https://doi.org/10.1007/s00392-025-02625-4
1Herzzentrum der Universität zu Köln Klinik III für Innere Medizin Köln, Deutschland; 2University of Barcelona Department of Cardiology Barcelona, Spanien; 3University of California San Francisco Division of Cardiology San Francisco, USA; 4University of Campania 'L. Vanvitelli' Department of Cardiology Napoli, Italien; 5Pulmonary Circulation Centre Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College Krakau, Polen; 6Hospital Clínic, University of Barcelona Department of Pulmonary Medicine Barcelona, Spanien; 7Universitätsklinikum Münster Klinik für Kardiologie III: Angeborene Herzfehler (EMAH) und Klappenerkrankungen Münster, Deutschland; 8Deutsches Herzzentrum München Klinik für Kinderkardiologie und angeborene Herzfehler München, Deutschland; 9Royal Brompton Hospital London, Großbritannien; 10Herzzentrum der Universität zu Köln Klinik für Kinderkardiologie Köln, Deutschland; 11Aristotle University of Thessaloniki First Department of Cardiology Thessaloniki, Griechenland; 12University Hospital Centre Zagreb Department of Cardiology Zagreb, Kroatien; 13Herz- und Diabeteszentrum NRW Allgemeine und Interventionelle Kardiologie/Angiologie Bad Oeynhausen, Deutschland; 14University of Bonn Department of Cardiology Bonn, Deutschland; 15Deutsches Herzzentrum München Klinik für angeborene Herzfehler und Kinderkardiologie München, Deutschland; 16Universitätsklinikum Münster Klinik und Poliklinik für angeborene (EMAH) & erworbene Herzfehler Münster, Deutschland; 17Hospital Universitario Doce de Octubre Cardiovasculares (CIBERCV), Instituto de Salud Carlos III (ISCIII) Madrid, Spanien; 18University of Campania 'L. Vanvitelli', A.O.R.N. dei Colli, Monaldi Hospital Department of Cardiology Napoli, Italien
Aims: Monitor the effects of DTT on hemodynamics, ESC/ERS risk status, and outcomes in patients with PAH-ACHD in real-world clinical practice.
Methods: Data from consecutive patients with ACHD-PAH who underwent assessment of cardiopulmonary hemodynamics by right heart catheterization (RHC) prior to initiation of DTT in adulthood (therapy-naïve) and at least one subsequent RHC on DTT between 2000 and 2022 in 14 expert centres in Europe and the U.S. were analyzed. Comprehensive analyses of RHC parameters was performed, and key non-invasive measures as well as ESC/ERS risk status were recorded. The correlation between hemodynamic parameters and the combined outcome of all-cause mortality or hospitalization for heart failure was assessed.
Results: 141 patients (77% female, median age 38 [27-52] years) were included. Underlying CHD was a pre- or post-tricuspid shunt in 35% and 59%, respectively. 17% had complex CHD, 7% had innate left heart disease, and 19% presented with Eisenmenger´s syndrome. Upon diagnostic RHC, DTT was started and maintained in all patients (38% upfront combination therapy; 62% monotherapy). The median time to follow-up RHC was 16 [8–27] months. DDT was associated with significant reductions in mean pulmonary artery pressure and pulmonary vascular resistance (both p<0.001), and improvement of stroke volume index, pulmonary arterial compliance, and cardiac index (all p<0.01). Clinical measures such as WHO-FC and NT-proBNP, and ESC/ERS risk status also improved. PVR and NT-proBNP at baseline, and the extent of PVR reduction during follow-up were associated with outcome (composite of all-cause mortality or hospitalization for heart failure). Repeat RHC resulted in treatment escalation in 48% of patients.
Conclusions: In PAH-ACHD, DTT led to a moderate but sustained hemodynamic and functional improvement, and the degree of PVR reduction was associated with outcomes. Repeat RHC resulted in subsequent treatment escalation in many patients, indicating that invasive follow-up is warranted.