Cardiovascular manifestations in inherited connective tissue disorders

Abstract

Heritable connective tissue disorders comprise a heterogeneous group of entities with widespread manifestations in most of them. Increased understanding of the structure and function of the connective tissue had led to the identification of many additional diseases over the last decade. The spectrum of disorders affecting the cardiovascular system has broadened significantly and also includes disorders with no- or very limited-systemic manifestations. Important clinical and genetic overlap between these different entities exists. In this chapter, we provide an overview of the most common cardiovascular manifestations occurring in the setting of HCTD and discuss the most relevant disease entities in this context.