What is Heterotaxy Syndrome?

Heterotaxy Syndrome is a rare condition associated with congenital cardiovascular and gastrointestinal anomalies. It means a disordered organ arrangement in the chest or abdomen.

The medical term comes from the Greek words ‘hetero’ meaning ‘other’ and ‘taxis’ meaning ‘arrangements’.

The condition is so complex that each patient has different anatomical complications, however the associated incidence of congenital heart disease ranges from 50-100 percent.

Treatment centres on surgery — considered some of the most challenging procedures in the medical profession. Antibiotics can also increase the survival rate, however the mortality rate is high. A recent study showed the mortality rate for five years from diagnosis as high as 50 percent.

What is Pulmonary Vein Stenosis?

Pulmonary vein stenosis is a condition in which there is a blockage in the blood vessels which bring the oxygen-rich blood from the lungs to the heart.

It occurs due an abnormal process that thickens and narrows the walls of veins, thought to be an uncontrolled growth of connective tissue cells.

The condition can occur as a complication of congenital heart disease but can also occur among children with normal hearts.

Infants quickly develop difficulty breathing and low oxygen levels.

Symptoms include: shortness of breath; fatigue; paleness; poor feeding; and fast heartbeat.

Treatment currently focuses on the removal of obstructions and the prevention of any recurrence of abnormal cell growth.