Pokima Ngowari, Gill Sunydip, Moradi Ramtin, Thida Aye M, Muthu John
Internal Medicine, Staten Island University Hospital, Northwell Health, Staten Isand, USA.
Internal Medicine, State University of New York Downstate Health Sciences University, Brooklyn, USA.
Cureus. 2025 Jul 29;17(7):e89012. doi: 10.7759/cureus.89012. eCollection 2025 Jul.
Pulmonary hypertension (PH) is a serious and increasingly recognized complication in sickle cell disease (SCD), particularly in individuals with the homozygous genotype. Elevated tricuspid regurgitant velocity (TRV) increases the potential for future development of PH, yet no standardized management approach currently exists, likely because of the varied pathophysiology of PH in these patients. Transthoracic echocardiogram-derived elevated TRV of more than 2.7 m/second in patients with SCD is associated with increased mortality and remains the only non-invasive approach to diagnosis. PH in patients with SCD can be due to various etiologies and can be considered either precapillary or post-capillary PH. This case series highlights the need to diagnose the underlying etiology of SCD-induced PH, which in turn will dictate appropriate management. Additionally, this case series will review recent management strategies available for treating patients with established PH and address significant risk factors for patients at risk for the development of PH.
肺动脉高压（PH）是镰状细胞病（SCD）中一种严重且日益被认识到的并发症，尤其是在纯合子基因型个体中。三尖瓣反流速度（TRV）升高增加了未来发生PH的可能性，但目前尚无标准化的管理方法，这可能是因为这些患者中PH的病理生理学各不相同。在SCD患者中，经胸超声心动图测得的TRV升高超过2.7米/秒与死亡率增加相关，并且仍然是唯一的非侵入性诊断方法。SCD患者的PH可能由多种病因引起，可分为毛细血管前性或毛细血管后性PH。本病例系列强调了诊断SCD所致PH潜在病因的必要性，这反过来将决定适当的管理措施。此外，本病例系列将回顾目前可用于治疗已确诊PH患者的最新管理策略，并探讨有PH发生风险患者的重要危险因素。
