Development and Validation of Quality Indicators for Pulmonary Arterial Hypertension Management in Japan: A Modified Delphi Consensus Study.

Background: Quality indicators (QIs) are used to standardize care and improve outcomes in patients with pulmonary arterial hypertension (PAH). It is important that QIs are validated within specific healthcare contexts. Therefore, this study aimed to validate QIs for PAH management in Japan using a modified Delphi consensus method.

Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy.

Background: The European Chronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, reported the major impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH. Since then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplasty (BPA), and an approved oral drug therapy with the guanylate cyclase stimulator riociguat. The current registry aimed to evaluate the effect of these new therapeutic approaches in a worldwide context.

Chronic thromboembolic pulmonary disease.

Chronic thromboembolic pulmonary hypertension is a complication of pulmonary embolism and a treatable cause of pulmonary hypertension. The pathology is a unique combination of mechanical obstruction due to failure of clot resolution, and a variable degree of microvascular disease, that both contribute to pulmonary vascular resistance. Accordingly, multiple treatments have been developed to target the disease components.

Effects of medical therapy and age on cardiac output changes following balloon pulmonary angioplasty: Implications for combination therapy in chronic thromboembolic pulmonary hypertension.

Background: Some patients with chronic thromboembolic pulmonary hypertension (CTEPH) exhibit exercise intolerance due to reduced cardiac output (CO) even after successful balloon pulmonary angioplasty (BPA). Medical therapy is a potential option for such cases; however, it is unclear which patients necessitate it even after BPA.

Methods: This study included 286 patients with CTEPH who underwent BPA and right heart catheterization 1 year after the final BPA and classified them into no-medication and withdrawal groups.

Status and Future Directions for Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Disease With and Without Pulmonary Hypertension: A Scientific Statement From the American Heart Association.

Balloon pulmonary angioplasty continues to gain traction as a treatment option for patients with chronic thromboembolic pulmonary disease with and without pulmonary hypertension. Recent European Society of Cardiology guidelines on pulmonary hypertension now give balloon pulmonary angioplasty a Class 1 recommendation for inoperable and residual chronic thromboembolic pulmonary hypertension. Not surprisingly, chronic thromboembolic pulmonary hypertension centers are rapidly initiating balloon pulmonary angioplasty programs.

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 results of genetic analysis and clinical outcomes after stent deployment in adult patients with isolated peripheral pulmonary artery stenosis.

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Combination of rescue balloon pulmonary angioplasty and riociguat therapy for chronic thromboembolic pulmonary hypertension with nonsustained polymorphic ventricular tachycardia: A case report.

We encountered a case of frequent nonsustained polymorphic ventricular tachycardia (NSPVT) due to hemodynamically unstable chronic thromboembolic pulmonary hypertension (CTEPH). A 78-year-old woman was taking anticoagulants for CTEPH. She had refused specific treatment for CTEPH, including pulmonary vasodilators, because she was then asymptomatic.

Rescue balloon pulmonary angioplasty for refractory heart failure in chronic thromboembolic pulmonary hypertension complicated with takotsubo cardiomyopathy.

Unlabelled: Balloon pulmonary angioplasty (BPA) seems promising for treating critically ill patients with chronic thromboembolic pulmonary hypertension (CTEPH) because of its less invasive and stepwise nature. However, there are only a few reports on rescue BPA. Herein, we present a case of CTEPH and takotsubo cardiomyopathy in an 82-year-old female.

Initial Triple Combination Therapy Including Intravenous Prostaglandin I for the Treatment of Patients with Severe Pulmonary Arterial Hypertension.

Upfront combination therapy including intravenous prostaglandin I (PGI-IV) is recognized as the most appropriate treatment for patients with severe pulmonary arterial hypertension (PAH). This retrospective study aimed to determine reasons why this therapy is not used for some patients with severe PAH and describe the hemodynamic and clinical prognoses of patients receiving initial combination treatment with (PGI-IV+) or without (PGI-IV-) PGI-IV.Data for patients with severe PAH (World Health Organization Functional Class III/IV and mean pulmonary arterial pressure [mPAP] ≥ 40 mmHg) were extracted from the Japan Pulmonary Hypertension Registry.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function.

The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important.

Antiproliferative effect of selexipag active metabolite MRE-269 on pulmonary arterial smooth muscle cells from patients with chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a group 4 pulmonary hypertension (PH) characterized by nonresolving thromboembolism in the central pulmonary artery and vascular occlusion in the proximal and distal pulmonary artery. Medical therapy is chosen for patients who are ineligible for pulmonary endarterectomy or balloon pulmonary angioplasty or who have symptomatic residual PH after surgery or intervention. Selexipag, an oral prostacyclin receptor agonist and potent vasodilator, was approved for CTEPH in Japan in 2021.

Long-term outcome of chronic thromboembolic pulmonary hypertension using direct oral anticoagulants and warfarin: a Japanese prospective cohort study.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) requires lifelong anticoagulation. Long-term outcomes of CTEPH under current anticoagulants are unclear.

Objectives: The CTEPH AC registry is a prospective, nationwide cohort study comparing the safety and effectiveness of direct oral anticoagulants (DOACs) and warfarin for CTEPH.

Comparison of the safety and efficacy of balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension patients with surgically accessible and inaccessible lesions.

Background: Although pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension, not all patients are eligible. While balloon pulmonary angioplasty is an alternative for such patients, its efficacy and safety may differ between patients with and without surgically accessible lesions.

Methods: This study involved 344 patients treated with balloon pulmonary angioplasty who were ineligible for pulmonary endarterectomy.

Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. It is caused by persistent obstruction of pulmonary arteries by chronic organised fibrotic clots, despite adequate anticoagulation. The pulmonary hypertension is also caused by concomitant microvasculopathy which may progress without timely treatment.

Treatment of Vascular Injury During Balloon Pulmonary Angioplasty in Patients With Chronic Thromboembolic Pulmonary Hypertension.

Background: Treatment strategy for vascular injury during balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) was uncertain.

Objectives: This study aimed to identify an optimal therapeutic strategy for vascular injury during BPA in patients with CTEPH.

Methods: This study reviewed 207 patients with CTEPH and 956 BPA procedures between November 1, 2012 and November 30, 2015.

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).