TY  - CHAP
M1  - Book, Section
TI  - Amyloid Heart Disease
A1  - Sher, Taimur
A1  - Gertz, Morie A.
A2  - Baliga, R. R.
A2  - Abraham, William T.
Y1  - 2018
N1  - 
T2  - Color Atlas and Synopsis of Heart Failure
AB  - JS,  a  45-year-old  Caucasian  man,  was  referred  to  the  Mayo  Clinic  in  consultation  for  progressive  congestive  heart  failure  (CHF)  of  unclear  etiology.  A  year  prior  to  his  symptoms,  he  had  been  a  healthy  and  active  person  playing  tennis  4  times  a  week  and  leading  a  very  busy  life  as  a  farmer  in  the  Midwest  and  as  a  father  of  3  young  children  with  no  medical  problems  except  for  well-controlled  essential  hypertension  (HTN).  Six  to  eight  months  prior  to  presentation  he  experienced  a  decline  in  exercise  capacity  and  gradually  gave  up  tennis.  Three  months  prior  to  presentation  he  developed  dyspnea  on  moderate  exertion  and  intermittent  swelling  of  his  legs.  Medical  evaluation  included  normal  renal  function,  blood  counts,  and  pulmonary  computed  tomography  (CT)  angiogram.  Nuclear  medicine  cardiac  stress  test  was  normal;  pulmonary  function  tests  indicated  a  mild  reversible  obstructive  defect  and  2D  echocardiogram  revealed  mildly  thickened  left  ventricle  with  an  ejection  fraction  of  60%  and  borderline  diastolic  dysfunction.  Treatment  with  bronchodilators  was  empirically  started  for  a  provisional  diagnosis  of  atypical  asthma.  His  symptoms  progressed  rather  rapidly  over  the  course  of  2  to  3  weeks  and  he  underwent  a  coronary  angiogram,  which  demonstrated  normal  coronary  arteries.  At  his  evaluation  at  Mayo  Clinic  he  was  found  to  be  in  New  York  Heart  Association  (NYHA)  class  III  CHF.  Based  on  the  review  of  his  electrocardiograph  (ECG)  and  echocardiogram  a  diagnosis  of  infiltrative  cardiomyopathy  was  suspected  and  further  work  demonstrated  an  immunoglobulin  lambda  light  chain  gammopathy  in  the  serum  with  8%  lambda  restricted  plasma  cells  in  the  bone  marrow.  Congo  red  staining  of  the  bone  marrow  and  abdominal  fat  aspirate  was  positive  for  extracellular  amorphous  material  that  demonstrated  apple  green  birefringence  under  polarized  microscope.  He  was  diagnosed  with  immunoglobulin  lambda  light  chain  amyloidosis  (AL)  with  stage  IV  cardiac  disease.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/23
UR  - accesscardiology.mhmedical.com/content.aspx?aid=1161016199
ER  -