Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with hypoplasia ofthe lung. This syndrome has varied presentations, from an asymptomatic state to severe pulmonary hypertension and/or heart failure.Newer diagnostic modalities have improved our understanding of this rare syndrome and hence providing newer treatment options tobe tried. Here, we present a case of a 4-year-old child with recurrent pneumonia with Scimitar syndrome. We are reporting this case inview of the rarity of this syndrome and its presentation as recurrent pneumonia

Basavanthappa, S P

Kumar, Naveen

Murthy, Srinivas

Pejaver, Rajath

Raghavendra, K

English

Atharva Scientific Publications (E-Jounals)

Vol 1 | Issue 3 | Oct - Dec 2014 Indian J Child Health 147
Murthy et al.  Scimitar syndrome
Case Report
A rare cause of recurrent pneumonia: Scimitar syndrome
Srinivas Murthy1, K Raghavendra1, Rajath Pejaver1, S P Basavanthappa1, Naveen Kumar2
From Departments of 1Pediatrics and 2Radiology, Basaveshwara Medical College Hospital and Research Centre, Chitradurga, 
Karnataka, India
Correspondence to: Dr. Rajath Pejaver, Departments of Pediatrics, Basaveshwara Medical College Hospital and Research Centre, NH 4, 
Near Housing Board Colony, Chitradurga - 577 501, Karnataka, India. Phone: +91-9880178343, E-mail: rajath.pejaver@gmail.com
Received – 18 September 2014 Initial Review – 31 October 2014 Published Online – 30 December 2014
Abstract
Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with hypoplasia of 
the lung. This syndrome has varied presentations, from an asymptomatic state to severe pulmonary hypertension and/or heart failure. 
Newer diagnostic modalities have improved our understanding of this rare syndrome and hence providing newer treatment options to 
be tried. Here, we present a case of a 4-year-old child with recurrent pneumonia with Scimitar syndrome. We are reporting this case in 
view of the rarity of this syndrome and its presentation as recurrent pneumonia.
Key words: Anomalous pulmonary venous return, Lung hypoplasia, Recurrent pneumonia, Scimitar syndrome
Recurrent pneumonia often proves to be a diagnostic challenge to the pediatrician due to its numerous causes. Scimitar syndrome is a very rare cause of recurrent 
pneumonia [1]. The name Scimitar syndrome is derived from 
the curvilinear vascular pattern created on a chest radiogram by 
the anomalous pulmonary vein coursing along the right side of 
the heart towards the diaphragm, which resembles a “Scimitar” 
or Turkish Sword. The term venolobar syndrome is used when 
scimitar syndrome is associated with certain other conditions 
such as right pulmonary hypoplasia, dextroposition of the heart, 
right pulmonary artery hypoplasia, pulmonary sequestration and 
anomalous communication between the right lower lobe of the 
lung, and infradiaphragmatic segment of the aorta [1,2]. Scimitar 
syndrome has varied presentations, from an asymptomatic state 
to severe pulmonary hypertension and/or heart failure [3-5]. 
A high index of suspicion is needed to diagnose scimitar 
syndrome in any child with recurrent or persistent pneumonia. 
Here, we present a child diagnosed to have scimitar syndrome 
who presented to us with a history of recurrent pneumonia.
CASE REPORT
A 4-year-old female child, first born to a third-degree 
consanguineous marriage was brought with complaints of 
repeated episodes of lower respiratory tract infections. Child 
had been hospitalized on four occasions in various hospitals and 
treated with intravenous antibiotics, the records of which were not 
available. Antenatal and neonatal history was non-contributory.
On physical examination, the child had respiratory distress. 
Heart rate was 110/min, respiratory rate - 40/min, temperature 
was 39°C, Blood pressure 78/52 mm Hg. The child’s weight 
was at 10th centile (13 kg) and height was <10th centile 
(92 cm). Respiratory system examination revealed small right 
hemithorax and reduced intensity of breath sounds on the right 
side. There were crepitations on the right side of the chest. 
Cardiac examination revealed shift of the apex beat to the right 
side in fourth intercostal space in right parasternal area without 
any murmur.
Blood investigations revealed elevated total counts with 
neutrophilia. Chest X-ray (Fig. 1) showed small right hemithorax 
with shift of trachea and heart toward right side. Right lung 
field was more opaque than left due to increased background 
reticulations. His electrocardiogram was normal and two-
dimensional echocardiogram revealed dilatation of the right sided 
chambers with intact septum. Main pulmonary artery and left 
pulmonary artery was well seen, but right pulmonary artery could 
not be visualized. Computed tomography (CT) chest (Fig. 2) 
revealed a hypoplastic right lung with the shift of the trachea 
and heart toward the right side. The right inferior pulmonary 
artery was seen draining into the inferior vena cava at the level 
of the diaphragm just above the inferior vena cava-hepatic vein 
junction. A diagnosis of scimitar syndrome was made. The need 
for surgical intervention was explained to the parents. However, 
they declined the same in view of financial constraints. Child 
was treated symptomatically with IV antibiotics and antipyretics.
DISCUSSION
Scimitar syndrome was first described in 1836 by George 
Cooper during an autopsy of a 10 month old infant. The 
Vol 1 | Issue 3 | Oct - Dec 2014 Indian J Child Health 148
Murthy et al.  Scimitar syndrome
incidence of scimitar syndrome is 1-3/100,000 live births [1,6]. 
However, its true incidence may be much higher in view of 
some asymptomatic cases. The disease often presents in early 
infancy but may present later in childhood or rarely in adults. 
In infants it usually presents with tachypnea, congestive heart 
failure, and pulmonary hypertension. It may lead to recurrent 
lower respiratory tract infections as in our case. Recurrent 
episodes of wheeze have also been described in the literature. 
Hemoptysis may be the presenting symptom rarely [7]. 
Scimitar syndrome is associated with congenital heart disease, 
mostly ostium secundum type atrial septal defect. In some 
cases, ventricular septal defect, patent ductus arteriosus, aortic 
stenosis, aortic arch anomalies, Shone complex, and Fallot’s 
tetralogy can be seen. Other anomalies associated with this 
syndrome are sequestration of the lower lobe of the right lung, 
horseshoe lung and anomalous perfusion from the abdominal 
aorta [5,8-10].
Diagnosis is made with the help of echocardiography, 
characteristic chest X-ray, CT chest findings. Echocardiography 
delineates both the Scimitar vein as well as any systemic arterial 
supply to the right lung. Congenital heart defects are associated 
with Scimitar syndrome in 75% of infants with severe symptoms, 
atrial septal defect (ASD) being the most common congenital 
heart disease. The flow velocity pattern in the Scimitar vein 
differs from the normal pulmonary venous flow. It is monophasic 
extending throughout the cardiac cycle with no reverse flow at 
atrial contraction in the Scimitar vein. CT or magnetic resonance 
angiography provides excellent visualization of vascular 
anatomy of this complex congenital defect noninvasively [11].
Cardiac catheterization and angiography are extremely 
useful tools to confirm the diagnosis and clarify the exact 
anatomy and degree of pulmonary hypertension. Radionuclide 
perfusion scan can reveal abnormal arterial supply while 
ventilation scan can identify bronchial connection in a patient 
with Scimitar syndrome. In sequestration, aeration can occur 
through pores of Kohn or through anomalous fistulous 
connection. Scintigraphic screening is helpful to rule out 
significant vascular shunting [12].
Treatment primarily involves surgical correction of the 
anatomical anomaly. However, the most effective surgical 
method has not been clearly identified. Surgical options include 
direct anastomosis of the Scimitar vein to the left atrium or 
division with reimplantation of the anomalous pulmonary vein 
into the right atrium with baffle insertion to redirect the flow 
into the left atrium. An intra-atrial patch may be used to create a 
tunnel, redirecting flow from the anomalous pulmonary vein to 
the left atrium through an ASD [13]. Uthaman et al. described 
coil embolization of Scimitar vein and artery, followed by 
definitive surgery later in life as a method of treatment [14]. 
Pneumonectomy is required in certain cases. Baskar et al. 
performed pneumonectomies for three of the six patients in 
their case series, citing low weight to be the reason for opting 
out of reimplantation surgery [10].
Pulmonary hypertension is a problem seen in infants and 
older children with scimitar syndrome [15]. Pulmonary artery 
pressure was significantly reduced by pulmonary venous stenting 
in combination with coiling of aberrant vascular supply to the 
sequestered lung segment in a report by Awasthy et al. [16]. Further 
experience with such procedures could be useful as it suggests 
that interventional procedures such as pulmonary venous stenting 
could be an emergent palliative treatment to relieve pulmonary 
hypertension in children with Scimitar syndrome.
The age of presentation and the presence of associated 
anomalies are important in predicting the outcome. In general, 
presentation in infancy and presence of heart failure are poor 
prognostic factors.
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Funding: None; Conflict of Interest: None Stated
How to cite this article: Murthy S, Raghavendra K, Pejaver R, 
Basavanthappa SP, Kumar N. A rare cause of recurrent pneumonia: 
Scimitar syndrome. Indian J Child Health. 2014;1(3):147-9.


A rare cause of recurrent pneumonia: Scimitar syndrome

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A rare cause of recurrent pneumonia: Scimitar syndrome

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