To evaluate respiratory morbidities and lung function tests in the cohort of patients with scimitar syndrome evaluated at our center since 1976.

Eighty-one children were investigated. Twenty-six patients died, all with the infantile form. The median duration of follow-up of surviving children was 7.2 years.

A high rate of respiratory morbidities was measured, with 38% and 43% of children reporting pulmonary infections or wheezing episodes during the last 12 months of follow-up, respectively. One-third of children have been rehospitalized for a respiratory cause. Lung function tests were obtained in 20 children. The median value of total lung capacity was 73.0% of the predicted value (IQR, 65.3-86.8), and the median value of the ratio of the forced expiratory volume in one second to the forced vital capacity was −1.26 Z score (−2.25; −0.31). Significantly lower total lung capacity values were obtained in children with the infantile form (P < .005) or with a history of thoracic surgery (P = .002). The ratio of the forced expiratory volume in one second to the forced vital capacity Z score values were significantly lower in boys (P < .05) and in children with a history of wheezing (P = .01). Wheezing episodes were not associated with significant salbutamol-induced reversibility.

Respiratory complications frequently are observed in children with scimitar syndrome. Pulmonary hypoplasia appears as an independent marker of long-term severity in these patients.