Carmelo Anfuso

MRI of Cardiac Involvement in Transthyretin Familial Amyloid Polyneuropathy

OBJECTIVE The purpose of this study was to evaluate cardiac MRI features in a group of patients with transthyretin familial amyloid polyneuropathy (FAP). SUBJECTS AND METHODS Sixteen patients with transthyretin FAP underwent 2D echocardiography with Doppler examination, cardiac MRI, and (99m)Tc-diphosphonate (DPD) scintigraphy. Four patients had peripheral polyneuropathy, three had carpal tunnel syndrome, one patient had symptoms and signs of heart failure, and eight patients had no symptoms but had a family history of FAP. At MRI, cardiac function parameters and delayed contrast enhancement findings were evaluated. RESULTS Six patients had cardiac radiotracer uptake at scintigraphy (FAP cardiac group), and 10 patients had no cardiac uptake (FAP noncardiac group). The FAP cardiac group included the four patients with peripheral neuropathy, one patient with carpal tunnel syndrome, and the only patient with heart failure. At MRI, abnormal contrast enhancement was found in all patients with positive scintigraphic findings and in no patient with negative scintigraphic findings. All patients had involvement of the left ventricle and other chambers or structures (atria, right ventricle, tricuspid valve leaflets). Left ventricular contrast enhancement was focal in four patients, subendocardial circumferential in one patient, and diffuse in one patient. The only patient with signs of heart failure had circumferential subendocardial enhancement. CONCLUSION Cardiac contrast-enhanced MRI can be used to identify cardiac amyloidosis in patients with FAP who do not have clinical signs of heart involvement. In these patients, the typical subendocardial circumferential pattern of contrast enhancement is rare. We observed unusual enhancement patterns as focal or diffuse left ventricular enhancement accompanied by enhancement of the atria, tricuspid valve, or right ventricle.

Simultaneous recognition of myocardial, pleural and pulmonary parenchyma inflammation by cardiac magnetic resonance.

Magnetic resonance imaging (MRI) showed high accuracy in the diagnosis of many inflammatory cardiac diseases. We report a case of a patient where cardiac MRI was able to identify inflammation of myocardium, pleura and pulmonary parenchyma.

Left ventricular pseudodiverticulum.

The differential diagnosis between left ventricular aneurysm and diverticulum remains a matter of debate. Cardiac magnetic resonance is dramatically helpful in the anatomical and functional characterization of the walls of any angiographical left ventricular outpouching.

Lipomatous metaplasia in ischemic cardiomyopathy.

Lipomatous metaplasia is characterized by substitution of muscle fibers with adipose tissue in the context of a myocardial scar. Cardiac magnetic resonance is an accurate cardiac imaging technique with high tissue spatial resolution that can easily characterize scar tissue, adipose tissue and lipomatous metaplasia. We report a case of lipomatous metaplasia in a 68-year-old man with a 14-year history of anterior myocardial infarction admitted to our cardiac department for dyspnea and palpitations.

An integrated imaging approach with magnetic resonance imaging can reveal pitfall pleural effusion at echocardiography.

We report an echocardiographic pitfall, where a hypoecogenic area mimicking a pleural effusion was detected by CMR as an area of normal pulmonary parenchyma between the posterior left atrial wall and the dilated right pulmonary vein.