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Table 4 Assessments for monitoring progression in cardiac dysfunction

From: Expert opinion on monitoring symptomatic hereditary transthyretin-mediated amyloidosis and assessment of disease progression

Technique

Indicator of progression

Frequency of assessment

Sensitivity to progressiona

Clinical examination

Progression indicated by:

3–6 months

High

 New signs and symptoms of CHF

 Unplanned cardiac hospitalization

Uncontrolled heart failure that would request to increase the diuretic dosage or the need of using intravenous diuretics

6MWT

If no disabling neuropathy, progression indicated by a decrease of 20–30 m

6 months

High

Check heart rate response during 6MWT for chronotropic incompetence

12-lead ECG

New bundle branch block or AV block of any degree

6 months

High/medium

New microvoltage or pseudo myocardial infarction pattern; new arrhythmias (atrial and ventricular, atrial fibrillation, bradycardia, AV block)

Holter ECG

New arrhythmias, burden of atrial fibrillation, need for pacing, VT/VF. If new syncope: repeat Holter for sinus dysfunction, atrial fibrillation, atrial or ventricular arrhythmias, and consider EPS

1 year

High

EPS

Asymptomatic conduction abnormalities (left or right bundle branch block and/or prolonged PR interval)

When clinically indicated based on clinical or ECG changes

Medium

New conduction abnormalities or indication for pacemaker or defibrillator implantation according to existing guidelines or clinical situation

Pacemaker memory

Check for bouts of asymptomatic atrial fibrillation requiring anticoagulation

6 months

Medium

Check for worsening of AV block degree if device has a function for preservation of physiologic AV conduction information

Echocardiographyb

Myocardial thickness and regional LV strain measurement mandatory. Doppler filling parameters, EF. Strain measurements

1 year

Medium

Progression indicated by:

 Increased myocardial thickness (wall thickness 2 mm increase with other symptoms/findings)

 Decreased basal strain

 Worsening diastolic dysfunction

 Decrease in EF

Same operator should be used for consecutive assessments

Cardiac magnetic resonance imagingb

Changes noted in the report; T1, ECV, wall thickness, EF

1 year when clinically indicated by ambiguous echo changes

High

Scintigraphy with bone tracersb

PYP or DPD cardiac uptake using qualitative Perugini grading 1–3, quantification using H/L ratio. Repeat scan only if initial scan was negative, and if > 3 years, and echo shows significant increase in wall thickness

3 years

High

Do not repeat once scan is positive

Cardiac staging system [70, 78]

Persistent change in the patients’ Grogan or Gillmore stage

6 months

Medium

Cardiac biomarkers: NT-proBNP, troponin I, troponin T

Progression indicated by trend increase

3–6 months

High

  1. 6MWT 6-min walk test, AV atrioventricular, CHF chronic heart failure, DPD 99mTc-3,3-diphosphono-1,2-propanodicarboxylicacid, ECG electrocardiogram, ECV extracellular volume, EF ejection fraction, EPS electrophysiologic study, H/L heart-to-lung, LV left ventricular, NT-proBNP N-terminal prohormone of brain-type natriuretic peptide, PYP 99mTc-pyrophosphate, T1 longitudinal relaxation time, VF ventricular fibrillation, VT ventricular tachycardia
  2. aIn the authors’ clinical experience
  3. bCardiac imaging should be performed at different visits by the same operator or radiologist, on the same machine, using the same software