TY - CHAP M1 - Book, Section TI - Restrictive Heart Diseases A1 - Arbustini, Eloisa A1 - Di Toro, Alessandro A1 - Giuliani, Lorenzo A1 - Dec, G. William A1 - Narula, Jagat A2 - Fuster, Valentin A2 - Narula, Jagat A2 - Vaishnava, Prashant A2 - Leon, Martin B. A2 - Callans, David J. A2 - Rumsfeld, John S. A2 - Poppas, Athena Y1 - 2022 N1 - T2 - Fuster and Hurst's The Heart, 15e AB - Chapter SummaryThis chapter provides an overview of restrictive heart diseases, conditions characterized by diastolic dysfunction in the presence of elevated left ventricular filling pressures and limited increase in volume. The diastolic dysfunction may be observed in myocardial (restrictive cardiomyopathy [RCM] and phenocopies), endocardial/endomyocardial (fibrosis, elastosis), and pericardial (constriction, effusion) diseases (see Fuster and Hurst’s Central Illustration). Primary RCMs are rare myocardial diseases and are defined by restrictive ventricular physiology in the presence of normal or reduced diastolic volumes of one or both ventricles, normal or reduced systolic volumes, and normal ventricular wall thickness, with significant atrial dilation. They carry a poor prognosis. Familial RCM are usually caused by mutations in genes encoding for sarcomeric proteins and intermediate filaments. Restrictive endomyocardial diseases include endomyocardial fibrosis of right, left, or both ventricles, frequently with involvement of valves, Hedinger syndrome of the right-sided heart valves in patients with neuroendocrine tumors and carcinoid syndrome, as well as endocardial fibroelastosis associated with congenital heart anomalies. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/15 UR - accesscardiology.mhmedical.com/content.aspx?aid=1202454401 ER -