Pulmonary Hypertension (PH)

Pulmonary hypertension (PH) is a frequent and clinically significant complication in patients with end-stage kidney disease (ESKD) undergoing maintenance hemodialysis (HD). However, PH assessment in this population is often confounded by volume overload, leading to potential overestimation and misclassification. This prospective, multicenter observational study aims to evaluate the prevalence, phenotype, and predictors of PH in HD patients under standardized euvolemic conditions. A structured pretrial phase including volume assessment and correction will be performed prior to echocardiographic evaluation, which will be conducted after confirmation of euvolemia, post-hemodialysis or on the following day. In participants with echocardiographic findings suggestive of PH, right heart catheterization may be performed when clinically indicated and after obtaining specific informed consent. The study integrates clinical, biological, dialysis-related, and echocardiographic data to provide a comprehensive characterization of PH in a well-defined ESKD population.

Previous data from our group showed reduced BMPR2 isoform A in pulmonary hypertension associated with left heart disease (PH-LHD) of valvular origin. Focusing on dysregulation of the BMPR2-activin pathway, a key regulator of vascular homeostasis, this project uses a multimodal approach combining molecular biology, advanced cardiac imaging, and histology. Its aim is to determine the role of the BMPR2-activin pathway in the development and regression of PH-LHD. We will conduct a prospective observational study with 1-year follow-up in 150 heart failure patients of different etiologies undergoing hemodynamic assessment. Clinical parameters, biomarkers, molecular profiles, and hemodynamic/echocardiographic variables will be analyzed. A nested substudy in 40 heart transplant candidates will include thoracic CT for vascular morphometric analysis and histological correlation in explanted hearts versus healthy controls. We will also assess reversibility of vascular remodeling one year after transplantation. Results may redefine PH-LHD classification, enable phenotypic precision-medicine models, guide targeted therapies such as activin inhibitors, and refine transplant criteria in combined pulmonary hypertension.

The goal of this observational study is to learn if advanced heart pressure wave analysis (called WIA and REPA) can help track and predict clinical improvement in adults (aged 18-85) with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) who are undergoing Balloon Pulmonary Angioplasty (BPA). The main questions it aims to answer are: Do changes in these advanced heart pressure wave patterns relate to improvements in a patient's walking distance, heart function, and overall quality of life after BPA treatment? Are these new measurements more effective than standard heart pressure tests at showing how much a patient has truly improved? Participants will: Undergo standard Balloon Pulmonary Angioplasty (BPA) sessions as part of their regular medical care for CTEPH. Have their heart pressure waves recorded through a catheter during the routine BPA procedure (this does not require any extra surgical steps). Complete walking tests (6-minute walk test), blood tests, and heart ultrasounds (echocardiography) before starting the treatment and after it is completed. Fill out short surveys regarding their daily physical activity and quality of life.

In view of the manifold options for mono- and combination therapy that have now emerged for patients with pulmonary (arterial) hypertension (PH/PAH), controlled clinical trials can only provide part of the information needed for optimal management. In order to gather adequate data on PAH/PH treatment in routine clinical care, the ongoing COMPERA registry prospectively documents consecutive patients with newly initiated treatment of PAH/PAH since May 2007. The internet-based registry fulfills high quality standards through several measures (planned minimum centre contribution of at least 10 patients per year, automated plausibility checks of data at entry, queries, monitoring with source data verification in \>50% of participating centers). It can be applied, among further purposes, for quality assurance: individual centers can confidentially compare their results with the combined outcome of other centers and the recommendations from guidelines. It is expected that the register contributes to optimization of specific drug therapy for PAH and PH. Since July 2013, also children of any age can be documented (COMPERA-KIDS).