Magnetic Resonance Unit, Department of Radiology, National Institute of Cardiology, ul. Alpejska 42, 04-628, Warsaw, Poland.
Department of Interventional Cardiology and Angiology, National Institute of Cardiology, Warsaw, Poland.
Sci Rep. 2020 Dec 3;10(1):21054. doi: 10.1038/s41598-020-78245-x.
We investigated factors associated with right ventricular (RV) function and size in hypertrophic cardiomyopathy (HCM) patients. Two hundred fifty-three consecutive HCM patients and 20 healthy volunteers underwent cardiac magnetic resonance examination. In addition to measuring RV function (ejection fraction-RVEF) and size (end-diastolic volume-RVEDV), each image was inspected for the presence of RV and left ventricular (LV) hypertrophy, and the maximal wall thickness of the left and right ventricles was recorded. HCM patients had higher RVEF and lower RVEDV than healthy volunteers and similar RV mass. The mean RV wall thickness was higher in HCM patients than in controls. LV late gadolinium enhancement (LGE) was present in 89.7% of patients, and RV LGE was present in 3.1% of patients (p < 0.0001). Univariate and multivariable analyses revealed that LVEF, peak LV outflow tract gradient, LV LGE, maximal LV wall thickness, and tricuspid regurgitation (TR) volume by magnetic resonance imaging were positive predictors of RVEF. In addition to TR volume, the only independent predictor of RVEF < 45% was LVEF (odds ratio = 0.80, 95% confidence interval 0.67-0.95). Multivariable analysis revealed that LVEDV and TR volume were positive predictors of RVEDV, whereas negative predictors were RVEF, maximal RV wall thickness, LV LGE, and age. Neither estimated systolic pulmonary artery pressure nor TR grade by echocardiography proved to be predictors of RVEF. There were no differences in either the maximal RV wall thickness or the maximal left ventricular (LV) wall thickness in patients stratified according to NYHA functional class (p = 0.93 and p = 0.15, respectively). There were no differences in mean RV wall thickness in patients categorised based on the number of clinical risk factors for sudden cardiac death (SCD), i.e., non-sustained ventricular tachycardia, family history of SCD, or unexplained syncope (p = 0.79). On the other hand, there was a weak positive association between RV hypertrophy and the estimated probability of SCD at 5 years (rho = 0.16, p = 0.01). RV systolic dysfunction measured as decreased RVEF was uncommon in HCM and was associated with poor LV systolic function. LV also had a significant impact on RV size.
我们研究了肥厚型心肌病（HCM）患者右心室（RV）功能和大小相关的因素。253 例连续 HCM 患者和 20 名健康志愿者接受了心脏磁共振检查。除了测量 RV 功能（射血分数-RVEF）和大小（舒张末期容积-RVEDV）外，还检查了每个图像是否存在 RV 和左心室（LV）肥大，并记录了左、右心室的最大壁厚度。HCM 患者的 RVEF 高于健康志愿者，RVEDV 低于健康志愿者，而 RV 质量相似。HCM 患者的 RV 壁厚度平均值高于对照组。89.7%的患者存在 LV 晚期钆增强（LGE），3.1%的患者存在 RV LGE（p<0.0001）。单因素和多因素分析显示，LVEF、LV 流出道峰值梯度、LV LGE、最大 LV 壁厚度和磁共振成像的三尖瓣反流（TR）量是 RVEF 的正预测因子。除了 TR 量外，唯一能独立预测 RVEF<45%的因素是 LVEF（比值比=0.80，95%置信区间 0.67-0.95）。多因素分析显示，LVEDV 和 TR 量是 RVEDV 的正预测因子，而 RVEF、最大 RV 壁厚度、LV LGE 和年龄是负预测因子。超声心动图估测的收缩期肺动脉压或 TR 分级均不能预测 RVEF。根据 NYHA 功能分级分层的患者，最大 RV 壁厚度或最大 LV 壁厚度均无差异（p=0.93 和 p=0.15）。根据发生心脏性猝死（SCD）的临床危险因素数量对患者进行分类时，平均 RV 壁厚度无差异，即非持续性室性心动过速、SCD 家族史或不明原因晕厥（p=0.79）。另一方面，RV 肥大与 5 年时 SCD 的估计概率呈弱正相关（rho=0.16，p=0.01）。作为 RVEF 降低的 RV 收缩功能障碍在 HCM 中并不常见，与 LV 收缩功能不良有关。LV 对 RV 大小也有显著影响。
