Department of Radiology, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
Circ Cardiovasc Imaging. 2010 May;3(3):290-7. doi: 10.1161/CIRCIMAGING.109.911313. Epub 2010 Mar 2.
Although arrhythmogenic right ventricular dysplasia (ARVD) predominantly affects the right ventricle (RV), genetic/molecular and histological changes are biventricular. Regional left ventricular (LV) function has not been systematically studied in ARVD.
The study population included 21 patients with suspected ARVD who underwent evaluation with MRI including tagging. Eleven healthy volunteers served as control subjects. Peak systolic regional circumferential strain (Ecc, %) was calculated by harmonic phase from tagged MRI based on the 16-segment model. Patients who met ARVD Task Force criteria were classified as definite ARVD, whereas patients with a positive family history who had 1 additional minor criterion and patients without a family history with 1 major or 2 minor criteria were classified as probable ARVD. Of the 21 ARVD subjects, 11 had definite ARVD and 10 had probable ARVD. Compared with control subjects, probable ARVD patients had similar RV ejection fraction (58.9+/-6.2% versus 53.5+/-7.6%, P=0.20), but definite ARVD patients had significantly reduced RV ejection fraction (58.9+/-6.2% versus 45.2+/-6.0%, P=0.001). LV ejection fraction was similar in all 3 groups. Compared with control subjects, peak systolic Ecc was significantly less negative in 6 of 16 (37.5%) segments in definite ARVD and 3 of 16 segments (18.7%) in probable ARVD (all P<0.05).
ARVD is associated with regional LV dysfunction, which appears to parallel degree of RV dysfunction. Further large studies are needed to validate this finding and to better define implications of subclinical segmental LV dysfunction.
尽管致心律失常性右室心肌病（ARVD）主要影响右心室（RV），但遗传/分子和组织学变化是双心室的。ARVD 中尚未系统研究局部左心室（LV）功能。
研究人群包括 21 名接受 MRI 评估的疑似 ARVD 患者，包括标记。11 名健康志愿者作为对照。基于 16 节段模型，通过来自标记 MRI 的谐相计算峰值收缩期局部周向应变（Ecc，%）。符合 ARVD 工作组标准的患者被归类为明确 ARVD，而有阳性家族史且有 1 项额外次要标准的患者和无家族史且有 1 项主要或 2 项次要标准的患者被归类为可能 ARVD。在 21 名 ARVD 受试者中，有 11 名患有明确 ARVD，有 10 名患有可能 ARVD。与对照组相比，可能 ARVD 患者的 RV 射血分数相似（58.9+/-6.2%与 53.5+/-7.6%，P=0.20），但明确 ARVD 患者的 RV 射血分数明显降低（58.9+/-6.2%与 45.2+/-6.0%，P=0.001）。三组的 LV 射血分数相似。与对照组相比，明确 ARVD 中有 6 个（37.5%）节段的收缩期峰值 Ecc 明显为负，可能 ARVD 中有 3 个（18.7%）节段（所有 P<0.05）。
ARVD 与局部 LV 功能障碍相关，这似乎与 RV 功能障碍的程度平行。需要进一步的大型研究来验证这一发现，并更好地定义亚临床节段性 LV 功能障碍的影响。
