The Journal of the Arkansas Medical Society Issue 11 Vol 114 | Page 18

SCIENTIFIC ARTICLE
Rheumatic Heart Disease in Marshallese Youth
in Northwest Arkansas: A Case Series
Hannah Baer, MD 1 ; Rupal Bhakta, MD 1,2
UAMS, Department of Pediatrics
1
Arkansas Children’s Hospital, Divisions of Cardiology and Critical Care Medicine
2
Abbreviations
RHD, rheumatic heart disease; ARF,
acute rheumatic fever; AR, Arkansas
Abstract
Rheumatic heart disease (RHD) is the
leading cause of pediatric acquired car-
diac disease worldwide. It is now rare in
developed countries, but still prevalent in
lower income countries with some of the
highest rates in Pacific Island nations. The
Marshallese population in northwest Ar-
kansas has significant risk factors for RHD
coupled with financial and cultural barriers
to health care access.
This paper describes the clinical
course of three Marshallese adolescents
with RHD, all of whom presented in criti-
cal condition and developed heart failure
requiring surgery. We review their risk fac-
tors and discuss ways to identify children
earlier in the disease course.
Introduction
R
heumatic heart disease (RHD)
is the leading cause of pediat-
ric acquired cardiac disease. It
is a long-term sequela of acute rheumatic fever
(ARF), a multi-system inflammatory disease fol-
lowing group A streptococcal pharyngitis. ARF is
diagnosed using the Jones criteria summarized in
Table 1. 1 Between 40-80% of children with ARF
have cardiac involvement during the initial illness.
Children with recurrent ARF or with moderate to
severe valve disease on initial presentation can
progress to chronic RHD. World Heart Federation
echocardiographic criteria for RHD are summa-
rized in Table 2. 2 immigrating, with lower socioeconomic status,
living conditions, and educational achievement
compared to the state average. 6,7
Of the estimated 336,000 cases of ARF and
282,000 cases of RHD each year, 95% occur in
less developed countries. In developed countries,
immigrants and indigenous minority groups are
disproportionately affected. 3,4 In our institution,
we recently treated three adolescent patients
with RHD. All presented in critical condition re-
quiring surgical intervention, and all were of Mar-
shallese origin. Cases
Historical Background
Patient 1. A 15-year-old Marshallese boy
presented with fever and arthralgia, progressing
to tachypnea, orthopnea, and chest pain. Exam in-
cluded bounding pulses, jugular venous distention,
pericardial friction rub, and respiratory distress.
Echocardiogram showed a large pericardial effu-
sion, moderate mitral insufficiency with a normal-
appearing valve, and mild-moderate aortic valve
insufficiency. Relevant laboratory studies are pre-
sented in Table 3. He was diagnosed with ARF with
carditis, requiring drainage of the pericardial effu-
sion. He completed a course of penicillin and was
discharged with furosemide, lisinopril, and aspirin.
Follow-up was arranged with monthly penicillin in-
jections for secondary prophylaxis. He was subse-
After WWII, the United States administered
the Marshall Islands as part of the UN Trust Terri-
tory of the Pacific Islands. Several atolls were used
as nuclear testing sites from 1946-1958. In 1982,
the U.S. government and the new Republic of the
Marshall Islands signed the
Compact of Free Association,
Table 1. Revised Jones Criteria for Diagnosis of
which provides compensa-
Initial ARF (1)
tion for claims related to the
effects of the nuclear test- Diagnosis requires laboratory evidence of recent streptococcal
infection, plus 2 major criteria or 1 major and 2 minor criteria.
ing and allows Marshallese
citizens to lawfully reside, Major criteria
work, and study in the United • Carditis
States without a visa. 5 Since • Polyarthritis
then, Marshallese have been
• Chorea
immigrating to northwest
Arkansas, drawn by jobs in • Erythem marginatum
the poultry industry and the • Subcutaneous nodules
relatively low cost of living. Minor criteria
An estimated 4,000+ Mar- • Polyarthralgia
shallese live in Arkansas, an
• Fever > 38.5ºC
increase of 250% from 2000-
2010. Sixty-two percent were • Peak ESR>60 mm/hr and/pr CRP greater than upper limit of
normal for laboratory
born overseas. Many face
significant challenges after • Prolonged PR interval, if carditis is not a major criterion
258 • THE JOURNAL OF THE ARKANSAS MEDICAL SOCIETY
VOLUME 114