Technetium-99m Pyrophosphate Scintigraphic Finding in

Cardiac Amyloidosis: A Case Report

Pei-Ying Hsu1,2, Yen-Wen Wu2,3, Lung-Chun Lin3, Su-Rong Hung2, Kai-Yuan Tzen2,
Ruoh-Fang Yen2
1

Division of Nuclear Medicine, National Taiwan University Hospital Yun-Lin Branch, Douliou City, Taiwan
2
Department of Nuclear Medicine, National Taiwan University Hospital and
National Taiwan University College of Medicine, Taipei, Taiwan
3
Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital and
National Taiwan University College of Medicine, Taipei, Taiwan

Amyloidosis is a rare systemic disorder caused by

extracellular deposition of insoluble fibrils in a wide
spectrum of tissues and organs. Among the variety of
affected organs, cardiac involvement contributes to the
majority of death. Echocardiography and technetium99m pyrophosphate (99mTc-PYP) scintigraphy are the
common non-invasive imaging modalities in the evaluation of cardiac amyloidosis. In this case report, we present a 65-year-old man with symptoms and signs of
congestive heart failure. Cardiac amyloidosis was suspected from his family history. Echocardiography
revealed diffuse myocardial hypertrophy and hyperechoic granular sparkling texture in the myocardium.
99m
99m

Tc-PYP scintigraphy showed diffuse and intense

Tc-PYP uptake in bilateral ventricular myocardium.

The pathological result from a biopsy of subcutaneous

fat from the chest wall later confirmed the diagnosis.
Key words: amyloidosis, heart, 99mTc-PYP, scintigraphy

Ann Nucl Med Sci 2009;22:115-119

Received 12/23/2008; revised 1/6/2009; accepted 2/10/2009.

For correspondence and reprints contact: Ruoh-Fang Yen, M.D., Department of
Nuclear Medicine, National Taiwan University Hospital. 7 Chung-Shan South
Road, Taipei 100, Taiwan. Tel: (886)2-23123456 ext. 65581, Fax: (886)223957855, E-mail: rfyen@ntu.edu.tw

Introduction
Amyloidosis, a rare systemic disorder caused by extracellular deposition of insoluble fibrils in a wide spectrum of
tissues and organs, results in various clinical manifestations
[1,2] one of which is cardiac involvement, a devastating
process with significant morbidity and mortality [1,2].
For the detection of cardiac amyloidosis, many noninvasive tests such as echocardiography, CT, MRI and technetium-99m pyrophosphate ( 99mTc-PYP) scintigraphy are
available, although definitive diagnosis lies in tissue biopsy
with special stain [3-5]. 99mTc-PYP is a bone seeking agent
and has been observed to induce uptake in tissue with amyloid deposition. The precise mechanism of amyloid affinity
for 99mTc-PYP is not understood. One commonly accepted
proposal for this mechanism is that the increase of calcium
content in the amyloid deposition tissue leads to the increase
of 99mTc-PYP uptake [6].
In this report, we present a case with suspicious cardiac
amyloidosis and with 99mTc-PYP scintigraphy revealing significant uptake in the myocardium. Amyloidosis for this case
was later confirmed by subcutaneous fat biopsy of the chest
wall.
Case Report
A 65-year-old male patient had suffered from progressive shortness of breath and leg edema for four months.
Paroxysmal nocturnal dyspnea, orthopnea and dizziness were
noted as well. This patient had already received endocardial


Hsu PY et al

biopsy at Tri-Service General Hospital with negative Congo

chest wall. The pathological result showed amorphorous

red stain in pathology. The patients younger brother had also

eosinophilic deposition in the perivascular region and posi-

experienced congestive heart failure, which was suspected to

tive apple green birefringence in Congo red staining from

be amyloidosis related but with inconclusive evidence from

polarized microscope. Amyloidosis was then confirmed. The

endocardial biopsy.

patient was thus treated symptomatically for heart failure

The physical examination for this patient gave the

result of low blood pressure (82/60 mmHg) and pedal

only, and discharged afterwards upon improvement of general condition.

edema. Hemogram and biochemistry, including cardiac

enzymes, liver function and renal function, were all within
normal ranges. Chest X ray showed normal heart size. 24-h
Holter revealed sinus rhythm with 1st degree atrioventricular
block. Sick sinus syndrome was noted during hospitalization
and pacemaker was placed. Echocardiography revealed fair
ejection fraction (55%), moderate mitral regurgitation, diffuse myocardial hypertrophy and hyperechoic granular
sparkling texture in the myocardium (Figure 1). But other
diagnostic workups of serum and urine immunofixation electrophoresis for possible disorder associated with amyloidosis
were all negative.
99m

Tc-PYP scintigraphy was then arranged and the

resulting images taken at 3 h after injection of 925 MBq (25

mCi) 99mTc-PYP showed diffuse intense tracer uptake (greater
than sternum) in the myocardium of both right and left ventricles. This result further enhanced the possibility of cardiac
amyloidosis and led to a subcutaneous fat biopsy from the

Figure 1. Echocardiography shows typical granular

sparkling texture in the hypertrophic ventricular myocardium, especially in the interventricular and interatrial septums.

Ann Nucl Med Sci 2009;22:115-119

116

Discussion
Amyloidosis is caused by deposition of amyloid fibrils
in multiple organs, and can be classified according to the
protein precursors [1,2]. Each type has different probabilities
of cardiac involvement, extracardiac manifestations and
median survival. It is also suggested that amyloidosis may be
hereditary. About one third of the patients with amyloidosis
are known to have overt heart disease [3].
Amyloid infiltration to the heart may interrupt contractile function and lead to mild diastolic dysfunction at the
beginning stage of the disease and then to deteriorating
restrictive cardiomyopathy and diastolic heart failure (rightside predominant). In addition, coronary blood flow is likely
to be hindered due to microvascular involvement with amyloid so that patients may experience anginal chest pain.
Furthermore, chance of arrhythmia is increased because
amyloid infiltration may interrupt the conduction of heart
[2].
Histological result from biopsy is generally used for the
final verdict in diagnosing amyloidosis. However, the biopsy
procedure bares the risk of bleeding from vascular integrity
disruption in amyloid-laden tissue, and sometimes insufficiently sampling of myocardial biopsy occurs and leads to
false negative result [7]. The subcutaneous abdominal fat
aspirate is a safe and sensitive procedure for the confirmation
of amyloidosis [4]. But, it lacks the important function of
providing extent of the disease.
Echocardiography is a non-invasive modality for diagnosing cardiac amyloidosis. The amyloid deposits in the ventricular myocardium may show granular sparkling texture in
echocardiography to signal the occurrence of cardiac amyloidosis [1,3,4]. Other echocardiographical readings such as
increased ventricular mass, biartrial dilatation, and thickening of the interventricular/interatrial septum and valve

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June 2009

99mTc-PYP
Tc-PYP imaging on cardiac amyloidosis

99m

Figure 2. 99mTc-PYP scintigraphic images taken at 3 h after injection of 925 MBq (25 mCi) 99mTc-PYP show diffuse intense
99m
Tc-PYP uptake (4+) in the myocardium of bilateral ventricles. (A) Planar images of anterior and left anterior oblique 60
degrees (LAO 60) views. (B) SPECT images of axial, coronal and sagittal views.

leaflets are typical findings of cardiac amyloidosis [1,3].

In radionuclide scintigraphy,

99m

Tc-PYP is bound to cal-

activity equal in density to the sternum; and 4+: uptake activity greater in intensity than the sternum [9]. Diffuse and
99m

cium phosphate complex known to accumulate in severely

intense uptake of

damaged myocardial cells [3,4]. Usually, image acquisition

to be attributed to the involvement of cardiac amyloidosis

is performed at 2 to 4 h after tracer injection for avoiding

[3,4,8,10].

false positive interpretation of tracer activity in the blood

pool early after tracer administration [8]. According to
Holman classification of visual assessment of

99m

99m

Tc-PYP (3+ or 4+) in the heart is likely

Tc-PYP scintigraphy used in conjunction with

echocardiography is able to provide noninvasive evidence of

Tc-PYP

cardiac amyloidosis for the suspected patients. Furthermore,

uptake in the myocardium, the intensity of myocardial tracer

the uptake maps in extra-cardiac organs such as liver, spleen

activity is graded as 1+: minimal or equivocal uptake; 2+:

or thyroid gland constructed from whole-body

definite uptake but intensity less than sternum; 3+: uptake

scintigraphy should also conveniently provide valuable

2009;22:115-119

99m

Tc-PYP

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117


Hsu PY et al

informations regarding the localization and extent of amyloi-

polyneuropathy. Am Heart J 1987;113:654-662.

6. Pepys MB, Dyck RF, de Beer FC, Skinner M, Cohen AS.

dosis infiltration [5,11].

Binding of serum amyloid P-component (SAP) by amy-

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99mTc-PYP
Tc-PYP imaging on cardiac amyloidosis

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1007 (02)-23123456 65581 : (02)-23957855 : rfyen@ntu.edu.tw

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