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frequent both before and after correction. These need to be excluded before certification can be
considered. Life-long periodic ambulatory ECG monitoring is required.
Ventricular septal defect
Isolated ventricular septal defect accounts for about one-third of congenital heart defects. Small
(pulmonary/systemic flow ratio < 1.5 : 1) defects either close spontaneously or remain stable lifelong.
There is no increased risk of sudden or insidious incapacitation, although there is a small risk of
endocarditis, and appropriate measures should be taken for its prophylaxis. Such candidates may be fit for
unrestricted certification. Closure in childhood likewise carries a good outcome –five percent mortality at
25 years, but larger defects that have undergone closure do not appear to have a normal life expectancy
with an 82 per cent 30-year survival compared with 97 per cent in age-matched controls. Age at surgery
ICAO Preliminary Unedited Version — October 2008 III-1-41
and the presence of pulmonary vascular change are predictors of survival. Applicants with such defects
should undergo full cardiological review.
Pulmonary stenosis
Pulmonary valvar stenosis accounts for one in ten subjects with congenital heart disease. Stenosis of the
infundibulum of the right ventricle and of the supravalvar region are much less common. The former may
be present as a fibromuscular ring or as concentric hypertrophy in an otherwise normal heart with an
intact interventricular septum. Valvar stenosis may also be present. Supravalvar stenosis may be
associated with multiple stenoses of the pulmonary trunk and its branches.
Mild degrees of pulmonary valvar stenosis (peak gradient < 30 mmHg across the valve — tricuspid
valve Doppler velocity < 2.5 m/s) are consistent with unrestricted certification. Following surgery, 25-
year survival is 95 per cent — not quite normal — but discretion may be exercised in “best-risk”
subjects, judged by non-invasive and invasive means. Supra-valvar stenosis should normally disbar from
all forms of certification to fly.
Aortic stenosis
Aortic stenosis has been reviewed above. Congenital abnormalities of the aortic valve or the aortic
outflow tract requiring surgery in childhood carry a relatively poor prognosis, the 25-year mortality being
17 per cent. Nevertheless, in one small study there were no late deaths in the 16-year period following
resection of isolated discrete subaortic stenosis. This condition is normally incompatible with certification
to fly.
Coarctation of the aorta
Coarctation of the aorta may be diagnosed in childhood or the diagnosis may be delayed until later years.
In terms of outcome the difference is significant. In about one-third of patients a bicuspid aortic valve will
also be present. Early intervention is important. The 20-year survival of patients aged 14 years or younger
at the time of operation was 91 per cent compared with an 84 per cent survival of those in whom surgery
was delayed. The best outcome was in those operated on under the age of nine years. Age at operation
predicted subsequent hypertension, which was also associated with an increased risk of sudden death,
myocardial infarction, stroke and aortic dissection.
Unrestricted certification can be considered in normotensive subjects who underwent correction of the
anomaly below the age of 12 to 14 years. Continuous subsequent review is required to monitor the blood
pressure. Echocardiographic follow-up should be determined by the presence or absence of a bicuspid
aortic valve. Ascending aortic dilation is not compatible with certification. Treated hypertension
following late closure may be compatible with restricted certification.
Tetralogy of Fallot1
The tetralogy of Fallot is classically the only cyanotic congenital heart condition that is consistent with
survival into adult life if uncorrected. Such survivors do not have a normal life expectancy and late
1 Tetralogy of Fallot: ventricular septal defect, pulmonary stenosis (which protects the pulmonary circulation),
overriding aorta, and right ventricular hypertrophy. After Étienne-Louis Arthur Fallot, French physician (1850-
1911).
ICAO Preliminary Unedited Version — October 2008 III-1-42
closure (> 12 years) carries a less favourable outlook than early closure. In one study, the 32-year
actuarial survival was 86 per cent overall compared with 96 per cent for an age- and sex-matched control
population; for patients operated on before the age of 12-years, the figure was 92 per cent — still not
normal. An increased frequency of complex rhythm disturbances has been noted as has a higher than
expected incidence of late SCD. The former do not appear to predict the latter reliably. In one study, the
　
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