Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial or total anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia and systemic arterial supply of a variable degree to the right lung. The origin of the name “scimitar” comes from the curvilinear shadow along the posterior-anterior chest x-ray, representing the anomalous pulmonary venous connection of the hypoplastic right lung.
Surgical correction of this malformation is usually accomplished in symptomatic patients or in patients with an increased pulmonary blood flow and signs of right heart chambers dilation. However the specific indication to surgery, the ideal treatment and the long term out come are still debating.
This monograph gives an overview of the various aspect of this unusual congenital heart malformation focusing also on recent therapeutic results.

Preface. Aldo R. Castañeda
Introduction. Vladimiro Vida
1. Definition
Vladimiro Vida
2. Historical Notes
Gaetano Thiene, Richard Van Praagh
3. Anatomy and Embryology
Carla Frescura, Annalisa Angelini, Gaetano Thiene
4. Epidemiology, Physiopathology and Diagnosis
Ornella Milanesi, Maddalena Padrini, Raffaella Motta, Vladimiro Vida
5. Medical and interventional therapies
Ornella Milanesi, Maddalena Padrini, Vladimiro Vida
6. Surgical treatment
Vladimiro Vida, Giovanni Stellin

Vladimiro L. Vida. Born in 1973. Studied Medicine and Cardiac Surgery at the University of Padua, Italy. Senior fellow in pediatric cardiac surgery in Guatemala with professor Aldo Castañeda (Emeritus Professor Harvard Medical School) in 2004-2005 and senior fellow and chief resident at the Children’s Hospital Boston-Harvard Medical School with professor Pedro del Nido in 2006-2007. Accomplished a PhD in cardiovascular science in 2009, a master in pediatric cardiology (focusing of hybrid surgery) in 2011 and a master in cardiovascular pathology in 2012. Since 2008 working as a staff pediatric cardiac surgeon at the University of Padua, Italy.