Scimitar syndrome with tetralogy of fallot and pulmonary atresia

Tetralogy of Fallot with Scimitar syndrome in VACTERL: a very rare association

“…The occurrence of Scimitar syndrome in VACTERL and its association with another cardiac congenital anomaly such as TOF is extremely rare. [5][6][7][8] In the present case, we observed that the conventional management of Scimitar syndrome was altered by the association of TOF with severe infundibular pulmonary stenosis. The child was severely cyanosed with frequent squatting episodes.…”

Surgical correction of rare anomaly of scimitar syndrome with tetralogy of Fallot

“…SS was first described by Cooper in London and Chassina G. in Paris in 1836; however, it was Neill and associates in 1960 who coined the term “scimitar syndrome.” 3 The incidence of SS was reported as 0.5%–1% of CHD 4 …”

“…Connection of scimitar vein most commonly occurs to IVC to infradiaphragmatic portion, however, variability exists and it may end in supradiaphragmatic portion also 3 . These anomalous vessels may be visible on chest X‐ray as a curvilinear shadow just above the right diaphragm resembling a Turkish sword or scimitar hence its name 4 …”

“…3 The incidence of SS was reported as 0.5%-1% of CHD. 4 Scimitar vein is usually single and most commonly drains the entire right lung, however, variations exist. 1 Connection of scimitar vein most commonly occurs to IVC to infradiaphragmatic portion, however, variability exists and it may end in supradiaphragmatic portion also.…”

“…3 These anomalous vessels may be visible on chest X-ray as a curvilinear shadow just above the right diaphragm resembling a Turkish sword or scimitar hence its name. 4 As it is a cardiopulmonary anomaly, pulmonary anomalies are common as well, the right lung is hypoplastic in 80%-90% of cases.…”