CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 7, August 2012
AFRICA
e1
Case Report
Severe cardiac failure due to rapidly progressive
rheumatoid arthritis-associated valvulopathy
JASON M TARKIN, NEARCHOS HADJILOIZOU, HENRY OLUWASEFUNMI SAVAGE, SANJAY K PRASAD,
MARY N SHEPPARD, NEIL E MOAT, SAM KADDOURA
Abstract
Cardiac failure due to rapidly progressive valve disease is a
rare complication of rheumatoid arthritis (RA) that can be
challenging to manage. A patient with severe heart failure
secondary to RA who, after failing to respond to medical
therapy, underwent high-risk valve surgery and did remark-
ably well, with dramatic symptomatic improvement and
essentially normalised left ventricular size and function as
seen on follow-up echocardiography.
Keywords:
cardiac failure, rheumatoid arthritis, valvular heart
disease
Submitted 14/6/11, accepted 23/2/12
Cardiovasc J Afr
2012;
23
: e1–e3
DOI: 10.5830/CVJA-2012-012
Cardiovascular involvement is present in up to 70% of patients
with rheumatoid arthritis (RA) at autopsy.
1,2
Pericardial disease
is the commonest cardiac manifestation, occurring in 30 to 50%
of patients.
1-3
Less frequently, myocarditis, cardiomyopathy,
conduction defects, coronary artery vasculitis, aortitis and
valvular heart disease can occur.
1,2
In post-mortem studies,
RA-associated cardiomyopathy was found in three to 30% of
patients, and evidence of coronary artery vasculitis in up to
20%.
3
The risk of developing congestive heart failure is twice
as high in patients with RA, and higher rates of premature
coronary artery disease are also seen.
2,3
Mitral regurgitation
(MR) has been reported in 30 to 80% of patients with RA,
and aortic regurgitation (AR) in nine to 33%.
3
Pathological
mechanisms underlying cardiovascular manifestations in RA
are multifactorial and include chronic inflammation, fibrosis,
rheumatoid nodules and amyloid deposition.
Medical treatments for RA may improve cardiovascular
disease via their anti-inflammatory properties. Long-term
treatment with methotrexate has been associated with reduced
cardiovascular mortality, and disease-modifying anti-rheumatic
drugs may also reduce hospitalisation for congestive heart
failure in RA.
4
However, this benefit remains unclear and
several therapies, such as corticosteroids and non-steroidal anti-
inflammatory drugs can lead to increased cardiovascular risk.
4
Case report
A 52-year-old man from Ethiopia was admitted with worsening
heart failure despite maximum medical therapy. He had a five-
month history of worsening shortness of breath, orthopnoea
and decreased exercise tolerance (NYHA III–IV), with two
previous hospital admissions during this period. Past medical
history included pulmonary sarcoidosis that had been treated
with steroids and seropositive erosive RA with multiple joint
involvement and typical rheumatoid nodules on his elbows.
Current medications were perindopril 8 mg once daily,
furosemide 40 mg twice daily, spironolactone 25 mg once daily,
and long-term methotrexate therapy.
On examination, blood pressure was 111/64 mmHg and heart
rate 91 beats per min. Heart sounds I and II were audible, with
an early diastolic murmur and a loud pan-systolic murmur heard.
There was evidence of decompensated congestive cardiac failure
with raised jugular venous pressure, bi-basal coarse crackles and
marked peripheral oedema.
Electrocardiogram showed sinus rhythm with non-specific
T-wave changes. Chest radiograph showed pulmonary oedema,
bilateral pleural effusions and increased cardiothoracic ratio.
Echocardiography showed grossly dilated left and right
ventricles [LV end-diastolic diameter (LVEDD) 6.5 cm], with
severely impaired systolic function, bi-atrial dilatation (left
atrium 6.1 cm), and no evidence of LV hypertrophy. There was
severe, broad AR due to failure of co-aptation of the aortic valve
cusps (aortic root 2.5 cm), occupying almost the entire left
ventricular outflow tract with flow reversal in the descending
and abdominal aorta. Left ventricular ejection fraction was 35
to 45% in the context of severe AR. Severe, eccentric MR and
severe tricuspid regurgitation (TR) were also seen. Pulmonary
artery pressure was elevated at 70 mmHg + right atrial pressure.
All three valves appeared thickened (Figs 1A–D).
Chest computed tomography confirmed pulmonary oedema
with no evidence of parenchymal sarcoidosis. Coronary
angiography showed unobstructed coronary arteries and
catheterisation showed raised pulmonary artery pressures with
Chelsea and Westminster Hospital, Fulham Road; Royal
Brompton Hospital, Sydney Street; Imperial College School
of Medicine, London, UK
JASON M TARKIN, MB BS
NEARCHOS HADJILOIZOU, MB BS, MRCP
SAM KADDOURA, BSc (Hons), BM BCh, DIC, PhD, FRCP, FESC,
FACC,
Royal Brompton Hospital, Sydney Street; Imperial College
School of Medicine, London, UK
HENRY OLUWASEFUNMI SAVAGE, MB BS, MRCP
SANJAY K PRASAD, MD, FRCP
MARY N SHEPPARD, MD, FRC Path
NEIL E MOAT, MS, FRCS
