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: 167 relevant articles (3 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pulmonary Vein Stenosis
2.	Birth Weight (Birth Weights)
3.	Pulmonary Hypertension
4.	Scimitar Syndrome
5.	Ventricular Heart Septal Defects (Ventricular Septal Defect)

Experts

1.	Bichell, David P: 2 articles (12/2022 - 11/2019)
2.	Andersen, Nicholas D: 2 articles (07/2021 - 10/2020)
3.	Turek, Joseph W: 2 articles (07/2021 - 10/2020)
4.	Lin, Angela E: 2 articles (11/2015 - 04/2011)
5.	Schranz, Dietmar: 2 articles (10/2013 - 03/2002)
6.	Chen, Yi-Yung: 2 articles (09/2013 - 09/2006)
7.	Kirshbom, P M: 2 articles (03/2008 - 01/2008)
8.	Kogon, B E: 2 articles (03/2008 - 01/2008)
9.	Masterson, K: 2 articles (03/2008 - 01/2008)
10.	Simsic, J M: 2 articles (03/2008 - 01/2008)

Drugs and Biologics

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

1.	GadoliniumIBA 10/01/2006 - "Twenty consecutive patients (10 male, mean age: 27+/-20 years) with suspected partial anomalous pulmonary venous return underwent a magnetic resonance study comprehensive of Gadolinium-enhanced three-dimensional magnetic resonance angiography and phase-velocity-contrast in order to evaluate pulmonary and systemic venous anatomy and QP/QS. " 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."
2.	esmolol (Brevibloc)FDA LinkGeneric 11/01/1996 - "Application of ultra short acting beta blockade (esmolol) in pediatric open heart surgery: a trial in total anomalous pulmonary venous return." 09/01/1996 - "[Repair of total anomalous pulmonary venous return using an ultra-short acting beta blockade (esmolol)--case reports]." 11/01/1996 - "In the repair of total anomalous pulmonary venous return (TAPVR) under cardiopulmonary bypass, esmolol, ultra short acting beta blocker, was applied to obtain low heart rate and weak ventricular contraction under mild hypothermic cardiopulmonary bypass. " 09/01/1996 - "In 2 infants with asplenic syndrome associated with total anomalous pulmonary venous return (TAPVR), a repair of TAPVR was done under mild hypothermic cardiopulmonary bypass (CPB) using esmolol, an ultra short-acting beta-blocker to obtain good operative field without aortic-cross clamping. "
3.	4- ((1,4,8,11- tetraazacyclotetradec- 1- yl)methyl)benzoic acidIBA 06/01/2012 - "The cases demonstrate that, although uncommon, partial anomalous pulmonary venous return can be a contributing factor to pulmonary hypertension and pulmonary veins should be carefully examined when reading a CTPA study."
4.	Nitric Oxide (Nitrogen Monoxide)FDA Link 05/01/1999 - "Adverse hemodynamic effects observed with inhaled nitric oxide after surgical repair of total anomalous pulmonary venous return." 05/01/1999 - "The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. " 12/01/1995 - "Transient effects of inhaled nitric oxide for prolonged postoperative treatment of hypoxemia after surgical correction of total anomalous pulmonary venous return." 07/01/1994 - "Successful use of inhaled nitric oxide for treatment of severe hypoxemia in an infant with total anomalous pulmonary venous return." 08/01/1993 - "Inhaled nitric oxide in pulmonary hypertension after total repair of total anomalous pulmonary venous return."
5.	Anesthetics (Anesthetic Agents)IBA 01/01/2023 - "This case report highlights the challenges during liver transplantation, and perioperative anesthetic management of a patient with an uncorrected Scimitar Syndrome." 11/09/2018 - "Anesthetic considerations for cesarean section in a parturient complicated by Scimitar syndrome-like pathophysiology." 11/01/2018 - "Herein, we discuss positional oxygenation and hemodynamic changes associated with scimitar syndrome, especially in the left lateral decubitus position, detected during preoperative anesthetic examination in an adult patient who underwent general anesthesia for left mastectomy." 02/01/2022 - "This educational review will focus on the pathophysiology, clinical manifestations, surgical approaches, and anesthetic management of congenital anomalies of the large intrathoracic airways: congenital tracheal stenosis, tracheal agenesis, tracheal diverticulum, bronchial anomalies (tracheal, esophageal, or bridging bronchus), congenital lung malformations, lung sequestrations and Scimitar syndrome, lobar emphysema, Williams-Campbell syndrome, and pleuropulmonary blastoma. "
6.	Coenzyme A (CoA)IBA 01/27/2023 - "Early mortality varied by birthweight (VLBW 56%, MLBW 15%, LBW 16%; p = 0.003) and CHD types (hypoplastic left heart syndrome (HLHS) 50%, total anomalous pulmonary venous return (TAPVR) 47%, transposition of the great arteries (TGA) 34%, coarctation of the aorta (CoA) 16%; p = 0.13). " 06/14/2015 - "Distinct cardiac phenotypes were present in four of these subjects with non-recurrent de novo deletions (range 0.08 Mb-1.4 Mb) in the subtelomeric region of 17q25.3. These included coarctation of the aorta (CoA), total anomalous pulmonary venous return (TAPVR), ventricular septal defect (VSD) and atrial septal defect (ASD). " 05/01/2010 - "Ventricular septal defect (VSD; 4.0) was the most common defect, followed by secundum atrial septal defect (ASDII; 3.2), patent ductus arteriosus (PDA; 2.0), pulmonary stenosis (PS; 1.2), tetralogy of Fallot (TOF; 0.63), coarctation of aorta (CoA; 0.25), transposition of great arteries (TGA; 0.21), endocardial cushion defect (ECD; 0.20), double outlet of right ventricle (DORV; 0.15), total anomalous pulmonary venous return (TAPVR; 0.11), aortic stenosis (0.09), hypoplastic left heart syndrome (HLHS; 0.062), Ebstein anomaly (0.047), and tricuspid atresia (0.046). "
7.	Warfarin (Coumadin)FDA LinkGeneric 01/01/2022 - "A diagnostic algorithm stratifying on absence of pulmonary veins followed by an abnormal Coumadin ridge, can correctly identify cases of TAPVR with high specificity (90.5%) and moderate sensitivity (61.9%). " 01/01/2022 - "The most common ultrasound marker of TAPVR was absence of pulmonary vein entry into the LA (42.9%), followed by abnormal Coumadin ridge (38.1%). " 04/13/1999 - "Twenty patients (15+/-14 years) with PHT and associated CHD (9 atrial septal defect, 7 ventricular septal defect, 4 transposition of the great vessels, 3 patient ductus arteriosus, 3 partial anomalous pulmonary venous return, and 1 aortopulmonary window) who failed conventional therapy (including digitalis; diuretics; oxygen; warfarin; calcium channel blockade, if indicated; and surgery, if operable) were treated with chronic PGI2. "
8.	Vasodilator Agents (Vasodilators)IBA 07/01/2001 - "OBJECTIVE: To report a fatal outcome from pulmonary hemosiderosis in an infant with scimitar syndrome after prolonged pulmonary vasodilator therapy. " 12/01/2022 - "Partial anomalous pulmonary venous return is a rare congenital heart defect that can escape detection until adulthood and can be misdiagnosed as pulmonary hypertension and managed with vasodilators before the anomaly is identified. "
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." 02/01/2004 - "[Prostacyclin oral internal therapy for severe pulmonary hypertension after total correction of cor triatriatum with partial anomalous pulmonary venous return; report of a case]."
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. " 09/01/2020 - "Left-sided partial anomalous pulmonary venous return (left pulmonary veins to left vertical vein) was repaired in a 70-year-old patient by ligation of the vertical vein and connection of the pulmonary veins to the left atrial appendage using a ringed polytetrafluoroethylene tube graft. "

Therapies and Procedures

1.	Cardiopulmonary Bypass (Heart-Lung Bypass) 10/01/1991 - "In the group of neonates with TAPVR, operative mortality improved to 7% (1 of 14) in Group II compared to that of 60% (3 of 5) in Group I. CVP and LAP measured immediately after cessation of cardiopulmonary bypass (CPB) were lower in Group II and the value of CPK-MB was significantly lower in Group II. There was a significant negative correlation (r = -0.695, p less than 0.05) between CPB time and urine volume for 72 hours after operation. " 06/01/2017 - "An alternative cardiopulmonary bypass strategy for intracaval baffle repair of scimitar syndrome." 11/01/2016 - "Since 2005, we have exclusively repaired all types of TAPVR using continuous cardiopulmonary bypass. " 11/01/2016 - "Continuous Cardiopulmonary Bypass During the Repair of Total Anomalous Pulmonary Venous Return." 10/01/2016 - "We report a case of ECMO performed in a 1,360-g premature infant with VLBW due to cardiopulmonary bypass weaning failure after repairing infracardiac total anomalous pulmonary venous return."
2.	Extracorporeal Membrane Oxygenation 02/01/2020 - "In addition, recent group had more comorbidities including chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies (5.6% vs. 3.6%, 2.3% vs. 1.7%, and 5.6% vs. 3.6%, respectively, p < 0.001); these were associated with a higher rate of extracorporeal membrane oxygenation utilization (9.2% vs. 4.5%, p < 0.001). " 10/01/2016 - "Extracorporeal Membrane Oxygenation in a 1,360-g Premature Neonate after Repairing Total Anomalous Pulmonary Venous Return." 03/01/2023 - "Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; P < .001), total anomalous pulmonary venous return (hazard ratio, 4.3; 95% confidence interval, 1.7-10.8; P = .002), and birth weight 2.5 kg or less (hazard ratio, 2.4; 95% confidence interval, 1.0-5.4; P = .041) to be independent risk factors for mortality in all-comers. " 11/01/2010 - "All infants with a diagnosis of TAPVR as an isolated lesion who underwent surgical repair at Children's Hospital of Wisconsin from 1991 to 2007 were reviewed to assess location of drainage, presence of obstruction, age at presentation, age at surgery, death, need for extracorporeal membrane oxygenation (ECMO), length of hospital stay, length of mechanical ventilation (MV), and late pulmonary venous obstruction. " 05/01/2015 - "The recipient was a 26-year-old woman with idiopathic pulmonary arterial hypertension and the graft ischemic time was prolonged significantly because of the time required to induce peripheral veno-arterial extracorporeal membrane oxygenation (V-A ECMO) under local anesthesia, and address the severe intrathoracic and pericardial adhesions from past surgery for partial anomalous pulmonary venous return, with concurrent annular plication of the tricuspid valve. "
3.	Therapeutics 11/01/1998 - "This anomaly is usually characterized by the presence of anomalous venous drainage of the right lung to the inferior vena cava, abnormal bronchial segmentation, and alterations of pulmonary artery and right lung vascularization.METHODS: The authors report a case of Scimitar Syndrome in an 8 year-old-child, discussing its diagnostic methods and surgical treatment.RESULTS: The child presented good outcome after surgical therapy, with an uneventful follow-up.CONCLUSION: The Scimitar Syndrome is a rare anomaly, which may present few clinical manifestations. " 06/30/1972 - "[Clinical features and therapy of anomalous pulmonary venous return]." 11/01/2020 - "Pumpless Lung Assist as a Bridge to Medical Therapy in a Teenager With Pulmonary Arterial Hypertension and Partial Anomalous Pulmonary Venous Return." 07/01/2001 - "OBJECTIVE: To report a fatal outcome from pulmonary hemosiderosis in an infant with scimitar syndrome after prolonged pulmonary vasodilator therapy. " 02/01/2004 - "[Prostacyclin oral internal therapy for severe pulmonary hypertension after total correction of cor triatriatum with partial anomalous pulmonary venous return; report of a case]."
4.	Length of Stay 02/01/2020 - " is new:• The study utilized a national database to compare in-hospital mortality and length of stay between the two time periods 1998-2005 and 2006-2014.• The recent group had more comorbidities (prematurity, chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies), and there was higher rate of ECMO and longer length of stay, while mortality rates decreased by 20%." 11/05/2020 - "The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. " 11/01/2010 - "All infants with a diagnosis of TAPVR as an isolated lesion who underwent surgical repair at Children's Hospital of Wisconsin from 1991 to 2007 were reviewed to assess location of drainage, presence of obstruction, age at presentation, age at surgery, death, need for extracorporeal membrane oxygenation (ECMO), length of hospital stay, length of mechanical ventilation (MV), and late pulmonary venous obstruction. "
5.	Arterial Switch Operation 12/01/2014 - "Notably, 63% of the neonates were extubated within 24 hours, including 67% of arterial switch operations and 54% of total anomalous pulmonary venous return repairs. " 01/01/2008 - "The device was used on six pediatric patients to facilitate correction or palliation of their cardiac defects, which included two arterial switch operations, two truncus arteriosus repairs, one stage 1 Norwood and one repair of total anomalous pulmonary venous return. " 11/01/1997 - "Double switch operation for corrected transposition with total anomalous pulmonary venous return." 11/01/1990 - "These lesions, encountered singly or in association, were single atrium (2), previous Mustard operation (1), hypoplastic left atrium (1), anomalous systemic venous return (3), anomalous pulmonary venous return (2), transposition or malposition of the great vessels (5), pulmonary artery hypoplasia (1). " 05/01/2021 - "We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. "