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|Number 09-06 Heterogeneous etiologies affecting a patient with cardiac amyloidosis|
Number 09-06 Heterogeneous etiologies affecting a patient with cardiac amyloidosis
Case from: Bastarrika G, Azcárate PM, Zudaire B, Barrio MT, Castaño S, Gavira JJ.
Institution: Departments of Radiology and Cardiology. Clínica Universidad de Navarra, Pamplona, Navarra, Spain
Clinical history: A 81 year-old male with history of atrial fibrillation, non-obstructive hypertrophic cardiomyopathy, moderate aortic stenosis and pulmonary hypertension by transthoracic echocardiography was admitted to the hospital with acute coronary syndrome. ECG at admission showed atrial Fibrillation, RBBB, and anterior Q waves with T wave inversion.
Movie 1 Movie 2a Movie 2b
Transthoracic echocardiography showed myocardial hypertrophy, septal akinesia, impaired left ventricular function, aortic stenosis and mild to moderate pericardial effusion (Movie 1, parasternal long axis). CMR was performed for diagnostic clarification.
Cine CMR: SSFP cine sequence demonstrated myocardial hypertrophy, impaired bi-ventricular function, septal akinesia, bi-atrial enlargement, moderate pericardial and bilateral pleural effusion (Movie 2a -two chamber view; Movie 2b -four chamber view).
Early gadolinium enhanced CMR: the images acquired in the early phase after gadolinium injection showed microvascular obstruction (black arrows) in the anteroseptum, in the region corresponding to the acute myocardial infarction (Figure 1, A two- and B four-chamber view).
Late gadolinium enhanced CMR: the images demonstrated a pattern of diffuse and circumferential myocardial enhancement (typical of cardiac amyloidosis) and a characteristic anteroseptal inner dark core suggestive of microvascular damage (white arrows) seen in acute coronary syndrome (Figure 2: short-axis views, A: horizontal long-axis, b:vertical long-axis).
In consideration of the CMR findings, the patient was brought to the cath lab. A LAD occlusion was identified and stented.
Angiogram after PCI of the occluded LAD
Perspective: The potential role of CMR is assessing ischemic heart disease and cardiac involvement in amyloidosis is well known (1, 2, 3). In this complex patient CMR was determinant for establishing the correct aetiology of LVH and indentifying a concurrent acute anterior myocardial infarction.
1. Kim RJ, Wu E, Rafael A, Chen EL, Parker MA, Simonetti O, Klocke FJ, Bonow RO, Judd RM. The use of contrast-enhanced magnetic resonance imaging to identify reversible myocardial dysfunction. N Engl J Med. 2000 Nov 16;343(20):1445-53.
2. Maceira AM, Prasad SK, Hawkins PN, Roughton M, Pennell DJ. Cardiovascular magnetic resonance and prognosis in cardiac amyloidosis. J Cardiovasc Magn Reson. 2008 Nov 25;10(1):54.
3. Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 2005 Jan 18;111(2):186-93.
COTW handling editor: Chiara Bucciarelli-Ducci