Hybrid Treat and Repair Strategy for Large Patent Ductus Arteriosus: A Proof-of-Concept Case Report

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 large patent ductus arteriosus (PDA) with PAH. A 10-year-old girl with trisomy 21 was referred to our hospital for the treatment of large PDA with PAH. Cardiac catheterisation and angiography revealed a type C tubular PDA with a minimal diameter of 8.1 mm, an increase in mean pulmonary artery pressure (mPAP) 60mmHg, the ratio of pulmonary to systemic blood flow (Qp/Qs) 2.7 and pulmonary artery resistance (Rp) 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 PDA. Postoperatively, we confirmed the flow-restricted duct with a minimal diameter of 3.3 mm, decreased Qp/Qs 1.38, high mPAP 40mmHg, 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. 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.