Respiratory outcome in children with scimitar syndrome.

OBJECTIVE

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

STUDY DESIGN

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.

RESULTS

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.

CONCLUSION

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