Rocha Lucas Oliveira, Miyague Nelson Itiro, Solarewicz Leo Agostinho, Fernandes-Silva Miguel Morita
Pediatric Cardiologists of Hospital Infantil Pequeno Príncipe, Curitiba, Brazil.
Interventional Cardiologist of Hospital Infantil Pequeno Príncipe, Curitiba, Brazil.
Pediatr Cardiol. 2024 Sep 2. doi: 10.1007/s00246-024-03636-0.
Complete atrioventricular septal defect (CAVSD) can lead to the development of pulmonary obstructive vascular disease due to high pulmonary blood flow and pressures. This study aimed to evaluate the changes in pulmonary hemodynamics with aging and with patent ductus arteriosus (PDA) in children with CAVSD. We retrospectively evaluated 137 children (94% with trisomy 21, median age of 195 (25-2963) days, 58.4% female) with CASVD referred to cardiac catheterization from January 2000 to December 2020. Those with associated congenital heart disease, except PDA, had been excluded. They were divided into three age terciles (T1, T2, and T3). Aging was directly associated with higher mean (T1: 34.2 ± 9.1; T2: 37.1 ± 5.8; T3: 42 ± 10.6 mmHg, p < 0.001) and diastolic (T1: 19.4 ± 5.3; T2 21.6 ± 5.0; T3: 26.0 ± 9.5 mmHg, P < 0.001) pulmonary arterial pressures, and with higher pulmonary vascular resistance (T1: 3.24 ± 1.69, T2: 3.47 ± 1.19; T3: 4.49 ± 3.91 Wu.m, p = 0.023). This resulted in a loss of eligibility for anatomical correction, which became evident only after 300 days of age. PDA was associated with a higher mean (37.2 [35.9; 38.5] vs. 41.3 [37.5; 45.0] mmHg, p = 0.049) and diastolic (21.7 [20.7; 22.6] vs. 26.4 [24.1; 29.0] mmHg, p = 0.001) pulmonary pressure, and resistor-compliance time (0.28 [0.26; 0.29] vs. 0.36 [0.31; 0.40], p = 0.001) after adjusting for age and sex. In children with CAVSD, aging was associated with worsening of pulmonary vascular hemodynamics, particularly when PDA was associated, resulting in loss of eligibility for anatomical correction after 10 months of age as the first surgical option.
完全性房室间隔缺损（CAVSD）可因肺血流量增加和压力升高导致肺阻塞性血管疾病的发生。本研究旨在评估CAVSD患儿随着年龄增长以及伴有动脉导管未闭（PDA）时肺血流动力学的变化。我们回顾性评估了2000年1月至2020年12月期间接受心导管检查的137例CAVSD患儿（94%为21三体综合征，中位年龄195（25 - 2963）天，58.4%为女性）。排除了伴有除PDA之外其他先天性心脏病的患儿。将他们分为三个年龄三分位数组（T1、T2和T3）。年龄增长与更高的平均肺动脉压（T1：34.2±9.1；T2：37.1±5.8；T3：42±10.6 mmHg，p<0.001）和舒张压（T1：19.4±5.3；T2：21.6±5.0；T3：26.0±9.5 mmHg，P<0.001）以及更高的肺血管阻力（T1：3.24±1.69，T2：3.47±1.19；T3：4.49±3.91 Wu.m，p = 0.023）直接相关。这导致失去解剖矫正的适应证，这种情况在300日龄后才变得明显。在调整年龄和性别后，PDA与更高的平均肺动脉压（37.2[35.9；38.5] vs. 41.3[37.5；45.0] mmHg，p = 0.049）、舒张压（21.7[20.7；22.6] vs. 26.4[24.1；29.0] mmHg，p = 0.001）以及电阻 - 顺应性时间（0.28[0.26；0.29] vs. 0.36[0.31；0.40]，p = 0.001）相关。在CAVSD患儿中，年龄增长与肺血管血流动力学恶化相关，尤其是伴有PDA时，导致10月龄后失去作为首选手术方式的解剖矫正适应证。
