly with IVS thickness and basal septal strain.
Transthyretin amyloid seems to be less harmful to myocytes than that of AL amyloid as evaluated by serum troponin T and I as well as by echocardiography. BNP appears to be a sensitive marker for cardiomyopathy in FAP, and could prove valuable for follow-up purposes as has been shown for AL-amyloidosis patients.
心肌病是家族性淀粉样多神经病(FAP)中一种众所周知的并发症。肌钙蛋白T和B型利钠肽(BNP)已被证明是AL淀粉样变性中心脏并发症的优秀标志物。本研究的目的是调查肌钙蛋白T、肌钙蛋白I和BNP作为FAP中心肌损伤和衰竭的标志物。
对FAP患者进行回顾性研究。
三级转诊中心。
29例接受FAP评估的患者。
二维M型和多普勒超声心动图以及应变超声心动图检查。测量肌钙蛋白T、肌钙蛋白I和BNP。
仅在3例患者中检测到肌钙蛋白T,这3例患者的室间隔(IVS)厚度均异常。6例患者(21%)的肌钙蛋白I异常,其中只有2例IVS厚度增加。尽管14例患者存在IVS肥厚,但总体心脏功能良好。22例患者(76%)的BNP升高,且与IVS厚度和基底间隔应变显著相关。
通过血清肌钙蛋白T和I以及超声心动图评估,转甲状腺素蛋白淀粉样变似乎比AL淀粉样变更少损害心肌细胞。BNP似乎是FAP中心肌病的敏感标志物,并且如对AL淀粉样变性患者所显示的那样,可能对随访有价值。