Scimitar Syndrome

An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.

Also Known As:

Networked: 65 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Bronchopulmonary Sequestration (Pulmonary Sequestration)
2.	Atrial Heart Septal Defects (Atrial Septal Defect)
3.	Pathologic Constriction (Stenosis)
4.	Bronchogenic Cyst
5.	Ventricular Heart Septal Defects (Ventricular Septal Defect)

Experts

1.	Peuster, Matthias: 2 articles (11/2011 - 01/2003)
2.	Giordano, Raffaele: 1 article (08/2015)
3.	Chakraborty, Subhasis: 1 article (08/2015)
4.	Chowdhury, Moti M: 1 article (08/2015)
5.	Cantinotti, Massimiliano: 1 article (08/2015)
6.	Spadoni, Isabella: 1 article (08/2015)
7.	Halkos, Michael E: 1 article (07/2015)
8.	Murphy, Douglas A: 1 article (07/2015)
9.	Moss, Emmanuel: 1 article (07/2015)
10.	Aldoss, Osamah: 1 article (06/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Scimitar Syndrome:

1.	VACTERL associationIBA 03/01/2015 - "In this study, we report on a neonate with prenatal suspicion of VACTERL association and small left-sided cardiac structures, which, only on postnatal angiography, could be revealed to be part of a Scimitar syndrome. " 03/01/2015 - "As this is the second reported case of VACTERL association and Scimitar syndrome, the presence of Scimitar syndrome should be considered in the prenatal and postnatal evaluation of VACTERL association. " 03/01/2015 - "VACTERL association and Scimitar syndrome are rare congenital diseases. " 03/01/2015 - "Scimitar syndrome in a case with VACTERL association." 04/01/2013 - "We report on a two-month-old infant with an unusual form of scimitar syndrome, associated with an absent right pulmonary artery, obstructed inferior vena cava, hemi-azygous continuation and the VACTERL association. "
2.	Pulmonary venous return anomalyIBA 09/01/2014 - "Scimitar syndrome is a congenital anomaly occurring in approximately 1/50,000 births, consisting of partial anomalous pulmonary venous return, right lung hypoplasia, and several associated defects. " 06/01/2014 - "Scimitar syndrome (pulmonary venolobar syndrome) is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. " 03/01/2014 - "Three-dimensional echocardiography images showing anomalous pulmonary venous return in an adult with scimitar syndrome." 10/01/2013 - "Scimitar syndrome is a rare and complex congenital anomaly characterized by partial or complete anomalous pulmonary venous return from the right or left lung into the inferior vena cava, through drainage into the hepatic vein, right atrium or left atrium. " 01/01/2013 - "Scimitar syndrome, a rare congenital cardiopulmonary condition, presents in both pediatric and adult populations as an anomalous pulmonary venous return of most of the right lung to the inferior vena cava. "
3.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 08/01/2015 - "We present a rare case of scimitar syndrome in which the scimitar vessel, collecting all the right pulmonary veins, was stenotic at its junction, with the inferior caval vein and two anomalous vessels, connecting to the same venous collector, draining most of the flow to the left atrium. " 10/01/2013 - "Scimitar syndrome of atypical, rare drainage of venous vessel to the superior vena cava. " 06/01/2012 - "The appearance of the vessel resembles Turkish scimitar; therefore, the syndrome is called scimitar syndrome. " 12/01/2011 - "In a girl suffering from "Scimitar syndrome", a rerouting of the scimitar vein was performed at the age of 6 years, but no embolisation of the aberrant systemic vessel was done. " 11/01/2005 - "Such cases have been described as "scimitar syndrome." We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. "
4.	GadoliniumIBA 01/01/2005 - "We report definition of both abnormal pulmonary venous drainage and anomalous systemic arterial supply by gadolinium-enhanced magnetic resonance angiography and three-dimensional computerized tomography in an eight month-old infant with scimitar syndrome. " 10/01/2003 - "Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI." 01/01/2000 - "Gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation."
5.	Lung agenesisIBA 10/01/2010 - "The most commonly encountered anomalies include lung agenesis-hypoplasia complex (pulmonary underdevelopment), congenital pulmonary airway malformations, congenital lobar overinflation, bronchial atresia, bronchogenic cysts, congenital high airway obstruction syndrome, scimitar syndrome, and bronchopulmonary sequestration. " 05/01/2011 - "Unilateral hyperlucent hemithorax has a broad differential diagnosis that includes unilateral emphysematous or bullous disease, pneumatocele, foreign body aspiration, Swyer-James syndrome, congenital lobar emphysema, endobronchial mass, unilateral pulmonary agenesis, proximal interruption of the pulmonary artery, scimitar syndrome, diaphragmatic hernia, and Poland syndrome. "
6.	Congenital lobar emphysemaIBA 01/01/2004 - "Specific topics addressed include tracheal conditions such as tracheal stenosis, tracheomalacia, tracheal bronchus, tracheal atresia, and bronchogenic cyst; anomalies of the lung such as lung underdevelopment (agenesis and hypoplasia), scimitar syndrome, congenital cystic adenomatoid malformation, congenital lobar emphysema, and pulmonary sequestration; esophageal anomalies such as esophageal atresia, tracheoesophageal fistula, and esophageal duplications; and vascular rings. " 05/01/2011 - "Unilateral hyperlucent hemithorax has a broad differential diagnosis that includes unilateral emphysematous or bullous disease, pneumatocele, foreign body aspiration, Swyer-James syndrome, congenital lobar emphysema, endobronchial mass, unilateral pulmonary agenesis, proximal interruption of the pulmonary artery, scimitar syndrome, diaphragmatic hernia, and Poland syndrome. "
7.	Coarctation of the aortaIBA 12/01/2005 - "Associated cardiac anomalies included ostium secundum atrial septal defect in 5 patients and ventricular septal defect in 2 patients, pulmonary artery sling in 4 patients, patent ductus arteriosus in 6 patients, atrioventricular septal defect in 1 patient, aortic arch hypoplasia in 1 patient, coarctation of the aorta in 1 patient, and partial anomalous pulmonary venous connection in 2 patients, one of them with "scimitar syndrome." Tracheal origin of the right upper lobe was diagnosed in 2 of the patients. " 09/01/2003 - "A male infant presenting with severe heart failure and respiratory distress was diagnosed with a hypoplastic right lung, scimitar syndrome with pulmonary sequestration and multiple anomalous systemic arteries, left bronchomalacia, a large atrial septal defect and coarctation of the aorta. "
8.	SiliconIBA 03/01/2005 - "Embolization of the systemic arterial supply via a detachable silicon balloon in a child with scimitar syndrome." 03/01/2005 - "We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. "
9.	Epoprostenol (Prostacyclin)FDA LinkGeneric 01/01/2003 - "We present an infant who had an operation for scimitar syndrome and abnormal drainage of the right pulmonary veins into the inferior vena cava who developed pulmonary edema while receiving a prostacyclin infusion. " 01/01/2003 - "Acute pulmonary edema caused by epoprostenol infusion in a child with scimitar syndrome and pulmonary hypertension."
10.	Polytetrafluoroethylene (Teflon)FDA Link 07/01/2015 - "We present two adult patients in whom anatomic correction of scimitar syndrome (SS) was accomplished by redirecting the anomalous pulmonary venous drainage into the left atrium using a reinforced polytetrafluoroethylene (PTFE) graft extension. "

Therapies and Procedures

1.	Pneumonectomy (Lung Volume Reduction) 10/01/2010 - "Emergency transmediastinal pneumonectomy for scimitar syndrome." 09/01/2006 - "Extrapleural pneumonectomy for scimitar syndrome." 01/01/2004 - "Scimitar syndrome treated with pneumonectomy: a case associated with bronchospastic attack." 05/01/1994 - "Scimitar syndrome complicated with right pneumonectomy syndrome: report of one case." 10/01/2010 - "Repair of scimitar syndrome presenting in infancy involves either tunneling or reimplantation of the anomalous vein to the left atrium and may be fraught with serious complications such as thrombosis and secondary pulmonary infarction necessitating pneumonectomy. "
2.	X-Ray Film 10/01/2010 - "The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. " 11/01/1996 - "On chest x-ray films, an abnormal shadow resembling scimitar syndrome was seen in two patients. " 01/01/1987 - "Therefore, the diagnosis of scimitar syndrome cannot be made with certainty from a plain x-ray film."
3.	Catheters 09/01/2014 - "Abnormal location of umbilical venous catheter due to Scimitar syndrome." 07/01/2006 - "Catheter-based closure of an atrial septal defect in scimitar syndrome."
4.	Thoracotomy 03/01/2014 - "Right thoracotomy, off-pump, scimitar syndrome repair in infants." 02/01/2003 - "This clinical experience indicates that an alternative surgical approach to scimitar syndrome is direct anastomosis of the scimitar vein to the posterior aspect of the left atrium using a right thoracotomy without cardiopulmonary bypass. "
5.	Replantation 01/01/2006 - "Direct reimplantation technique via a right minithoracotomy for scimitar syndrome: a case report." 10/01/2010 - "Repair of scimitar syndrome presenting in infancy involves either tunneling or reimplantation of the anomalous vein to the left atrium and may be fraught with serious complications such as thrombosis and secondary pulmonary infarction necessitating pneumonectomy. "