Prevalence and clinical significance of right ventricular pulmonary arterial uncoupling in cardiac amyloidosis

Copyright © 2023 Elsevier B.V. All rights reserved..

BACKGROUND: This study aims to evaluate the prevalence and the clinical significance of the right ventricular pulmonary arterial (RV-PA) uncoupling in patients with cardiac amyloidosis (CA).

METHODS: The study population consisted in 92 consecutive patients with CA (age 71.1 ± 12.2 years, 71% males; 47% with immunoglobulin light chain (AL), 53% with transthyretin [ATTR]). A pre-specified tricuspid anulus plane systolic excursion on pulmonary arterial systolic pressure (TAPSE/PASP) value <0.31 mm/mmHg was used to define RV-PA uncoupling and to dichotomize the study population.

RESULTS: Thirty-two patients (35%) showed RV-PA uncoupling at baseline evaluation (15/44 [34%] AL and 17/48 [35%] ATTR). Patients with RV-PA uncoupling, in both AL and ATTR, showed worse NYHA functional class, lower systemic blood pressure, and more pronounced left ventricular and RV systolic dysfunction than those with RV-PA coupling. During a median follow-up of 8 months (IQR 4-13), 26 patients (28%) experienced cardiovascular death. Patients with RV-PA uncoupling showed lower survival at 12 months follow-up than those with RV-PA coupling (42.7% [95%CI 21.7-63.7%] vs. 87.3% [95%CI 78.3-96.3%], p-value<0.001). Multivariate analysis identified high-sensitivity troponin I values (HR 1.01 [95%CI 1.00-1.02] per 1 pg/mL increase; p-value 0.013) and TAPSE/PASP (HR 1.07 [95%CI 1.03-1.11] per 0.01 mm/mmHg decrease; p-value 0.002) as independent predictors of cardiovascular death.

CONCLUSIONS: RV-PA uncoupling is common among patient with CA, and it is a marker of advanced disease and worse outcome. This study suggest that TAPSE/PASP ratio has the potential to improve risk stratification and guide management strategies in patients with CA of different etiology and advanced disease.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:388

Enthalten in:

International journal of cardiology - 388(2023) vom: 01. Okt., Seite 131147

Sprache:

Englisch

Beteiligte Personen:

Palmiero, Giuseppe [VerfasserIn]
Monda, Emanuele [VerfasserIn]
Verrillo, Federica [VerfasserIn]
Dongiglio, Francesca [VerfasserIn]
Caiazza, Martina [VerfasserIn]
Rubino, Marta [VerfasserIn]
Lioncino, Michele [VerfasserIn]
Diana, Gaetano [VerfasserIn]
Vetrano, Erica [VerfasserIn]
Fusco, Adelaide [VerfasserIn]
Cirillo, Annapaola [VerfasserIn]
Mauriello, Alfredo [VerfasserIn]
Ciccarelli, Giovanni [VerfasserIn]
Ascione, Luigi [VerfasserIn]
De Rimini, Maria Luisa [VerfasserIn]
D'Alto, Michele [VerfasserIn]
Cerciello, Giuseppe [VerfasserIn]
D'Andrea, Antonello [VerfasserIn]
Golino, Paolo [VerfasserIn]
Calabrò, Paolo [VerfasserIn]
Bossone, Eduardo [VerfasserIn]
Limongelli, Giuseppe [VerfasserIn]

Links:

Volltext

Themen:

Cardiac amyloidosis
Echocardiography
Journal Article
Outcome

Anmerkungen:

Date Completed 14.08.2023

Date Revised 16.09.2023

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1016/j.ijcard.2023.131147

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM359246184