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cardiological follow-up with regular echocardiography and Holter monitoring is required.
The cause of death in dilated cardiomyopathy may be divided more or less equally into those perishing
from pump failure and those suffering a sudden arrhythmic event. The presence of high-grade ventricular
rhythm disturbances is both common and predictive of outcome.
In view of the generally poor prognosis, the diagnosis of dilated cardiomyopathy is inconsistent with any
form of certification to fly. Mild global reduction in left ventricular systolic function (with the ejection
fraction > 50 per cent) that has been stable for a period of at least one year and with no evidence of
electrical instability may be considered for restricted certification, subject to close follow-up with
echocardiography and Holter monitoring.
Sarcoidosis
Sarcoidosis presents special problems in certification due to its ubiquity and its occasional involvement of
the heart. It is commonly a self-limiting condition seen in young adults with the extent of systemic
involvement being largely unknown. There is often no significant systemic illness and presentation may
be fortuitous with bilateral hilar lymphadenopathy on routine chest X-ray. Or there may be erythema
nodosum, malaise, arthralgia, iridocyclitis, respiratory symptoms or other constitutional upset. In those
with systemic involvement, five percent will also have cardiac involvement. Its aetiology is not
understood, but a genetically determined sensitivity to pine pollen or an infective agent may be involved.
Involvement of the heart is associated with a poor prognosis and a significant risk of sudden death; half of
those diagnosed with the condition die from the disease. Cardiac involvement may exist without
concomitant involvement of other systems. Sudden death may be due to life threatening ventricular
rhythm disturbance or granulomatous involvement of the conducting system. Dilation of the ventricles
due to patchy involvement of the myocardium may lead to the development of a dilated or restrictive
cardiomyopathy.
There are no characteristic ECG features although Holter monitoring may be premonitory of rhythm and
conduction disturbance. Echocardiography may show patchy or generalized hypokinesia, especially if the
basal myocardium is affected, with ventricular dilation and reduction of the ejection fraction. Deposits
thicker than 3mm may be detected non-invasively. Multiple Gated Acquisition (MUGA) and thallium
MPI are inconclusive but magnetic resonance imaging (MRI) scanning may demonstrate localized highintensity
lesions with gadolinium enhancement. Raised plasma angiotensin-converting enzyme (ACE)
activity is not diagnostic but may give an indication of active disease. A scalene node biopsy will confirm
ICAO Preliminary Unedited Version — October 2008 III-1-39
systemic sarcoidosis if present but myocardial biopsy is often unhelpful due to the patchy nature of the
disease.
The diagnosis of sarcoidosis (sometimes by way of the chance discovery of bilateral hilar
lymphadenopathy) requires that the pilot should be made unfit. Satisfactory evaluation for restricted
Class I certification should attempt to establish that the disease is inactive and include:
• no increase in hilar lymphadenopathy on serial chest radiography
• stable gas transfer factor
• no evidence of active disease elsewhere (including scalene node biopsy)
• normal resting and exercise ECG (to at least nine minutes of the Bruce protocol)
• no significant rhythm or conduction disturbance on Holter monitoring
• normal echocardiogram.
Trans-oesophageal echocardiography and/or MRI scanning will be required in the event of possible
myocardial abnormality.
Restricted certification may be permitted subject to six-monthly cardiological follow-up for at least two
years. Minimum re-investigation should include echocardiography and Holter monitoring. Full
certification may be considered no sooner than two years after the initial observation, subject to regular
follow-up. Any evidence of systemic involvement (except erythema nodosum) requires permanent
restriction to multi-crew operation. Evidence of involvement of the heart disbars for all licences.
Right ventricular cardiomyopathy
Right ventricular cardiomyopathy (previously arrhythmogenic right ventricular dysplasia (ARVC)) is
characterized by dilation of the right ventricle with regional or global replacement of the myocardium
with fibro-fatty tissue. It may also involve the left ventricle.
It may account for up to 25 per cent of sudden cardiac death (SCD) in young adults and is transmitted as
an autosomal dominant gene with incomplete penetrance in at least 30 per cent of those affected. The
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Manual of Civil Aviation Medicine 1(98)
