Kristen Arnt V, Perz Jolanta B, Schonland Stefan O, Hansen Alexander, Hegenbart Ute, Sack Falk-Udo, Goldschmidt Hartmut, Katus Hugo A, Dengler Thomas J
Department of Cardiology, Angiology, and Respiratory Medicine, University Hospital Heidelberg, Heidelberg, Germany.
J Heart Lung Transplant. 2007 Dec;26(12):1313-9. doi: 10.1016/j.healun.2007.09.014. Epub 2007 Nov 26.
Cardiac amyloidosis (CA) is the most problematic cause of heart failure because medical treatment strategies are not well tolerated. Due to its high mortality, identification of patients at high risk is crucial for treatment strategies such as heart transplantation prior to chemotherapy for amyloid disease.
Left ventricular wall thickness (LVT) progression was retrospectively compared with electrocardiographic and echocardiographic parameters for risk prediction in 39 patients with histologically proven cardiac amyloidosis.
Seventeen deaths occurred, equivalent to 1- and 3-year survival rates of 62.1% and 55.0%, respectively. LVT progression in deceased patients was 2.02 +/- 0.85 mm/month compared with 0.19 +/- 0.03 mm/month in survivors (p < 0.001). Autologous stem-cell transplantation (n = 22, or 54%) reduced LVT progression as compared with not receiving stem cells (0.21 +/- 0.04 mm/month vs 1.45 +/- 0.57 mm/month, p < 0.005). LVT progression correlated with maximal LVT and absolute LVT increase. Progression of LVT was more rapid in patients with impaired LV ejection fraction (LVEF) than preserved LVEF (2.16 +/- 1.04 mm/month vs 0.30 +/- 0.13 mm/month, p < 0.001). LVT closely correlated with survival, whereas initial, maximum or absolute increase in LVT did not. Further predictors of survival were LVEF, autologous stem-cell transplantation and low voltage, but not diastolic dysfunction. Multivariate analysis identified LVT progression as the strongest independent parameter for survival.
LVT progression is a powerful risk predictor in light-chain CA, superior to parameters such as LVEF, LVT or a low-voltage pattern. Improved survival by high-dose chemotherapy and stem-cell transplantation is paralleled by a reduction in LVT progression. Repetitive echocardiographic assessment appears indicated in CA patients to identify candidates for heart transplantation in amyloidosis.
心脏淀粉样变性（CA）是心力衰竭最棘手的病因，因为药物治疗策略耐受性不佳。鉴于其高死亡率，识别高危患者对于诸如在淀粉样疾病化疗前进行心脏移植等治疗策略至关重要。
回顾性比较39例经组织学证实的心脏淀粉样变性患者的左心室壁厚度（LVT）进展与心电图和超声心动图参数，以进行风险预测。
17例患者死亡，1年和3年生存率分别为62.1%和55.0%。死亡患者的LVT进展为2.02±0.85mm/月，而存活患者为0.19±0.03mm/月（p<0.001）。与未接受干细胞治疗相比，自体干细胞移植（n=22，占54%）可降低LVT进展（0.21±0.04mm/月对1.45±0.57mm/月，p<0.005）。LVT进展与最大LVT及LVT绝对增加值相关。左心室射血分数（LVEF）受损的患者LVT进展比LVEF保留的患者更快（2.16±1.04mm/月对0.30±0.13mm/月，p<0.001）。LVT与生存率密切相关，而LVT的初始值、最大值或绝对增加值则不然。生存率的其他预测因素为LVEF、自体干细胞移植和低电压，但不包括舒张功能障碍。多变量分析确定LVT进展是生存的最强独立参数。
在轻链型CA中，LVT进展是一个强大的风险预测指标，优于LVEF、LVT或低电压图形等参数。高剂量化疗和干细胞移植改善生存率的同时，LVT进展也有所降低。对于CA患者，似乎需要反复进行超声心动图评估，以识别淀粉样变性中适合心脏移植的候选者。
