This situation occurs when there was a large left to right shunt due to holes in the heart and was not treated at a proper time and so presents later in life and that time; patient cannot be treated either by surgery or by intervention. (E.g. large VSD, PDA, atrio-ventricular septal defect etc) and this causes severe irreversible pulmonary hypertension and results in cyanosis.

If female patient is having this situation, then pregnancy is contra-indicated as it can be life threating to mother as well as to the fetus. There is a very high risk maternal mortality and may be due to bleeding from lungs, syncopal attacks due to low cardiac output, air or blood embolism to the brain resulting into stroke etc.

Clinical Presentation

  • Bluishness of skin n mucus membrane along with thickening of fingers and toes
  • Loss of consciousness
  • Right Heart Failure leading to swelling of the body
  • ECG abnormalities
  • Bleeding into the lungs resulting into bleeding from mouth
  • Lack of blood in the body etc.

If child is diagnosed early with significant large shunt, then it can be treated early by surgery or cath lab interventions so that pulmonary hypertension can be avoided.
When it is not repaired early, then option left is either medical management or heart-lung transplantation. But transplantation is in itself needs a very close monitoring of patient after tis and may need lifelong medication.
Medical management includes:

  • Avoid dehydration
  • Daily routine activities
  • Pulmonary vasodilators
  • IE prophylaxis
  • Avoid pregnancy
  • Anticoagulation: not routinely