[Chronic heart failure in patients with cardiac amyloidosis]

Area of Science:

• Cardiology
• Pathology
• Internal Medicine

Background:

• Amyloidosis involves amyloid protein deposition in tissues, frequently causing cardiac disorders and chronic heart failure (CCF).
• AL-amyloidosis is a primary form of amyloidosis, often leading to cardiac complications and CCF, characterized by its rarity, severity, and poor prognosis without specific treatment.

Purpose of the Study:

• To examine the cardiohemodynamics (CHD) and clinical course of CCF in patients with cardiac amyloidosis (CA).
• To understand the characteristics and outcomes of CCF in the context of CA due to its rarity and severity.

Main Methods:

• Analysis of 12 patients diagnosed with cardiac amyloidosis and clinical manifestations of CCF.
• Diagnosis verification through autopsy (5 patients), biopsies (gingival, rectal, pleural; 4 patients), and clinical/instrumental findings (3 patients).
• Assessment of cardiohemodynamics, clinical course, patient longevity, and pre-CCF conditions (renal disorders, weight loss).

Main Results:

• All 12 patients with CCF and CA died during the study period.
• Mean longevity since CCF onset was significantly shorter for patients over 70 years old (5.9 months vs. 28 months).
• 83.3% of CA patients experienced renal disorders and weight loss prior to CCF; 83.3% showed restrictive cardiomyopathy and 16.7% dilatational cardiomyopathy, with thickened myocardial walls.

Conclusions:

• CCF associated with CA presents with severely disturbed hemodynamics and resistance to standard therapies.
• Cardiac amyloidosis leads to significant myocardial thickening, predominantly manifesting as restrictive cardiomyopathy.
• The findings underscore the critical need for specific therapeutic strategies for cardiac amyloidosis to improve outcomes in CCF patients.