Stöllberger Claudia, Blazek Gerhard, Gessner Martin, Bichler Katharina, Wegner Christian, Finsterer Josef
Krankenanstalt Rudolfstiftung, Vienna, Austria.
Hanusch Krankenhaus, Vienna, Austria.
Am J Cardiol. 2015 May 1;115(9):1287-92. doi: 10.1016/j.amjcard.2015.02.014. Epub 2015 Feb 12.
Left ventricular hypertrabeculation/noncompaction (LVHT) is diagnosed in all ages and is frequently associated with neuromuscular disorders (NMDs). The aim of the study was to compare patients with LVHT depending on age at diagnosis. Included were 232 patients with LVHT (72 women, mean age 52±17 years) diagnosed from 1995 to 2014 at 1 echocardiographic laboratory. In 2014, their survival was assessed. Seventy-six percent of the patients were neurologically investigated, revealing specific NMDs in 18%, unspecific NMDs in 60%, and normal findings in 22%. Forty-five patients (19%) received electronic devices: implantable cardioverter-defibrillators in 26 patients, combined with cardiac resynchronization systems (n=14) or an antibradycardic pacemaker (n=1); antibradycardic pacemakers (n=8); cardiac resynchronization systems (n=4); implantable loop recorders (n=4); life vests (n=2); and a left ventricular assist device as a bridge to transplantation (n=1). During 72-month follow-up, mortality was 4.9% per year. In younger age groups, more patients were referred for syncope or palpitations, whereas in older age groups, more patients were referred for heart failure. Classic cardiovascular risk factors such as hypertension and diabetes, as well as coronary artery stenosis, were rare in the young age groups but were more prevalent in older age groups. Differences between age groups were found regarding cardiac symptoms, NMDs, electrocardiographic findings, rate of device implantation, and mortality but not in location and extension of LVHT. None of the neurologically investigated patients≥70 years of age was neurologically normal. Prevalence of heart failure, electrocardiographic abnormalities, and mortality were highest in the oldest age group. In conclusion, LVHT must be considered as an echocardiographic diagnosis in all age groups. The morphologic pattern of LVHT is similar, whereas clinical manifestations and prognosis are variable among age groups.
左心室致密化不全（LVHT）在各年龄段均可诊断，且常与神经肌肉疾病（NMDs）相关。本研究的目的是比较不同诊断年龄的LVHT患者。纳入了1995年至2014年在1个超声心动图实验室诊断的232例LVHT患者（72例女性，平均年龄52±17岁）。2014年，评估了他们的生存情况。76%的患者接受了神经学检查，其中18%发现特定的NMDs，60%发现非特异性NMDs，22%检查结果正常。45例患者（19%）接受了电子设备治疗：26例患者植入了植入式心律转复除颤器，其中14例联合心脏再同步系统，1例联合抗心动过缓起搏器；8例患者植入抗心动过缓起搏器；4例患者植入心脏再同步系统；4例患者植入植入式循环记录仪；2例患者使用救生背心；1例患者植入左心室辅助装置作为移植过渡。在72个月的随访期间，年死亡率为4.9%。在较年轻的年龄组中，更多患者因晕厥或心悸就诊，而在较年长的年龄组中，更多患者因心力衰竭就诊。高血压、糖尿病等经典心血管危险因素以及冠状动脉狭窄在年轻年龄组中少见，但在年长年龄组中更普遍。在心脏症状、NMDs、心电图表现、设备植入率和死亡率方面发现了年龄组间的差异，但LVHT的部位和范围无差异。年龄≥70岁且接受神经学检查的患者中，无一例神经学检查结果正常。心力衰竭、心电图异常和死亡率在最年长年龄组中最高。总之，LVHT在所有年龄组中均应被视为一种超声心动图诊断。LVHT的形态学模式相似，而各年龄组的临床表现和预后各不相同。
