Transthyretin Amyloidosis Cardiomyopathy in Patients With HFpEF in Russia

Prior treatment

• Patients with previously established (and documented) ATTR-CM.

Other illness

• Any severe condition that, in opinion of the physician, will limit patient's life span to 12 months or less from the inclusion to this study.

Lab values

• Patients with previous positive hematological test result on evaluating AL-amyloidosis in the anamnesis (monoclonal protein is identified by SIFE/UIF) and the serum FLC ratio (kappa/lambda) is in outside of the normal range).
  • If the hematological test on evaluating AL amyloidosis is negative, or there is no such test performed in the patient's medical history, the patient may be enrolled to this study.

Other

• Current participation in any interventional trial (i.e. at the time of inclusion to this study).
  • The following criteria apply for exclusion of patients from the prospective part of the study:

• for the retrospective phase are:
• Patients participating/participated in the PRIORITY-CHF study (may be alive or deceased at the time of inclusion to this study).
• Established diagnosis of HFpEF (presence of typical signs and symptoms of HF (see Appendix A) and LVEF ≥50%) at any time during participation in the PRIORITY-CHF study.
  • The following criteria apply for inclusion of patients into the prospective part of the study:
• Provided written informed consent for the prospective phase of the study.
• Presence of left ventricular wall thickness >12 mm.
• Presence of at least three of "red flags" or additional signs for having high-risk of ATTR-CM (evaluation based on medical history of HF and concomitant diseases, family history, results of previous ECG, Echo-CG/cardiac MRI, laboratory data):
  • >65 age (during participation in the PRIORITY-CHF study)
  • Conduction system disease (atrioventricular block, left bundle branch block, long QRS complex, sick sinus syndrome)/pacemaker
  • Atrial fibrillation
  • Pseudoinfarct pattern on the ECG (when hemodynamically significant stenosis is absent);
  • Disproportion of QRS voltage to the degree of increased LV wall thickness on imaging methods
  • Grade 2 or worse diastolic dysfunction
  • Decreased longitudinal strain with apical sparing
  • Diffuse subendocardial or transmural late gadolinium enhancement on cardiac MRI with increased extracellular volume fraction
  • Persistent low-level troponin elevation
  • Elevated NT-proBNP level
  • Right ventricular (RV) wall thickness more than 6 mm
  • Pericardial effusion in patients with LV-hypertrophy
  • Restrictive pattern in patients with LV-hypertrophy
  • Atrial enlargement with the normal ventricular volumes
  • Bilateral carpal tunnel syndrome
  • Lumbar/cervical spinal stenosis
  • Spontaneous biceps tendon rupture
  • Hip or knee replacement
  • Peripheral neuropathy
  • Family history of neuropathy
  • Intolerance/poor tolerance to vasodilating antihypertensive medications
  • Orthostatic hypotension
  • Gastroparesis
  • Urinary incontinence (neurogenic bladder), recurrent urinary infections,
  • Erectile dysfunction
  • Other autonomic dysfunction, such as gastrointestinal motility disorders (constipation, early satiety, chronic diarrhea, nausea/vomiting), syncope, anhidrosis)