Scimitar Syndrome

Occurs in 1 to 3–5:100.000 live births. The incidence could be higher because of asymptomatic cases in adult population.

Significant risk of pulm Htn, respiratory failure, and cardiac failure in the periop period.

Intraop pulm Htn crisis, severe bleeding with hypovolemia, worsening left to right shunt, and in critical cases, reverse shunt right to left with acute cardiac failure and cardiac arrest.

Disease characterized by cardiopulmonary anomalies as partial or total anomalous pulmonary venous return connection of the right lung to the inferior caval vein, leading a left to right shunt.

Associated with other anomalies such as hypoplastic right lung, anomalous systemic arterial supply to the right lung with or without pulmonary sequestration, pulm Htn, dextroposition of the heart, heart failure, and atrial septal defect (with ostium secundum being the most frequent).

Etiology is unknown, but in some pts, the anomalous pulmonary venous return has been coded genetically in the chromosome 4q12.