title: Evaluation of patients with cardiac amyloidosis using echocardiography, ECG and right heart catheterization.
abstract: OBJECTIVE
  To characterize patients with cardiac amyloidosis using echocardiography, electrocardiogram (ECG) and right heart catheterization (RHC).
  
  
  RESULTS
  Fourteen patients with biopsy verified light chain or transthyretin cardiac amyloidosis were included. All patients had heart failure with markedly elevated NT-proBNP. Echocardiography demonstrated biventricular hypertrophy, left atrial enlargement and normal to slightly reduced left ventricular ejection fraction. Tissue Doppler septal é was low and median E/é was high. Within 6 months RHC was performed in eight of the patients. The restrictive filling pattern demonstrated by echocardiography corresponded well to median pulmonary wedge pressure (21 mmHg). Systolic pulmonary artery pressure (SPAP) was increased, whereas cardiac output and stroke volume were seen to be decreased with both methods. ECG demonstrated: low voltage (36%), abnormal R-progression (65%), ST-T abnormalities (71%) and high incidence of fibrillation (36%). In addition, a case report following the treatment of melphalan and dexamethasone is presented with improvement of hypertrophy, SPAP, left ventricular mass and é.
  
  
  CONCLUSIONS
  These findings should lead to a suspicion of cardiac amyloidosis and suggest further investigation.
authors: Department of Medical Sciences: Clinical Physiology, Uppsala University, Uppsala, Sweden. Sven-Olof.Granstam@medsci.uu.se
authors: Sven-Olof
authors: SO
authors: Granstam
authors: Sara
authors: S
authors: Rosengren
authors: Ola
authors: O
authors: Vedin
authors: Tanja
authors: T
authors: Kero
authors: Jens
authors: J
authors: Sörensen
authors: Kristina
authors: K
authors: Carlson
authors: Frank A
authors: FA
authors: Flachskampf
authors: Gerhard
authors: G
authors: Wikström
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D028227:Amyloid Neuropathies, Familial / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology; D006328:Cardiac Catheterization; D006332:Cardiomegaly / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology; D003907:Dexamethasone / Q000494:pharmacology / Q000627:therapeutic use; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D006333:Heart Failure / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008297:Male; D008558:Melphalan / Q000494:pharmacology / Q000627:therapeutic use; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000032:analysis* / Q000378:metabolism; D010446:Peptide Fragments / Q000032:analysis* / Q000378:metabolism; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D013318:Stroke Volume / Q000187:drug effects; D016277:Ventricular Function, Left / Q000187:drug effects
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D003907:Dexamethasone; D008558:Melphalan