Abstract Background and Aims Studies of sodium-glucose co-transporter 2 inhibitors (SGLT2i) therapy on ATTR cardiomyopathy are scarce and focus wild-type and V122I. In ATTRv, namely V30M, SGLT2i introduction to preserve kidney function or improve cardiorenal syndrome is not an outlined practice. Our aim was to assess the clinical evolution and difficulties with SGLT2i therapy in ATTR V30M amyloidosis, a population with neurogenic bladder, dysautonomia and prone to dehydration. Methods Patients with ATTR V30M amyloidosis and criteria for SGLT2i started standard dosages. Gillmore score (GS) for ATTR cardiomyopathy, kidney failure risk equation (KFRE) and the renal system score (RSS) of the FASTEX model (Fabry disease) were calculated. Estimation of heart amyloid load was based on Tc-99-DPD scintigraphy. Results Eight patients were followed on median of 7 months [IQR 5.6-9.4]; 7 treated with dapagliflozin, 1 with empagliflozin. 6 had type-2 diabetes. 4 were treated with tafamidis, 1 inotersen and 1 patisiran. 3/7 had positive DPD scintigraphy. Heart failure decompensation, new arrhythmias and renal adverse events were absent. Neurogenic bladder was present in 4 patients, 2 had urinary tract infections. 1 had symptomatic hypotension, leading to discontinuation of therapy. EGFR and RSS improved in 5 and 3 patients, respectively; NT-proBNP/albuminuria/proteinuria and KFRE at 2 and 5 years decreased. In terms of cardiomyopathy, 1 patient improved to GSI; 2 using CKD-EPI creatinine and none using CKD-EPI cystatin C. 1 progressed to GSII in all equations. Conclusion In this pilot study, we evidenced that ATTR V30M patients meet the criteria for SGLT2i therapy. Dysautonomia and neurogenic bladder were not an impediment for therapy. In addition to disease modifying drugs, SGLT2i may improve kidney and cardiovascular outcomes in ATTR V30M amyloidosis.