Clinical significance of the estimation of pulmonary-right ventricular uncoupling in patients with transthyretin amyloid cardiomyopathy

Hiroki Usuku; Eiichiro Yamamoto; Kasumi Miyazaki; Ryudai Higashi; Atsushi Nozuhara; Fumi Oike; Naoto Kuyama; Noriaki Tabata; Masanobu Ishii; Shinsuke Hanatani; Tadashi Hoshiyama; Hisanori Kanazawa; Daisuke Sueta; Yuichiro Arima; Seitaro Oda; Hiroaki Kawano; Yasushi Matsuzawa; Yasuhiro Izumiya; Mitsuharu Ueda; Yasuhito Tanaka; Kenichi Tsujita

doi:10.1093/ehjimp/qyae113

Clinical significance of the estimation of pulmonary-right ventricular uncoupling in patients with transthyretin amyloid cardiomyopathy

Eur Heart J Imaging Methods Pract. 2025 Jan 17;3(1):qyae113. doi: 10.1093/ehjimp/qyae113. eCollection 2025 Jan.

Authors

Hiroki Usuku^{1

2

3}, Eiichiro Yamamoto^{2

3}, Kasumi Miyazaki¹, Ryudai Higashi^{2

3}, Atsushi Nozuhara^{2

3}, Fumi Oike^{2

3}, Naoto Kuyama^{2

3}, Noriaki Tabata^{2

3}, Masanobu Ishii^{2

3}, Shinsuke Hanatani^{2

3}, Tadashi Hoshiyama^{2

3}, Hisanori Kanazawa^{2

3}, Daisuke Sueta^{2

3}, Yuichiro Arima^{2

3}, Seitaro Oda⁴, Hiroaki Kawano^{2

3}, Yasushi Matsuzawa^{2

3}, Yasuhiro Izumiya^{2

3}, Mitsuharu Ueda^{3

5}, Yasuhito Tanaka¹, Kenichi Tsujita^{2

3}

Affiliations

¹ Department of Laboratory Medicine, Kumamoto University Hospital, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.
² Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.
³ Center of Metabolic Regulation of Healthy Aging, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.
⁴ Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.
⁵ Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.

Abstract

Aims: There are few data on the prognostic impact of pulmonary-right ventricular (RV) uncoupling in patients with wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM).

Methods and results: Among the 174 patients who were diagnosed with ATTRwt-CM at Kumamoto University Hospital from 2002 to 2021, 143 patients who met the current Japanese guideline and had sufficient information for two-dimensional speckle tracking echocardiography were retrospectively analysed. During a median follow-up of 1209 days, 39 cardiac deaths occurred. Compared with patients in the non-event group, those in the cardiac death group were significantly older (79.3 ± 6.7 vs. 76.4 ± 6.2, respectively; P < 0.05). Additionally, RV global longitudinal strain (RV-GLS)/systolic pulmonary artery pressure (sPAP), an index of pulmonary-RV uncoupling, was significantly lower in patients in the cardiac death group vs. the non-event group [0.29 (0.18-0.35) vs. 0.40 (0.29-0.57), P < 0.01]. Multivariate Cox proportional hazards regression analysis demonstrated that RV-GLS/sPAP was significantly associated with cardiac death after adjusting for tricuspid annular plane systolic excursion/sPAP (P < 0.01), sPAP (P < 0.05), and conventional prognostic factors including age and hospitalization for heart failure (<0.01), laboratory finding including high-sensitivity cardiac troponin T, and B-type natriuretic peptide (P < 0.01). Receiver operating characteristic analysis showed that the area under the curve for RV-GLS/sPAP for cardiac death was 0.72 and that the best cut off value for RV-GLS/sPAP was 0.34 (sensitivity, 76%; specificity, 65%). In the Kaplan-Meier analysis, patients with ATTRwt-CM who had low vs. high RV-GLS/sPAP (cut-off value 0.34) had a significantly higher probability of cardiac death (P < 0.01).

Conclusion: Pulmonary-RV uncoupling has significantly higher prognostic value compared with conventional prognostic factors in ATTRwt-CM.

Keywords: pulmonary-right ventricular uncoupling; transthyretin amyloid cardiomyopathy; two-dimensional speckle tracking imaging.