Tesi etd-12012025-095103
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Tipo di tesi
Corso Ordinario Ciclo Unico 6 Anni
Autore
TAMEZE, MARIEL SERGE
URN
etd-12012025-095103
Titolo
Prediction of the final diagnosis
in patients with suspected amyloid cardiomyopathy
and indication to tissue biopsy
Struttura
Classe Scienze Sperimentali
Corso di studi
SCIENZE MEDICHE - SCIENZE MEDICHE
Commissione
Tutor Prof. RECCHIA, FABIO ANASTASIO
Relatore Dott. AIMO, ALBERTO
Presidente Prof. PASSINO, CLAUDIO
Membro Prof. EMDIN, MICHELE
Membro Dott.ssa durante, angela
Membro Dott.ssa TOGNINI, PAOLA
Relatore Dott. AIMO, ALBERTO
Presidente Prof. PASSINO, CLAUDIO
Membro Prof. EMDIN, MICHELE
Membro Dott.ssa durante, angela
Membro Dott.ssa TOGNINI, PAOLA
Parole chiave
- AL Amyloidosis
- Cardiac Amyloidosis
- Carpal Tunnel Syndrome
- Monoclonal Gammopathy
- Transthyretin Amyloidosis
Data inizio appello
15/12/2025;
Disponibilità
completa
Riassunto analitico
Background: In suspected amyloid cardiomyopathy with a monoclonal protein, biopsy is mandatory, but only AL-CM needs urgent therapy.
Methods: We studied a multicenter cohort referred for biopsy for suspected amyloid CM. We derived age cut-offs overall and by carpal tunnel syndrome (CTS), and searched for risk modifiers in a gray zone.
Results: Of 606 patients (median 76 years, interquartile range 69–81; 81% male), 524 (86%) had a monoclonal protein; diagnoses were ATTR-CM 49%, AL-CM 30%, other 21%. AL-CM was unlikely at age >84 (sensitivity 98%; negative predictive value 0.94). ATTR-CM was ruled in by age ≥85 without CTS (specificity 97%), ≥86 with monolateral CTS, or ≥87 with bilateral CTS (both 100% specificity). Gray zone was defined as 74–84 (no CTS), 74–85 (monolateral), 74–86 (bilateral), 74–92 (CTS unknown). In this zone, each additional year of age favored ATTR-CM (OR 1.12, 95% CI 1.02–1.23), as did CTS (monolateral 2.36, 0.89–6.22; bilateral 3.53, 1.48–8.43). Modifiers toward ATTR-CM were Perugini grade (per level 8.80, 5.09–15.23), male sex (5.30, 2.40–11.69), interventricular septal thickness (per standard deviation [SD] 1.55, 1.14–2.12), and aortic stenosis (2.14, 1.06–4.33); modifiers toward AL-CM were abnormal free light-chain ratio (0.19, 0.08–0.46), positive urine immunofixation (0.23, 0.10–0.51), proteinuria (0.26, 0.12–0.57), log–troponin T (per SD 0.58, 0.37–0.90), and log–N-terminal pro-B-type natriuretic peptide (per SD 0.67, 0.48–0.94).
Conclusions: An age and CTS-based framework yields actionable pre-biopsy probabilities and may help prioritize biopsy for likely AL-CM.
Methods: We studied a multicenter cohort referred for biopsy for suspected amyloid CM. We derived age cut-offs overall and by carpal tunnel syndrome (CTS), and searched for risk modifiers in a gray zone.
Results: Of 606 patients (median 76 years, interquartile range 69–81; 81% male), 524 (86%) had a monoclonal protein; diagnoses were ATTR-CM 49%, AL-CM 30%, other 21%. AL-CM was unlikely at age >84 (sensitivity 98%; negative predictive value 0.94). ATTR-CM was ruled in by age ≥85 without CTS (specificity 97%), ≥86 with monolateral CTS, or ≥87 with bilateral CTS (both 100% specificity). Gray zone was defined as 74–84 (no CTS), 74–85 (monolateral), 74–86 (bilateral), 74–92 (CTS unknown). In this zone, each additional year of age favored ATTR-CM (OR 1.12, 95% CI 1.02–1.23), as did CTS (monolateral 2.36, 0.89–6.22; bilateral 3.53, 1.48–8.43). Modifiers toward ATTR-CM were Perugini grade (per level 8.80, 5.09–15.23), male sex (5.30, 2.40–11.69), interventricular septal thickness (per standard deviation [SD] 1.55, 1.14–2.12), and aortic stenosis (2.14, 1.06–4.33); modifiers toward AL-CM were abnormal free light-chain ratio (0.19, 0.08–0.46), positive urine immunofixation (0.23, 0.10–0.51), proteinuria (0.26, 0.12–0.57), log–troponin T (per SD 0.58, 0.37–0.90), and log–N-terminal pro-B-type natriuretic peptide (per SD 0.67, 0.48–0.94).
Conclusions: An age and CTS-based framework yields actionable pre-biopsy probabilities and may help prioritize biopsy for likely AL-CM.
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