Cardiac Amyloidosis: A Case Series from India.

OBJECTIVE

Cardiac amyloidosis (CA) is not well recognized in Indian literature. The aim of this communication is to highlight the difficulties in diagnosis.

METHODS

A retrospective analysis of data of six patients of CA diagnosed during 2008 to 2015 was done. Clinical, investigative, management and follow-up data is analyzed.

RESULTS

The mean age was 51 years with male preponderance. Heart failure was the commonest manifestation. Atrial arrhythmias were seen in 33%. Syncope, peripheral neuropathy and macroglossia were striking features. A speckled inter-ventricular septum (66%) or thickened inter-atrial septum (16%) on echocardiography strongly favoured CA. Other echocardiography features include thickened ventricular wall, enlarged atria and pericardial effusion (PE). Late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) was characteristic of CA. Plasma cell dyscrasia confirmed light chain amyloidosis. Abdominal fat pad and rectal biopsy confirmed the diagnosis. Chemotherapy for plasma cell dyscrasia was administered in 50% of patients.

CONCLUSION

Echocardiography and CMR imaging enable the diagnosis of CA in background of strong clinical suspicion. Abdominal fat pad biopsy is a simple and reliable method for confirming the diagnosis.

IMPLICATIONS AND PRACTICE: The awareness of this entity can enable an early diagnosis and improve the survival with timely novel chemotherapy.