Cardiovascular Journal of Africa: Vol 27 No 3 (May/June 2016)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 27, No 3, May/June 2016 AFRICA e11
The total surgical time was 150 min; fluoroscopy time was 45
min. Pulmonary-to-systemic flow ratio (Qp/Qs) dropped from 2
to 1.2 and pulmonary arterial pressure decreased from 70/41 (50)
mmHg to 45/14 (24) mmHg.
After intervention, the patient improved clinically and was
treated with asprin and frusemide. Three months later he was
asymptomatic, and he was clinically well at the 12-month
follow-up assessment. The indicators of right cardiac chamber
and pulmonary arterial pressure estimated by echocardiography
were normal.
Discussion
Scimitar syndrome has a wide complex of anomalies, including
drainage of all or part of the right lung to the inferior caval
vein, hypoplasia of the right lung, pulmonary hypertension,
dextroposition of the heart and anomalous systemic arterial
supply.9 The anomaly was first described by Cooper in London
in 1836 during autopsy of an infant. 4
The aetiology of scimitar syndrome is not clear. Clinical
presentation of patients with scimitar syndrome depend on
the degree of pulmonary arterial hypertension, which is often
secondary to left-to-right shunt from the pulmonary vein,
co-existing intracardiac shunt, pulmonary hypoplasia, resulting
in reduction of the vascular bed, and systemic arterial supply to
the right lung.
A surgical approach has been recognised as the gold-
standard therapy for scimitar syndrome, including rerouting
of the aberrant pulmonary vein and repair of the other cardiac
defects.2,10 However, a two-staged strategy with catheter-based
embolisation of the APC, followed later by correction of the
anomalous veins, has also been recommended in some cases.
There is no consensus on which is the best option.
Recently, multiple reports have shown transcatheter
intervention, including embolisation of the APC and closure of
the cardiac defects, may improve symptoms, decrease pulmonary
arterial pressure,11,12 and postpone or even eliminate the need for
surgical correction.7,8,13 Instead of a surgical approach, patients
with scimitar syndrome who have significant left-to-right shunt
due to APC and other cardiac defects are largely suitable for
transcatheter intervention.
In our case, the patient’s pulmonary arterial hypertension
was attributed to the ASD and APC, which could be improved
by decreased pulmonary arterial pressure after transcatheter
intervention. We opted for embolisation of the APC and closure
of the ASD. The patient had satisfactory clinical improvement
and no surgery was performed during follow up. No pulmonary
infarction was observed during the follow-up period.
Many kinds of devices, such as detachable coils, AVPII and
AVPIV have been reported to be appropriate for embolisation of
the APC. In this case, we used detachable coils due to their ease
of deployment, extractability, small size of catheter required, low
metal content and affordability.
Besides the therapies mentioned above, others have also been
used in scimitar syndrome. Pneuomonectomy or lobectomy is
performed cautiously in patients with persistent haemoptysis,
thrombosed intra-atrial baffle, obvious hypoplasia of the
right lung,14 or right pulmonary vein obstruction at risk of
scoliosis post operation2, and respiratory insufficiency post
pneuomonectomy. 10,12,15
Conclusion
Although the traditional surgical approach is still the gold
standard in symptomatic patients, transcatheter intervention of
embolisation of the aortopulmonary collaterals and repair of
co-existing cardiac defects may be an option for selected patients
with scimitar syndrome.
We thank Yingyi He and Xiaomeng Wang for the language editing in this report.
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