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characteristic ECG pattern is one of QRS prolongation with T wave inversion in V1-V3. Epsilon waves
may also be present1. Monomorphic ventricular rhythm disturbances with left bundle branch block and
right-axis deviation, including sustained ventricular tachycardia, are commonly seen. An early sign may
be minor T wave changes in the right ventricular leads. Exercise-induced ventricular tachycardia and
SCD are common. A family history has an uncertain predictive value but early presentation (< age 20
years) is likely to be an adverse factor. Syncope is an adverse event but QT dispersion, Holter monitoring,
exercise ECG and programmed electrical stimulation are not reliable predictors of ventricular tachycardia.
Although right ventricular outflow tract tachycardia should prompt the search for dysplasia, isolated
ventricular premature beats with a right ventricular outflow tract pattern may be benign in young adults.
However, our ability to disentangle those with “innocent” (and, perforce, asymptomatic) ventricular
tachycardia from those with a potentially fatal outcome is not yet secure. For these reasons, associated
right ventricular dilation disbars from all forms of certification to fly.
CONGENITAL HEART DISEASE
Improvements in diagnostic and interventional techniques in the management of congenital heart disease
have led to the emergence of the specialty of "grown-up congenital heart disease" (GUCH). A patient
1 Epsilon waves are seen on the ST segments of leads V1 and V2 as small ‘crinkles’. They are best seen in
Fontaine leads (SI, SII, SIII in the parasternal position). They are not diagnostic of ARVC and may be seen in right
ventricular hypertrophy and sarcoidosis. They probably represent late potentials in the right ventricle.
ICAO Preliminary Unedited Version — October 2008 III-1-40
with such an anomaly on achieving adulthood naturally expects to lead as normal a life as possible which
includes carrying on employment and pursuing hobbies and pastimes, some of which will have defined
fitness requirements. These pursuits are not confined to aviation but include activities such as diving,
vocational driving, and motor-racing.
In general terms the principles applied to other cardiovascular problems are equally applicable to GUCH,
the defining requirement being that the risk of sudden or insidious incapacitation does not exceed that
appropriate to the age of the individual. As we learn more about the long-term outcomes of these
conditions, it is increasingly possible to make certificatory recommendations that are both safe and fair,
although an individual may not remain fit for a conventional career span. At present only those who have
a normal, or almost normal, event-free outlook with or without surgery can be considered. Many forms of
congenital heart disease are not consistent with flying status. Cardiological review with appropriate,
usually non-invasive, investigation and follow-up is mandatory in those accepted.
Atrial septal defect
Atrial septal defect is one of the most common congenital anomalies of the heart accounting for onequarter
of all. Three-quarters are ostium secundum defects, one-fifth are ostium primum defects and one
in 20 are sinus venosus defects.
The life expectancy with all but small (pulmonary/systemic flow ratio < 1.5 : 1) uncorrected secundum
defects is not normal with an increasing risk of atrial rhythm disturbances including flutter and fibrillation
from the fourth decade, and the eventual onset of right-sided heart failure in the sixth and seventh
decades. Early (age <24 years) closure of the defect carries a very low operative mortality and normal life
expectancy, but later closure is associated with a poorer outcome — increasingly poor as the age of
intervention rises — due to atrial fibrillation, thrombo-embolism and the onset of right heart failure. The
use of clam-shell and angel-wing devices is accepted and may encourage the closure of smaller defects
although long-term outcome data are not yet available.
Small or early-corrected ostium secundum defects are consistent with unrestricted certification, subject to
occasional review. Larger defects, or those complicated by atrial rhythm disturbance, may lead to
unfitness or restricted certification only.
Ostium primum defects present additional problems to those outlined above because the mitral valve and
conducting system may be involved. Such involvement significantly worsens the outcome.
Applicants with this condition can be considered only for restricted certification. Regular review is
required. Mitral regurgitation should be minimal and there should be no significant disturbance of
rhythm or conduction. Sinus venosus defects bear the problem that significant rhythm disturbances are
　
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