Department of Pediatrics, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu Mie, 514-8507, Japan.
Eur J Pediatr. 2024 Nov 19;184(1):26. doi: 10.1007/s00431-024-05843-6.
To evaluate pulmonary hemodynamics in patients who had been followed up for bronchopulmonary dysplasia (BPD)-associated pulmonary hypertension (PH) in the mid-term by right heart catheterization (RHC). A retrospective study was conducted among 56 patients who were born at a gestational age < 28 weeks during 2018-2020, and the consecutive patients complicated by BPD and ultrasound-defined PH (n = 10, 18%), of whom 7 patients were treated with sildenafil, were examined by RHC in the mid-term follow-up (median age: 25 months [IQR: 19-32]). Ultrasound study at RHC showed improvement in PH as represented by left ventricular end-systolic eccentricity index (median [IQR], 1.05 [IQR: 1.03-1.06] vs. 1.18 [IQR: 1.15-1.33]), pulmonary artery acceleration time (PAAT) (mean ± SD, 97.3 ± 18.2 vs. 55.2 ± 10.1) and a ratio of PAAT to right ventricular ejection time (0.35 ± 0.05 vs. 0.25 ± 0.04) compared with those at 40 weeks of postmenstrual age (PMA) (p < 0.05, respectively). RHC (mean pulmonary artery [PA] pressure: 21 [19-22] mmHg; pulmonary vascular resistance index [PVRi]: 2.63 [1.95-2.94] Wood Units·m) revealed that 5 infants (50%) had mild PH (21-24 mmHg) and reduced peripheral PAs by pulmonary wedge angiography. The presence of emphysema on chest CT at 40 weeks of PMA and moderate/marked reduction of contrast filling in peripheral PAs on angiography were correlated with increased PVRi (p < 0.05, respectively).
We observed that even after apparent resolution of ultrasound-defined PH, half of patients had catheterization-defined PH and vasculopathy at 2 years of age. These findings warrant long-term follow-up studies of BPD-associated PH.
• Pulmonary hypertension (PH) is associated with increased mortality in infants with bronchopulmonary dysplasia (BPD). Mortality in patients with BPD-associated PH predominantly occurs within the first 6 months of life and most patients who survive beyond 6 months show resolution of PH on echocardiography by 2 to 3 years of age.
• Even after the resolution of echocardiography-derived PH in infants with BPD-associated PH in the mid-term, the majority of such patients have abnormal pulmonary vasculature with PH or mildly elevated pulmonary artery pressure on right heart catheterization (RHC), indicating that echocardiography is insufficient and RHC may be necessary to follow up this population of patients.
通过右心导管检查（RHC）评估在中期随访中患有支气管肺发育不良（BPD）相关肺动脉高压（PH）的患者的肺血流动力学。
对 2018 年至 2020 年期间胎龄 <28 周出生的 56 例患者进行了一项回顾性研究，其中 10 例（18%）并发 BPD 和超声定义的 PH（n=10），其中 7 例患者接受了西地那非治疗，在中期随访中进行了 RHC 检查（中位年龄：25 个月[IQR：19-32]）。RHC 上的超声研究显示 PH 有所改善，表现为左心室收缩末期偏心指数（中位数[IQR]，1.05[IQR：1.03-1.06] vs. 1.18[IQR：1.15-1.33]）、肺动脉加速时间（PAAT）（平均值±SD，97.3±18.2 vs. 55.2±10.1）和 PAAT 与右心室射血时间的比值（0.35±0.05 vs. 0.25±0.04）与 40 周龄（PMA）时相比（p<0.05）。RHC（平均肺动脉[PA]压：21[19-22]mmHg；肺血管阻力指数[PVRi]：2.63[1.95-2.94]Wood 单位·m）显示，5 名婴儿（50%）有轻度 PH（21-24mmHg），肺动脉楔压造影显示外周 PA 减少。40 周龄时 PMA 的胸部 CT 上存在肺气肿和造影剂在外周 PA 中的中度/明显充盈减少与 PVRi 增加相关（p<0.05）。
我们观察到，即使超声定义的 PH 明显缓解，仍有一半的患者在 2 岁时存在导管定义的 PH 和血管病变。这些发现需要对 BPD 相关 PH 进行长期随访研究。
•支气管肺发育不良（BPD）患儿的肺动脉高压（PH）与死亡率增加有关。患有 BPD 相关 PH 的患者的死亡率主要发生在生命的前 6 个月内，大多数存活超过 6 个月的患者在 2 至 3 岁时通过超声心动图显示 PH 缓解。
•即使在患有 BPD 相关 PH 的婴儿的中期超声心动图 PH 缓解后，大多数此类患者的肺血管仍存在异常，或在右心导管检查（RHC）时肺动脉压轻度升高，表明超声心动图不足，RHC 可能有必要对该人群进行随访。
