Rationale, Objectives and Study Design of the Taiwan Registry of Hypertrophic Cardiomyopathy (THIC)

The Taiwan Registry of Hypertrophic Cardiomyopathy (THIC) is a multicenter national registry containing the clinical and imaging data of patients with hypertrophic cardiomyopathy (HCM) in Taiwan. The aim of the registry is to systematically evaluate the clinical, genetic and biochemical features, possible natural course, and outcomes of HCM and relevant rare diseases that mimic HCM such as Fabry disease (FD) and transthyretin amyloid cardiomyopathy in Taiwan, and to identify their specific "red-flag" signs, which are especially valuable from the perspective of unique genetic mutations or clinical manifestations in Taiwanese patients. Herein, we present the design and initial baseline data from the registry.

The THIC is an observational program that aims to collect prospective and/or retrospective data of patients with HCM in Taiwan. The registry plans to recruit 800 individuals with unexplained left ventricular hypertrophy, including 200 with FD, with a follow-up period of at least 12 months, and the project is expected to run for 5 years. Data on baseline characteristics, laboratory and imaging results, deaths, major adverse cardiovascular, cerebrovascular and renal events are collected.

The THIC has been in the enrollment phase since December 2022, and has enrolled 534 patients (age 62.37 ± 13.41 years, male 65.6%) as of March 15, 2025 from 13 centers. At enrollment, 284 of these patients had HCM, 227 had FD, and 23 had ATTR-CM. Family history was found to be an important diagnostic clue; however, common echocardiographic and laboratory data including N-terminal pro-brain natriuretic peptide were not significantly different between the three groups.

The THIC will contain comprehensive clinical and imaging data of patients with HCM, FD and ATTR-CM in Taiwan, and provide an opportunity to extend our knowledge on the clinical presentations and long-term consequences of these disease entities. It will aid in understanding patients with unexplained LVH in the context of the genetic background of Taiwanese patients, and in identifying predictors of LVH and important clinical events.