PDF(Pubmed)
Abstract：
UNASSIGNED: In cases of atrial septal defect with pulmonary arterial hypertension (PAH), a treat-and-repair strategy that adopts pulmonary vasodilator therapy and subsequent defect closure is postulated to be effective. However, this strategy has not been applied to the large patent ductus arteriosus (PDA) with PAH.
UNASSIGNED: A 10-year-old girl with trisomy 21 was referred to our hospital for the treatment of a large PDA with PAH. Cardiac catheterization and angiography revealed a type C tubular PDA with a minimal diameter of 8.1 mm, an increase in mean pulmonary artery pressure (mPAP) of 60 mmHg, a ratio of pulmonary to systemic blood flow (Qp/Qs) of 2.7, and pulmonary artery resistance (Rp) of 7.1 U/m2. Because she was categorized in the grey zone for operability, we adopted a hybrid treat-and-repair strategy in which palliative surgical duct banding was performed before pulmonary vasodilator therapy to prevent excessive pulmonary blood flow and was followed by transcatheter closure of the PDA. Postoperatively, we confirmed the flow-restricted duct with a minimal diameter of 3.3 mm, decreased Qp/Qs 1.38, high mPAP 40 mmHg, and Rp 7.3 U/m2. Six months after treatment with macitentan and tadalafil, we confirmed a decrease in Rp 4.1 U/m2 as well as low Qp/Qs 1.12, which was low enough for the duct occlusion. The transcatheter occlusion of the surgically created type A conical duct was easily and safely performed. In the mid-term follow-up, favourable haemodynamics and improved exercise were confirmed.
UNASSIGNED: This is the first proof-of-concept case report to show the successful hybrid treat-and-repair strategy for large PDA, which warrants further investigation.
UNASSIGNED: A 10-year-old girl with trisomy 21 was referred to our hospital for the treatment of a large PDA with PAH. Cardiac catheterization and angiography revealed a type C tubular PDA with a minimal diameter of 8.1 mm, an increase in mean pulmonary artery pressure (mPAP) of 60 mmHg, a ratio of pulmonary to systemic blood flow (Qp/Qs) of 2.7, and pulmonary artery resistance (Rp) of 7.1 U/m2. Because she was categorized in the grey zone for operability, we adopted a hybrid treat-and-repair strategy in which palliative surgical duct banding was performed before pulmonary vasodilator therapy to prevent excessive pulmonary blood flow and was followed by transcatheter closure of the PDA. Postoperatively, we confirmed the flow-restricted duct with a minimal diameter of 3.3 mm, decreased Qp/Qs 1.38, high mPAP 40 mmHg, and Rp 7.3 U/m2. Six months after treatment with macitentan and tadalafil, we confirmed a decrease in Rp 4.1 U/m2 as well as low Qp/Qs 1.12, which was low enough for the duct occlusion. The transcatheter occlusion of the surgically created type A conical duct was easily and safely performed. In the mid-term follow-up, favourable haemodynamics and improved exercise were confirmed.
UNASSIGNED: This is the first proof-of-concept case report to show the successful hybrid treat-and-repair strategy for large PDA, which warrants further investigation.
摘要：
■在房间隔缺损伴肺动脉高压（PAH）的病例中，采用肺血管扩张剂治疗和随后的缺损闭合的治疗和修复策略被认为是有效的.然而,该策略尚未应用于患有PAH的大型动脉导管未闭（PDA）。
一名患有21三体的10岁女孩被转诊到我们医院治疗患有PAH的大型PDA。心导管插入术和血管造影显示最小直径为8.1mm的C型管状PDA，平均肺动脉压（mPAP）增加60mmHg，肺与全身血流量（Qp/Qs）为2.7，肺动脉阻力（Rp）为7.1U/m2。因为她被归类为灰色地带的可操作性，我们采用了混合治疗-修复策略,即在肺血管扩张治疗前进行姑息性手术导管绑扎术,以防止肺血流过多,然后经导管封堵PDA.术后,我们确认了最小直径为3.3毫米的限流管道，降低Qp/Qs1.38，高mPAP40mmHg，Rp7.3U/m2。在接受马替坦和他达拉非治疗六个月后，我们证实了Rp4.1U/m2的降低以及Qp/Qs1.12的降低,这对于导管闭塞来说足够低.手术产生的A型锥形导管的经导管闭塞容易且安全地进行。在中期随访中，证实了良好的血流动力学和运动改善.
■这是第一个概念验证病例报告，展示了大型PDA的成功混合治疗和修复策略，这需要进一步调查。
一名患有21三体的10岁女孩被转诊到我们医院治疗患有PAH的大型PDA。心导管插入术和血管造影显示最小直径为8.1mm的C型管状PDA，平均肺动脉压（mPAP）增加60mmHg，肺与全身血流量（Qp/Qs）为2.7，肺动脉阻力（Rp）为7.1U/m2。因为她被归类为灰色地带的可操作性，我们采用了混合治疗-修复策略,即在肺血管扩张治疗前进行姑息性手术导管绑扎术,以防止肺血流过多,然后经导管封堵PDA.术后,我们确认了最小直径为3.3毫米的限流管道，降低Qp/Qs1.38，高mPAP40mmHg，Rp7.3U/m2。在接受马替坦和他达拉非治疗六个月后，我们证实了Rp4.1U/m2的降低以及Qp/Qs1.12的降低,这对于导管闭塞来说足够低.手术产生的A型锥形导管的经导管闭塞容易且安全地进行。在中期随访中，证实了良好的血流动力学和运动改善.
■这是第一个概念验证病例报告，展示了大型PDA的成功混合治疗和修复策略，这需要进一步调查。
