Ty -jour t1-严重的小儿肺动脉高压JF的前期三重联合疗法JF-欧洲呼吸杂志Lévy，Marilyne au -Roofthooft，Marcus T.R.Au -Douwes，Johannes M.Au -Vissia -Kazemier，Theresia R.Au -Szezepanski，Isabelle au -Berger，Rolf M.F.AU-邦内特，达米安Y1-2021/01/01 ur -http：//www.qdcxjkg.com/content/57/57/1/1/2001120.Abstract N2-小儿肺动脉高压（PAH）的治疗策略已在最后一场演变多年，但生存仍然很差。最近，在患有严重PAH的成年人中，据报道，诊断的前期三重联合疗法（UTCT）显示出明显的临床改善和出色的长期结局。这项回顾性观察性研究旨在评估UTCT在2010年至2019年间被诊断为PAH的儿童PAH的功效，并开始使用UTCT。世界卫生组织功能类别（WHO-FC），血液动力学，超声心动图，步行6分钟的步行距离和血清N末端促脑 - 脑尿素肽的水平在基线，3个月和6个月后评估向上。事件被定义为死亡，肺移植或POTTS分流。21名儿童（中位年龄4.8岁（2.5-12.8），57％女性）包括在内。除一个儿童外，所有儿童均为WHO-FC III或IV（分别为28％和67％）。 After 3 months, one child had died and one child had received a Potts shunt. The remaining 19 children showed clinical and echocardiographic improvement, which persisted at 6 months. Children with idiopathic and heritable PAH showed one-, two- and three-year transplant-free survival estimates of 100%, 94% and 87%, albeit 47% of them receiving a Potts shunt during follow-up.Children with severe PAH, but not pulmonary veno-occlusive disease, improved significantly with uTCT and showed beneficial up to 3-year survival rates, albeit 47% of them receiving a Potts shunt during follow-up. The role of a Potts shunt in conjunction to uTCT in paediatric PAH needs to be further established.Upfront triple combination therapy in severe paediatric PAH resulted in significant clinical, haemodynamic and echocardiographic improvement and favourable 1-, 2- and 3-year survival rates, albeit with 47% receiving a Potts shunt during follow-up https://bit.ly/3iG92PA ER -