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Benchmarks of clinical management are essential for improving the quality of care. However, the lack of established quality metrics for pulmonary arterial hypertension (PAH) contributes to practice heterogeneity. We assessed our center's diagnostic practices, therapeutic practices, and risk-adjusted survival patterns over time for the purpose of establishing quality benchmarks. We analyzed the demographics, clinical characteristics, and diagnostic evaluation of 702 PAH patients enrolled between 1999 and 2019. We examined outcomes in this cohort, including an analysis of risk stratification, therapeutic practice patterns, hospitalizations, organ transplant, and survival. Initial diagnostic workup of incident PAH cases demonstrated excellent completion of echocardiographic (99%) and pulmonary function testing (91%), with improved completion of VQ scanning over the study time period (90% between 2015 and 2019). Right heart catheterization (RHC) was performed in all patients; RHC performed at our center was more likely to include complete hemodynamic data than those performed at referring institutions (55.4% and 30.4% respectively). The average number of PAH-specific medications prescribed increased over time; however, there was no significant increase in the use of parenteral therapy over time, even when stratified by the REVEAL risk score. Survival rates in the cohort were 94% at 1 year, 75% at 5 years, and 60% at 10 years, comparable to those of other PAH cohorts. Analysis of our well-characterized cohort of PAH patients reveals the extent to which guideline-directed diagnostic and therapeutic care is delivered at our specialty center, and the associated outcomes; these data may serve as a benchmark for continued improvements in quality of PAH care.
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http://dx.doi.org/10.1002/pul2.70063 | DOI Listing |
Clin J Gastroenterol
September 2025
Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
Portopulmonary hypertension (POPH), a subtype of pulmonary arterial hypertension (PAH), develops with portal hypertension and may persist after liver transplantation. While there have been successes using balloon-occluded retrograde transvenous obliteration (BRTO) for POPH, no reports exist on long-term follow-up. A 60-year-old man with hepatitis C cirrhosis developed POPH.
View Article and Find Full Text PDFJACC Case Rep
September 2025
Pulmonary Hypertension Multidisciplinary Unit, Cardiology Department, Hospital Universitario 12 de Octubre, and CIBERCV, Madrid, Spain; Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), Madrid, Spain.
Background: BMPR2 mutations cause heritable pulmonary arterial hypertension (PAH) and may also influence epithelial carcinogenesis.
Case Summary: We report 3 women with BMPR2-related PAH who developed early onset epithelial cancers: 2 breast cancers (34 and 54 years of age) and 1 colorectal cancer (47 years of age). All were on advanced PAH therapy at diagnosis.
ESC Heart Fail
September 2025
Department of Cardiac-, Thoracic-, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany.
Aims: Non-pharmacological therapies for acute decompensated heart failure (HF) and cardiogenic shock have evolved considerably in recent decades. Short-term mechanical circulatory support (MCS) devices can be used as circulatory backup. While nearly all available devices use continuous flow, evidence indicates that pulsatile flow can be more effective.
View Article and Find Full Text PDFJTCVS Open
August 2025
Department of Paediatric Cardiac Surgery, Kinderspital and University Zurich, Zurich, Switzerland.
Objectives: Primary repair of the common arterial trunk is still burdened by high mortality rates. Because of the low incidence and complexity of common arterial trunk, evidence is limited to reports with small sample sizes, and issues such as the ideal surgical timing to address the primary repair are still debated. We performed a systematic review and meta-analysis to estimate the pooled mortality, morbidity, and reoperation rates after common arterial trunk primary repair.
View Article and Find Full Text PDFJTCVS Open
August 2025
Department of Critical Care Medicine, Mayo Clinic, Jacksonville, Fla.
Objective: Ex vivo lung perfusion (EVLP) has resulted in a significant increase in the use of extended-criteria donor lungs without negatively impacting survival outcomes. However, in-house EVLP is resource-intensive, thereby limiting accessibility. Remote, centralized EVLP (rc-EVLP) has been used with acceptable outcomes in a highly protocolized feasibility study, although has not been assessed in a clinical setting.
View Article and Find Full Text PDF