Gillmore Staging System for Transthyretin Amyloid Cardiomyopathy (ATTR-CM)
Three-stage clinical staging system for transthyretin amyloid cardiomyopathy (ATTR-CM) based on NT-proBNP and eGFR. Predicts median survival and guides treatment eligibility (tafamidis, CTRN gene silencers).
Score interpretation
Stage I: NT-proBNP <= 3000 AND eGFR > 45 -- early disease with best prognosis
→ Initiate disease-modifying therapy: tafamidis 61 mg OD (ATTR-ACT trial -- reduced all-cause mortality and CV hospitalisations in wtATTR and variant ATTR); consider patisiran (siRNA) or inotersen (ASO) for hereditary ATTR with polyneuropathy; symptom management: loop diuretics for congestion (furosemide titrated to weight), beta-blocker caution in fixed heart rate dependency; avoid: CCB (verapamil, diltiazem -- negative inotropes), digoxin (binding to amyloid fibrils), high-dose ACEi (hypotension risk); ICD if EF < 35% or high-risk arrhythmia; pacemaker if conduction disease; 6-monthly NT-proBNP, TTE, 6MWT; genetic testing for TTR variants; family screening if hereditary.
Stage II: one criterion met -- intermediate stage; disease-modifying therapy urgent
→ Tafamidis 61 mg OD if not already started; reevaluate Stage I management; optimise diuresis: loop diuretic + spironolactone (low dose); avoid fluid overload; mobility assessment and physiotherapy; 99mTc-DPD/PYP scintigraphy for non-invasive diagnosis confirmation; cardiac MRI for disease characterisation; electrophysiology review if syncope or conduction disease; palliative care involvement for symptom management; advance care planning discussion; cardiomyopathy specialist centre review; consider orthotopic heart transplant (ATTR-CM eligibility variable by programme -- discuss with transplant centre).
Stage III: both criteria met -- advanced disease; palliative approach may predominate
→ Disease-modifying therapy (tafamidis) may still provide benefit even in stage III -- continue or initiate; aggressive symptom control: diuresis, fluid restriction, salt restriction < 2 g/day; heart failure specialist review; advance care planning: DNACPR, hospitalisation preferences, preferred place of care; palliative care team involvement; ICD/CRT: reassess if EF <= 35% (ICD benefit unclear in advanced amyloid); consider percutaneous EP mapping if arrhythmia; heart transplantation: limited data but may be considered at select centres; amyloid specialist centre referral; family and carer support; spiritual/religious care if desired.
Interpretation bands for the Gillmore ATTR-CM Staging. Apply clinical judgement and local guidance.
References
- Gillmore JD et al. A new staging system for cardiac transthyretin amyloidosis. Eur Heart J. 2018;39(30):2799-2806.
- Maurer MS et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy (ATTR-ACT). N Engl J Med. 2018;379(11):1007-1016.
Related
Curated clinical cross-links plus same-class fallbacks.
- Acute Heart Failure · ESC 2021 Heart Failure Guidelines; NICE NG106
- NSTEMI / Unstable Angina · ESC 2020 NSTEMI Guidelines; NICE NG185
- New-Onset Atrial Fibrillation · ESC 2020 AF Guidelines; NICE NG196
- Hypertensive Emergency · ESC/ESH 2018 Hypertension Guidelines; NICE NG136
- Bradycardia Management · Resuscitation Council UK ABCDE; ESC 2021 Pacing Guidelines
- Ventricular Tachycardia / Fibrillation · Resuscitation Council UK ACLS; ESC 2022 Ventricular Arrhythmia Guidelines
Decision support only — verify against a current formulary, NICE, or your local guideline before clinical use.