CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 3, April 2013
e6
AFRICA
case in our patient. The abnormal pulmonary vein draining
into the IVC can often be seen as a classic curvilinear density
on the right chest on plain radiography and is shaped like a
Turkish sword or scimitar. This may not always be evident. It
is also known as congenital pulmonary veno-lobar syndrome,
hypogenic lung syndrome, mirror image lung syndrome, vena
cava bronchovascular syndrome, epibronchial right pulmonary
artery syndrome or Halasz’s syndrome.
1-3
Halasz first used the term ‘scimitar’ in 1956,
3
but Neill coined
the term ‘scimitar syndrome’ in 1960.
1-3,6
The aetiology remains
unknown. It occurs more frequently in females and may be
familial.
1,6
It remains unclear why it predominantly involves the
right lung, although there have been a few case reports involving
the left lung.
7
There are several associations described with the scimitar
syndrome, including the horse-shoe lung and absence of the RPA.
It has rarely been described with a hypoplastic or interrupted
IVC. Agnoletti
et al
. described a similar case with scimitar
syndrome, absent RPA and a persistent primitive hepatic venous
plexus.
8,9
The IVC was also stenosed in their case.
The pathophysiology in scimitar syndrome resembles that
of PAPVC or left-to-right shunting at atrial level with volume
overload and dilatation of the RA, RV and pulmonary arteries.
Pulmonary hypertension may develop, especially where there
is associated obstruction of the scimitar vein. The systemic
collateral supply from the aorta to the right lung also functions as
a left-to-right shunt, increasing the flow through the scimitar vein
and worsening the pulmonary hypertension. In our patient, the
left lung received the whole cardiac output and this exacerbated
the pulmonary hypertension. The abnormal systemic supply
leads to sequestration of the left lung. This may lead to recurrent
chest infections and bronchiectasis.
There is a wide clinical spectrum in scimitar syndrome,
ranging from severely ill infants to asymptomatic adults. The
infantile type is the severe form and presents in the first three
months of life with respiratory and cardiac failure and often
has a poor long-term outcome.
10,11
This group usually has
other associated cardiac defects, commonly atrial septal and
ventricular septal defects and tends to have severe pulmonary
hypertension. The adult type is often asymptomatic, presenting
later in life as an incidental finding on chest radiography and is
usually benign.
Treatment for symptomatic patients with scimitar syndrome
consists of surgical repair of the PAPVC, with the anomalous
scimitar vein being redirected to the LA and ligation or coil
embolisation of the systemic collateral supply to the right
lung.
12-15
Obstruction by thrombosis and fibrosis of the redirected
pulmonary vein is a common post-operative complication.
12
Lobectomy, and in some cases, pneumonectomy of the affected
lung has been performed.
3,12
VACTERL association is an acronym used to describe a series
of non-randomly associated birth defects (vertebral defects, anal
atresia, cardiac anomalies, tracheo-esophageal fistula, renal
abnormalities and limb anomalies). To our knowledge, this
rare association of scimitar syndrome, absent RPA, stenosis of
the IVC with hemi-azygous continuation and the VACTERL
association has not been previously described in the English
literature.
Scimitar syndrome as the cardiac component of theVACTERL
association is very uncommon. In their series of 32 patients with
scimitar syndrome over 20 years, Najm and colleagues describe
only one patient with the VACTERL association.
16
References
1.
Grech V, Xuereb R, Xuereb M, Manche A,
et al
. Late presentation and
successful treatment of classical scimitar syndrome.
Images Paediatr
Cardiol
2003;
16
: 49–62.
2.
Muktachand L, Rokade RV, Devdas S,
et al.
Scimitar syndrome.
Ind J
Paediatr
2005;
72
: 245–247
3.
Patel AM, Joshi R, Vaghela D,
et al.
A face of scimitar syndrome.
Ind J
Radiol Imag
2004;
14
: 401–404.
4.
Beitzke A, Zobel G, Rigler B,
et al.
Scimitar syndrome with absence of
the right pulmonary artery: a case with volume-induced, reversible left-
sided pulmonary hypertension
. Paediatr Cardiol
1992;
13
: 119–121.
5.
Saltik IL, Eroglu AG, Oztune F, Sarioglu A. Scimitar syndrome with
absence of the right pulmonary artery: a case report.
Turk J Paediatr
1999;
41
: 399–402.
6.
Neill CA, Ferenca C, Sabiston DC. The familial occurrence of hypo-
plastic right lung with anomalous systemic arterial supply and venous
return, ‘scimitar syndrome’.
Bull Johns Hopkins Hospital
1960;
107
:
1–21.
7.
Rutledge JM, Hiatt PW, Wesley VG 3rd,
et al
. A sword for the left hand:
an unusual case of left sided scimitar syndrome.
Paediatr Cardiol
2001;
22
: 350–352.
8.
Mac Donald C, Mikhailian H, Yoo S,
et al
. Angiographic findings of
persistent primitive hepatic venous plexus with underdevelopment of
the infrahepatic inferior vena cava in paediatric patients.
Am J Radiol
2000;
175
: 1397–1401.
9.
Agnoletti G, Bonnet D, De Blic J. Scimitar syndrome associated with
absence of the right pulmonary artery and a persistent primitive hepatic
venous plexus.
Cardiol Young
2005;
15
: 216–218.
10. Sehgal A, Loughran-Fowlds A. Scimitar syndrome.
Indian J Paediatr
2005;
72
: 249–251.
11. Gikonyo DK, Tandon R, Lucas RV Jnr,
et al
. Scimitar syndrome in
neonates: Report of 4 cases and review of the literature.
Paediatr
Cardiol
1986;
6
: 193–197.
12. Schramel FMNH, Westermann CJJ, Knaepen PJ,
et al
. The scimitar
syndrome: Clinical spectrum and surgical treatment.
Eur Resp J
1995;
8
: 196–201.
13. Desai PR, Babu M. Scimitar syndrome as a differential diagnosis in a
child with recurrent wheeze.
Arch Dis Child
2002;
87
: 357.
14. Oshima Y, Hashimoto I, Shimazu C,
et al.
Atypical infantile form of
scimitar syndrome with bronchomalacia.
Interact Cardiovasc Thorac
Surg
2003;
2
: 298–300.
15. Mordue B. A case series of 5 infants with scimitar syndrome.
Adv
Neonat Cardiol
2003;
3
: 121–132.
16. Najm HK, Williams WG, Coles JG,
et al.
Scimitar syndrome: Twenty
years’s experience and results of repair.
J Thorac Cardiovasc Surg
1996;
112
: 1161–1168.
