SCIMITAR SYNDROME

PONTIFICAL CATHOLIC UNIVERSITY OF ECUADOR

Daniela Naranjo
Patient history and Physical examination

Parents reported he had

This patient is a male
an abnormal breathing
infant born at 39 weeks
Birth weight was 3000 He was discharged home pattern noted very early
gestation after an He developed a chronic,
grams, APGAR Scores 8 on day 2 of life with in life with mild
uneventful pregnancy wet cough at 1 month of
and 9 at 1 and 5 respiratory rate of 36 intermittent labored
via cesarean section due age.
minutes, respectively. and a normal lung exam. respirations and
to pelvic disproportion
retractions occasional
as a third child.
stridor.

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 He was admitted for further
studies. A thorough
infectious disease evaluation
was negative for bacterial or
It was found a pulmonary
viral infections.
pressure increase
(90mmHg), of worsening
respiratory distress with a
Normal monthly routine
supplemental oxygen
check ups until the 3rd
requirement of 2 liter per
month.
minute (lpm) via nasal
An echocardiogram at the cannula, worsening cough
referring hospital revealed a and fever.
patent ductus arteriosus, a
secundum atrial septal
defect and pulmonary
hypertension (70mm Hg).

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Hospital course

A chest x-ray showed findings suggestive of scimitar syndrome with a semi-circular

density at the medial right lung base and normal right lung size.

Electrocardiogram (EKG) showed right axis deviation and right ventricular hypertrophy.

Angiotomography: situs solitus, anomalous pulmonary venous drainage was found.

From the right pulmonary vein to the inferior vena cava. Lobar opacity in the right lower
pulmonary lobe was seen. Left pulmonary veins drain to the left auriculae.

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Cardiac catheterization
demonstrated elevated pulmonary
artery pressures. Angiographic
phase of the right pulmonary artery
showed partial anomalous venous
drainage via a vertical vein into the
inferior caval vein together with
unobstructed drainage to the left
atrium. There was also a pulmonary
vasculature sequestration from the
aorta and subclavian.
One venous access and one arterial
access were achieved via the
femoral route. The anomalous
pulmonary vein was identified and
According to the angiographic cannulated.
findings, a decision was made to
correct the anomaly through a
transcatheter approach. The
procedure was performed under
general anaesthesia; the patient
was under high- frecuency
oscillation (HFO)
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• Scimitar syndrome, also known as congenital
pulmonary venolobar syndrome, is a rare
association of congenital anomalies.
• Named by its similarity to a Turkish sword,
known as the scimitar, since its anomalous
drainage of pulmonary veins has a curved path
to the inferior vena cava, usually of the right
lung.
• Estimated prevalence of 1 to 3 / 100,000 births.
• It is estimated that it is responsible for 0.5 to
1% of congenital heart disease.
• The severe symptoms and pulmonary
hypertension that are found have many causes.

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 • The diagnosis is made by clinical
suspicion of a newborn who
presents with cardiac failure,
tachypnea or permanent
respiratory distress or who is
aggravated accompanied by the
imaging diagnosis where the
scimitar sign can be observed in
70%.

Gaillard, F., & Murphy, A. (2011). Scimitar syndrome  | Radiology Reference Article | Radiopaedia.org. Retrieved
October 20, 2019, from Radiopaedia.org website: https://radiopaedia.org/articles/scimitar-syndrome-lungs
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Conclusion
• Scimitar syndrome is a rare congenital syndrome that requires an
interprofessional team approach that includes a pediatrician,
pediatric cardiologist, cardiac surgeon, and radiologist.
• If it is not appropriately diagnosed, scimitar syndrome can have poor
outcomes, especially with the infantile form.
• An early diagnosis with advance imaging facilitates the surgical
strategy, contributing to low morbidity and mortality rates after
corrective surgery.
Diaz-Frias J, Widrich J. Scimitar Syndrome. [Updated 2019 Sep 4].
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK546602