Akangire Gangaram G, Manimtim Winston, Agarwal Amit, Alexiou Stamatia, Aoyama Brianna C, Austin Eric D, Bansal Manvi, Fierro Julie L, Hayden Lystra P, Kaslow Jacob A, Lai Khanh V, Levin Jonathan C, Miller Audrey N, Rice Jessica L, Tracy Michael C, Baker Christopher D, Bauer Sarah E, Cristea A Ioana, Dawson Sara K, Eldredge Laurie, Henningfeld Jennifer K, McKinney Robin L, Siddaiah Roopa, Villafranco Natalie M, Abman Steven H, McGrath-Morrow Sharon A, Collaco Joseph M
Division of Neonatology, Children's Mercy-Kansas City and University of Missouri Kansas City School of Medicine, Kansas City, MO, USA.
Division of Pulmonary Medicine, Arkansas Children's Hospital and University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Pediatr Res. 2025 Jan;97(1):387-394. doi: 10.1038/s41390-024-03495-8. Epub 2024 Aug 24.
To characterize a cohort of ventilator-dependent infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) and to describe their cardiorespiratory outcomes.
Subjects with BPD on chronic home ventilation were recruited from outpatient clinics. PH was defined by its presence on ≥1 cardiac catheterization or echocardiogram on or after 36 weeks post-menstrual age. Kaplan-Meier analysis was used to compare the timing of key events.
Of the 154 subjects, 93 (60.4%) had PH and of those, 52 (55.9%) required PH-specific medications. The ages at tracheostomy, transition to home ventilator, and hospital discharge were older in those with PH. Most subjects were weaned off oxygen and liberated from the ventilator by 5 years of age, which did not occur later in subjects with PH. The mortality rate after initial discharge was 2.6%.
The majority of infants with BPD-PH receiving chronic invasive ventilation at home survived after initial discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen and PH medications, ventilator liberation, and tracheostomy decannulation. While the presence of PH was not associated with later ventilator liberation or decannulation, the use of PH medications may be a marker of a more protracted disease trajectory.
There is limited data on long-term outcomes of children with bronchopulmonary dysplasia (BPD) who receive chronic invasive ventilation at home, and no data on those with the comorbidity of pulmonary hypertension (PH). Almost all subjects with BPD-PH who were on chronic invasive ventilation at home survived after their initial hospital discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen, PH medications, liberation from the ventilator, and tracheostomy decannulation. The presence of PH did not result in later ventilator liberation or decannulation; however, the use of outpatient PH medications was associated with later ventilation liberation and decannulation.
对一组依赖呼吸机的支气管肺发育不良相关性肺动脉高压（BPD-PH）婴幼儿进行特征描述，并阐述其心肺功能转归。
从门诊招募长期家庭通气的BPD患儿。PH定义为在月经龄36周及以后的≥1次心导管检查或超声心动图检查中发现PH。采用Kaplan-Meier分析比较关键事件的发生时间。
154例受试者中，93例（60.4%）患有PH，其中52例（55.9%）需要使用针对PH的药物。有PH的患儿气管切开、转至家庭呼吸机以及出院时的年龄更大。大多数受试者在5岁时停用氧气并脱离呼吸机，PH患儿未出现更晚停用的情况。初次出院后的死亡率为2.6%。
大多数在家接受长期有创通气的BPD-PH婴儿初次出院后存活。随着时间推移，BPD-PH患儿情况有所改善，表现为停用氧气和PH药物、脱离呼吸机以及气管切开拔管。虽然PH的存在与后期脱离呼吸机或拔管无关，但使用PH药物可能是疾病病程更长的一个标志。
关于在家接受长期有创通气的支气管肺发育不良（BPD）儿童的长期转归数据有限，关于合并肺动脉高压（PH）的儿童则尚无数据。几乎所有在家接受长期有创通气的BPD-PH受试者初次出院后存活。随着时间推移，BPD-PH患儿情况有所改善，表现为停用氧气、PH药物、脱离呼吸机以及气管切开拔管。PH的存在并未导致后期脱离呼吸机或拔管；然而，门诊使用PH药物与后期脱离呼吸机和拔管有关。
