Hussain Ali Shabbir, Ali Rehan, Ahmed Shakeel, Naz Farah, Haroon Anila
Department of Pediatrics and Child Health, The Aga khan University Hospital Karachi, Pakistan.
J Ayub Med Coll Abbottabad. 2017 Oct-Dec;29(4):677-680.
The prevalence of PPHN has been estimated at 1.9 per 1000 live births. After the discovery of iNO's, its efficacy and benefit in PPHN is well established. Even in the best of centers equipped with iNo and ECMO the mortality is around 20%. Also, iNO is expensive and difficult to administer and monitor which makes it difficult choice in our part of the world. Furthermore About 40% of patients do not respond or have rebound pulmonary hypertension after discontinuation. Owing to these reasons, other treatment modalities like phosphodiesterase inhibitors such as Sildenafil need to be evaluated.
We report a retrospective case series of eighteen patients with PPHN admitted in NICU and treated with oral sildenafil.
Three (17%) babies had mild, 5 (28%) moderate and 10 (55%) severe PPHN based on echocardiography. Sildenafil was started on all patients on a mean of 1.67 days and stopped on mean 12.6 days. Initial fio2 was 100%, which after starting sildenafil decreased gradually to 40% on mean 10 days. Average length of stay in NICU was 13 days. Twelve (67%) patients survived whereas 6 (33%) expired (Figure 2). No improvement in oxygen Index after 36 hours (p<0.05) was the independent predicting risk factor for PPHN related mortality in the expired patients.
Oral sildenafil can be a used in conjunction with other treatment modalities for PPHN especially in resource limited settings. However further studies regarding its comparative efficacy need to be done.
据估计，持续性肺动脉高压（PPHN）的发病率为每1000例活产中有1.9例。一氧化氮（iNO）被发现后，其在PPHN中的疗效和益处已得到充分证实。即使在配备了iNO和体外膜肺氧合（ECMO）的最佳中心，死亡率仍约为20%。此外，iNO价格昂贵，给药和监测困难，这使得在我们所在的地区难以选择使用。此外，约40%的患者在停药后无反应或出现肺动脉高压反弹。由于这些原因，需要评估其他治疗方式，如磷酸二酯酶抑制剂西地那非。
我们报告了一组18例入住新生儿重症监护病房（NICU）并接受口服西地那非治疗的PPHN患者的回顾性病例系列。
根据超声心动图，3例（17%）婴儿为轻度PPHN，5例（28%）为中度PPHN，10例（55%）为重度PPHN。所有患者均在平均1.67天开始使用西地那非，并在平均12.6天停药。初始吸氧浓度（FiO2）为100%，开始使用西地那非后，平均10天逐渐降至40%。在NICU的平均住院时间为13天。12例（67%）患者存活，6例（33%）死亡（图2）。36小时后氧指数无改善（p<0.05）是死亡患者中PPHN相关死亡率的独立预测危险因素。
口服西地那非可与其他治疗方式联合用于PPHN，尤其是在资源有限的环境中。然而，需要进一步研究其比较疗效。
