Division of Neonatology - Department of Pediatrics - McGill University, Montreal Children's Hospital - McGill University Health Centre, Montreal, Canada.
Neonatal and Developmental Medicine, Stanford University School of Medicine - Lucile Packard Children's Hospital, Stanford, CA, USA.
J Perinatol. 2019 Mar;39(3):415-425. doi: 10.1038/s41372-018-0303-8. Epub 2019 Jan 7.
The primary objective was to describe the early "natural history" of pulmonary hypertension (PH) in the premature population. The secondary objective was to describe factors associated with poor outcomes in the premature population with PH at 36 weeks post-menstrual age (PMA).
Retrospective chart review of patients followed at our institution from 2000 to 2017 with echocardiographic (ECHO) evidence of PH at 36 weeks PMA, and born ≤ 32 weeks estimated gestational age (GA). Cox regression was used for survival analysis.
Sixty-one patients with PH (26.5 ± 1.5 weeks at birth) were included. All PH patients had bronchopulmonary dysplasia (BPD), with 89% considered severe; 38% were small for gestational age. Necrotizing enterocolitis requiring surgery was common (25%). Use of post-natal steroids (HR 11.02, p = 0.01) and increased severity of PH (HR 1.05, p < 0.001) were associated with mortality. Pulmonary vein stenosis (PVS) was documented in 26% of the PH cohort, but not associated with increased mortality. ECHO estimation of pulmonary artery pressure (PAP) was available in 84%. PAP was higher in those who died (sPAP/sBP ratio 1.09 ± 27 vs 0.83 ± 20 %, p = 0.0002). At follow-up (mean 250 ± 186 weeks PMA), 72% of the PH cohort was alive. Most survivors (66%) had resolution of their PH on their most recent ECHO; 31% remained on PH therapy.
PH resolved in most survivors in this study population. Mortality in those with BPD-PH was associated with male sex, post-natal steroid use, and increased severity of PH, but not with PVS.
主要目的是描述早产儿人群中肺动脉高压（PH）的早期“自然史”。次要目的是描述在 36 周胎龄（PMA）时具有 PH 的早产儿人群中与不良结局相关的因素。
对 2000 年至 2017 年在我院就诊并在 36 周 PMA 时具有超声心动图（ECHO）证据的 PH 且出生胎龄（GA）≤32 周的患者进行回顾性图表审查。使用 Cox 回归进行生存分析。
共有 61 例 PH 患者（出生时 26.5±1.5 周）入选。所有 PH 患者均患有支气管肺发育不良（BPD），其中 89%为重度；38%为小于胎龄儿。常见需要手术治疗的坏死性小肠结肠炎（25%）。使用产后类固醇（HR 11.02，p=0.01）和 PH 严重程度增加（HR 1.05，p<0.001）与死亡率相关。PH 队列中有 26%的患者记录到肺静脉狭窄（PVS），但与死亡率增加无关。84%的 PH 患者可进行 ECHO 估计肺动脉压（PAP）。死亡患者的 PAP 更高（sPAP/sBP 比值 1.09±27 对 0.83±20%，p=0.0002）。在随访（平均 36 周 PMA）时，PH 队列中有 72%的患者存活。在本研究人群中，大多数幸存者（66%）最近的 ECHO 显示 PH 已缓解；31%仍在接受 PH 治疗。
在本研究人群中，大多数幸存者的 PH 得到缓解。BPD-PH 患者的死亡率与男性、产后类固醇使用和 PH 严重程度增加相关，但与 PVS 无关。
