Primary Systemic Amyloidosis and High Levels of Angiotensin-Converting Enzyme: Two Case Reports

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. 1. Introduction 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. The majority of restrictive cardiomyopathies are secondary to a systemic disorder such as amyloidosis, sarcoidosis, scleroderma, haemochromatosis, eosinophilic heart disease, malignancy infiltration, or as a result of radiation treatment [1]. The symptoms of heart failure are dyspnoea and low exercise tolerance. In the physical examination, leg oedemas, ascites, hepatomegaly, and increased central venous pressure can be present. The changes in electrocardiogram are often unspecific. Diastolic dysfunction with preserved systolic function is often the only echocardiographic abnormality that may be noted. 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. Although 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. Echocardiography is a multimodality imaging technique that, when used appropriately and expertly in its full capacity, allows for the comprehensive description of cardiac involvement. Further imaging techniques should be adapted to the perceived clinical suspicion: in the majority of cases, cardiac magnetic resonance imaging will be performed to confirm and describe cardiomyopathies. In rare occasions, the definitive diagnosis of the etiology needs invasive endomyocardial biopsy, due to recent developments in imaging techniques and