Molecular and physiological characterization of RV remodeling in a murine model of pulmonary stenosis

摘要

jhe right ventricle is uniquely at risk in patients with complex congenital heart disease involving right-sided obstructive lesions (e.g., tetralogy of Fallot, tetralogy/pulmonary atresia) and inpatients with systemic right ventricles (1, 40, 46). Increased stress on the right ventricle in the form of increased hemodynamic loading (pressure and/or volume) may result in abnormalities in cardiac structure, function, metabolism, coronary perfusion, neurohormonal activation, and molecular signaling. These stresses lead to both adaptive and maladaptive structural and molecular remodeling of the right ventricle and deleterious effects on the left ventricle through ventricular-ventricular interaction and may limit long-term survival (3, 7, 29, 42). For many of these patients, detrimental conditions for the right ventricle exist throughout life, even after successful repair or Palliation. As surgical techniques for the primary repair of complex forms of congenital heart disease improve, long-term survival and quality of life will depend on our ability to preserve long-term right ventricular (RV) function. Decisionsregarding the ideal time for surgical reintervention will need to be based on more quantitative rather than qualitative measures.