Uthaman et al. (2008) conducted a systematic study to assess the influence of anomalous systemic arterial supply (ASAS) on the clinical course and management outcome of scimitar syndrome (SS) represented throughout infancy. The study included 23 children diagnosed with SS and the diagnosis was confirmed through cardiac catheterization. Out of the 23 children, 16 (9 males and 7 females) were found to have ASAS with a mean age of 1 to 10 months. Throughout their first year, subjects experienced acute respiratory distress (81%) and recurrent chest infections (19%), of those, nine patients suffered during the neonatal period and three on ventilator. Patients underwent a chest X-ray which revealed an abnormality in all-varying degrees of dextroposition of the heart, hypoplasia of the right lung, right lower lobe opacity, and cardiomegaly. Fifteen infants suffered from pulmonary hypertension and significant left to right shunt, therefore, interruption of the ASAS was performed and reduced the left to right shunt significantly in 93% of patients while one patient passed away post surgically as a result of septicemia. Uthaman et al. (2008) proposed the use of coil embolization of ASAS to avoid surgery for SS during infancy.

Venugopalan et al. (2000) reported a four month old Omani infant with an uncommon type of TAPVD. The patient presented with pneumonia, intermittent cyanosis and poor feeding, since he was two weeks old. He was found to be marasmic, pale, cyanosed, with poor peripheral circulation and oxygen saturation of 85%. Cardiovascular examination revealed cardiomegaly, left parasternal heave, well-split second heart sound, and grade 2/6 ejection systolic murmur over the 3rd and 4th intercostal spaces, with crepetation heard on lung bases and hepatomegaly. Chest X-ray revealed cardiomegaly, patch of pneumonia on right mid zone, and pulmonary congestion. The electrocardiogram showed right axis deviation, right atrial enlargement, and right ventricular hypertrophy, while the echo-doppler study showed marked dilatation of the right atrium, right ventricle and pulmonary arteries, unobstructed connection of the left pulmonary veins to the innominate vein through the vertical vein and superior vena cava, and an obstructed connection of the right pulmonary veins to the coronary sinus along with patent foramen ovale and ductus arteriosus. Diagnosis of a mixed type TAPVD was confirmed by cardiac catheterization and angiography, and surgical correction was carried out. The confluence of the left pulmonary veins was anastamosed to the left atrium and the left vertical vein ligated, while the confluence of the right pulmonary veins was enlarged and the coronary sinus deroofed into the left atrium, followed by closure of foramen ovale. Postoperatively, the child's condition deteriorated, and he required high doses of inotropes to maintain the blood pressure. The infant died in a low output state. The vast contrast between the clinical findings and the underlying condition in this case made Venugopalan and colleagues (2000) to emphasize the need for high suspicion index and the prompt use of echocardiography for early diagnosis of this condition.

Kabbani et al. (2004) described a newborn boy who was admitted to hospital due to congenital cardiac disease.  He presented with a grade 3/6 murmur with hepatomegaly.  Chest radiograph showed shifting of the enlarged heart to the right side, a cystic radiolucent lesion in the middle lobe adjacent to the right cardiac border, and an unusual vertical curvilinear shadow resembling the shape of a scimitar in the left lung.  There were anomalous pulmonary venous connection to the inferior caval vein, horseshoe lungs, and right pulmonary cyst.

Al-Naami and Abu-Sulaiman (2006) described the first two variant forms of Scimitar Syndrome within the same family.  The older affected brother was diagnosed with classical scimitar syndrome. The younger brother presented at 2-months of age with unrestricted VSD, an ASD of moderate size, repeated lung infections, and a failure to thrive. Radiological and other analysis revealed cardiomegaly, congested lung fields, and biventricular hypertrophy. Cross-sectional ECG showed a systemic collateral artery originating from the abdominal aorta and supplying the lower left pulmonary lobe, as well as an abnormal tortuous course of the right lower pulmonary vein, which formed a loop before entering the left atrium. This was confirmed by cardiac catheterization, during which the collateral artery was coiled. The VSD and ASD were both closed, and the patient improved significantly. Considering the variability in morphology in this family, Al-Naami and Abu-Sulaiman (2006) recommended that the genetic variability of abnormalities of the pulmonary venous system needed further studied.