Scimitar Syndrome

What is Scimitar syndrome?

Scimitar syndrome (also known as hypogenetic lung syndrome) is a rare form of congenital heart disorder which occurs due an abnormal arrangement of pulmonary veins. This condition generally affects people in their early childhood and may be present in newborns.

Scimitar syndrome also causes some or all the pulmonary veins to drain into the inferior vena cava – the large vein that carries deoxygenated blood from the lower and middle body into the right atrium of the heart.

Associated anomalies of this disorder usually include hypoplasia of right lung, systemic arterial supply to right lung from the descending aorta, Dextrocardia and various congenital cardiac diseases like ASD.

What are the symptoms of Scimitar syndrome?

Some cases of Scimitar syndrome are asymptomatic, meaning it does not present any symptoms.

In other cases, common symptoms include:

Respiratory distress
Rapid breathing
Shortness of breath
Cyanosis
Retraction of the rib cage muscle

What causes Scimitar syndrome?

The exact cause of Scimitar syndrome is currently unknown. It often occurs as a congenital disorder, which means that patients are born with it.

How is Scimitar syndrome diagnosed?

A doctor may perform various tests and examinations to see if this disease is present in patients suffering from dizziness, chest pain, weakness and irregular heart beat.

Various imaging and other tests to diagnose Scimitar syndrome include:

Chest x-ray
Transesophageal or transthoracic echocardiography
Angiography
CT angiography
MR Angiography
MRI

What are the treatments for Scimitar syndrome?

The most common way to treat serious symptoms of Scimitar syndrome is by surgery.

The principal object of surgery is to create an inter-atrial baffle for redirecting the pulmonary venous circulation into the patient’s left atrium. There is another surgical operation which involves re-implanting the abnormal vein directly into the patient’s left atrium. Patients suffering from pulmonary hypertension as an associated condition require surgical management. The form of treatment is decided by the physician depending on the severity and nature of the symptoms as well as the associated disorders.