Jacquemyn Xander, Van De Bruaene Alexander, Budts Werner, Dresselaers Tom, Bogaert Jan
Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
Department of Cardiovascular Diseases, UZ Leuven, Leuven, Belgium.
Eur Heart J Cardiovasc Imaging. 2025 Mar 3;26(3):569-578. doi: 10.1093/ehjci/jeaf001.
Atrial septal defect (ASD) and partial abnormal pulmonary venous connection (PAPVC) are non-cyanotic congenital heart defects (CHDs) that produce a left-to-right shunt. This single-centre retrospective study aimed to assess the haemodynamic impact of isolated ASD, isolated PAPVC, and ASD-associated PAPVC using cardiovascular magnetic resonance (CMR).
From our CMR registry (2002-24), 110 patients were included: isolated ASD (n = 64), isolated PAPVC (n = 18), and ASD-associated PAPVC (n = 28, mostly sinus venosus septal defects). CMR included geometric assessment of the cardiac chambers and great vessels, assessment of ventricular function, and flow patterns through the systemic (Qs) and pulmonary circulation (Qp). Patients were compared with a 40 control subjects with normal CMR findings. Significant differences were observed between controls, isolated ASD, isolated PAPVC, and ASD-associated PAPVC groups in left ventricular end-diastolic volumes (EDVs) indexed to body surface area (BSA) [80 mL/m2 (75-90) vs. 71 mL/m2 (57-79) vs. 71 mL/m2 (63-80) vs. 64 mL/m2 [57-72], P < 0.001], right ventricular EDVs/BSA [82 mL/m2 (74-88) vs. 110 mL/m2 (101-127) vs. 150 mL/m2 (120-165) vs. 154 mL/m2 (128-181), P < 0.001)] and Qp/Qs ratio [0.99 (0.95-1.01) vs. 1.49 (1.34-1.67) vs. 1.96 (1.55-2.40) vs. 2.15 (1.81-2.64), P < 0.001]. No significant modulating factors were found for cardiac chambers or great vessels geometry, ventricular function, or flow patterns between the patient groups.
Despite phenotypic differences, no specific anatomical influences were found, suggesting that outcomes in isolated ASD, isolated PAPVC, and ASD-associated PAPVC are mainly driven by the degree of shunting.
房间隔缺损（ASD）和部分性肺静脉异常连接（PAPVC）是导致左向右分流的非青紫型先天性心脏病（CHD）。本单中心回顾性研究旨在使用心血管磁共振（CMR）评估孤立性ASD、孤立性PAPVC以及与ASD相关的PAPVC对血流动力学的影响。
从我们的CMR登记系统（2002 - 2024年）中纳入了110例患者：孤立性ASD（n = 64）、孤立性PAPVC（n = 18）以及与ASD相关的PAPVC（n = 28，大多为静脉窦型房间隔缺损）。CMR包括对心腔和大血管的几何形态评估、心室功能评估以及体循环（Qs）和肺循环（Qp）的血流模式评估。将患者与40例CMR检查结果正常的对照者进行比较。在对照组、孤立性ASD组、孤立性PAPVC组以及与ASD相关的PAPVC组之间，观察到以体表面积（BSA）校正的左心室舒张末期容积（EDV）存在显著差异[80 mL/m²（75 - 90）对比71 mL/m²（57 - 79）对比71 mL/m²（63 - 80）对比64 mL/m²[57 - 72]，P < 0.001]，右心室EDV/BSA也存在显著差异[82 mL/m²（74 - 88）对比110 mL/m²（101 - 127）对比150 mL/m²（120 - 165）对比154 mL/m²（128 - 181），P < 0.001]，以及Qp/Qs比值存在显著差异[0.99（0.95 - 1.01）对比1.49（1.34 - 1.67）对比1.96（1.55 - 2.40）对比2.15（1.81 - 2.64），P < 0.001]。在患者组之间，未发现对心腔或大血管几何形态、心室功能或血流模式有显著调节作用的因素。
尽管存在表型差异，但未发现特定的解剖学影响，这表明孤立性ASD、孤立性PAPVC以及与ASD相关的PAPVC的预后主要由分流程度驱动。
