Abstract
Infiltrative heart diseases are caused by a heterogeneous group of disorders; amyloidosis and sarcoidosis are two frequent causes of myocardial infiltration, which differ in clinical and biological outcome and treatment issues. The presence of high levels of angiotensin-converting enzyme (ACE) in a patient with infiltrative heart disease may increase suspicion of sarcoidosis. Nevertheless, no mention about increased ACE levels in extracerebral primary systemic amyloidosis is available. We present two cases of primary systemic amyloidosis, which are cardiac involvement and elevated ACE levels.
Highlights
Restrictive cardiomyopathies (RCMs) constitute a heterogeneous group of heart muscle conditions characterized by an increased stiffness of the myocardium that causes a decrease of ventricular filling and cardiac output
We report two patients with RCM, in whom high levels of plasma angiotensin-converting enzyme (ACE) were present; the final diagnosis in both cases was cardiac amyloidosis as a clinical manifestation of primary systemic amyloidosis associated with the overproduction of immunoglobulin light chains
Our two patients presented a suspected systemic infiltrative disease affecting the heart with signs, symptoms, and results of tests compatible with both amyloidosis and sarcoidosis
Summary
Restrictive cardiomyopathies (RCMs) constitute a heterogeneous group of heart muscle conditions characterized by an increased stiffness of the myocardium that causes a decrease of ventricular filling and cardiac output. Restrictive cardiomyopathy is diagnosed based on medical history, physical examination, and tests, such as blood tests, electrocardiogram, chest X-ray, echocardiography, and magnetic resonance imaging. There are some typical imaging characteristics of the various conditions that can affect the filling of the heart under the generic classification of restrictive cardiomyopathies, imaging findings are often unspecific and each condition has a spectrum of cardiac manifestations from a very early involvement with mild and subclinical disease to a very severe appearance with huge pathological findings. We report two patients with RCM, in whom high levels of plasma ACE were present; the final diagnosis in both cases was cardiac amyloidosis as a clinical manifestation of primary systemic amyloidosis associated with the overproduction of immunoglobulin light chains
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
