Viliani Dafne, Pozo Eduardo, Aguirre Norma, Cecconi Alberto, Olivera María J, Caballero Paloma, Jiménez-Borreguero Luis J, Alfonso Fernando
Cardiology Department, Hospital Universitario de la Princesa, IIS-IP, Universidad Autónoma de Madrid, Calle Diego de León, 62, 28006, Madrid, Spain.
Radiology Department, Hospital Universitario de la Princesa, IIS-IP, Universidad Autónoma de Madrid, Calle Diego de León, 62, 28006, Madrid, Spain.
Int J Cardiovasc Imaging. 2017 Nov;33(11):1771-1780. doi: 10.1007/s10554-017-1161-8. Epub 2017 May 23.
Recently a novel pattern of helical distribution of hypertrophy has been described in patients with hypertrophic cardiomyopathy (HCM). Our aim was to determine its prevalence and potential implications in an unselected cohort. One-hundred- and eight consecutive patients diagnosed with HCM by cardiac magnetic resonance (CMR) were included (median clinical follow up of 1718 days). All clinical and complementary test information was prospectively collected. The presence of a helical pattern was assessed by a simple measurement of the maximal left ventricle (LV) wall thickness (LVWT) for each of the 17 classical LV segments and it was classified in one of three types according to its extension. A helical distribution was detected in 58% of patients, and was associated to a higher incidence of left ventricular outflow tract obstruction (LVOT; 35% vs. 10%; p = 0.005) and systolic anterior motion of the mitral valve (SAM; 30% vs. 13%, p = 0.053). No significant difference in the maximal LVWT was observed. However, the presence of a helical pattern showed a significant association with non sustained ventricular tachycardia (NSVT; 22% vs. 7%; p = 0.029) and was associated to a higher risk of sudden cardiac death (SCD) calculated with the European society of cardiology (ESC) calculator (p = 0.006). Notably, patients with a more extense spiral had a higher incidence of heart failure (75% vs. 34%, p = 0.012) and all-cause death (21 vs. 3%, p = 0.049). A helical pattern is frequent in HCM and can be readily assessed on CMR standard cine sequences. In conclusion, a helical pattern carries negative clinical implications and is associated to a higher estimated risk of SCD.
最近，在肥厚型心肌病（HCM）患者中描述了一种新的肥厚螺旋分布模式。我们的目的是确定其在未经选择的队列中的患病率及其潜在影响。纳入了108例通过心脏磁共振成像（CMR）诊断为HCM的连续患者（中位临床随访时间为1718天）。前瞻性收集了所有临床和辅助检查信息。通过简单测量17个经典左心室（LV）节段中每个节段的最大左心室壁厚度（LVWT）来评估螺旋模式的存在，并根据其范围将其分为三种类型之一。在58%的患者中检测到螺旋分布，并且与左心室流出道梗阻（LVOT）的发生率较高相关（35%对10%；p = 0.005）以及二尖瓣收缩期前向运动（SAM；30%对13%，p = 0.053）。未观察到最大LVWT有显著差异。然而，螺旋模式的存在与非持续性室性心动过速（NSVT；22%对7%；p = 0.029）有显著关联，并且与使用欧洲心脏病学会（ESC）计算器计算出的心脏性猝死（SCD）风险较高相关（p = 0.006）。值得注意的是，螺旋范围更广的患者心力衰竭发生率更高（75%对34%，p = 0.012）以及全因死亡率更高（21%对3%，p = 0.049）。螺旋模式在HCM中很常见，并且可以在CMR标准电影序列上轻松评估。总之，螺旋模式具有负面临床意义，并且与更高的SCD估计风险相关。
