Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, 1-8-1, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka, 806-8501, Japan.
Department of Cardiovascular Surgery, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan.
Pediatr Cardiol. 2020 Oct;41(7):1408-1413. doi: 10.1007/s00246-020-02400-4. Epub 2020 Jun 18.
This study aimed to investigate risk factors for poor outcomes in infants with isolated atrial septal defect (ASD) and pulmonary hypertension who had unexpectedly fatal course. We retrospectively reviewed 22 infants with isolated ASD and pulmonary hypertension, and analyzed the relationship between clinical outcomes and pulmonary hemodynamic parameters including pulmonary arterial resistance (Rp) and compliance (Cp) based on cardiac catheterization among them. Age and weight at cardiac catheterization were 5 (1-11) months and 4.9 (3.1-9.2) kg, respectively. There were 17 individuals with Down syndrome. Pulmonary hemodynamic parameters were shown as follows: the ratio of pulmonary to systemic blood flow: 2.0 (0.6-3.8), mean pulmonary arterial pressure: 41 (20-60) mmHg, the ratio of pulmonary to systemic mean pressure (Pp/Ps): 0.67 (0.46-1.13), Rp: 4.11 (0.68-15.80) Wood units m, and Cp: 1.80 (0.63-6.16) mL/mmHg m. There were 4 deaths during the follow-up period of 40 (7-241) months. Univariable logistic regression analysis revealed that Pp/Ps (odds ratio [OR]: 18,500, 95% confidential interval [CI] 1.48-230,659,690, P = 0.041) and Cp (OR: 0.03, 95% CI 0.001-0.73, P = 0.031) were significantly related to death. A Pp/Ps cutoff value more than 0.94 and a Cp cutoff value less than 0.97 mL/mmHg m yielded as the predictors of death with sensitivity of 98% and 94%, specificity of 75% and 75%, respectively. Decreased Cp is a previously unrecognized predictor for poor outcome in infants with isolated ASD and pulmonary hypertension.
本研究旨在探讨伴有肺动脉高压的孤立性房间隔缺损（ASD）婴儿中，意外致命病例的不良预后的危险因素。我们回顾性分析了 22 例伴有孤立性 ASD 和肺动脉高压的婴儿，根据其中的心脏导管检查结果，分析了临床结果与肺血流动力学参数（包括肺动脉阻力（Rp）和顺应性（Cp））之间的关系。心脏导管检查时的年龄和体重分别为 5（1-11）个月和 4.9（3.1-9.2）kg。其中 17 例为唐氏综合征患儿。肺血流动力学参数如下：肺血流量与体循环血流量之比：2.0（0.6-3.8），平均肺动脉压：41（20-60）mmHg，肺循环与体循环平均压比值（Pp/Ps）：0.67（0.46-1.13），Rp：4.11（0.68-15.80）Wood 单位·m，Cp：1.80（0.63-6.16）mL/mmHg·m。随访 40（7-241）个月期间有 4 例死亡。单变量逻辑回归分析显示，Pp/Ps（比值比[OR]：18500，95%置信区间[CI]：1.48-230659690，P=0.041）和 Cp（OR：0.03，95%CI：0.001-0.73，P=0.031）与死亡显著相关。Pp/Ps 大于 0.94 和 Cp 小于 0.97 mL/mmHg·m 的截断值可作为死亡的预测因子，其敏感性分别为 98%和 94%，特异性分别为 75%和 75%。Cp 降低是伴有肺动脉高压的孤立性 ASD 婴儿不良预后的一个以前未被认识的预测因子。
