Dores Hélder, Toste Alexandra, Cardim Nuno
Hospital da Luz, Lisbon, Portugal; CHRC, NOVA Medical School, Lisbon, Portugal; NOVA Medical School, Lisbon, Portugal.
Hospital da Luz, Lisbon, Portugal; NOVA Medical School, Lisbon, Portugal.
Int J Cardiol. 2023 Nov 1;390:131260. doi: 10.1016/j.ijcard.2023.131260. Epub 2023 Aug 12.
Although the 12‑lead electrocardiogram (ECG) is abnormal in most patients with hypertrophic cardiomyopathy (HCM), some present normal ECG. This study aimed to analyse the baseline characteristics, clinical presentation and outcomes of HCM patients with normal ECG and to compare them with those with abnormal ECG.
Baseline characteristics, clinical presentation, data from complementary exams and clinical outcomes of 1070 consecutive patients included in the Portuguese Registry of HCM (Pro-HCM registry) were compared between two groups of patients: normal Vs. abnormal ECG. Among this population, 98 (9.2%) patients had normal ECG at presentation; they were significantly younger and had lower frequency of hypertension, symptoms at presentation, heart failure, angina, cardiac and non-cardiac diseases. ESC and AHA risk scores for Sudden Cardiac Death (SCD) were not significantly different between the two groups. Patients with normal ECG had higher prevalence of family history of SCD and lower degree of left ventricular (LV) hypertrophy, LV systolic dysfunction, LV outflow tract obstruction and myocardial fibrosis. The combined endpoint of cardiac death, SCD, cardiac arrest, appropriate ICD shocks or evolution to systolic dysfunction, during a mean follow-up of 5 years was significantly less frequent in patients with normal ECG (2.1% Vs. 6.5%; p = 0.043).
A normal ECG is not a marker of an overall benign profile in HCM patients. Though a normal ECG at presentation is associated with a less severe phenotype and a lower probability of evolution to heart failure at 5-years, this finding did not show a protective effect in other clinical outcomes.
尽管大多数肥厚型心肌病（HCM）患者的12导联心电图（ECG）异常，但部分患者心电图表现正常。本研究旨在分析心电图正常的HCM患者的基线特征、临床表现及预后，并与心电图异常的患者进行比较。
比较葡萄牙HCM注册研究（Pro-HCM注册研究）中连续纳入的1070例患者的基线特征、临床表现、辅助检查数据及临床预后，分为两组：心电图正常组与心电图异常组。在该人群中，98例（9.2%）患者就诊时心电图正常；他们明显更年轻，高血压、就诊时症状、心力衰竭、心绞痛、心脏和非心脏疾病的发生率更低。两组间心脏性猝死（SCD）的ESC和AHA风险评分无显著差异。心电图正常的患者SCD家族史患病率更高，左心室（LV）肥厚程度、LV收缩功能障碍、LV流出道梗阻及心肌纤维化程度更低。在平均5年的随访期间，心电图正常的患者发生心脏死亡、SCD、心脏骤停、ICD恰当电击或进展为收缩功能障碍的复合终点事件的频率显著更低（2.1%对6.5%；p = 0.043）。
心电图正常并非HCM患者整体良性特征的标志。尽管就诊时心电图正常与较轻的表型及5年内进展为心力衰竭的可能性较低相关，但这一发现并未在其他临床结局中显示出保护作用。
