Impaired right ventricular to pulmonary artery coupling is associated with worse survival in cardiac amyloidosis

Aiste Monika Jakstaite (Hannover)1, A. Hänselmann (Hannover)1, S. Soltani (Hannover)1, E. Angelini (Hannover)1, J. Kösterke (Hannover)1, P. Bianchi-Pereira (Hannover)1, M. Heuser (Hannover)2, V. Gödecke (Hannover)3, S. Gingele (Hannover)4, T. Skripuletz (Hannover)4, J. Bauersachs (Hannover)1, U. Bavendiek (Hannover)1, D. Berliner (Hannover)1

1Medizinische Hochschule Hannover Kardiologie und Angiologie Hannover, Deutschland; 2Medizinische Hochschule Hannover Hämatologie, Hämostaseologie, Onkologie und Stammzelltransplantation Hannover, Deutschland; 3Medizinische Hochschule Hannover Klinik für Nieren- und Hochdruckerkrankungen Hannover, Deutschland; 4Medizinische Hochschule Hannover Klinik für Neurologie Hannover, Deutschland


Background: Right ventricular (RV) dysfunction has been reported as an independent prognostic parameter in patients with cardiac amyloidosis (CA). The afterload-adjusted parameters of RV function may provide a more comprehensive insight into RV performance. This study aims to assess the prognostic value of echocardiographic surrogates of RV-pulmonary artery (PA) coupling in cardiac immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis.

Methods: We retrospectively analyzed RV-PA coupling in consecutive patients with AL or ATTR-CA from our center diagnosed between 2014 and 2023. RV-PA coupling was assessed using three different echocardiographic surrogates: tricuspid annular plane systolic excursion/pulmonary artery systolic pressure (TAPSE/PASP), fractional area change/PASP (FAC/PASP), and RV free wall strain/PASP (RVFWS/PASP) ratios. Median values were determined to identify RV-PA uncoupling and stratify the population. The primary endpoint was all-cause mortality.

Results: 133 patients with CA (83% with ATTR-CA, 17% with AL-CA) were included in the study (median age 77 years, 87% male). During a median follow-up period of 23 months (IQR: 15-34) the primary endpoint of all-cause mortality occurred in 29 patients (22%). The median values of TAPSE/PASP, FAC/PASP, and RVFWS/PASP were 0.41 mm/mmHg (IQR: 0.32-0.60), 0.88 %/mmHg (IQR: 0.62-1.18), and 0.41 %/mmHg (IQR: 0.29-0.63), respectively. RV-PA uncoupling at the diagnosis of CA was observed in 52% of the study patients when using TAPSE/PASP and FAC/PASP, and 51% when using the RVFWS/PASP ratio. Patients exhibiting RV-PA uncoupling were older, more symptomatic, had worse systolic left ventricular (LV) and RV function as well as higher NT-proBNP levels and poorer laboratory parameters indicative of secondary organ damage (Table 1). Impaired RV-PA coupling was associated with an increased all-cause mortality as indicated by a TAPSE/PASP ratio <0.41 (hazard ratio (HR) 4.19, 95% confidence interval (CI) 2.02–8.69, p = 0.001; figure 1), and these findings remained consistent in both AL- and ATTR-CA patient cohorts. Receiver operating characteristic (ROC) analysis revealed that the TAPSE/PASP ratio exhibited the best predictive value for outcomes (area under the curve (AUC) = 0.700, 95% CI: 0.560–0.840) when compared to FAC/PASP (AUC = 0.676, 95% CI: 0.541–0.811) and RVFWS/PASP (AUC = 0.662, 95% CI: 0.532–0.793).

Conclusions: Early RV-PA uncoupling is linked to a higher risk of all-cause mortality in CA patients. TAPSE/PASP outperforms FAC/PASP and RVFWS/PASP in predicting long-term survival.

Table 1. Baseline characteristics of the study population stratified by RV-PA uncoupling (TAPSE/PASP <0.41 mm/mmHg)

 Variable All
N = 133
 Impaired RV-PA coupling 
N = 69
 Normal RV-PA coupling
N = 64
 P value
 Age, years77 (72-81)79 (75-84)76 (72-80)0.008
 NYHA class ≥3, n (%)39 (29)26 (37)13 (21)0.048
 LVEF, %48 ± 11 44 ± 1252 ± 9<0.001
 LVGLS, %-11.8 ± 4.2-10.4 ± 3.7-13.1 ± 4.3 <0.001
 TAPSE, mm 16 ± 513 ± 3 19 ± 4 <0.001 
 FAC, %34 ± 1029 ± 939 ± 8 <0.001 
 RVFWS, %-16.0 ± 5.8-12.6 ± 4.0-19.6 ± 5.2<0.001
 RVGLS, %-12.1 ± 4.7-9.6 ± 3.1-15 ± 4.5<0.001
 NT-proBNP, ng/l2583 (1255-5728)5100 (2681-10279)1661 (729-3172)<0.001
 Creatinine, μmol/l108 (93-143)117 (99-159)101 (84-118)0.003
 Bilirubin, mg/dl12 (9-18)15 (10-25)11 (8-15)



Figure 1. Kaplan-Meier survival analysis in CA patients with vs. without RV-PA uncoupling
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