Author + information
- Hiroto Shimokawahara1,
- Mitsutaka Nakashima1,
- Takanori Naito1,
- Masataka Shigetoshi1,
- Aiko Ogawa1,
- Mitsuru Munemasa1 and
- Hiromi Matsubara1
Peripheral pulmonary artery stenosis (PPS) presenting in adulthood is an uncommon disease and may be misdiagnosed as idiopathic pulmonary artery hypertension or chronic thromboembolic pulmonary hypertension. Standard curative treatment has not fully established because of the limited experience of catheterization therapy that is considered the only effective treatment for PPS. The aim of this study is to review the clinical course and interventional therapeutic outcomes for patients with PPS from 2007 to 2016 in our hospital.
23 consecutive patients (12 women, 29.0 ± 15.1 years old) with PPS including a patient with Williams syndrome who underwent interventional catheterization therapy (total 228 sessions) were studied. Mean follow-up period is 2.7 ± 2.6 years. Three patients were treated by only conventional balloon dilatation and the other 20 patients underwent stent implantation. 14 lesions were treated with bare metal stents (BMS), and 183 lesions with drug-eluting stents (DES). We evaluated hemodynamic improvements before and after interventional therapy and we also investigated the incidence of target lesion revascularization (TLR) at follow-up pulmonary angiography.
Mean pulmonary artery pressure (mPAP) decreased from 54.5 ± 18.9 mmHg to 40.7 ± 13.8 mmHg (p<0.001) and pulmonary vascular resistance (PVR) also decreased from 11.2 ± 5.8 Wood units to 7.8 ± 5.4 Wood units (p<0.001) after interventional catheter therapy. However, both of mPAP and PVR did not significantly decrease in three patients treated by only conventional balloon dilatation (mPAP; 57.7 ± 9.0 mmHg vs. 56 ± 13.5 mmHg, PVR; 14.2 ± 5.0 Wood units vs. 14.2 ± 5.6 Wood units). Moreover, all the three patients treated without stent implantations died during the follow-up period. Stent implantation was successful in 193 of 197 lesions, and the incidence of TLR was 52 lesions (26.4%).
Although we have to resolve the high incidence of TLR, stent implantation therapy for patients with PPS in adulthood could become an effective and safe treatment.