Vol.:(0123456789) 1 3
The International Journal of Cardiovascular Imaging
https://doi.org/10.1007/s10554-020-01879-5
IMAGES IN CV APPLICATIONS
A heart with red fags
Cátia Costa Oliveira
1,2,3
· Vitor Hugo Pereira
1,2,3
· Carla Rodrigues
1
· Catarina Vieira
1
· Alberto Salgado
1
Received: 25 April 2020 / Accepted: 5 May 2020
© Springer Nature B.V. 2020
A 67 year old woman complaining of nausea, anorexia,
weight loss (8 pounds in 3 months), asthenia and dyspnea
for minor eforts was admitted for investigation. Her physical
examination revealed an arrhythmic heartbeat, without other
alterations. The ECG revealed atrial fbrillation and low volt-
age in limb and precordial leads. Transthoracic echocar-
diogram showed preserved systolic biventricular function
with impaired left ventricular (LV) diastolic function and
moderate pulmonary hypertension. A severe LV concentric
hypertrophy pattern more pronounced at the interventricular
septum (26 mm vs posterior wall 18 mm) and hypertrophy
of the free right ventricular wall (12 mm) were identifed
(Fig. 1a, b). Cardiac magnetic resonance confrmed the LV
hypertrophy with septal predominance (24 mm) associated
with late difuse subendocardial gadolinium enhancement
(LGE) and difculty at nulling the myocardium (Fig. 1c).
(99m)Tc-DPD scintigraphy was negative for amyloid tran-
sthyretin deposition. The patient underwent bone marrow
and salivary gland biopsies. Salivary gland biopsy con-
frmed the deposition of amyloid substance. Bone marrow
biopsy was consistent with multiple myeloma. Patient was
referred for hematology and started chemotherapy. She died
6 months later due to disease progression.
Cardiac amyloidosis (CA) is a rare condition with an esti-
mated prevalence of 8 to 12 per million. When left and right
ventricular myocardial thickening, valvular thickening, peri-
cardial efusion, diastolic dysfunction and a characteristic
pattern of global longitudinal strain (GLS) with apical spar-
ing are observed in echocardiographic studies, CA should
be suspected [1]. GLS pattern appreciated in CA is both
sensitive and specifc to identify and diferentiate CA from
other causes of LV hypertrophy [2]. The ‘speckled’ pattern
because of the higher echogenicity of amyloid protein than
the surrounding myocardial tissue is a typical feature of CA.
In CMR, a global subendocardial late gadolinium enhance-
ment with diferent contrast kinetics and a difculty in nul-
ling the myocardium (“nulling pattern”) are the most typical
fndings of CA [2]. This case remembers CA as a cause of
heart failure and its most relevant imaging fndings.
* Cátia Costa Oliveira
catiaandreiaoliveira@gmail.com
1
Cardiology Department, Hospital de Braga, Braga, Portugal
2
University of Minho, School of Medicine, Braga, Portugal
3
ICVS/3B’s-PT Government Associate Laboratory,
Braga/Guimarães, Portugal