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Aims <p>The RAISE Score, encompassing five domains (<i>R</i>emodeling, <i>A</i>ge, <i>I</i>njury, <i>S</i>ystemic, <i>E</i>lectrical), was proposed to screen for transthyretin amyloid cardiomyopathy (ATTR-CM) in patients with aortic stenosis (AS), but is not routinely used. This study evaluated its performance following aortic valve replacement (AVR).</p> Methods <p>This single-center, prospective, observational study included patients aged ≥ 60&#xa0;years with end-diastolic interventricular septum thickness (IVSd) ≥ 12&#xa0;mm, who underwent hydroxydiphosphonate (HDP) bone scintigraphy after AVR between March 2021 and July 2024. The diagnostic performance of the RAISE Score assessed at 30-day follow-up and of&#xa0;simpler screening parameters for pathological HDP uptake (Perugini 1–3) were analyzed, along with their association with all-cause mortality and heart failure (HF) hospitalization.</p> Results <p>Among 131 included patients (median age 81&#xa0;years; 64% male) pathological HDP uptake was found in 21 (16%), and 11 (8.4%) were diagnosed with ATTR-CM. A RAISE Score ≥ 2 demonstrated 76% sensitivity and 56% specificity for pathological bone scintigraphy, while a Score ≥ 3 showed 62% sensitivity and 78% specificity. The parameters age ≥ 83&#xa0;years and the combination of carpal tunnel syndrome (CTS) and/or N-terminal pro-B-type natriuretic peptide (NT-proBNP) ≥ 1,400&#xa0;pg/mL performed similarly to the RAISE Score. Cardiac HDP uptake showed a strong trend toward HF hospitalization (HR&#xa0;[95%-CI]:&#xa0;5.81&#xa0;[0.93–36.20];&#xa0;p = 0.060) and all-cause mortality (HR&#xa0;[95%-CI]:&#xa0;3.27&#xa0;[0.95–11.24]; p = 0.060). CTS and/or NT-proBNP ≥ 1,400&#xa0;pg/mL was significantly associated with higher all-cause mortality (HR&#xa0;[95%-CI]:&#xa0;3.92&#xa0;[1.04–14.84];&#xa0;p = 0.044), whereas the&#xa0;RAISE Score was not.</p> Conclusion <p>After AVR, the complex RAISE Score showed lower sensitivity than originally reported, while simpler parameters demonstrated comparable predictive value for ATTR-CM screening. Advanced age, CTS history and elevated NT-proBNP levels seem most valuable for identifying candidates for bone scintigraphy.</p> Graphical Abstract <p>A total of 131 patients aged ≥ 60&#xa0;years, with an IVSd ≥ 12&#xa0;mm and an AVR between March 2021 and July 2024, underwent standard diagnostic evaluation for ATTR-CM, including HDP bone scintigraphy. Pathological findings on bone scintigraphy (Perugini Score 1–3) were observed in 16% of the patients and were associated with worse clinical outcomes. The performance of the proposed RAISE Score [<CitationRef CitationID="CR11">11</CitationRef>], along with simplified or modified screening parameters, was evaluated in predicting both pathological scintigraphy results and clinical outcomes. ATTR-CM = transthyretin amyloidosis cardiomyopathy; AVR = aortic valve replacement; CTS = carpal tunnel syndrome; HDP = hydroxydiphosphonate; IVSd = interventricular septum thickness at end-diastole; NT-proBNP = N-terminal pro-B-type natriuretic peptide.</p> <p></p>

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Cardiac transthyretin amyloidosis in aortic valve replacement: RAISE score performance in the postoperative setting

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月4日

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  • Richard J. Nies,
  • Svenja Ney,
  • Jasper F. Nies,
  • Katharina Seuthe,
  • Jan Grobecker,
  • Friedrich Gruenagel,
  • Stephan Nienaber,
  • Merve Kural,
  • Sascha Macherey-Meyer,
  • Matthieu Schäfer,
  • Clemens Metze,
  • Matti Adam,
  • Maria Papathanasiou,
  • Can Öztürk,
  • Amin Polzin,
  • Fabian Voß,
  • Stephan Baldus,
  • Roman Pfister

摘要

Aims

The RAISE Score, encompassing five domains (Remodeling, Age, Injury, Systemic, Electrical), was proposed to screen for transthyretin amyloid cardiomyopathy (ATTR-CM) in patients with aortic stenosis (AS), but is not routinely used. This study evaluated its performance following aortic valve replacement (AVR).

Methods

This single-center, prospective, observational study included patients aged ≥ 60 years with end-diastolic interventricular septum thickness (IVSd) ≥ 12 mm, who underwent hydroxydiphosphonate (HDP) bone scintigraphy after AVR between March 2021 and July 2024. The diagnostic performance of the RAISE Score assessed at 30-day follow-up and of simpler screening parameters for pathological HDP uptake (Perugini 1–3) were analyzed, along with their association with all-cause mortality and heart failure (HF) hospitalization.

Results

Among 131 included patients (median age 81 years; 64% male) pathological HDP uptake was found in 21 (16%), and 11 (8.4%) were diagnosed with ATTR-CM. A RAISE Score ≥ 2 demonstrated 76% sensitivity and 56% specificity for pathological bone scintigraphy, while a Score ≥ 3 showed 62% sensitivity and 78% specificity. The parameters age ≥ 83 years and the combination of carpal tunnel syndrome (CTS) and/or N-terminal pro-B-type natriuretic peptide (NT-proBNP) ≥ 1,400 pg/mL performed similarly to the RAISE Score. Cardiac HDP uptake showed a strong trend toward HF hospitalization (HR [95%-CI]: 5.81 [0.93–36.20]; p = 0.060) and all-cause mortality (HR [95%-CI]: 3.27 [0.95–11.24]; p = 0.060). CTS and/or NT-proBNP ≥ 1,400 pg/mL was significantly associated with higher all-cause mortality (HR [95%-CI]: 3.92 [1.04–14.84]; p = 0.044), whereas the RAISE Score was not.

Conclusion

After AVR, the complex RAISE Score showed lower sensitivity than originally reported, while simpler parameters demonstrated comparable predictive value for ATTR-CM screening. Advanced age, CTS history and elevated NT-proBNP levels seem most valuable for identifying candidates for bone scintigraphy.

Graphical Abstract

A total of 131 patients aged ≥ 60 years, with an IVSd ≥ 12 mm and an AVR between March 2021 and July 2024, underwent standard diagnostic evaluation for ATTR-CM, including HDP bone scintigraphy. Pathological findings on bone scintigraphy (Perugini Score 1–3) were observed in 16% of the patients and were associated with worse clinical outcomes. The performance of the proposed RAISE Score [11], along with simplified or modified screening parameters, was evaluated in predicting both pathological scintigraphy results and clinical outcomes. ATTR-CM = transthyretin amyloidosis cardiomyopathy; AVR = aortic valve replacement; CTS = carpal tunnel syndrome; HDP = hydroxydiphosphonate; IVSd = interventricular septum thickness at end-diastole; NT-proBNP = N-terminal pro-B-type natriuretic peptide.