Right ventricular to pulmonary artery coupling and outcome in patients with cardiac amyloidosis

Daniela Tomasoni; Marianna Adamo; Aldostefano Porcari; Alberto Aimo; Giovanni Battista Bonfioli; Vincenzo Castiglione; Maria Franzini; Riccardo Maria Inciardi; Anas Khalil; Carlo Mario Lombardi; Laura Lupi; Matilde Nardi; Chiara Oriecuia; Matteo Pagnesi; Giorgia Panichella; Maddalena Rossi; Nicola Saccani; Claudia Specchia; Giuseppe Vergaro; Marco Merlo; Gianfranco Sinagra; Michele Emdin; Marco Metra

doi:10.1093/ehjci/jead145

Right ventricular to pulmonary artery coupling and outcome in patients with cardiac amyloidosis

Eur Heart J Cardiovasc Imaging. 2023 Sep 26;24(10):1405-1414. doi: 10.1093/ehjci/jead145.

Authors

Daniela Tomasoni¹, Marianna Adamo¹, Aldostefano Porcari², Alberto Aimo^{3

4}, Giovanni Battista Bonfioli¹, Vincenzo Castiglione^{3

4}, Maria Franzini⁵, Riccardo Maria Inciardi¹, Anas Khalil³, Carlo Mario Lombardi¹, Laura Lupi¹, Matilde Nardi¹, Chiara Oriecuia^{6

7}, Matteo Pagnesi¹, Giorgia Panichella³, Maddalena Rossi², Nicola Saccani¹, Claudia Specchia⁷, Giuseppe Vergaro^{3

4}, Marco Merlo², Gianfranco Sinagra², Michele Emdin^{3

4}, Marco Metra¹

Affiliations

¹ Cardiology, ASST Spedali Civili di Brescia and Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Piazzale Spedali Civili, Brescia 25123, Italy.
² Department of Cardiovascular, Azienda Sanitaria Universitaria Integrata Giuliano Isontina, University of Trieste, Trieste, Italy.
³ Health Science Interdisciplinary Center, Scuola Superiore Sant'Anna, Pisa, Italy.
⁴ Cardiology and Cardiovascular Medicine Division, Fondazione Toscana Gabriele Monasterio, Pisa, Italy.
⁵ Dipartimento di Ricerca Traslazionale e delle Nuove Tecnologie in Medicina e Chirurgia, Università di Pisa, Pisa, Italy.
⁶ Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
⁷ Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

PMID: 37379445
DOI: 10.1093/ehjci/jead145

Abstract

Aims: To investigate the prognostic value of the right ventricle-to-pulmonary artery (RV-PA) coupling in patients with either transthyretin (ATTR) or immunoglobulin light-chain (AL) cardiac amyloidosis (CA).

Methods and results: Overall, 283 patients with CA from 3 Italian high-volume centres were included (median age 76 years; 63% males; 53% with ATTR-CA, 47% with AL-CA). The RV-PA coupling was evaluated by using the tricuspid annular plane systolic excursion/pulmonary artery systolic pressure (TAPSE/PASP) ratio. The median value of TAPSE/PASP was 0.45 (0.33-0.63) mm/mmHg. Patients with a TAPSE/PASP ratio <0.45 were older, had lower systolic blood pressure, more severe symptoms, higher cardiac troponin and N-terminal pro-B-type natriuretic peptide levels, greater left ventricular (LV) thickness, and worse LV systolic and diastolic function. A TAPSE/PASP ratio <0.45 was independently associated with a higher risk of all-cause death or heart failure (HF) hospitalization [hazard ratio (HR) 1.98, 95% confidence interval (CI) 1.32-2.96; P = 0.001] and all-cause death (HR 2.18, 95% CI 1.31-3.62; P = 0.003). The TAPSE/PASP ratio reclassified the risk of both endpoints [net reclassification index 0.46 (95% CI 0.18-0.74) P = 0.001 and 0.49 (0.22-0.77) P < 0.001, respectively], while TAPSE or PASP alone did not (all P > 0.05). The prognostic impact of the TAPSE/PASP ratio was significant both in AL-CA patients (HR for the composite endpoint 2.47, 95% CI 1.58-3.85; P < 0.001) and in ATTR-CA (HR 1.81, 95% CI 1.11-2.95; P = 0.017). The receiver operating characteristic curve showed that the optimal cut-off for predicting prognosis was 0.47 mm/mmHg.

Conclusion: In patients with CA, RV-PA coupling predicted the risk of mortality or HF hospitalization. The TAPSE/PASP ratio was more effective than TAPSE or PASP in predicting prognosis.

Keywords: TAPSE/PASP; cardiac amyloidosis; heart failure; prognosis; right ventricular function; right ventricular to pulmonary artery coupling.