Tsuboya Naoki, Mitani Yoshihide, Ohashi Hiroyuki, Sawada Hirofumi, Hirayama Masahiro
Department of Pediatrics, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu City, Mie Prefecture 514-8507, Japan.
Eur Heart J Case Rep. 2024 Jul 23;8(8):ytae354. doi: 10.1093/ehjcr/ytae354. eCollection 2024 Aug.
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.
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/m. 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/m. Six months after treatment with macitentan and tadalafil, we confirmed a decrease in Rp 4.1 U/m 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.
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.
在患有肺动脉高压（PAH）的房间隔缺损病例中，采用肺血管扩张剂治疗并随后闭合缺损的治疗与修复策略被认为是有效的。然而，该策略尚未应用于伴有PAH的大型动脉导管未闭（PDA）。
一名患有21三体综合征的10岁女孩因治疗伴有PAH的大型PDA被转诊至我院。心导管检查和血管造影显示为C型管状PDA，最小直径为8.1mm，平均肺动脉压（mPAP）升高60mmHg，肺循环与体循环血流量之比（Qp/Qs）为2.7，肺动脉阻力（Rp）为7.1U/m。由于她被归类为手术可行性的灰色区域，我们采用了一种混合治疗与修复策略，即在肺血管扩张剂治疗前进行姑息性外科导管绑扎以防止肺血流量过多，随后通过经导管闭合PDA。术后，我们确认了直径最小为3.3mm的血流受限导管，Qp/Qs降至1.38，mPAP仍较高为40mmHg，Rp为7.3U/m。在用马昔腾坦和他达拉非治疗6个月后，我们确认Rp降至4.1U/m，Qp/Qs也降至1.12，低至足以进行导管封堵。经导管封堵手术形成的A型圆锥形导管轻松且安全地完成。在中期随访中，证实了血流动力学良好且运动能力有所改善。
这是首例证明大型PDA成功采用混合治疗与修复策略的概念验证病例报告，值得进一步研究。
