Clinical impact of mean pulmonary arterial pressure after balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension.

Background: Balloon pulmonary angioplasty (BPA) improves haemodynamics in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Previous studies on BPA have set the treatment objective to achieve a mean pulmonary arterial pressure (mPAP) of <30 mm Hg. However, the clinical impact of mPAP after BPA remains unclear.

Sequential Combination of Balloon Pulmonary Angioplasty for Distal Lesions in the Left Pulmonary Artery Followed by Pulmonary Endarterectomy for Central Lesions in the Right Pulmonary Artery: A Case Report.

Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). While balloon pulmonary angioplasty (BPA) is an emerging treatment for distal CTEPH, a standard therapeutic strategy for CTEPH with unilateral central lesions has yet to be established. Herein, we describe the successful treatment of a patient with CTEPH who underwent BPA for left distal lesions, followed by PEA for unilateral right central lesions, without serious complications.

Development of severe pre-capillary pulmonary hypertension 7 years after heart transplant.

We present the case of a patient who developed severe pre-capillary pulmonary hypertension 7 years after heart transplantation. The transplantation was performed due to the dilated phase of hypertrophic cardiomyopathy with combined post- and pre-capillary pulmonary hypertension (CpcPH), which had initially improved. This case highlights the potential underlying factors contributing to pulmonary arterial hypertension in patients with CpcPH and the importance of careful long-term follow-up after heart transplantation.

Long-term outcome of manual aspiration thrombectomy for acute proximal deep vein thrombosis.

Manual aspiration thrombectomy (MAT) is a catheter intervention for cases with a high risk of bleeding. It is a rapid, simple, inexpensive, and single-session intervention for patients with proximal deep vein thrombosis (DVT). We evaluated the long-term outcome of MAT for patients with proximal DVT.

Hypopituitarism Induced by Continuous Infusion of PGI2 Analogues: A Case Series and the Role of ACTH Screening and Hydrocortisone Treatment.

Hypopituitarism has been reported in patients receiving continuous infusions of prostaglandin I2 (PGI2) analogues for pulmonary hypertension (PH). However, these patients' clinical characteristics, treatment, and prognoses remain unclear. This retrospective multicentre study included 22 patients who developed hypopituitarism while on continuous PGI2 analogue infusion between 1999 and 2021.

Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: Results of an International Multicenter Prospective Registry.

Background: Chronic thromboembolic pulmonary hypertension results from mechanical obstruction of major pulmonary artery lumina with fibrotic tissue. Main treatment has been pulmonary endarterectomy, a complex surgical procedure removing vascular obstruction. However, at least 40% of patients are not candidates for pulmonary endarterectomy because of technical inoperability, comorbidities, or limited access to surgery.

Balloon Pulmonary Angioplasty in Heterozygous p.Arg4810Lys Variant Carriers Diagnosed With Chronic Thromboembolic Pulmonary Hypertension.

Background: The heterozygous ring finger protein 213 p.Arg4810Lys variant has been identified in patients with chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to clarify the influence of the p.

Phase 2 Study to Evaluate the Efficacy and Safety of Inhaled Nitric Oxide Therapy in Patients With Severe Right Heart Failure Associated With Pulmonary Hypertension - Protocol for the PHiNO Study.

Background: Acute right heart failure (RHF) is a syndrome characterized by sudden right ventricular dysfunction leading to systemic hypoperfusion, which carries a poor prognosis, particularly in patients with pulmonary hypertension (PH). Early reduction of pulmonary vascular resistance (PVR) is crucial for improving RHF and reducing acute mortality. Compared with pulmonary vasodilators approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH), inhaled nitric oxide (iNO) therapy has the advantages of being fast acting, an excellent selective pulmonary vasodilation, and has less effect on systemic blood pressure.

Exercise-induced Right-to-left Shunt in a Patient with Combined Pulmonary Fibrosis and Emphysema.

Combined pulmonary fibrosis and emphysema (CPFE) is characterized by emphysematous lesions in the upper lung field and pulmonary fibrosis in the lower lung field and is often associated with pulmonary hypertension and severe exercise-induced hypoxemia (EIH). We herein report a 62-year-old man with CPFE who presented with severe EIH despite relatively preserved lung volumes. Cardiopulmonary exercise testing suggested exercise-induced right-to-left shunt (EIS) through a patent foramen ovale (PFO).

Pulmonary Artery Denervation for Medication Refractory Pulmonary Hypertension (PARPH Study) - Study Protocol for a Prospective, Open-Label, Single-Arm Clinical Trial.

Background: Despite the development of effective pulmonary vasodilators, the prognosis for patients with pulmonary hypertension (PH) remains poor, particularly in medication-refractory patients. Catheter-based pulmonary artery denervation (PADN) is an emerging therapeutic strategy targeting the sympathetic nervous system in various types of PH. However, data on its safety and efficacy in refractory patients with PH who truly require non-pharmacotherapy are lacking.

A Novel Missense Variant of in Pulmonary Arterial Hypertension.

Background: Because Regnase-1, encoded by , suppresses the development of pulmonary arterial hypertension (PAH) by controlling pro-inflammatory cytokines, we aimed to identify variants in patients with PAH.

Methods And Results: We analyzed whole-genome sequence data of patients with PAH to search for disease-associated variants. The Regnase-1 p.

The clinical impact of acute vasoreactivity testing in patients with severe pulmonary hypertension associated with lung disease: A retrospective exploratory analysis.

Background: The clinical impact of acute vasoreactivity testing with inhaled nitric oxide (NO testing) in severe pulmonary hypertension associated with lung disease (LD-PH) remains unknown.

Methods: We retrospectively reviewed 48 consecutive patients with severe LD-PH (73 ± 9 years; male: 81%) who underwent NO testing between 2014 and 2022. We conducted an exploratory analysis of the association between the response to NO testing and both the efficacy of pulmonary vasodilators and prognosis.

Survival trends in patients with chronic thromboembolic pulmonary hypertension: an observational study.

Background: Treatment options for patients with chronic thromboembolic hypertension (CTEPH) have increased over the past decade. However, it is unknown whether the outcomes of patients with CTEPH have changed as well.

Methods: This retrospective study analysed the data of 834 patients with CTEPH, categorised into early (April 1980-December 1999), middle (January 2000-September 2010) and current (October 2010-December 2023) eras.

Relationship Between p.R4810K and Echocardiographic Findings in Patients with Cerebrovascular Diseases: A Multicenter Prospective Cohort Study.

Background: RING finger protein 213 () p.R4810K is an established risk factor for moyamoya disease and intracranial artery stenosis in East Asian people. Recent evidence suggests its potential association with extracranial cardiovascular diseases, including pulmonary hypertension.

The selective serotonin reuptake inhibitor paroxetine improves right ventricular systolic function in experimental pulmonary hypertension.

Background: Pulmonary hypertension (PH) often leads to right ventricle (RV) failure, a significant cause of morbidity and mortality. Despite advancements in PH management, progression to RV maladaptation and subsequent failure remain a clinical challenge. This study explored the effect of paroxetine, a selective serotonin reuptake inhibitor (SSRI), on RV function in a rat model of PH, hypothesizing that it improves RV function by inhibiting G protein-coupled receptor kinase 2 (GRK2) and altering myofilament protein phosphorylation.

Familial onset of venous thromboembolism due to inherited antithrombin deficiency with a novel gene variant (p.Arg14Gly).

Unlabelled: Inherited antithrombin deficiency is an autosomal dominant thrombophilia, resulting from genetic variations in the serpin family C member 1 (SERPINC1) gene. Antithrombin deficiency increases the risk of venous thromboembolism (VTE) compared to the general population. In this report, a novel missense variant of , c.

Effect of direct oral anticoagulant therapy on pulmonary artery clot dissolution in intermediate high-risk pulmonary thromboembolism.

Background: Direct oral anticoagulants are the established drugs for treating pulmonary thromboembolism. The advantage of direct oral anticoagulants over conventional therapy for clot lysis and right ventricular unloading in the acute phase remains unclear. This study aimed to evaluate the effect of acute treatment with direct oral anticoagulants on clot dissolution and right ventricular unloading in intermediate high-risk pulmonary thromboembolism.

Feasibility of revascularization after gelatin sponge embolization for hemoptysis during balloon pulmonary angioplasty.

Background: Vessel injury is a common complication during balloon pulmonary angioplasty (BPA). For persistent hemoptysis, gelatin sponge embolization (GSE) is considered, but its impact on subsequent perfusion in embolized vessels remains unknown. This study explores the feasibility of revascularization in vessels post-GSE.