Anaesthesia Considerations in Scimitar Syndrome

Abstract

Scimitar syndrome is a rare congenital heart disorder characterised by abnormal drainage of pulmonary veins into the inferior vena cava, associated with hypoplasia of the right lung, pulmonary artery, atrial septal defects and other congenital cardiac anomalies. Scimitar syndrome has a very low prevalence in the population but it has a very high perioperative morbidity and mortality due to pulmonary artery hypertension and its sequel. Hence understanding the anaesthetic implications becomes important. This study presents the anaesthetic management of an 11-year-old case of scimitar syndrome planned for adenotonsillectomy under general anaesthesia. After a complete evaluation of the present cardiopulmonary status and obtaining consent from the guardians, the child was taken up for surgery. Perioperatively, all the factors that precipitate pulmonary artery hypertension like hypovolemia, pain, hypoxia, hypothermia, hypercarbia and metabolic acidosis were avoided. Intermittent positive pressure ventilation with low tidal volumes for the management of any underlying lung hypoplasia was maintained. Vasoconstrictors like noradrenaline and inotropes like dobutamine and milrinone were readily available in the event of any untoward cardiac complications. Haemodynamic parameters were stable intraoperatively. Adequate recovery was ensured after which tracheal extubation was performed followed by an uneventful post-operative period.