{"id":"379382dd317b","type":"article","url":"https://hartvaat.nl/2026/11/04/gdmt-verbetert-rechterventrikel-functie-en-overleving-bij-cc-tga-met-matige-tot-/","title":"GDMT verbetert rechterventrikel-functie en overleving bij cc-TGA met matige tot ernstige RV-dysfunctie","title_en":"Heart failure therapy in congenitally corrected transposition of the great arteries: effect on right ventricular function and survival.","category":"algemeen","category_label":"Algemeen","professions":["cardioloog"],"tags":[],"journal":"European heart journal","doi":"10.1093/eurheartj/ehag854","source_url":"https://doi.org/10.1093/eurheartj/ehag854","authors":["Sara Ahmed","Yehia Karara","Rana Rashwan","William R Miranda","Heidi M Connolly","Alexander C Egbe"],"significance":6,"published":"2026-11-12","source_date":"2026-11-04","image":"","kennis":["https://hartvaat.nl/kennis/kleplijden/aortastenose/","https://hartvaat.nl/kennis/coronairlijden/stemi/"],"congress":"","summary_en":"A cohort of 213 adults with congenitally corrected transposition of the great arteries (cc-TGA) and systemic right ventricular dysfunction was evaluated for the impact of guideline-directed medical therapy (GDMT) optimization. Higher GDMT scores were associated with improved RV free wall strain, lower NT-proBNP levels, and reduced mortality, though benefits were confined to patients with moderate or severe dysfunction. These findings support early and aggressive GDMT titration in this high-risk congenital heart disease population to mitigate heart failure progression and improve survival.","created":"2026-11-05T02:26:40Z","updated":"2026-11-05T02:26:40Z","licence":"Citeer vrij, met bronvermelding en een link naar hartvaat.nl (de url van het record). Samenvattingen zijn redactioneel werk van HartVaat; de oorspronkelijke publicaties blijven van hun uitgevers (doi). Geen medisch advies.","body_markdown":"Een cohortstudie van 213 volwassenen met congenitaal gecorrigeerde transpositio magna arteriarum (cc-TGA) en een systeemrechterventrikel toont aan dat optimalisatie van richtlijnconforme hartfalenmedicatie (GDMT) gepaard gaat met verbeterde RV-functie, lagere NT-proBNP-waarden en een lager sterftecijfer. Het voordeel was alleen zichtbaar bij patiënten met een matige tot ernstige RV-dysfunctie; bij milde dysfunctie leverde GDMT-uptitratie geen meetbaar klinisch voordeel op. Deze bevindingen ondersteunen het tijdig en intensief inzetten van GDMT bij deze kwetsbare groep om hartfalenprogressie en mortaliteit te beperken.","abstract_original":"BACKGROUND AND AIMS: The aim of the study was to assess the effect of guideline-directed medical therapy (GDMT) on cardiac function, neurohormonal activation, and mortality in adults with congenitally corrected transposition of the great arteries (cc-TGA) presenting with systemic right ventricular (RV) systolic dysfunction. The study hypothesis was that GDMT use (assessed using the GDMT score) was associated with improved cardiac function (higher RV free wall strain [RVFWS]), less neurohormonal activation (lower N-terminal pro-B-type natriuretic peptide [NT-proBNP] levels), and lower mortality. METHODS: GDMT score, RVFWS, and NT-proBNP levels were assessed at baseline and 1-year follow-up. GDMT uptitration was assessed using ΔGDMT (GDMT at 1-year minus GDMT at baseline). Improvements in RV systolic function and neurohormonal activation were defined as a relative increase in RVFWS of >20% and a relative decrease in NT-proBNP levels of >20%, respectively. RESULTS: Of 213 patients (age 43 ± 15 years; 54% male), baseline GDMT score was 1.97 ± 1.27, and ΔGDMT score was 0.74 ± 0.39. Baseline RVFWS was -18 ± 5%; 25% had improved RV systolic function. Baseline NT-proBNP was 369 (142, 1004) pg/mL; 56% had improved neurohormonal activation. Higher baseline GDMT score and ΔGDMT score were associated with improvement in RV systolic function and neurohormonal activation. Overall, 34 (16%) patients died, and higher baseline GDMT score and ΔGDMT score were associated with a lower risk of mortality and of the composite adverse event endpoint (heart failure hospitalization, death, and heart transplantation). Clinical benefits of GDMT use were observed only in patients with moderate/severe RV systolic dysfunction, and not in patients with mild RV systolic dysfunction. CONCLUSIONS: GDMT use was associated with improved outcomes in patients with moderate/severe RV systolic dysfunction."}