RT Book, Section
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
SR Print(0)
ID 1202454401
T1 Restrictive Heart Diseases
T2 Fuster and Hurst's The Heart, 15e
YR 2022
FD 2022
PB McGraw-Hill Education
PP New York, NY
SN 9781264257560
LK accesscardiology.mhmedical.com/content.aspx?aid=1202454401
RD 2024/04/19
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.