Our study shows the following findings: (1) RV reverse remodeling with an improvement in RV FW-GLS and RV-PA coupling was documented in 22.6% of patients during 1 year of treatment with tafamidis; (2) patients treated with tafamidis at an earlier stage of disease are more likely to experience RV reverse remodeling, as RV-ESV, NT-proBNP, and the degree of pulmonary hypertension were the main independent predictors of RV reverse remodeling. Here, NPPB is linked to pulmonary arterial hypertension.