Das Bibhuti B
Department of Pediatrics, Division of Pediatric Cardiology, University of Mississippi Medical Center, Jackson, MS 39216, USA.
Children (Basel). 2025 Apr 7;12(4):476. doi: 10.3390/children12040476.
Pulmonary hypertension (PH) is a progressive disorder characterized by obstructive changes in the pulmonary vasculature, leading to increased pulmonary vascular resistance (PVR), right ventricular (RV) strain, and eventual RV failure (RVF). Despite advancements in medical therapy, PH remains associated with significant morbidity and mortality, particularly in children. RVF is a clinical syndrome resulting from complex structural and functional remodeling of the right heart, leading to inadequate pulmonary circulation, reduced cardiac output, and elevated venous pressure. Management paradigms for pediatric PH diverge significantly from those in adults, particularly due to the predominance of congenital heart disease (CHD) and the dynamic nature of pediatric cardiovascular and pulmonary development. CHD remains a principal driver of PH in children, and its associated pathophysiology demands a nuanced approach. In patients with unrepaired left-to-right shunts, elevated pulmonary blood flow can lead to progressive pulmonary vascular remodeling and increased PVR. The postoperative persistence or progression of PH may occur if irreversible vascular changes have already developed. Current PH treatments primarily focus on reducing PVR, yet distinguishing between therapeutic approaches that target the pulmonary vasculature and those aimed at improving RV function remain challenging. In pediatric patients with progressive PH despite optimal therapy, additional targeted interventions may be necessary to mitigate RV dysfunction and disease progression. This review provides a comprehensive analysis of the mechanisms underlying RVF in PH, incorporating insights from clinical studies in adults and experimental models, while highlighting the unique considerations in children. Furthermore, it explores current pharmacological and interventional treatment strategies, emphasizing the need for novel therapeutic approaches aimed at directly reversing RV remodeling. Given the complexities of RV adaptation in pediatric PH, further research into disease-modifying treatments and innovative interventions is crucial to improving long-term outcomes in affected children.
肺动脉高压（PH）是一种进行性疾病，其特征为肺血管系统出现阻塞性改变，导致肺血管阻力（PVR）增加、右心室（RV）负荷加重，最终出现右心衰竭（RVF）。尽管药物治疗取得了进展，但PH仍然与显著的发病率和死亡率相关，尤其是在儿童中。RVF是一种临床综合征，由右心复杂的结构和功能重塑引起，导致肺循环不足、心输出量减少和静脉压升高。小儿PH的管理模式与成人有很大不同，特别是由于先天性心脏病（CHD）的 predominance 以及小儿心血管和肺发育的动态性质。CHD仍然是儿童PH的主要驱动因素，其相关的病理生理学需要细致入微的方法。在未修复的左向右分流患者中，肺血流量增加可导致进行性肺血管重塑和PVR升高。如果已经发生不可逆的血管变化，术后PH可能持续存在或进展。目前的PH治疗主要集中在降低PVR，但区分针对肺血管系统的治疗方法和旨在改善RV功能的治疗方法仍然具有挑战性。在尽管接受了最佳治疗但仍有进行性PH的小儿患者中，可能需要额外的靶向干预措施来减轻RV功能障碍和疾病进展。本综述对PH中RVF的潜在机制进行了全面分析，纳入了来自成人临床研究和实验模型的见解，同时强调了儿童的独特考虑因素。此外，它还探讨了当前的药物和介入治疗策略，强调了需要旨在直接逆转RV重塑的新型治疗方法。鉴于小儿PH中RV适应的复杂性，进一步研究疾病修饰治疗和创新干预措施对于改善受影响儿童的长期结局至关重要。 （注：“predominance”原词有误，推测可能是“predominance”，意为“主导地位”，翻译时按此理解）
