Progression of ventricular wall thickening after liver transplantation for familial amyloidosis
Progression of ventricular wall thickening after liver transplantation for familial amyloidosis
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DOI：
10.1097/00007890-199707150-00014
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发表时间：
1997-07-15
期刊：
影响因子：
6.2
通讯作者：
Falk, RH
中科院分区：
文献类型：
--
作者：
Dubrey, SW;Davidoff, R;Falk, RH
背景。家族性淀粉样变性（FAP）的特点是神经系统和心脏损害进展，最终导致发病后7至15年内死亡。肝移植是治疗此病的唯一确定方法，自1990年以来一直在进行。为了确定肝移植对疾病进展的影响，对11例FAP患者进行了肝移植前0.8 ~ 8.6年和肝移植后0.8 ~ 4.1年的心电图和多普勒超声心动图随访，并进行了盲目分析。肝移植后，5例患者左心室壁增厚进展，左心室质量增加，其中3例尽管血清中突变蛋白消除，但心电图电压降低。在表现进行性壁增厚的5名患者中，4名患者患有转甲状腺素变体Glu 42 Gly， 1名患者患有Ala 36 Pro变体；其余6例患者（均为Val 30met变异）均未出现超声心动图变化。虽然11例患者中有9例神经系统症状出现症状性改善，但1例患者发生心力衰竭，2例患者发生心源性猝死。肝移植后，FAP患者应定期进行临床评估，包括心电图和超声心动图检查，以寻找心脏结构或功能的持续恶化。
Background. Familial amyloidosis (FAP) is characterized by the progression of neurologic and cardiac impairment ultimately leading to death within 7 to 15 years after the onset of the disease. Liver transplantation represents the only definitive therapy for this disease and has been performed since 1990.Methods. To determine the effect of liver transplantation on disease progression, electrocardiography and Doppler echocardiography were performed and blindly analyzed on 11 patients with FAP who were followed 0.8 to 8.6 years before liver transplantation and 0.8 to 4.1 years after liver transplantation.Results. After liver transplantation, five patients showed progression of left ventricular wall thickening with increased left ventricular mass, and three of these five showed a reduction in electrocardiographic voltage despite abolition of the mutant protein hom the serum. Of the five patients showing progressive wall thickening, four had the transthyretin variant Glu 42 Gly and one patient had the Ala 36 Pro variant; none of the remaining six patients, all of whom possessed the Val 30 Met variant, showed echocardiographic changes. Although 9 of the 11 patients have shown symptomatic improvement in neurologic symptoms, 1 patient has developed heart failure and a second patient has suffered a sudden cardiac death.Conclusions. After liver transplantation, patients with FAP should have regular clinical evaluations including electrocardiographic and echocardiographic examinations to look for continued deterioration in heart structure or function.