Cellular and Molecular Mechanisms Underlying Tricuspid Valve Development and Disease

Abstract

Tricuspid valve (TV) disease is highly prevalent in the general population. For ages considered “the forgotten valve” because of the predominant interest in left-side valve disease, the TV has now received significant attention in recent years, with significant improvement both in diagnosis and in management of tricuspid disease. TV is characterized by complex anatomy, physiology, and pathophysiology, in which the right ventricle plays a fundamental role. Comprehensive knowledge of molecular and cellular mechanisms underlying TV development, TV disease, and tricuspid regurgitation-related right-ventricle cardiomyopathy is necessary to enhance TV disease understanding to improve the ability to risk stratify TR patients, while also predicting valve dysfunction and/or response to tricuspid regurgitation treatment. Scientific efforts are still needed to eventually decipher the complete picture describing the etiopathogenesis of TV and TV-associated cardiomyopathy, and future advances to this aim may be achieved by combining emerging diagnostic imaging modalities with molecular and cellular studies. Overall, basic science studies could help to streamline a new coherent hypothesis underlying both the development of TV during embryogenesis and TV-associated disease and its complications in adult life, providing the conceptual basis for the ultimate and innovative field of valve repair and regeneration using tissue-engineered heart valves.

The mechanics of congenital heart disease: from a morphological trait to the functional echocardiographic evaluation

“…Ebstein's Anomaly (EA) is a rare CHD involving the tricuspid valve and the RV. It is caused by a failure in the delamination process during embryologic development which results in septal and posterior leaflets typically adherent to the underlying myocardium and in a dysplastic anterior leaflet, with varying degrees of redundancy and fenestration ( 58 ). In EA, the RV can be divided into two distinct regions ( 58 , 59 ).…”

“…It is caused by a failure in the delamination process during embryologic development which results in septal and posterior leaflets typically adherent to the underlying myocardium and in a dysplastic anterior leaflet, with varying degrees of redundancy and fenestration ( 58 ). In EA, the RV can be divided into two distinct regions ( 58 , 59 ). The inlet portion becomes in fact functionally integrated into the right atrium due to the TV malformation.…”

Complete Transposition of the Great Arteries in the Pediatric Field: A Multimodality Imaging Approach

“…Although the exact etiology and pathogenesis of TGA remains unknown [ 4 ], several theories have been postulated; the most accredited one involves an abnormal twisting of the pulmonary artery (PA) around the aorta during embryogenesis, with consequent misalignment of great vessels with the respective ventricular chamber [ 5 , 6 ]. An alternative theory suggests that an abnormal enlargement of the subaortic conus and resorption of the subpulmonary conus could be involved, resulting in the anterior and rightward positioning of the aorta in relation to the PA [ 7 ].…”