Ginting Munte Fatwiadi Apulita, Elen Elen, Lelya Olfi, Rudiktyo Estu, Prakoso Radityo, Lilyasari Oktavia
Department of Cardiology and Vascular Medicine, Faculty of Medicine, University of Indonesia, National Cardiovascular Center Harapan Kita, Jakarta, Indonesia.
Division of Cardiovascular Imaging and Nuclear Cardiology, Department of Cardiology and Vascular Medicine, Faculty of Medicine, University of Indonesia, National Cardiovascular Center Harapan Kita, Jakarta, Indonesia.
Front Cardiovasc Med. 2024 May 30;11:1395382. doi: 10.3389/fcvm.2024.1395382. eCollection 2024.
Right ventricular (RV) fibrosis represents both adaptive and maladaptive responses to the overloaded RV condition. Its role in pulmonary hypertension (PH) associated with secundum atrial septal defect (ASD), which is the most common adult congenital heart disease (CHD), remains poorly understood.
We enrolled 65 participants aged ≥18 years old with uncorrected secundum ASD who had undergone clinically indicated right heart catheterization (RHC), divided into the non-PH group ( = 7), PH group ( = 42), and Eisenmenger syndrome (ES) group ( = 16). We conducted cardiovascular magnetic resonance (CMR) studies with late gadolinium enhancement (LGE) imaging, native T1 mapping, and extracellular volume (ECV) measurement to evaluate the extent and clinical correlates of RV fibrosis.
LGE was present in 94% of the population and 86% of the non-PH group, mostly located at the right ventricular insertion point (RVIP) regions. LGE in the septal and inferior RV region was predominantly observed in the ES group compared to the other groups ( = 0.031 and < 0.001, respectively). The mean LGE scores in the ES and PH groups were significantly higher than those in the non-PH group (3.38 ± 0.96 vs. 2.74 ± 1.04 vs. 1.57 ± 0.79; = 0.001). The ES and PH groups had significantly higher degrees of interstitial RV fibrosis compared to those in the non-PH group, indicated by native T1 (1,199.9 ± 68.9 ms vs. 1,131.4 ± 47.8 ms vs. 1,105.4 ± 44.0 ms; < 0.001) and ECV (43.6 ± 6.6% vs. 39.5 ± 4.9% vs. 39.4 ± 5.8%; = 0.037). Additionally, native T1 significantly correlated with pulmonary vascular resistance ( = 0.708, < 0.001), RV ejection fraction ( = -0.468, < 0.001) and peripheral oxygen saturation ( = -0.410, = 0.001).
In patients with uncorrected secundum ASD, RV fibrosis may occur before the development of PH and progressively intensify alongside the progression of PH severity. A higher degree of RV fibrosis, derived from CMR imaging, correlates with worse hemodynamics, RV dysfunction, and poorer clinical conditions.
右心室（RV）纤维化代表了对右心室负荷过重状况的适应性和适应不良性反应。其在继发孔型房间隔缺损（ASD）相关肺动脉高压（PH）中的作用仍知之甚少，继发孔型房间隔缺损是最常见的成人先天性心脏病（CHD）。
我们纳入了65名年龄≥18岁、未矫正继发孔型ASD且接受了临床指征性右心导管检查（RHC）的参与者，分为非PH组（n = 7）、PH组（n = 42）和艾森曼格综合征（ES）组（n = 16）。我们进行了心血管磁共振（CMR）研究，采用延迟钆增强（LGE）成像、原生T1映射和细胞外容积（ECV）测量来评估RV纤维化的程度及其临床相关性。
94%的研究对象存在LGE，非PH组中这一比例为86%，LGE主要位于右心室插入点（RVIP）区域。与其他组相比，ES组中隔膜和右心室下壁区域的LGE更为常见（分别为P = 0.031和P < 0.001）。ES组和PH组的平均LGE评分显著高于非PH组（3.38 ± 0.96 vs. 2.74 ± 1.04 vs. 1.57 ± 0.79；P = 0.001）。原生T1（1,199.9 ± 68.9 ms vs. 1,131.4 ± 47.8 ms vs. 1,105.4 ± 44.0 ms；P < 0.001）和ECV（43.6 ± 6.6% vs. 39.5 ± 4.9% vs. 39.4 ± 5.8%；P = 0.037）显示，与非PH组相比，ES组和PH组的右心室间质纤维化程度显著更高。此外，原生T1与肺血管阻力显著相关（P = 0.708，P < 0.001）、右心室射血分数（P = -0.468，P < 0.001）和外周血氧饱和度（P = -0.410，P = 0.001）。
在未矫正继发孔型ASD患者中，RV纤维化可能在PH发生之前就已出现，并随着PH严重程度的进展而逐渐加重。CMR成像显示的更高程度的RV纤维化与更差的血流动力学、右心室功能障碍和更差的临床状况相关。
