Cite Share Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome.
Cite Share Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome.
复制标题
引用分享唐氏综合症患者 Fontan 手术后的血流动力学特征。
DOI：
10.1007/s00246-021-02727-6
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发表时间：
2022
影响因子：
1.6
通讯作者：
Sagawa K.
中科院分区：
文献类型：
--
作者：
Otsuka M;Kodama Y;Kuraoka A;Ishikawa Y;Nakamura M;Nakano T;Kado H;Umemoto S;Ishikita A;Sakamoto I;Ide T;Tsutsui H;Sagawa K.
患有唐氏综合征（DS）的患者通常被认为不是Fontan手术的良好候选人。然而，随着时间的推移，详细的血流动力学变化尚未完全阐明。对所有接受Fontan手术的DS患者以及在福冈儿童医院和九州大学医院接受Fontan手术的5倍于此数量的未接受DS的Fontan患者进行了回顾性病历审查。确定了7例Fontan DS患者，招募了35例无DS的Fontan患者。Fontan手术后平均观察期为14.7年和15.0年（分别为DS和非DS），仅1名DS患者死亡。Fontan手术后DS患者的中心静脉压（CVP）和跨室压差显著增加，动脉血氧饱和度随时间显著降低，与未发生DS的患者相比。与非DS患者相比，Fontan手术后DS患者的CVP随时间推移而增加。更好的管理，包括肺动脉高压特异性治疗的疗效，应在进一步的研究中阐明。
Patients with Down’s syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children’s Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient significantly increased, and arterial oxygen saturation significantly decreased over time in DS patients after the Fontan procedure compared with those without DS. CVP in DS patients after the Fontan procedure increased over time compared with non-DS patients. Better management including the efficacy of Pulmonary arterial hypertension-specific therapy should be clarified in further studies.