Two Cases of Scimitar Syndrome: An Adolescent and an Older Adult with Late Diagnosis

Abstract

Introduction: Scimitar syndrome, a rare form of congenital heart disease (CHD), is characterized by anomalous pulmonary venous return into the inferior vena cava, hypoplasia of the right lung, and dextroposition of heart. The most common form of the syndrome known as infantile form, is diagnosed in infancy and is surgically corrected. Asymptomatic cases are occasionally diagnosed in adolescence. However, there are rare cases which remain undiagnosed until older ages despite some manifestations of the disease. This is in part due to the nonspecific nature of this manifestations which necessitates a specific workup for the diagnosis and treatment. Case Presentation: We report two cases of scimitar syndrome in female patients, one 17 years old and another 48 years old. Here we described the symptoms, diagnosis and clinical course of these patients and reviewed the literature on Scimitar syndrome particularly in adolescents and adults. Conclusions: Although Scimitar syndrome has early manifestations in most of the patients, its late diagnosis is sometimes seen in adolescents and adults due to misdiagnosis. Surgical repair can correct the blood flow route and improve the outcomes.