Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients
Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients
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DOI：
10.1093/ejcts/ezu445
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发表时间：
2015-03-01
影响因子：
3.4
通讯作者：
Bonnet, Damien
中科院分区：
文献类型：
--
作者：
Baruteau, Alban-Elouen;Belli, Emre;Bonnet, Damien
目的：姑息性Potts分流术已被提议用于治疗患有系统上性肺动脉高压（PAH）的儿童。方法：进行了一项回顾性多中心研究，以评估Potts分流术后的短期和长期结局。结果：从2003年到2014年，24名儿童接受了Potts分流术[19例手术，中位年龄：7.7岁（1.5-17岁），中位体重：19.5 kg（10.2-47 kg）和5例经导管，中位年龄：8.1岁（2.3-9.7岁），中位体重：22 kg（12.5-31 kg）]。在人类中，我们首次对1例接受依前列醇静脉给药的下肺动脉高压儿童进行了单向带瓣Potts吻合术，该儿童发生了反复中心线感染。6例患者（25.0%，均来自手术组）发生严重术后并发症，包括3例与低心排血量相关的早期死亡（12.5%）。中位随访（FU）2.1年后21例生存者的WHO功能分级显著改善，均为WHO功能1级或2级随访期间无晕厥发生，无明显右心衰竭;平均6分钟步行距离从年龄和性别调整值的42.3 +/- 10.0%改善至81.2 +/- 9.7% BNP/NT-proBNP水平恢复正常（P < 0.001）;在接受三联疗法作为Potts吻合术前治疗的所有患者中，静脉依前列醇均已停用。最后，所有幸存者都赶上了正常的生长曲线。末次随访时上肢和下肢之间的动脉血氧饱和度梯度持续存在（94.7 +/- 3.6% vs 81.6 +/-5.1%，P < 0.001）。一名患者需要双肺移植6年后，外科Potts shunt.CONCLUSIONS：姑息性Potts分流允许延长生存期和戏剧性的，持久的改善功能能力在儿童严重，药物难治性PAH。Potts分流术可被视为治疗重度、药物难治性PAH儿童的第一个手术或介入步骤，为进一步肺移植（如需要）敞开大门。
OBJECTIVES: Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH).METHODS: A retrospective multicentre study was performed to assess short-and long-term outcomes after Potts shunt.RESULTS: From 2003 to 2014, 24 children underwent a Potts shunt [19 surgical, median age: 7.7 years (1.5-17 years), median weight: 19.5 kg (10.2-47 kg) and 5 transcatheter, median age: 8.1 years (2.3-9.7 years), median weight: 22 kg (12.5-31 kg)] for drug-refractory PAH. For the first time in humans, we performed an unidirectional valved Potts anastomosis in a child with infrasystemic PAH on intravenous epoprostenol who experienced repeated central line infections. Severe postoperative complications occurred in 6 patients (25.0%, all from the surgical group) including 3 early deaths (12.5%) related to low cardiac output. After a median follow-up (FU) of 2.1 years (range, 3 months to 14.3 years, >= 8 years in 7 patients), World Health Organization (WHO) functional class was dramatically improved in the 21 survivors, all being in WHO-functional class 1 or 2 (P < 0.05); none experienced syncope during the FU; none had overt right ventricular failure; mean 6-min walk distance improved from 42.3 +/- 10.0% to 81.2 +/- 9.7% of adjusted values for age and sex (P < 0.001), BNP/NT-proBNP levels normalized in all; and weaning of intravenous epoprostenol was obtained in all patients who received triple combination as pre-Potts anastomosis therapy. Finally, all survivors caught up to normal growth curves. Arterial oxygen saturation gradient between upper and lower limbs persisted at the last FU (94.7 +/- 3.6% vs 81.6 +/- 5.1%, P < 0.001). One patient required double lung transplantation 6 years after a surgical Potts shunt.CONCLUSIONS: Palliative Potts shunt allows prolonged survival and dramatic, long-lasting improvement in functional capacities in children with severe, drug-refractory PAH. The Potts shunt might be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH, leaving the door open for further lung transplantation, if needed.