CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 3, April 2013
AFRICA
e3
Case Report
Unusual variant of scimitar syndrome associated with
an absent right pulmonary artery, stenosis of the inferior
vena cava, hemi-azygous continuation and the VACTERL
association
FARIRAI F TAKAWIRA, FAREED OMAR
Abstract
We report on a two-month-old infant with an unusual form
of scimitar syndrome, associated with an absent right pulmo-
nary artery, obstructed inferior vena cava, hemi-azygous
continuation and the VACTERL association. The infant
posed a major management problem and eventually died
from a lower respiratory tract infection.
Keywords:
scimitar syndrome, absent pulmonary artery, hemi-
azygous, stenosis, inferior vena cava, VACTERL association
Submitted 4/5/10, accepted 28/11/12
Cardiovasc J Afr
2013;
24
: e3–e6
DOI: 10.5830/CVJA-2012-079
Scimitar syndrome is a rare and complex congenital anomaly that
often involves hypoplasia of the right lung, partial anomalous
pulmonary venous connection (PAPVC) of the right lung
draining into the inferior vena cava (IVC) and a systemic
collateral artery from the descending aorta supplying the right
lower lobe (lobar sequestration). The right pulmonary artery
(RPA) is often hypoplastic but may be completely absent.
1-3
Scimitar syndrome may be associated with other congenital heart
defects or occur in isolation.
We describe a young infant with scimitar syndrome associated
with an absent RPA, an obstructed IVC and the VACTERL
association (Vertebral defects, Anal atresia, Cardiac anomalies,
Tracheo-oEsophageal fistula, Renal abnormalities and Limb
anomalies). These conditions have not previously been described
together in the English literature.
The infant posed a major management problem and eventually
died from a lower respiratory tract infection.
Case report
A female infant was noted soon after birth to have anal atresia,
a recto-vaginal fistula and hypoplasia of the right thumb. She
was the product of the first pregnancy of a 19-year-old mother.
She was transferred to our institution at two months of age for
elective surgical repair of the fistula.
She was noted to be tachypnoeic and on chest radiography
was suspected to have ‘dextrocardia’. She was therefore referred
to our cardiology service for evaluation.
On clinical examination she weighed 3.3 kg and was not
thriving. She was not cyanosed but had saturations of 90% on 2 l/
min of nasal oxygen. Her pulse was 160 beats/min and the blood
pressure 75/44 mmHg. The trachea deviated to the right due to a
right mediastinal shift. The apex beat was felt in the fourth right
intercostal space/mid-clavicular line. The second heart sound
was loud and a soft 2/6 ejection systolic murmur was audible
along the upper sternal border. She was in congestive cardiac
failure, as evidenced by tachypnoea, tachycardia, a gallop rhythm
and hepatomegaly. There was dullness to percussion of the right
hemithorax with decreased air entry.
Steve Biko Academic Hospital and Department of Paediatric
Cardiology, University of Pretoria, South Africa
FARIRAI F TAKAWIRA, FCPaed (SA),
FAREED OMAR, MBCHB, FCPaed (SA)
Fig. 1. Chest radiography demonstrating dextroposition
and right mediastinal shift. Hemivertabrae and scoliosis
are also evident.
