Boudjemline Younes, Sizarov Aleksander, Malekzadeh-Milani Sophie, Mirabile Cristian, Lenoir Marien, Khraiche Diala, Lévy Marilyne, Bonnet Damien
Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Universitaire Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Paris, France; Université Paris V Descartes, Sorbonne Paris Cité, Paris, France.
Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Universitaire Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Paris, France.
Can J Cardiol. 2017 Sep;33(9):1188-1196. doi: 10.1016/j.cjca.2017.06.004. Epub 2017 Jun 13.
The reversed Potts shunt improves right ventricular (RV) function in patients with suprasystemic pulmonary arterial hypertension (PAH). The proximity of the left pulmonary artery (LPA) to the descending aorta (DAo) permits the creation of a transcatheter connection. We sought to assess the safety, feasibility, and hemodynamic efficacy of the transcatheter Potts shunt (TPS) in children.
The TPS procedure was performed using radiofrequency energy for vessel perforation and deployment of a covered stent to connect the DAo and LPA. Procedural details and clinical follow-up data were collected prospectively.
A TPS was successfully created in 6 children (mean age, 11.0 ± 4.2 years) with drug-refractory suprasystemic PAH and deteriorating RV function. All patients exhibited nearly complete equalization of aortic and pulmonary pressures and improvement in RV contractility within days after TPS placement. Two patients with pre-existing severe biventricular dysfunction and pericardial effusion experienced acute low-output states immediately after shunt creation because of sudden reductions in left ventricular (LV) preload, resulting in cardiac arrest, irreversible brain damage, and death. Stent dislodgement and embolization into the iliac artery occurred in 1 patient. The stent was successfully secured and followed by placement of a second stent at the target location. The procedure was uncomplicated in 4 patients, who remain alive after a mean follow-up of 10 ± 2.6 months. Intravenous vasodilator therapy was weaned uneventfully after TPS in 3 patients.
TPS creation in children is feasible and results in hemodynamic improvement. Further insights into high-risk markers, such as reduced preprocedural LV function and preload reserves, are important for guiding patient selection.
反向Potts分流术可改善超体循环性肺动脉高压（PAH）患者的右心室（RV）功能。左肺动脉（LPA）与降主动脉（DAo）位置接近，便于建立经导管连接。我们旨在评估经导管Potts分流术（TPS）在儿童中的安全性、可行性和血流动力学疗效。
采用射频能量进行TPS手术，用于血管穿孔并植入覆膜支架以连接DAo和LPA。前瞻性收集手术细节和临床随访数据。
6例（平均年龄11.0±4.2岁）药物难治性超体循环性PAH且RV功能恶化的儿童成功实施了TPS。所有患者在TPS植入后数天内均表现出主动脉和肺动脉压力几乎完全均衡，RV收缩力改善。2例术前存在严重双心室功能障碍和心包积液的患者，在分流建立后因左心室（LV）前负荷突然降低而立即出现急性低输出状态，导致心脏骤停、不可逆脑损伤和死亡。1例患者发生支架移位并栓塞至髂动脉。支架成功固定，随后在目标位置植入第二个支架。4例患者手术过程顺利，平均随访10±2.6个月后仍存活。3例患者在TPS术后顺利停用静脉血管扩张剂治疗。
儿童实施TPS是可行的，并可改善血流动力学。进一步深入了解高风险标志物，如术前LV功能降低和前负荷储备减少，对于指导患者选择很重要。
