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Cardiac Amyloidosis

Cardiac amyloidosis is a cardiomyopathy characterized by the extracellular deposition of  amyloid fibrils in the heart tissue. This leads to stiffening of the myocardium, impaired diastolic filling, and, eventually, heart failure. According to Braunwald’s Heart Disease, cardiac amyloidosis is defined as a condition where abnormal protein deposits disrupt normal cardiac structure and function, leading to restrictive cardiomyopathy.

Types of Cardiac Amyloidosis

AL (Light Chain) Amyloidosis

Caused by misfolded immunoglobulin light chains, often associated with plasma cell disorders like multiple myeloma.

ATTR Amyloidosis (Transthyretin Amyloidosis):

  • Hereditary (hATTR): Due to mutations in the transthyretin gene.
  • Wild-type (wtATTR): Occurs with aging, also known as senile systemic amyloidosis.

AA Amyloidosis

Rare in cardiac involvement, associated with chronic inflammatory diseases.

Causes of Cardiac Amyloidosis

  • Genetic Mutations: Inherited forms like hATTR.
  • Plasma Cell Dyscrasias: AL amyloidosis linked to abnormal plasma cell proliferation.
  • Aging: Wild-type ATTR amyloidosis occurs in elderly individuals without genetic mutations.
  • Chronic Inflammatory Conditions: Rarely leading to AA amyloidosis.

Clinical Features of Cardiac Amyloidosis

  • Fatigue and Weakness: Reduced cardiac output.
  • Shortness of Breath: Orthopnea
  • Edema: Swelling in the legs and abdomen due to fluid retention.
  • Arrhythmias: Atrial fibrillation, heart block, or sudden cardiac death.
  • Angina-like Chest Pain: Due to amyloid infiltration of coronary vessels.
  • Syncope: Resulting from arrhythmias or autonomic dysfunction.
  • Macroglossia: Enlarged tongue

Why Should You Choose A Cardiologist At Aster Hospitals?

Aster Hospitals emerges as a premier destination for cardiac amyloidosis care through its integrated, precision-driven approach combining diagnostics with multidisciplinary expertise. The hospital’s advanced cardiac imaging suite features 4D echocardiography, cardiac MRI with late gadolinium enhancement analysis, and nuclear medicine scans to detect amyloid deposits early, even in asymptomatic stages. These tools enable differentiation between AL and ATTR subtypes, critical for tailored treatment plans.

Why Choose a Cardiologist?

A cardiologist ensures:

  • Accurate differentiation between AL and ATTR amyloidosis
  • Timely initiation of disease-modifying treatments
  • Advanced diagnostic techniques for early detection
  • Personalized care plans for heart failure management
  • Long-term follow-up to monitor disease progression

FAQ's

Want to find out more about the treatment? The answer to your questions can be found below.

What are the early symptoms of cardiac amyloidosis?

Fatigue, shortness of breath, swelling in the legs, and irregular heartbeats are common early signs.

What is the treatment for cardiac amyloidosis?

Treatment includes managing heart failure symptoms, chemotherapy (for AL), and TTR stabilizers (for ATTR).

Is cardiac amyloidosis genetic?

Hereditary ATTR amyloidosis is genetic, while AL and wild-type ATTR are acquired forms.

How is cardiac amyloidosis diagnosed?

Diagnosis involves ECG, echocardiography, cardiac MRI, biopsy, and specialized blood tests.

Can cardiac amyloidosis be cured?

Early diagnosis and treatment can significantly improve quality of life and slow disease progression.

Why is follow-up important in cardiac amyloidosis?

Regular follow-up helps monitor heart function, adjust treatments, and manage complications.

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