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