Stenting of isolated pulmonary artery stenosis in an adult patient with RNF213 p.Arg4810Lys variant.

Isolated peripheral pulmonary artery stenosis (PPAS) in adulthood related to the RNF213 p.Arg4810Lys variant is characterized by bilateral nonuniform pulmonary artery stenoses without congenital heart disease or systemic congenital syndromes. Progressive stenoses worsening results in elevated pulmonary artery pressure (PAP), eventually leading to patient fatality. However, established treatments for PPAS are currently lacking. We present the case of a 40-year-old woman diagnosed with isolated PPAS with the RNF213 p.Arg4810Lys variant. She underwent 27 stent deployments in 20 procedures, leading to dramatic symptomatic and hemodynamic improvements, with a mean PAP decrease from 83 to 25 mmHg.

Flow Grade-Based Success Rates, Complication Rates, and Balloon Pulmonary Angioplasty Patency for Total Occlusions.

BACKGROUND: The number of successfully recanalized total occlusions affects hemodynamic improvement after balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to clarify the current efficacy, patency, and success rate of BPA for total occlusions. METHODS: Between April 2016 and August 2021, 178 BPAs were performed in 100 patients with CTEPH and total occlusions. The primary success and subsequent patency rates immediately before the second BPA procedure (follow-up) were compared between the segmental and subsegmental groups, based on the flow grade, which was defined as follows: 0, no reperfusion; 1, minimal reperfusion; 2, partial reperfusion; and 3, complete reperfusion. RESULTS: Total occlusions were mainly located in the right lung (70%) and lower lobes (48%). The primary success rate was 88%, with significant improvements in oxygenation, hemodynamic parameters, and 6-minute walk test. The primary flow grade did not differ between groups. However, the proportion of lesions with a flow grade of 2 or 3 at follow-up was significantly higher in the subsegmental group than in the segmental group (84% vs 45%, respectively; P < 0.01). In multivariate analysis, flow grade in the acute phase (odds ratio [OR], 46.9; 95% confidence interval [CI], 12.54-176.78; P < 0.01) and subsegmental lesions (OR, 13.8; 95% CI, 3.24-58.94; P < 0.01) were independently associated with better patency (flow grade of 2 or 3) at follow-up. CONCLUSIONS: Total occlusions can be safely and effectively treated with BPA. BPA for total occlusions may be preferable for subsegmental over segmental lesions.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel-Trenaunay-Weber syndrome.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital disorder characterized by cutaneous capillary malformations, bone hypertrophy, and multiple venous or lymphatic malformations. KTWS is associated with chronic thromboembolic pulmonary hypertension (CTEPH), presumably due to thromboembolism from multiple vascular malformations. Here, we report the first case series of patients with KTWS-CTEPH who underwent balloon pulmonary angioplasty (BPA). Both patients are alive 20 years and 1 year after the initial diagnosis of CTEPH, respectively, and are stable with improved hemodynamics. BPA may be an effective treatment option for patients with KTWS-CTEPH.

Comparative evaluation of standard maintenance-dose clopidogrel versus low-dose prasugrel in patients with stable coronary artery disease after percutaneous coronary intervention.

BACKGROUND: Treatment with low-dose prasugrel might be more beneficial even in chronic stable coronary artery disease (CAD) patients treated with clopidogrel. We compared platelet reactivity between standard maintenance-dose and low-dose prasugrel in stable CAD patients. METHODS: This multicenter study enrolled 164 stable CAD patients receiving dual antiplatelet therapy with aspirin and clopidogrel. Patients were randomly assigned to continue treatment with 75-mg clopidogrel daily (n = 80) or switch to 3.75-mg prasugrel daily (n = 84). Platelet reactivity was evaluated by measuring P2Y12 reaction unit (PRU) before randomization and at 5 and 30 days thereafter using the VerifyNow® assay. Patients were classified into three groups according to CYP2C19-clopidogrel metabolic phenotype: extensive (without a *2 or *3 allele), intermediate (one *2 or *3 alleles), or poor (two *2 or *3 alleles) metabolizers. RESULTS: The PRU level was comparable between the two groups at baseline but was significantly lower in the prasugrel group than in the clopidogrel group on days 5 (133.0 vs. 156.8 PRU, P = 0.005) and 30 (124.3 vs. 158.0 PRU, P < 0.001). On day 30, the PRU level was lower in the prasugrel group among patients categorized as poor and intermediate metabolizers but not among extensive metabolizers. CONCLUSIONS: Low-dose prasugrel achieves more consistent antiplatelet effects than clopidogrel irrespective of the metabolic phenotype in Japanese patients with stable CAD. Low-dose prasugrel might be also beneficial in the chronic phase without increasing the bleeding risk among stable CAD patients in other countries.