RHEUMATIC HEART DISEASE:
FORGOTTEN BUT NOT GONE
AUTHOR Andrea Beaton, MD
GETTING TO THE HEART OF MEDICINE
‘Cause it’s better to be gone but not forgotten, than to be forgotten
but not gone.’ – Keith Palmer
SCHOOL SCREENING IN UGANDA
When I first met Michael, he was standing on the sidelines at his
school in Kampala, Uganda, watching his friends in a pick-up
soccer game. The ball was far from regulation – a wobbly sphere
created from plastic bags, strips of fabric and string – and the field
was merely a patch of red dirt worn from students walking back
and forth between the open-air classrooms. Yet, it was obvious
that he longed to play. His bright eyes watched the other children
intently; when he raised his arms to cheer a goal, he caught my eye
and flashed a brilliant wide smile, his “signature move.”
Michael’s P3 (third grade) class showed up that afternoon for
cardiac screening. About 100 boys were lined up in the dirt-floor
classroom we had set up as our makeshift screening room. The orange
extension cord to our echo machine, an ultrasound that takes
images of the heart, ran across the road to a small grocery stand
which was allowing us to borrow electricity. Screening was going
fairly well except when the occasional chicken got snagged, unplugging
the cord. As the boys removed their shirts, jostling each other
and breaking into spontaneous dance (which seemed to happen as
each class undressed), I noticed that Michael was exceedingly thin.
His gangly limbs and his prominent ribs contrasted starkly with his
friends’ healthy frames.
When it was Michael’s turn, he jumped up on the table and laid
down, flashing that signature smile. The echo machine showed the
typical black and white images, the left-sided heart valves opening
and closing. But the thick valves on the left side of Michael’s heart
weren’t closing well. Turning on the echo machine’s color flow confirmed
a severe leak at both valves. Michael had advanced rheumatic
heart disease (RHD).
UNANSWERED QUESTIONS ABOUT RHD
Michael was hardly alone. Between one and two of every 100 children
I scanned that fall showed signs of RHD. 1 RHD occurs when
children have frequent exposure to Group A streptococcal infection,
the common strep sore throat. However, when these infections occur
at high rates and go untreated or undertreated, an immune system
overreaction known as acute rheumatic fever can cause damage
to the heart valves. More than 80% of the world’s children live in
areas considered to be endemic for RHD. Current global estimates
put the burden of RHD at 39 million prevalent cases with at least
230,000 annual deaths. 2
Because RHD is most common in areas of the world that lack
well-developed health care systems, diagnosis is challenging. When
I met Michael, I was a second-year cardiology fellow conducting
research in Uganda. My goal was to validate a new study that had
emerged, showing echo screening could uncover a high burden of
RHD in an at-risk population. 3 The idea was revolutionary. It suggested
we could find children with RHD at the earliest stages and
perhaps prevent advanced RHD; which carried a nearly 20% risk of
death within one year of diagnosis. 4 Indeed, our research and that of
others contributed to echo screening for RHD being named a 2012
American Heart Association “Top 10 Advance in Cardiovascular
Disease and Stroke.” 5 But learning that we could identify latent RHD
in large numbers of children only raised more questions: Should
we be screening for RHD in low-resource communities, and if so
how can we make screening practical, affordable and sustainable?
The answer to the second question proved to be easier to address.
Over the next four years, our research team conducted several
additional studies in Uganda that showed echo screening using
abbreviated protocols, handheld echo equipment and short training
sessions could effectively enable screening to be task-shared with
non-physician providers. 6, 7 These findings were bolstered by complementary
research in the French Caribbean and Fiji, and several
studies touted echo screening as cost-effective. 8, 9
But what about the first question: should we be screening children
for early RHD? This question really has two parts. First, “Did
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