Quantification of Extracellular Matrix Expansion by CMR in Infiltrative Heart Disease

Mongeon, François‐Pierre; Jerosch‐Herold, Michael; Coelho‐Filho, Otávio R.; Blankstein, Ron; Falk, Rodney H.; Kwong, Raymond Y.

doi:10.1016/j.jcmg.2012.04.006

Cited by 125 publications

(74 citation statements)

References 28 publications

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“…41 Recent data have shown promising role for equilibrium contrast CMR for quantification of extracellular volume fraction as a more robust method that correlates with amyloid burden, and hence quantify it objectively. 42,43 More recently, alteration of T2 kinetics with decreased T2 myocardium/skeletal muscle ratio, has been described in cardiac amyloidosis, in part due to pronounced local field inhomogeneity. 44 None of the CMR techniques, however, can differentiate subtypes of cardiac amyloidosis.…”

Section: Echocardiographymentioning

confidence: 99%

Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

AlJaroudi

Desai

Tang

et al. 2014

Journal of Nuclear Cardiology

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Amyloidosis is an infiltrative disease characterized by deposition of amyloid fibrils within the extracellular tissue of one or multiple organs. Involvement of the heart, cardiac amyloidosis, is recognized as a common cause of restrictive cardiomyopathy and heart failure. The two major types of cardiac amyloidosis are cardiac amyloid light-chain (AL) and transthyretin-related cardiac amyloidosis (ATTR, mutant and wild types) (Nat Rev Cardiol 2010;7:398-408). While early recognition of cardiac amyloidosis is of major clinical importance, so is the ability to differentiate between subtypes. Indeed, both prognosis and therapeutic options vary drastically depending on the subtype. While endomyocardial biopsy with immunostaining is considered the gold standard, advances in imaging provide an attractive non-invasive alternative. Currently, electrocardiography, echocardiography, and cardiac magnetic resonance imaging are all used in the evaluation of cardiac amyloidosis with varying diagnostic and prognostic accuracy. Yet, none of these modalities can effectively differentiate the cardiac amyloid subtypes. Recent data with 99m Tc-phosphate derivatives, previously used as bone seeking radioactive tracers, have shown promising results; these radiotracers selectively bind ATTR, but not AL subtype, and can differentiate subtypes with high diagnostic accuracy. This review will initially present the non-radionuclide imaging techniques and then focus on the radionuclide imaging techniques, particularly 99m Tc-DPD and 99m Tc-PYP, mechanism of action, performance and interpretation of the study, diagnostic accuracy, prognostic value, future clinical perspective, and outlook.

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Section: Echocardiographymentioning

confidence: 99%

Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

AlJaroudi

Desai

Tang

et al. 2014

Journal of Nuclear Cardiology

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“…3 Noncontrast T1 times >1090 ms and extracellular volume fraction >40% have been shown to be highly specific for cardiac amyloidosis. 3,4 In our patient, the noncontrast T1 time was 1207 ms, and the extracellular volume fraction was 42%. …”

Section: Discussionmentioning

confidence: 66%

Left Ventricular Hypertrophy in a Runner

et al. 2014

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A 63-year-old male runner presented for outpatient cardiology evaluation of decreased exercise tolerance and increased left ventricular (LV) wall thickness noted on an echocardiogram obtained by his primary care provider. The patient had been an avid runner since a young age; however, over the previous months he had noted decreased exercise tolerance prompting evaluation by his primary physician. His medical history was only significant for hypertension that had been well controlled with a single medication. ECG revealed sinus bradycardia with markedly elevated voltage in the anterolateral precordial leads (Figure 1). Echocardiography with strain imaging demonstrated increased LV wall thickness measuring 1.8 cm at the basal septum and 1.7 cm at the basal inferolateral wall (Figure 2A). Ejection fraction was noted to be normal; however, longitudinal strain imaging was markedly abnormal throughout the septum, extending into adjacent regions of the anterior and inferior walls and normalizing in the lateral wall and apex (Figure 2B and 2C).Differential diagnosis at this time included hypertrophic cardiomyopathy, cardiac amyloidosis, Fabry disease, LV hypertrophy attributed to longstanding hypertension, and athlete's heart, as he was an avid runner. Athlete's heart generally does not reach wall thicknesses >15 mm, and his hypertension had been well controlled; thus, hypertrophic cardiomyopathy was thought to be the most likely diagnosis. A cardiac magnetic resonance image subsequently demonstrated LV thickening most prominent throughout the septum that spared the middle and apical lateral walls ( Figure 2D). Late gadolinium enhancement (LGE) was seen throughout the thickened segments ( Figure 2E and 2F, black arrows) corresponding with areas of abnormal longitudinal strain seen on echocardiography. Precontrast and postcontrast T1 mapping demonstrated abnormal precontrast (1207 ms) and postcontrast (345 ms) T1 times, resulting in an extracellular volume percentage of 42% ( Figure 2G and 2H). The pattern of LGE and T1 mapping was most consistent with an infiltrative process prompting endomyocardial biopsy. The biopsy specimen stained positive for Thioflavin S under UV microscopy ( Figure 3A, white arrows), indicating amyloid deposits and that immunohistochemical staining for transthyretin was positive ( Figure 3B, black arrows). Genetic testing failed to identify a heritable mutation on the transthyretin (TTR) gene; thus diagnosis of wild-type TTR cardiac amyloidosis was made. DiscussionThis case demonstrates an atypical presentation of TTR cardiac amyloidosis with many findings that, although described, are not typical in cardiac amyloidosis, as well as the additive diagnostic support that multimodality imaging can provide. Classically, patients with cardiac amyloidosis have low voltage on ECG, although our patient had normal limb lead voltages with increased voltage in V4/V5. Interestingly, 1 series of patients with TTR amyloidosis found that 25% actually had electrocardiograms that met LV hypertrophy criteria. 1Echo...

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“…Although LGE remains an excellent tool for detecting focal myocardial fibrosis, detection of diffuse fibrosis (Fig. 4) remains challenging since the normal myocardium is used as a reference to highlight patchy areas of focal myocardial fibrosis [36]. Extracellular volume (ECV) quantification by T1 mapping with CMR has overcome this limitation of LGE for detecting diffuse fibrosis in HCM.…”

Section: The Role Of T1 Mapping For Detection Of Diffuse Myocardial Fmentioning

confidence: 99%

Cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy: Current state of the art

2016

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Quantification of Extracellular Matrix Expansion by CMR in Infiltrative Heart Disease

Cited by 125 publications

References 28 publications

Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

Left Ventricular Hypertrophy in a Runner

Cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy: Current state of the art

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