Transthyretin Amyloidosis Cardiomyopathy in Patients With HFpEF in Russia

Inclusion Criteria:

for the retrospective phase are:

• Patients participating/participated in the PRIORITY-CHF study (may be alive ordeceased at the time of inclusion to this study).

• Established diagnosis of HFpEF (presence of typical signs and symptoms of HF (seeAppendix A) and LVEF ≥50%) at any time during participation in the PRIORITY-CHFstudy.

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-riskof 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 isabsent);

• Disproportion of QRS voltage to the degree of increased LV wall thickness onimaging methods

• Grade 2 or worse diastolic dysfunction

• Decreased longitudinal strain with apical sparing

• Diffuse subendocardial or transmural late gadolinium enhancement on cardiac MRIwith 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)

Exclusion Criteria:

Exclusion criteria for the retrospective phase are:

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

• Current participation in any interventional trial (i.e. at the time of inclusion tothis study).

The following criteria apply for exclusion of patients from the prospective part of the study:

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

• Patients with previous positive hematological test result on evaluatingAL-amyloidosis in the anamnesis (monoclonal protein is identified by SIFE/UIF) andthe 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.