Hereditary transthyretin amyloidosis (ATTRv) is a multisystem disorder caused by pathogenic variants in the TTR gene, increasingly recognized for its impact on cardiac function. This review explores the genetic underpinnings of ATTRv and their implications for clinical care, including the complexities of diagnosis, family screening, and management. The importance of genetic testing and variant interpretation is emphasized, alongside the critical role of genetic counseling in patient care. Current and emerging targeted therapies, as well as strategies for monitoring asymptomatic carriers, are discussed. By integrating genetic evaluation into clinical practice and fostering collaboration between geneticists and cardiologists, early diagnosis, personalized treatment, and the identification of at-risk individuals can be achieved, ultimately enhancing the management of ATTRv.