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clinical significance is unknown. The endocarditis, cor pulmonale or are insensitive and non-specific first-line
overwhelming majority of patients are constrictive pericarditis. investigations. Echocardiography allows
asymptomatic. However, the compact evaluation of both cardiac anatomy and
anatomy and relationship of the atrio- function, and may be helpful in several
Ischaemic heart disease
ventricular node to the aortic root and situations8,9,16:
interventricular septum make it Myocardial perfusion imaging under
vulnerable to damage from inflam- pharmacological stress detects IHD in
pericarditis (fluid and thickening)
mation of adjacent structures and can about 50% of RA patients, a prevalence imminent tamponade (diastolic
collapse)
lead to conduction defects. Complete double that of matched controls18. This is
heart block has been reported in RA reflected in the incidence of MI and heart constriction (preserved LV function
but abrupt termination of
and penicillamine-induced myositis12,13. failure as causes of cardiovascular mor-
ventricular filling)
tality in RA1. Alarmingly, more than half
Arteritis
of RA patients with IHD have no valvular lesions (grading of
ischaemic symptoms. Classical cardio- regurgitation and serial assessment
Arteritis is present in up to 20% of vascular risk factors appear to be of LV end-diastolic dimension)
autopsy cases, affecting mainly medium important, but RA, like diabetes, confers amyloidosis
and small intramyocardial arteries57. significant extra risk18. The long-term assessment of LV systolic and
This may lead to patchy myocardial significance of this is obvious, but diastolic function.
necrosis due to microinfarction or strategies to prevent it are yet to be In cases of constriction, computed
ischaemia. Severe arteritis of the epi- established. tomography (CT) scanning is useful to
cardial vessels has been reported and confirm pericardial thickening, and
tends to be non-occlusive. Its relation- helps to differentiate constrictive peri-
Investigation of cardiovascular
ship to myocardial infarction (MI) is carditis from restrictive cardiomyopathy
involvement in rheumatoid
controversial14,15. (Table 2). Cardiac catheterisation is
arthritis
essential if pericardectomy is considered.
Myocardial dysfunction Overall cardiovascular risk in RA, as in Exercise testing provides evidence of
other conditions, can be assessed on the ischaemia, but may be impossible or
Several processes operating alone or in basis of history, blood pressure, lipids and difficult due to physical disability. A
tandem may lead to myocardial dysfunc- ECG. A range of other investigations is useful alternative is nuclear perfusion
tion in RA. Heart failure may be one of also available to identify specific cardiac imaging under pharmacological stress.
the main causes of increased cardio- pathologies, assess their effects and allow This may show ischaemia, whether due
vascular mortality in RA, particularly targeted treatment. These should be used to epicardial or small vessel abnormali-
in men1. Diastolic LV dysfunction on judiciously, and they require collabora- ties, and inform the need for further
Echo-Doppler, found in 3040% of RA tion between rheumatologists and invasive investigation 18,19. Coronary
patients without overt heart disease16, cardiologists. angiography will reveal epicardial
is thought to be an early sign of IHD or ECG and chest X-ray are useful, but disease, but can neither differentiate
heart failure and has adverse prognostic
significance. Restriction due to amyloid
can lead to diastolic heart failure; in the Table 2. Differential diagnostic features between constrictive pericarditis and
past it was found in 1020% of rheuma- restrictive cardiomyopathy.
toid hearts, but is now rare. Pancarditis
and small vessel vasculitis can lead to Diagnostic feature Constrictive Restrictive
pericarditis cardiomyopathy
systolic pump failure17, while pulmonary
fibrosis can cause right ventricular S3 Gallop Absent May be present
failure. Overall, however, heart failure in Pericardial knock May be present Absent
RA, as in the general population, is more Palpable systolic apical impulse Absent May be present
likely to be the result of atherosclerotic Pulsus paradoxus May be present May be present
disease. Symptoms of myocardial dys- Pericardial calcification Present 50% Absent
function such as dyspnoea are unusual in CT scan, MRI,
RA, possibly due to reduced physical echocardiography Thickened pericardium Normal pericardium
activity, while signs are non-specific. Equal RV and LV
Patients suddenly developing overt heart diastolic filling pressures Usually present LV>RV
failure should be investigated for many Rate of LV filling 80% in first half of 40% in first half of
possible causes, including acute MI or diastole diastole
hypertensive heart disease, but also CT = computed tomography; MRI = magnetic resonance imaging; LV = left ventricle; RV = right ventricle.
vasculitis, valvular disease/bacterial
epiphyseal dysplasia
metaphyseal dysplasia Soft connective tissues
disorders
spondyloepiphyseal dysplasias, etc.
The presence of skeletal disproportion The heritable disorders of the soft con-
and its distribution can be useful nective tissues are best exemplified by the
clinically, for example: heterogeneous Ehlers-Danlos syndrome
(EDS), characterised broadly by exces-
rhizomelic short limbs in
sive skin elasticity, joint hypermobility
achondroplasia
and bruising, and the Marfan syndrome.
relatively short trunk in
spondyloepiphyseal dysplasia.
Ehlers-Danlos syndrome
Several distinct families can be recog-
nised within the skeletal dysplasias based Although 10 classic forms of EDS are
on the underlying genetic abnormalities. described, many patients cannot be