Clinical Brief

Scimitar Syndrome
Arvind Sehgal and Alison Loughran-Fowlds

Grace Neonatal Intensive Care Unit, The Children's Hospital at Westmead, Sydney, New South Wales, Australia

Abstract. Scimitar syndrome is a relatively uncommon constellation of cardio-pulmonary anomalies, its typical feature being
partial anomalous pulmonary venous connection. It can present in the neonatal period as well as later in life. We present the
case of a girl diagnosed in the newborn period, along with a brief review of literature. [Indian J Pediatr 2005; 72 (3) : 249-251]
E-mail : jyotiarvind@hotmail.com

Key words : Newborn; Scimitar syndrome

The Scimitar syndrome or pulmonary venolobar showed severe pulmonary hypertension out of proportion
syndrome is a rare, complex and variable malformation of to lung disease, hypertrophied and dilated right ventricle,
the right lung characterised by an abnormal right sided dilated right atrium & inferior venacava (IVC), along with
pulmonary venous drainage in the inferior vena cava, trivial tricuspid and pulmonary incompetence. At this
malformation of the right lung, abnormal arterial supply stage she was transferred to a tertiary centre.
and sometimes cardiac malformations. About half the A cardiac catheterization was performed which
patients with Scimitar syndrome are asymptomatic or revealed supra-systemic pulmonary pressures (>30 mm
midly symptomatic when the diagnosis is made, despite Hg above systemic). She also underwent magnetic
varying degrees of pulmonary hypoplasia and
pulmonary artery hypertension.1,2 Neonates have severe
symptoms and worse prognosis while older children
come to light because of recurrent respiratory infections,
a murmur or an abnormal chest radiograph (CXR). 1,2
Although the diagnosis can frequently be made on a CXR,
further imaging is needed to confirm the diagnosis and
demonstrate other associated abnormalities. We present
the case of a newborn who had an early presentation and
had almost the full constellation of features of Scimitar
syndrome along with a review of literature.

CASE REPORT

A female child was born to a gravida 2 mother at term by

normal vaginal delivery. The pregnancy was complicated Fig. 1. Chest radiograph of the patient.
by growth restriction and her birth weight was 2375 gm.
Apgar scores were 6 and 8 at one and five minutes
respectively. On sixth day, she developed tachypnea and
was noted to have a systolic murmur. The initial CXR
suggested a non-specific pathology in right upper/
middle zone (Fig. 1) and the initial echocardiogram
(ECHO) showed a bi-directional shunt through a patent
duct, atrial septal defect (ASD) and significant pulmonary
hypertension. Drainage of left pulmonary veins into left
atria could be visualized (Fig. 2). She was treated with
oxygen and antibiotics. In view of non-improvement, she
had a computerised tomography (CT) scan of chest,
which revealed right lung hypoplasia. A repeat ECHO
Correspondence and Reprint requests : Dr. Arvind Sehgal, Room Fig. 2. Echocardiogram showing dilated right atria & drainage of
No. 262, John Astor House, 3, Foley Street, London W1W 6DN. left pulmonary veins into left atria.

Indian Journal of Pediatrics, Volume 72—March, 2005 249

 Arvind Sehgal and Alison Loughran-Fowlds
resonance imaging with magnetic resonance angiography
(MRI/MRA) which revealed anomalous drainage of right
pulmonary veins to the supra-hepatic portion of IVC
(Partial Anomalous Pulmonary) Venous Return, PAPVR)
and upper middle and lower lobe veins forming “scimitar
vein” (Fig. 3).
Fig. 3. Magnetic resonance angiograph showing right partial
anomalous pulmonary vein connection into IVC.
Associated findings were right lung hypoplasia with
mediastinal shift, sequestered posterobasal segment of
the right lower lobe with its blood supply from the
abdominal aorta, right pulmonary artery hypoplasia,
small left IVC draining into the left atria, marked coronary
sinus dilatation and vertebral scoliosis. After being
initially treated with digoxin, captopril and frusamide,
she underwent baffle repair and ligation of blood supply
to sequestered lobe. Her post-operative period was
complicated by right-hemidiaphragm neuropraxia. She
was transferred to the referring institution at two months
of age but was readmitted with worsened respiratory
symptoms two weeks later. A repeat MRI showed right
lung collapse and severely impaired perfusion. A repeat
ECHO at this stage showed supra-systemic pulmonary
artery pressures. She developed severe respiratory failure,
was ventilated and failed of nitric therapy. She died at age
of four months.
DISCUSSION
The Scimitar syndrome, first described by Chassinat in
1836 is a rare but well described constellation of cardio-
pulmonary anomalies,1-4 accounting for 0.5-1% of CHD.
250
The characteristic abnormality is anomalous pulmonary
venous return of part (PAPVR) or the entire right lung to
the IVC, though drainage into the hepatic vein or the
portal vein can occur. Isolated partially anomalous
pulmonary venous return to the inferior caval vein is also
called “incomplete Scimitar syndrome”. Two main forms
of Scimitar syndrome have been described. In the infantile
form, the anomaly is usually associated with a variety of
thoracic abnormalities and a high proportion of other
vascular malformation. PAPVR of the right lung is
usually associated with an ASD and an intact atrial
septum is exceptional. Approximately 10-15% of patients
with ostium secundum ASD have PAPVR.5 Other cardiac
malformation include coarctation of aorta, Tetrology of
Fallot's, patent ductus arteriosus and ventricular septal
defect.6,7 The adult form is characterised by a small shunt
with minor symptoms and lack of associated anomalies.
Other manifestations of the syndrome are hypoplasia
of the right lung and right pulmonary artery,
abnormalities of tracheo-bronchial architecture, anomalies
of pulmonary lobation, sequestration, diaphragmatic
eventration, Bochdalek hernia, accessory diaphragm, and
rarely an absent IVC. Many of the typical manifestations
were present in the present case. The age of presentation
is very variable. In a series of 32 patients, over a period of
20 years, the median age at diagnosis was 7 months. 8
Severe respiratory insufficiency is always present in
symptomatic cases in early age group as Scimitar
syndrome results in pulmonary hypertension, heart
failure and right lung infection. The severe symptoms and
pulmonary hypertension found in such infants have
many causes. Anomalous systemic arterial supply,
pulmonary vein stenosis and associated cardiac
anomalies play a significant role. The recurrence of
pulmonary hypertension in the present case could be
explained by occurrence of pulmonary venous stenosis. 9
Classic appearance on chest radiograph-the scimitar sign-
appears because the anomalous venous connection causes
a curvilinear shadow adjacent to the right heart border
that resembles a curved Turkish sword. However, in
some, when the scimitar vein is masked by the overlying
cardiac shadow, other modalities are required. In the
present case, the localized haziness on right side could be
because of vascular congestion due to blockage of
pulmonary venous drainage from the right lung though
the initial suspicion was of underlying infection.
Doppler US conventional X-ray angiography have
been traditionally considered to be the most accurate
methods for pulmonary venous abnormalities though
current MR technology enables excellent visualisation of
vascular anatomy. Cine MRI10 and 3-D contrast enhanced
MR angiography11 provides a non-invasive diagnostic
technique in the evaluation of anomalous pulmonary
venous return. Gadolinium enhanced 3-D MR
angiography that provides concurrent non-invasive
complete anatomical (arterial and venous supply) and
functional (calculation of left to right shunt using phase
Indian Journal of Pediatrics, Volume 72—March, 2005
 Scimitar Syndrome
contrast MRI) diagnosis avoids the need for more
traditional invasive techniques.12 A Velocity Encoded
Cine MRI (VEC-MR) is useful for quantitative assessment
of cardiac and great vessel flow. Surgical treatment of
Scimitar syndrome may be required in the symptomatic
patient, with other associated cardiac abnormalities or
when left to right shunt is greater than 2:1.12 Surgical
options in such cases include redirecting the venous
drainage to left atria, ligation/embolization of vascular
supply to the sequestered lobe and pneumonectomy.
Fetal echocardiography permits prenatal diagnosis in
which spectral and color Doppler provide clues to the
presence of an obstructed pulmonary venous pathway.
Visualisation of a confluence behind the right atrium and
a vertical vein are the most consistent ECHO clues.13
REFERENCES
1. Canter CE, Martin TC, Spray TL, Weldon CS, Strauss AW.
Scimitar syndrome in childhood. Am J Cardiol 1986; 58 : 652.
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remarquables de 1'appareil circulatoire avec hepatocele cong's
enitale a'yant donne' lieu pendant la vie a aucum symptome
particulier. Arch Gen Med Paris 1836; 11 : 80.
4. Dupuis C, Charaf LAC, Breviere G, Apou P, Remy-Jardine M,
Indian Journal of Pediatrics, Volume 72—March, 2005
Helmius G. The “adult” form of Scimitar syndrome. Am J
Cardiol 1992; 70 : 502.
5. Gotsman MS, Astley R, Parsons CG. Partial anomalous
pulmonary venous drainage in association with atrial septal
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6. Roehm JOF, Jue KL. Amplatz radiographic features of the
scimitar syndrome. Radiology 1966; 86 : 856-859.
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8. Najm HK, Williams WG, Coles JG, Rebeyka IM. Freedom RM.
Scimitar syndrome: twenty year's experience and results of
repair. J Thorac Cardiovasc Surg 1996; 112(5) : 1161-1168.
9. Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom
RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993; 22(3)
: 873-882.
10. Baxter R, McFadden M, Gradman M, Wright A. Scimitar
syndrome: cine magnetic resonance imaging demonstration of
anomalous pulmonary venous drainage. Am Thorac Surg 1990;
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11. Ferrari VA, Reilly MP, Axel L, Sutton MG. Scimitar syndrome.
Circulation 1998; 98 : 1583-1584.
12. Lucas EM, Canga, A, Sadaba P, Martin-Duaran R, Otero M,
Cerezal L. Scimitar syndrome: complete anatomical and
functional diagnosis with gadolinium-enhanced and velocity-
encoded cine MRI. Pediatr Radiol 2003; 33 : 716-718.
13. Valsangiacomo ER, Hornberger LK, Barrea C, Smallhorn JF,
Yoo S. Partial and total anomalous pulmonary venous
connection in fetus: two-dimensional and Doppler
echocardiographic findings. Ultrasound Obstet Gynecol 2003;
22(3) : 257-263.
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