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Cyanosis in late teens

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Q1: What is the most likely cause of the cyanosis?

A form of congenital cyanotic heart disease with a right to left shunt is the most likely cause of cyanosis in this case. Pulmonary causes for the cyanosis are virtually eliminated by the normal chestx ray and non-correction of hypoxia with 100% oxygen.

Q2: What does the transoesophageal echocardiogram show?

The TOE shows an abnormal connection (indicated by the arrow) between the left atrium and dilated right pulmonary artery. The cause of this patient's cyanosis is a left atrial to right pulmonary artery fistula.

Q3: What treatment options are available for this patient?

Several treatment options are available. Surgery can be undertaken to close the fistula by ligation or dissection, or it can be closed during cardiac catheterisation and pulmonary angiography by embolisation using a steel coil, umbrella, or detachable balloon.

Discussion

A fistula between the pulmonary artery and left atrium is a rare type of pulmonary …

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