Department of Cardiovascular Imaging, Hôpital Cardiologique du Haut-Lévêque, CHU de Bordeaux, Avenue de Magellan, 33604 Pessac, France.
Department of Healthcare Technologies, IHU LIRYC, Université de Bordeaux-Inserm, Avenue du Haut Lévêque, 33604, Pessac, France.
Eur Heart J Cardiovasc Imaging. 2019 Sep 1;20(9):990-1003. doi: 10.1093/ehjci/jez068.
To identify the correlates of focal scar and diffuse fibrosis in patients with history of tetralogy of Fallot (TOF) repair.
Consecutive patients with prior TOF repair underwent electrocardiogram, 24-h Holter, transthoracic echocardiography, exercise testing, and cardiac magnetic resonance (CMR) including cine imaging to assess ventricular volumes and ejection fraction, T1 mapping to assess left ventricular (LV) and right ventricular (RV) diffuse fibrosis, and free-breathing late gadolinium-enhanced imaging to quantify scar area at high spatial resolution. Structural imaging data were related to clinical characteristics and functional imaging markers. Cine and T1 mapping results were compared with 40 age- and sex-matched controls. One hundred and three patients were enrolled (age 28 ± 15 years, 36% women), including 36 with prior pulmonary valve replacement (PVR). Compared with controls, TOF showed lower LV ejection fraction (LVEF) and RV ejection fraction (RVEF), and higher RV volume, RV wall thickness, and native T1 and extracellular volume values on both ventricles. In TOF, scar area related to LVEF and RVEF, while LV and RV native T1 related to RV dilatation. On multivariable analysis, scar area and LV native T1 were independent correlates of ventricular arrhythmia, while RVEF was not. Patients with history of PVR showed larger scars on RV outflow tract but shorter LV and RV native T1.
Focal scar and biventricular diffuse fibrosis can be characterized on CMR after TOF repair. Scar size relates to systolic dysfunction, and diffuse fibrosis to RV dilatation. Both independently relate to ventricular arrhythmias. The finding of shorter T1 after PVR suggests that diffuse fibrosis may reverse with therapy.
确定法洛四联症（TOF）修复后患者局灶性瘢痕和弥漫性纤维化的相关因素。
连续的 TOF 修复后患者接受了心电图、24 小时 Holter、经胸超声心动图、运动试验和心脏磁共振（CMR）检查，包括电影成像以评估心室容积和射血分数、T1 映射以评估左心室（LV）和右心室（RV）弥漫性纤维化以及自由呼吸式晚期钆增强成像以高空间分辨率量化瘢痕面积。结构成像数据与临床特征和功能成像标志物相关。比较电影和 T1 映射结果与 40 名年龄和性别匹配的对照。共纳入 103 名患者（年龄 28±15 岁，36%为女性），其中 36 名患者有既往肺动脉瓣置换术（PVR）。与对照组相比，TOF 的 LV 射血分数（LVEF）和 RV 射血分数（RVEF）较低，RV 容积、RV 壁厚度以及心室的原始 T1 和细胞外容积值较高。在 TOF 中，瘢痕面积与 LVEF 和 RVEF 相关，而 LV 和 RV 原始 T1 与 RV 扩张相关。多变量分析表明，瘢痕面积和 LV 原始 T1 是室性心律失常的独立相关因素，而 RVEF 不是。有 PVR 病史的患者 RV 流出道有较大的瘢痕，但 LV 和 RV 原始 T1 较短。
TOF 修复后 CMR 可对局灶性瘢痕和双心室弥漫性纤维化进行特征描述。瘢痕大小与收缩功能障碍有关，弥漫性纤维化与 RV 扩张有关。两者都与室性心律失常独立相关。PVR 后 T1 缩短提示弥漫性纤维化可能随治疗而逆转。
