syndrome and evolution of managements
Mahdi Kahrom 1,&, Hadi Kahrom1
1 Division of Cardiothoracic Surgery, Imam Reza Hospital, Mashhad
University of Medical Sciences, Mashhad, Iran
Division of Cardiothoracic Surgery, Imam
Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran. Phone:
+98-915-5017276. Fax: +98-511-8674939
Scimitar syndrome is a rare
association of congenital cardiopulmonary anomalies, and first described by
Cooper in 1836 [1,2].
This syndrome is a form of anomalous pulmonary venous drainage (APVD). APVD
implies partial or total failure of the pulmonary veins to reach the left
atrium. Instead, pulmonary venous drainage is anomalously connected to systemic
vein/s, typically to the superior or inferior vena cava (SVC or IVC) or
directly to the right atrium (RA). APVD accounts for 2% of all congenital heart
disease and is usually associated with left to right shunting at atrial level
through some form of atrial septal defect (ASD) .
The haemodynamic effects of APVD are also those of left to right shunting at
atrial level, thereby exacerbating right heart volume overload.
The most common form of
partial APVD is connection to the SVC or SVC-RA junction in association with a
superior sinus venosus defect. Scimitar syndrome is rarer, with the right
pulmonary vein/s draining to the inferior vena cava.
Associated anomalies are
variable and include hypoplasia of the right lung, dextroposition of the heart,
hypoplasia of the right pulmonary artery (RPA), and anomalous systemic arterial
supply from the aorta to the right lung .
The term Scimitar syndrome was coined by Naill in 1960, because of the
radiographic appearance of the anomalous vein, which appears as a tubular
opacity paralleling the right heart border resembling a curved Turkish sword or
This is so-called as Scimitar sign (Figure 1).
This rare anomaly has an
incidence of approximately 1 to 3 per 100,000 live births ;
the true incidence may be higher because many patients are asymptomatic. It has
been reported most widely in adults and older children and is usually found
during a workup for dyspnea, fatigue, recurrent respiratory infections, or as
an incidental finding on a routine chest radiograph [7-9].
This adult form of Scimitar syndrome usually is not associated with pulmonary
hypertension and typically has mild symptoms and a benign prognosis.
A second, infantile group
of patients become symptomatic soon after birth. Their course is often
complicated by severe pulmonary hypertension and cardiac failure, making
management difficult and mortality high [8-13].
The surgical history of Scimitar syndrome along with current diagnostic
strategies, surgical techniques, results, and modalities of follow-up is
Clinical presentation and
Scimitar syndrome overlaps
with pulmonary sequestration and the term "venolobar syndrome" has
been coined to include these associated pulmonary and vascular malformations [14,15].
The condition is associated with :
agenesis or hypoplasia of the right lung with bronchial isomerism.
or hypoplasia of the right bronchial system.
or agenesis of the right pulmonary artery .
This may cause mediastinal shift to the right side and Scimitar vein may be
difficult to appreciate or even completely obscured .
systemic blood supply to at least part of the right lung, most frequently the
posterior basal segment of the lower lobe, usually arising from the
infradiaphragmatic descending aorta [18-22].
of the heart due to right lung hypoplasia with mediastinal shift .
diaphragm, eventration or partial absence of the diaphragm .
and gastric lung.
10. Absence of the
11. Other congenital
cardiac malformations (25% of cases) including ASD ,
ventricular septal defect, coarctation of the aorta, tetralogy of Fallot [28,29],
pulmonary stenosis, absent inferior vena cava with azygus continuation to
superior vena cava [30,31].
Moreover, the APVD may also be partially obscured, further contributing to
pulmonary hypertension .
The detection of any of
these anomalies should lead to a search for other components. The clinical spectrum
of Scimitar syndrome ranges from severely ill infants to asymptomatic adults,
and cases may present in one of three ways [11,32]:
the neonatal period with respiratory and/or cardiac failure .
This is most commonly caused by pulmonary hypertension due to cardiac and/or
right lung anomalies. Treatment is surgical and outcome is dependent on the
nature and severity of the anomalies .
Heart failure may also be caused because of a large arterial supply from the
abdominal aorta to a sequestered lobe. In these situations, cardiac
catheterization may be used to embolise the aberrant pulmonary blood supply.
any stage in life due to recurrent respiratory infections, usually affecting
the right lower lobe that often has an abnormal blood supply and venous
drainage. Severity and frequency of infections is related to the degree of
pulmonary hypoplasia. Lobectomy or even right pneumonectomy may be required to
deal with bronchiectasis and prevent further respiratory infections. Affected
individuals may also present with haemoptysis due to pulmonary hypertension .
any stage in life as an incidental finding e.g. due to the detection of a
murmur or due to the evident CXR abnormalities [7,36,37].
Classic findings on
Classic findings on
physical examination include a shift in heart sounds and cardiac impulse to the
right and a systolic murmur. Auscultation of the lung is usually normal,
although breath sounds may be diminished on the right. Curiously, Scimitar
syndrome is almost exclusively a right-sided anomaly. Only five cases involving
the left lung and left pulmonary vein have been reported [38-42].
Pulmonary hypertension as a
marker for Scimitar syndrome
Pulmonary hypertension is
often recognized as the cause of the severe symptoms and poor outcome of
Scimitar syndrome during infancy [7,9-11,43,44].
Multiple factors may be responsible for the pulmonary hypertension. These
include the following [7,9,11,41,45,46]:
left to right shunt via the anomalous pulmonary vein.
to right shunt from the systemic arterial supply to the right lung .
lung hypoplasia with reduction of the pulmonary vascular bed.
vein stenosis and obstruction.
congenital cardiac malformations.
pulmonary hypertension of the newborn.
Some studies have
emphasized that the pulmonary hypertension may be caused by multiple factors
including; large left to right shunting by the anomalous pulmonary vein or
other intracardiac lesions such as an ASD or VSD [9,13,46].
Pulmonary venous stenosis is a well known cause of pulmonary hypertension in
infants with total anomalous pulmonary venous connection and has been
recognized as the source of pulmonary hypertension in some patients with
Scimitar syndrome [9,12,13,44,48].
The abnormal venous return is
the main component of Scimitar syndrome, and gives a characteristic abnormal
radiographic shadow descending along the right cardiac border, which resembles
a curved Turkish sword (i.e., Scimitar) 
or a women's leg 
(Figure 1). However this radiologic sign may be may be obscured because of the
associated dextrocardia .
Sometimes a "meandering" but normally draining pulmonary vein may be
confused for a Scimitar vein .
The most striking radiographic feature in each of these patients is the
dextroposition of the heart along with varying degrees of opacity of the right
A small, opaque hemithorax generally implies volume loss. Mediastinal shift
toward the opacified hemithorax with compensatory hyperinflation of the
abnormal lung can be seen with atelectasis or pulmonary agenesis .
Pulmonary hypoplasia can also cause a unilateral small lung with the heart
shifted toward the affected side .
The term dextroposition, rather than dextrocardia, is used when the heart is
shifted into the right chest but the cardiac chambers maintain a normal
relationship and the cardiac apex still points to the left .The
Scimitar sign is usually absent in infants .
delineate both the Scimitar vein as well as any systemic arterial supply to the
right lung [54-55].
Additional cardiovascular anomalies have been noted in 75% of infants with
severe symptoms .
ASD is the most common associated defect [7,13],
but VSD, PDA, tetralogy of Fallot, and left-sided lesions such as aortic arch hypoplasia
or coarctation and hypoplastic left heart syndrome have also been reported [8,9,13,30,31].
Fetal echocardiography permits prenatal diagnosis in which spectral and color
Doppler provides clues to the presence of an obstructed pulmonary venous
Visualization of a confluence behind the right atrium and a vertical vein are
the most consistent Echo clues .
The flow velocity pattern
in the Scimitar vein is different from the normal pulmonary venous flow .
The normal flow is biphasic or triphasic, which one or two peaks in systole and
one peak in diastole (peak velocity of about 0.5 m/s), and reverse flow at
atrial contraction (peak velocity of about 0.2 m/s). The flow pattern in
Scimitar vein is monophasic extending throughout the cardiac cycle with no
reverse flow at atrial contraction .
tomography (CT) [61,62]
and cardiac-gated magnetic resonance imaging (MRI) 
are useful in visualizing the anomalous pulmonary vein (Figure 2). They can be
particularly helpful in detecting an associated horseshoe lung, in which there
is posterior fusion of portions of the right and left lungs behind the heart
and in front of the esophagus and spine [15,64].
Approximately 80% of infants with horseshoe lung also have Scimitar syndrome .
Cine MRI [65,66]
and 3-D contrast enhanced MR angiography 
provides a non-invasive diagnostic technique in the evaluation of anomalous
pulmonary venous return. Gadolinium enhanced 3-D MR angiography [67-70]
that provides concurrent non-invasive complete anatomical (arterial and venous
supply) and functional (calculation of left to right shunt using phase contrast
MRI) diagnosis avoids the need for more traditional invasive techniques .
Cardiac catheterization and
angiography are probably the most useful procedures for confirming the
diagnosis and clarifying the exact anatomy and degree of pulmonary hypertension
(Figure 3). Oxygen saturations in the IVC and RA may be increased .
The RPA is almost always hypoplastic, atretic, or otherwise abnormal in those
infants with pulmonary hypertension [8,42,43,45,72].
An aortogram should also be performed to visualize the presence or absence of an
anomalous systemic artery entering the right lower lobe.
Scimitar syndrome is also
known as Halasz's syndrome, mirror-image lung syndrome, hypogenetic lung
syndrome, epibronchial right pulmonary artery syndrome and vena cava bronchovascular
syndrome. It occurs more commonly in females and is occasionally familial .
The left lung is very rarely involved and the reason for this, is unknown .
The true incidence of this
syndrome is unknown since the syndrome may remain undetected in asymptomatic
patients who do not undertake a CXR. Patients with Scimitar syndrome may be
seen in infancy, childhood or adulthood. Infants typically have features of
congestive heart failure from a significant left to right shunt from the
anomalous pulmonary venous drainage or more commonly from an additional cardiac
defect such as ASD. Older children or adults may have symptoms, and the
diagnosis may be made from a chest radiograph that demonstrates the Scimitar
sign in as many as 70% of patients, because hypoplasia of the right lung is
usually absent in this population. If the atrial septum is open (25%-50% of
older patients), examination of the heart may elicit the salient
characteristics of an ASD, and the features of Scimitar syndrome will become
Functionally, Scimitar syndrome resembles an atrial septal defect (ASD);
however, there are no guidelines for surgical correction in adults .
Left to right shunt in adults is less than 50% in 82% of patients, pulmonary
artery pressures are normal in 77%
of patients and slightly elevated in a further 23%, and these patients lead a
normal life without surgical correction .
However, in cases with a left to right shunt, more than 50% of the patients
develop dyspnea, chest infections and pulmonary hypertension .
Prognosis after intracardiac repair of Scimitar syndrome is excellent in
patients without pulmonary hypertension .
The indications for surgical
repair include the presence of Scimitar syndrome, especially in association
with ASD, pulmonary hypertension, or stenosis of the anomalous vein. Scimitar
vein stenosis has been noted in 10% to 20% of cases and in conjunction with the
ASD may cause pulmonary hypertension .
It is important that the surgical procedure chosen, correct the stenosis in the
Scimitar vein; if pulmonary hypertension persists after repair, lung
transplantation should be pursued because the long-term mortality among these
patients is high.
In neonates and young
infants a trial of medical therapy as the initial approach is reasonable to allow
an increase in size before repair of the defect. However the presence of
pulmonary hypertension or lack of response to medical therapy demands prompt
The operative approach to
correct Scimitar syndrome is quite variable. The first report of surgical
therapy for Scimitar syndrome was 1950 by Drake and Lynch ,
who performed a right lower lobectomy. However, efforts should be made to avoid
lung resection. Simple ligation of the anomalous pulmonary vein usually results
in pulmonary edema, infarction, or both and is not advised.
In 1956, Kirklin et al 
reported the first total correction without cardiopulmonary bypass in a patient
with the infradiaphragmatic type of Scimitar syndrome and an atrial septal
defect. Since the vein was not long enough to be implanted directly into the
left atrium, it was anastomosed to the right atrium; by means of a Bailey 
atrioseptostopexy, the flow was directed to the left atrium through the ASD.
The experimental basis for this method was established by work of Gerbode and
who demonstrated in dogs that such an anastomosis would heal well and remain patent.
After Kirklin's report, several cases of total correction using open and closed
techniques have been reported [79-84].
Several methods with cardiopulmonary bypass have been
recommended to repair this anomaly, including direct anastomosis of the
Scimitar vein to the left atrium, as reported by Honey ,
or division with reimplantation of the anomalous pulmonary vein into the right
atrium with baffle insertion to redirect the flow into the left atrium, as
proposed by Shumacker and Judd .
Honey suggested that operative management should differ according to the
presence or absence of an associated ASD. With an intact atrial septum, the
anomalous vein may be reimplanted if possible into the back of the left atrium;
otherwise, the anomalous venous return can be baffled through an existing or
created ASD to the left atrium .
Baffling can be complicated by stenosis resulting in pulmonary venous
However, successful Inoue balloon percutaneous dilation of a stenotic inferior
vena cava anastomosis has been described with good haemodynamic and symptomatic
intra-atrial patch may be used to create a tunnel, redirecting flow from the
anomalous pulmonary vein to the left atrium through an ASD, as described by
Zubiate and Kay in 1962 .
Puig-Massana and Revuelta 
described use of free wall of the right atrium to create a tunnel from the
Scimitar vein to the left atrium across an ASD. These last two operations
require hypothermic circulatory arrest for accurate suturing of the
intra-atrial baffle around the orifice of the anomalous vein within the IVC.
There have been two case
reports utilizing a 14 mm Dacron graft 
and a 20 mm Dacron graft 
interposed between the orifice of the anomalous pulmonary vein and an enlarged
ASD as an intra-atrial conduit.
The authors also have an
experience in the use of 14 mm hemoshield Dacron graft as an extra-cardiac
conduit, between the Scimitar vein and the left atrium, as an alternative
approach. Although the fate of the used Dacron grafts in venous positions and
their long term patency is not clear, however, in our four year post-op
follow-ups by trans-esophageal echocardiography (TEE), the graft is patent and
well functioning. Use of anticoagulation therapy is an attempt to maximize the
Dacron graft patency [91,92].
Figure 1: Chest X-ray. The
heart is shifted into the right chest. The right lung is hypoplastic. Note the
“Scimitar sign”- a curvilinear opacity widening its course to the inferior vena
Figure 2: Chest
CT slice showing descending Scimitar vein in right lung field (arrow).
Figure 3: Cardiac
catheterization displaying the anomalous right pulmonary vein.
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