Right Ventricle Outflow Obstruction in Biventricular Hypertrophic Cardiomyopathy in Amyloidosis

Abstract

Light-chain (AL) amyloidosis is the most common type of amyloidosis; cardiac involvement is rare but has a poor prognosis. Biventricular hypertrophic cardiomyopathy is an exceptional finding in amyloidosis and its association with obstructive right ventricular gradient is even rarer. We report the case of a male patient with biventricular hypertrophy suggesting amyloidosis, with an obstructive gradient in the right ventricle. LEARNING POINTS• Consider amyloidosis in a patient presenting with signs of congestive heart failure and low or microvoltage on ECG.• It is important to examine right ventricle myocardium thickness, as hypertrophy not only occurs in the left ventricle, and look for a right ventricle outflow tract gradient.• Analysis of speckle tracking of the myocardium will show deterioration of the basal and medium segments with normal apical segment motion. KEYWORDSBiventricular hypertrophic cardiomyopathy, right ventricle outflow tract, amyloidosis, low voltage INTRODUCTION Biventricular hypertrophic cardiomyopathy (HCM) is an uncommon condition that is caused in most cases by systemic infiltrative disease. Its association with an obstructive right ventricular gradient is even rarer. We report the case of a male patient with biventricular hypertrophy suggesting amyloidosis with an obstructive gradient in the right ventricle.

Role of Cardiac Magnetic Resonance in Detecting Biventricular Apical Hypertrophic Cardiomyopathy

“…First-line therapy is an AV nodal blocking agent. Those who remain symptomatic can be offered resection of accessory RV muscles in the outflow tract [ 4 , 5 ]. Despite being the most sensitive and accurate imaging modality for BiApHCM, C-MRI has not been widely obtained due to cost-related limitations [ 6 ].…”