Scimitar syndrome

Scimitar syndrome consists of a partial anomalous pulmonary venous drainage of right lung, right lung hypoplasia, dextraposition of heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung. This syndrome has varied presentations, from an asymptomatic state¹ to severe pulmonary hypertension and/or heart failure.² Those who present early in life usually have associated congenital heart disease also.³ Hemoptysis⁴ and pulmonary arterial hypertension (PAH)^5,6 both are uncommon presenting complaints of this rare syndrome beyond infancy.