RAMB - Julho 2016

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Leal GN et al.
490 Rev Assoc Med Bras 2016; 62(6):490-493
AT THE BEDSIDE
What are the benefits of two-dimensional speckle tracking
echocardiography for diagnosis and treatment follow-up of
childhood-onset systemic lupus erythematosus myocarditis?
Gabriela Nunes Leal1*, Maria de F átima Diniz1, J uliana Brunelli2, Alessandro C. Lianza1, Adriana M. E. S allum2,
Clovis A. Silva2
1Echocardiography Service, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil
2Pediatric Rheumatology Unit, Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil
Study conducted at the Echocardiography Service and at Pediatric Rheumatology Unit, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina,
Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil
Article received: 6/29/2016
Accepted for publication: 7/26/2016
*Correspondence:
Address: Av. Dr. Enéas de Carvalho Aguiar, 647
São Paulo, SP – Brazil
Postal code: 05403-000
Financial support: Supported by grants from Conselho Nacional de Desenvolvimento Científico e Tecnológico
(CNPq 472155/2012-1 and 303422/2015-7 - 1A to CAS), Federico Foundation to CAS and by Núcleo de Apoio à Pesquisa
“Saúde da Criança e do Adolescente” / USP (NAP-CriAd) to CAS.
Case report
A 17-year-old white female had a previous diagnosis of
childhood-onset systemic lupus erythematosus (c-SLE)
based on American College of Rheumatology classification
criteria: arthritis, pericarditis, proteinuria > 0.5 g/day, and
the presence of antinuclear (ANA), double-stranded DNA
(ds-DNA) and antiphospholipid autoantibodies.1 She pre-
sented to the emergency room with a one-day history of
fever (38oC), shortness of breath, chest pain on inspiration,
and orthopnea. Regarding classic cardiovascular risk fac-
tors, there was no history of arterial hypertension or chron-
ic renal failure. Her lipid profile was considered normal for
age and sex and her body mass index was 27.5 kg/m2 (be-
tween 90th and 95th percentile). Previously to emergency
room admission, she had never shown signs or symptoms
of heart failure, and left ventricle (LV) ejection fraction (EF)
documented by a routine echocardiogram one month ear-
lier was 66% (normal ≥ 55%, by Teichholz method). There
was no chamber enlargement, ventricular hypertrophy or
signs of diastolic dysfunction. Despite that, a concomitant
deformation analysis of LV by two-dimensional speckle
tracking echocardiography (2DST) identified reduced lon-
gitudinal peak systolic strain in the apical 4-chamber view:
-15.2%; normal range -22.18%±3.06 (My Lab 60 – Esaote,
Florence, Italy).2 Of note, LV circumferential peak systolic
strain in the mid cavity was still preserved: -25.7% (normal
range -24%±6). 2 At that time, the disease activity param-
eter (SLE Disease Activity Index 2000 – SLEDAI-2K)3 was
18. At the emergency room, physical examination revealed
fever (38.5oC), tachypnea (respiratory rate of 40/min),
tachycardia (heart rate of 150/min), cool extremities, de-
layed capillary filling time (5 sec), arterial hypotension
(70 x 40 mmHg), and inspiratory crackles. Chest X-ray
showed slightly enlarged cardiac silhouette and pulmo-
nary edema. A bedside echocardiogram detected a small
pericardial effusion and reduced ejection fraction (52%),
with diffuse hypocontractility of the LV. C-reactive protein
was 190 mg/L (normal range < 5), erythrocyte sedimenta-
tion rate of 61 mm 1st/h (normal < 20), troponin 0.046 ng/
mL (normal < 0.014), and ds-DNA antibodies > 200 IU/mL
(normal < 20). Complement system components were low:
C4: 8.3 mg/dL (normal range 10-40) and C3: 71 mg/dL
(normal range 90-180 mg/dL). Both serum urea and cre-
atinine were slightly elevated (urea 41 mg/dL; normal range
11-38.5 mg/dL and creatinine 1.02 mg/dL; normal range
0.5-0.9 mg/dL). Hemoglobin was 7.2 g/dL, leukocytes count
was 5,880/mm,3 and platelets count was 408 K/µL. Urinary
casts were present. Blood and urine cultures were negative.
At admission, SLEDAI-2K was 20. A lupus flare with myo-
carditis was diagnosed and she was promptly sent to pedi-
atric intensive care unit (ICU), where oxygen supply, ino-
tropic support (milrinone) and endovenous diuretics were