Int J Angiol 2022; 31(01): 040-047
DOI: 10.1055/s-0041-1735948
Original Article

Magnetic Resonance Imaging in Cardiac Amyloidosis: Unraveling the Stealth Entity

1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
,
Naseer Choh
1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
,
Tahleel Shera
1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
,
Faiz Shera
1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
,
Tariq Gojwari
1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
,
Feroze Shaheen
1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
,
Irfan Robbani
1   Department of Radiology, Sher-I-Kashmir Institute of Medical Sciences, Soura, Jammu and Kashmir, India
› Author Affiliations
Funding None.

Abstract

Amyloidosis is a systemic disease involving many organs. Cardiac involvement is a significant cause of morbidity and mortality in these patients. Diagnosis of cardiac amyloidosis is based on endomyocardial biopsy which however is invasive and associated with complications. Noninvasive methods of diagnosis include magnetic resonance imaging (MRI) with various methods and sequences involved. Our study aims at describing MRI features of cardiac amyloidosis including new imaging sequences and to prognosticate the patients based on imaging features. We included 35 patients with suspected cardiac amyloidosis who underwent MRI at our center over 4 years. All images were retrieved from our archive and assessed by an experienced radiologist. Common morphological features in our patients included increased wall thickness of left ventricle (LV) (16. 1 ± 4.1 mm), right ventricle (RV) (6.3 ± 1.1 mm), and interatrial septum (6.2 ± 0.8 mm). Global late gadolinium enhancement (LGE) (n = 21 [65%]) including subendocardial or transmural was the most common pattern followed by patchy enhancement. Global transmural LGE was associated with worse prognosis. Four types of myocardial nulling patterns were observed on postcontrast time to invert (TI) scout imaging: normal nulling pattern (myocardium nulls after blood and coincident with spleen) and abnormal nulling pattern (ANP) which is further divided into three types: Type 1—myocardium nulls before blood pool but coincident with spleen, Type 2—myocardium nulling coincident with blood but not coincident with spleen, and Type 3—features of both Type 1 and Type 2. Type 3 ANP was the most common (n = 23) nulling pattern in our patients. Cardiac MRI is an essential in noninvasive diagnosis of cardiac amyloidosis. Transmural global LGE serves as a poor prognosticator in these patients. “Three-tier” TI scout imaging is essential to avoid false-negative enhancement results. Type 3 ANP is the most specific nulling pattern in cardiac amyloidosis.

Ethics Approval and Consent to Participate

Our study was an observational study with no requirement for ethical clearance in our institution. The consent from the patients was however taken in all the cases.


Authors' Contribution

O.S.: Study design, data collection, statistical analysis, data interpretation, manuscript preparation, and literature search.


N.C.: Study design, data collection, data interpretation, and literature search.


T.S: Study design, data interpretation, and manuscript preparation.


F.S.: Data collection, statistical analysis, and manuscript preparation


T.G.: Study design, statistical analysis, and data interpretation.


F.S.: Data collection, data interpretation, and manuscript preparation.


I.R.: Study design.


Data Interpretation

All authors have read and approved the manuscript.




Publication History

Article published online:
21 September 2021

© 2021. International College of Angiology. This article is published by Thieme.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 
  • References

  • 1 Banypersad SM, Moon JC, Whelan C, Hawkins PN, Wechalekar AD. Updates in cardiac amyloidosis: a review. J Am Heart Assoc 2012; 1 (02) e000364
  • 2 Cotran SC, Kumar V, Collins T, Schmitt B. eds. Robbins Pathologic Basis of Disease. 6th ed.. Philadelphia, PA: Saunders; 1999
  • 3 Soler R, Rodríguez E, Remuiñán C, Bello MJ, Díaz A. Magnetic resonance imaging of primary cardiomyopathies. J Comput Assist Tomogr 2003; 27 (05) 724-734
  • 4 Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation 2003; 107 (19) 2446-2452
  • 5 Georgiades CS, Neyman EG, Fishman EK. Cross-sectional imaging of amyloidosis: an organ system-based approach. J Comput Assist Tomogr 2002; 26 (06) 1035-1041
  • 6 Hancock EW. Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis. Heart 2001; 86 (03) 343-349
  • 7 Ammash NM, Seward JB, Bailey KR, Edwards WD, Tajik AJ. Clinical profile and outcome of idiopathic restrictive cardiomyopathy. Circulation 2000; 101 (21) 2490-2496
  • 8 Papantoniou V, Valsamaki P, Kastritis S. et al. Imaging of cardiac amyloidosis by (99m)Tc-PYP scintigraphy. Hell J Nucl Med 2015; 18 (Suppl. 01) 42-50
  • 9 Mahalingam H, Chacko BR, Irodi A, Joseph E, Vimala LR, Thomson VS. Myocardial nulling pattern in cardiac amyloidosis on time of inversion scout magnetic resonance imaging sequence - a new observation of temporal variability. Indian J Radiol Imaging 2018; 28 (04) 427-432
  • 10 vanden Driesen RI, Slaughter RE, Strugnell WE. MR findings in cardiac amyloidosis. AJR Am J Roentgenol 2006; 186 (06) 1682-1685
  • 11 Ikeda S, Sekijima Y, Tojo K, Koyama J. Diagnostic value of abdominal wall fat pad biopsy in senile systemic amyloidosis. Amyloid 2011; 18 (04) 211-215
  • 12 Paulsson Rokke H, Sadat Gousheh N, Westermark P. et al. Abdominal fat pad biopsies exhibit good diagnostic accuracy in patients with suspected transthyretin amyloidosis. Orphanet J Rare Dis 2020; 15 (01) 278
  • 13 Cueto-Garcia L, Tajik AJ, Kyle RA. et al. Serial echocardiographic observations in patients with primary systemic amyloidosis: an introduction to the concept of early (asymptomatic) amyloid infiltration of the heart. Mayo Clin Proc 1984; 59 (09) 589-597
  • 14 Eriksson P, Backman C, Bjerle P, Eriksson A, Holm S, Olofsson BO. Non-invasive assessment of the presence and severity of cardiac amyloidosis. A study in familial amyloidosis with polyneuropathy by cross sectional echocardiography and technetium-99m pyrophosphate scintigraphy. Br Heart J 1984; 52 (03) 321-326
  • 15 Hongo M, Ikeda S. Echocardiographic assessment of the evolution of amyloid heart disease: a study with familial amyloid polyneuropathy. Circulation 1986; 73 (02) 249-256
  • 16 Cueto-Garcia L, Reeder GS, Kyle RA. et al. Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival. J Am Coll Cardiol 1985; 6 (04) 737-743
  • 17 Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1981; 63 (01) 188-196
  • 18 Phelan D, Collier P, Thavendiranathan P. et al. Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart 2012; 98 (19) 1442-1448
  • 19 Syed IS, Glockner JF, Feng D. et al. Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis. JACC Cardiovasc Imaging 2010; 3 (02) 155-164
  • 20 aus dem Siepen F, Kammerer R, Scherer KA. et al. Thickness of the midventricular left ventricular wall is predictive of survival in different forms of cardiac amyloidosis. J Cardiovasc Magn Reson 2013; 15 (Suppl. 01) 93
  • 21 Maceira AM, Joshi J, Prasad SK. et al. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2005; 111 (02) 186-193
  • 22 Fontana M, Pica S, Reant P. et al. Prognostic value of late gadolinium enhancement cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2015; 132 (16) 1570-1579
  • 23 Ojha V, Jagia P, Seth S, Ganga K. Diagnosis of cardiac amyloidosis based on temporal sequence of nulling on TI scout: a poor man's T1 map. J Am Coll Cardiol 2020; 75: 1764