A case series of five infants with scimitar syndrome

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply to the right lung. To date, this syndrome has not been reported or discussed in the nursing literature. In this article, 5 patients are presented to illustrate the variable presentation of this syndrome, ranging from infants who are asymptomatic to those with heart failure and severe pulmonary hypertension. The clinical signs and symptoms, diagnostic dilemmas, and medical and surgical management are discussed. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to the possibility of this syndrome.