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Aims: Repeated risk assessments and treatment patterns over long time are sparsely studied in chronic thromboembolic pulmonary hypertension (CTEPH); thus, we aimed to investigate changes in risk status and treatment patterns in incident patients with CTEPH over a 5 year period.
Methods And Results: Descriptive and explorative study including 311 patients diagnosed with CTEPH 2008-2019 from the Swedish pulmonary hypertension registry, stratified by pulmonary endarterectomy surgery (PEA). Risk and PH-specific treatment were assessed in surgically treated (PEA) and medically treated (non-PEA) patients at diagnosis and up to 5 years follow-up. Data are presented as median (Q1-Q3), count or per cent. Prior to surgery, 63% in the PEA-group [n = 98, age 64 (51-71) years, 37% female] used PH-specific treatment and 20, 69, and 10% were assessed as low, intermediate or high risk, respectively. After 1 year post-surgery, 34% had no PH-specific treatment or follow-up visit registered despite being alive at 5 years. Of patients with a 5 year visit (n = 23), 46% were at low and 54% at intermediate risk, while 91% used PH-specific treatment. In the non-PEA group [n = 213, age 72 (65-77) years, 56% female], 28% were assessed as low, 61% as intermediate and 11% as high risk. All patients at high risk versus 50% at low risk used PH-specific treatment. The 1 year mortality was 6%, while the risk was unchanged in 57% of the patients; 14% improved from intermediate to low risk, and 1% from high to low risk. At 5 years, 27% had a registered visit and 28% had died. Of patients with a 5 year visit (n = 58), 38% were at low, 59% at intermediate and 1% at high risk, and 86% used PH-specific treatment.
Conclusions: Risk status assessed pre-surgery did not foresee long-term post-PEA risk and pre-surgery PH-specific treatment did not foresee long-term post-PEA treatment. Medically treated CTEPH patients tend to remain at the same risk over time, suggesting a need for improved treatment strategies in this group.
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http://dx.doi.org/10.1002/ehf2.14033 | DOI Listing |
Int J Cardiovasc Imaging
August 2025
Department of Cardiovascular Medicine, Mayo Clinic, Phoenix, AZ, USA.
Purpose: Right ventricular (RV) systolic dysfunction is crucial to the prognosis of pulmonary hypertension (PH). The value of incorporating right ventricular longitudinal strain (RVLS), a sensitive parameter for evaluating RV systolic function, in risk assessment tools has not been thoroughly investigated.
Methods: This study included adult patients with PH Group I or IV who had a transthoracic echocardiogram (TTE) within a year before starting PH-specific treatment.
J Heart Lung Transplant
August 2025
Thoracic Surgery Department, Marie Lannelongue Hospital and Paris Saclay University, Le Plessis Robinson, France.
Background: Treatments for chronic thromboembolic pulmonary hypertension (CTEPH) include PH-specific pharmacotherapy (PHSP), balloon pulmonary angioplasty (BPA), and pulmonary endarterectomy (PEA). We evaluated a sequential multimodal strategy (SMS) combining PHSP, BPA, and PEA in selected high-surgical-risk patients with distal lesions in one lung and proximal lesions in the other.
Methods: In this prospective observational study, patients were selected to the SMS by a multidisciplinary panel, based on hemodynamic severity, location of lesions, and comorbidity profile.
BMC Pulm Med
July 2025
Center for Health Optimization & Implementation Research, VA Boston Healthcare System, Boston, MA, USA.
Background: Pulmonary hypertension (PH) is a progressive disease leading to right heart failure and early mortality. Early recognition of the disease and timely initiation of PH-specific therapies for qualifying PH subgroups are crucial for improving patient outcomes. Yet delays in diagnosis and treatment of PH persist.
View Article and Find Full Text PDFZhejiang Da Xue Xue Bao Yi Xue Ban
July 2025
Division of Nanobiomedicine, Suzhou Institute of Nano-Tech and Nano-Bionics, Chinese Academy of Sciences, Suzhou 215123, Jiangsu Province, China.
Myocardial infarction is a cardiovascular disease with high morbidity and mortality rates. Hydrogel biomaterials mimicking the extracellular matrix have recently been shown to demonstrate excellent biocompatibility, low immunogenicity, favorable biodegradability, and multifunctionality, showcasing significant potential for treatment of myocardial infarction. Hydrogels can provide mechanical support to the damaged myo-cardium, alleviating pathological remodeling.
View Article and Find Full Text PDFCurr Opin Pulm Med
September 2025
Department of Clinical Sciences and Community Health Università degli Studi di Milano, Milan, Italy.
Purpose Of Review: Pulmonary hypertension (PH) is a significant complication of various lung diseases, including rare conditions such as lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH). This review explores the pathophysiology, diagnostic challenges, and therapeutic strategies for managing PH in these conditions, emphasizing recent findings and gaps in knowledge.
Recent Findings: In LAM, PH primarily results from parenchymal destruction and hypoxic vasoconstriction rather than direct vascular involvement, leading to its reclassification from Group 5 to Group 3 PH.