# Vutrisiran stabiliseert rechterventrikel-functie bij ATTR-cardiomyopathie — HELIOS-B

*geplaatst 2026-09-03 · Hartfalen · JAMA cardiology · doi 10.1001/jamacardio.2026.3390 · https://hartvaat.nl/2026/08/26/vutrisiran-stabiliseert-rechterventrikel-functie-bij-attr-cardiomyopathie-helios/*

In een secundaire analyse van het HELIOS-B-randomisatieonderzoek bij patiënten met transthyretine-amyloïdose met cardiomyopathie (ATTR-CM) bleek dat rechterventrikel-functieverslechtering (RVFWS) zeer prevalent is en onafhankelijk voorspellend is voor mortaliteit en cardiovasculaire gebeurtenissen. Behandeling met vutrisiran stabiliseerde RVFWS en verbeterde de RVFWS/PASP-verhouding na 30 maanden, terwijl conventionele echocardiografische maatregelen minder sterk met uitkomsten correleerden. Deze bevindingen onderstrepen het belang van geavanceerde rechterventrikel-imaging in de follow-up van ATTR-CM en bevestigen het cardiale voordeel van vutrisiran.

## English: Right Ventricular Function, Clinical Outcomes, and Effect of Vutrisiran in Transthyretin Amyloidosis With Cardiomyopathy: Secondary Analysis of the HELIOS-B Randomized Clinical Trial.

In a post hoc analysis of the HELIOS-B trial in transthyretin amyloidosis with cardiomyopathy (ATTR-CM), impaired right ventricular free wall strain (RVFWS) was highly prevalent and independently predicted mortality and recurrent cardiovascular events, whereas conventional RV measures lost prognostic significance after adjustment. Vutrisiran treatment stabilized RVFWS and improved RVFWS indexed to pulmonary artery pressure at 30 months. These findings highlight the prognostic value of advanced right ventricular imaging in ATTR-CM and reinforce the cardioprotective effects of vutrisiran beyond left-sided metrics.

## Abstract (original, from the publication)

IMPORTANCE: Right ventricular (RV) dysfunction is common among patients with transthyretin amyloidosis with cardiomyopathy (ATTR-CM) and portends worse prognosis. The effects of vutrisiran on RV function remain incompletely characterized. OBJECTIVE: To determine the prevalence and prognostic importance of RV dysfunction in ATTR-CM, and to evaluate the effect of vutrisiran on RV function. DESIGN, SETTING, AND PARTICIPANTS: This post hoc analysis of the HELIOS-B randomized clinical trial (December 2019 and August 2021) included participants with ATTR-CM who had adequate echocardiographic images. Median (IQR) follow-up was 36 (33-36) months. Data analysis was performed from September to December 2025. INTERVENTIONS: Vutrisiran, 25 mg, subcutaneously every 12 weeks vs placebo. MAIN OUTCOMES AND MEASURES: The primary outcome was a composite of all-cause mortality and recurrent cardiovascular events. RV function was assessed using tricuspid annular systolic myocardial velocity (RV S'), RV fractional area change (RV FAC), RV free wall strain (RVFWS), and RVFWS indexed to pulmonary artery systolic pressure (RVFWS/PASP). Associations with outcomes and treatment effects were evaluated. RESULTS: Among 655 participants with ATTR-CM who were randomized, 548 had adequate echocardiographic images. The mean (SD) age was 75 (7) years; 506 patients (92%) were men and 42 (8%) were women. RV dysfunction was more prevalent if defined by abnormal RVFWS (absolute RVFWS ≤20%, 85%) vs RV S' (≤9.5cm/s, 56%) or RV FAC (≤35%, 31%). Patients in the worst RVFWS quartile (<10.8%, n = 137) had lower estimated glomerular filtration rate, lower left ventricular ejection fraction, and more advanced National Amyloidosis Centre (NAC) stage. Worse RVFWS and RVFWS/PASP were significantly associated with greater risk of all-cause mortality and recurrent cardiovascular events, independent of clinical characteristics, NAC stage, and LV global longitudinal strain. In contrast, associations of RV S' and RV FAC with outcomes were attenuated after multivariable adjustment. At 30 months, vutrisiran stabilized RVFWS (between-group difference: 1.6%, 95% CI, 0.7 to 2.6%) and improved RVFWS/PASP compared with placebo (between-group difference: +0.08%/mm Hg; 95% CI, 0.03%/mm Hg to 0.13%/mm Hg), with no significant effect on RV FAC (-1.6%; 95% CI, -3.7% to 0.6%). CONCLUSIONS AND RELEVANCE: RV dysfunction assessed by RVFWS is highly prevalent in ATTR-CM and independently predicts mortality and recurrent CV events, whereas conventional RV measures may underestimate RV dysfunction and lack independent prognostic value. This study found that, consistent with its beneficial effects on other measures of cardiac structure and function, vutrisiran stabilized RVFWS and improved RVFWS/PASP at 30 months. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04153149.

Auteurs: Karola S Jering, Alireza Manafi, Brian L Claggett, Marianna Fontana, Julian D Gillmore, Bernard E Bulwer, Farideh Roshanali, Narayana Prasad, Patrick Y Jay, Scott D Solomon, Hicham Skali

---
Bron: JAMA cardiology, https://doi.org/10.1001/jamacardio.2026.3390. Bijgewerkt 2026-08-27T01:23:00Z. 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.