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cardiology

Grogan Staging System for Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

Mayo Clinic three-stage staging for wild-type transthyretin cardiac amyloidosis using NT-proBNP and troponin T (derived in 360 patients). Complements the Gillmore system, which uses eGFR.

Score interpretation

Stage I — 4-Year Survival 57 % 0

Stage I: NT-proBNP and troponin T both below the cut-offs (4-year survival 57 %).

→ Initiate tafamidis 61 mg OD (adults) if not already started; monitor for disease progression (6-monthly NT-proBNP, hs-TnT, echo, 6MWT); genetic testing for TTR variants (V122I common in Black/African heritage; V30M common in Portuguese/Swedish/Japanese); family screening if hereditary ATTR; echocardiographic surveillance: septal thickness, diastolic function, longitudinal strain; avoid drug toxicity (see gillmore_attr_cm for medication cautions). Children: use a children's formulary.

Stage II — 4-Year Survival 42 % 1

Stage II: one of NT-proBNP or troponin T above the cut-off (4-year survival 42 %).

→ Disease-modifying therapy (tafamidis) essential; symptomatic management: diuretics for congestion, anticoagulation if AF (NOACs -- data limited in ATTR-CM, digoxin caution due to amyloid binding); 3-monthly biomarker monitoring; echocardiography 6-monthly; ICD if high-risk arrhythmia or EF <= 35%; pacemaker if conduction disease (common in ATTR-CM); advance care planning introduction; cardiomyopathy centre review.

Stage III — 4-Year Survival 18 % 2

Stage III: NT-proBNP and troponin T both above the cut-offs (4-year survival 18 %).

→ End-stage management as per Gillmore Stage III; palliative care involvement; advance care planning; tafamidis continuation (may slow progression even in stage III); heart transplantation at select centres (discussion warranted); symptom control: aggressive diuresis, nutritional support; DNACPR discussion; family support; spiritual care; referral to specialist amyloid service (National Amyloidosis Centre or equivalent).

Interpretation bands for the Grogan ATTR-CM Staging. Apply clinical judgement and local guidance.

References

Related

Curated clinical cross-links plus same-class fallbacks.

Decision support only — verify against a current formulary, NICE, or your local guideline before clinical use.