Echocardiography shows typical features in cardiac amyloidosis: concentric left (and sometimes right) ventricular wall thickening, infiltration of the atrial septum, dilation of the atria, often inceased echogenicity of the valves.
Echocardiography shows typical features in cardiac amyloidosis: concentric left (and sometimes right) ventricular wall thickening, infiltration of the atrial septum, dilation of the atria, often inceased echogenicity of the valves.Tags: Essay Time Management ArtCollege Board EssaysPoverty In The Third World EssayProblem Solving ProcessesVideo Violence Argument EssayWrite A Personal Essay On YourselfBad Essay Habit
Based on the clinical history and examination as well as the above investigations, the diagnosis was of congestive heart failure (functional class NYHA III) due to restrictive cardiomyopathy (CMR). Still frame from cine image in 4 chamber-view showing concentric LV hypertrophy, dilated left atrium and small pericardial effusion. Delayed sequences after gadolinium administration in short axis shows diffuse intramyocardial delayed enhancement.
The association between increased left ventricle wall thickness on ultrasound and low QRS voltage on ECG pointed towards a diagnosis of infiltrative cardiomyopathy, with a first line suspicion of cardiac amyloidosis beeing the most frequent cause of CMR. In order to obtain a histological confirmation of the disease, an abdominal fat aspirate was performed.
At presentation the physical exam shows good general status, a blood pressure of 100/60 mm Hg, without postural hypotension, normal lung examination, normal heart sounds, and bilateral lower extremities edema.
The ECG (Figure 1) shows sinus rhythm with low voltage in the limb leads, with a QS pattern in leads DII, DIII, a VF and no R wave progression in V1-V3.
The laboratory workup shows elevated NT-pro BNP (2800 pg/ml) and troponin I (0.079 ng/ml), the rest was unremarkable.
Renal function was normal, and there was no proteinuria.Cardiac involvment carries a worse prognosis than any other organ involvement3.Patients with cardiac amyloidosis present with signs and symptoms of heart failure, with progressive dyspnea, peripheral edema and angina, typical or atypical, due to involvement of the small vessels of the heart by the amyloid deposition. A recent medical evaluation for these symptoms identified important pleural effusion particularly on the left, a pleural tap evacuated 400 ml transsudate, and the cardiac ultrasound showed left ventricular hypertrophy. Iliescu” Institute of Emergency for Cardiovascular Diseases 2 Department of Hematology, Fundeni Clinical Institute 3 University of Medicine and Pharmacy “Carol Davila”, Bucharest We present the case of a 60 years-old man, presenting with progressive shortness of breath on exertion for the last 2 months.Our patient having no history of chronic inflammatory disease, secondary amyloidosis (deposition of serum amyloid A, an acute phase protein) was ruled out.Serum protein electrophoresis was normal, as well as the level of immunoglobulin; bone marrow biopsy showed only a slight increase of plasmocyte number (5%).The dermatological manifestations of the disease include easy bruising and periorbital purpura, the latter being pathognomonic for AL amyloidosis. Blood pressure is often low, due to decreased cardiac output in conjunction with early autonomic dysfunction, and in hypertensive patients there is frequently a history of normalization of blood pressure values with the onset of amyloidosis.Pleural effusions and hepatomegaly are frequent, both as a manifestation of congestive heart failure and as a sign of pleural or hepatic involvement of the disease3.The appearance is that of an infiltrative disease, of which amyloidosis is the most frequent cause.The left ventricular ejection fraction is normal or nearly normal until late in the disease.