Isolated cardiac involvement in primary amyloidosis: presenting as sick sinus syndrome and heart failure

Area of Science:

• Cardiology
• Cardiovascular Medicine
• Cardiac Electrophysiology

Background:

• Cardiac amyloidosis is a severe infiltrative cardiomyopathy with poor prognosis.
• Clinical features often include restrictive cardiomyopathy, diastolic heart failure, and arrhythmias.
• Isolated cardiac involvement is rare, making diagnosis challenging.

Observation:

• A 76-year-old man presented with sick sinus syndrome requiring pacemaker implantation.
• He later developed heart failure symptoms, prompting further investigation.
• An endomyocardial biopsy confirmed primary cardiac amyloidosis.

Findings:

• The patient had isolated cardiac involvement by primary amyloidosis.
• Sick sinus syndrome was the initial manifestation, a rare presentation.
• Medical management led to symptomatic improvement.

Implications:

• This case underscores the importance of considering primary amyloidosis in elderly patients with unexplained cardiac conduction abnormalities and heart failure.
• Early diagnosis and treatment can improve outcomes in cardiac amyloidosis.
• Further research into the specific mechanisms and management of isolated cardiac amyloidosis is warranted.