Scimitar Syndrome is a rare disease and arises due to the presence of a pulmonary vein, in the form of a Turkish sword called a scimitar, which drains the right lung into the inferior vena cava rather than into the left atrium of the heart.
The change in vein shape causes changes in the size of the right lung, increased contraction force of the heart, deviation of the heart to the right side, decrease of the right pulmonary artery and abnormal circulation of blood to the right lung.
The severity of Scimitar Syndrome varies from individual to individual, there are patients who have the disease but do not manifest any signs or symptoms throughout life and other individuals who have serious health problems such as pulmonary hypertension, which can lead to death.
Symptoms of Scimitar Syndrome
Symptoms of Scimitar Syndrome can be:
- Shortness of breath;
- Purple skin due to lack of oxygen;
- Chest pain;
- Fatigue;
- Dizziness;
- Catarrh with blood;
- Pneumonia;
- Cardiac insufficiency.
The diagnosis of Scimitar Syndrome is performed through examinations such as chest x-ray, computed tomography and angiography to identify changes in the shape of the pulmonary artery.
Treatment of Scimitar Syndrome
The treatment of the Scimitar Syndrome consists of a surgery that redirects the anomalous pulmonary vein of the inferior vena cava to the left atrium of the heart, normalizing the drainage of the lung.
Treatment should be performed only when there is almost total deviation of blood from the right pulmonary vein to the inferior vena cava or in the case of pulmonary hypertension.
Useful link:
Cardiovascular system
