Scimitar syndrome associated with absence of the right pulmonary artery and a persistent primitive hepatic venous plexus

2005 ◽  
Vol 15 (2) ◽  
pp. 216-218 ◽  
Author(s):  
Gabriella Agnoletti ◽  
Damien Bonnet ◽  
Jacques De Blic
Keyword(s):  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Venous Plexus ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Right Pulmonary Artery ◽  
Unusual Variant ◽  
The Right

An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.

Variants of the scimitar syndrome

2015 ◽  
Vol 26 (5) ◽  
pp. 941-947 ◽  
Author(s):  
Ilaria Bo ◽  
Julene S. Carvalho ◽  
Emma Cheasty ◽  
Michael Rubens ◽  
Michael L. Rigby
Keyword(s):  
Venous Drainage ◽  
Scimitar Syndrome ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Inferior Caval Vein ◽  
Small Series ◽  
Right Pulmonary Artery ◽  
Aortopulmonary Collateral ◽  
The Right ◽  
Lost To Follow Up

AbstractIntroductionThe scimitar syndrome comprises hypoplastic right pulmonary artery and lung, anomalous right pulmonary venous drainage to the inferior caval vein, aortopulmonary collateral(s) to the right lung, and bronchial anomalies.AimThe aim of this study was to describe the morphological and clinical spectrum of variants from the classical scimitar syndrome in a single institution over 22 years.ResultsIn total, 10 patients were recognised. The most consistent feature was an aortopulmonary collateral to the affected lung (90%), but there was considerable variation in the site and course of pulmonary venous drainage. This was normal in 3 (one with meandering course), anomalous right to superior caval vein in 1, to the superior caval vein and inferior caval vein in 2, and to the superior caval vein and the left atrium in 1; one patient had a right pulmonary (scimitar) vein occluded at the insertion into the inferior caval vein but connected to the right upper pulmonary vein via a fistula. There were two left-sided variants, one with anomalous left drainage to the coronary sinus and a second to the innominate vein.Among all, three patients had an antenatal diagnosis and seven presented between 11 and 312 months of age; 90% of the patients were symptomatic at first assessment.All the patients underwent cardiac catheterisation; collateral embolisation was performed in 50% of the patients. Surgical repair of the anomalous vein was carried out in two patients, one patient had a right pneumonectomy, and one patient was lost to follow-up. There was no mortality reported in the remainder of patients during the study period.ConclusionThe heterogeneity of this small series confirms the consistent occurrence of an anomalous arterial supply to the affected lung but considerable variation in pulmonary venous drainage.

Systemic arterial supply of the right lung with venous drainage to the pulmonary arterial circuit

2011 ◽  
Vol 21 (6) ◽  
pp. 710-712 ◽  
Author(s):  
Peter Fritsch ◽  
Freyja-Maria Smolle-Juettner ◽  
Andreas Gamillscheg
Keyword(s):  
Pulmonary Artery ◽  
Venous Drainage ◽  
Left Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Arterial Supply ◽  
Respiratory Problems ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right ◽  
Systemic Arterial Supply

AbstractIn 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. She presented with recurring respiratory problems 13 years later. An angiography revealed an invert flow from the aberrant systemic vessel via the right pulmonary artery into the left pulmonary artery. After pneumonectomy, she recovered well.

Congenitally palliated scimitar syndrome

2014 ◽  
Vol 25 (6) ◽  
pp. 1218-1220 ◽  
Author(s):  
Massimiliano Cantinotti ◽  
Raffaele Giordano ◽  
Isabella Spadoni
Keyword(s):  
Left Atrium ◽  
Rare Case ◽  
Pulmonary Veins ◽  
Scimitar Syndrome ◽  
Caval Vein ◽  
Anatomical Variant ◽  
Inferior Caval Vein ◽  
The Right

AbstractWe 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.We arbitrarily defined this rare anatomical variant as a congenitally palliated scimitar syndrome.

Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced, reversible, left-sided pulmonary hypertension

1992 ◽  
Vol 13 (2) ◽  
pp. 119-121 ◽  
Author(s):  
Albrecht Beitzke ◽  
Gerfried Zobel ◽  
Bruno Rigler ◽  
J�rg Ingolf Stein ◽  
Christa Suppan
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Right Pulmonary Artery ◽  
The Right

Unusual right pulmonary artery–to-left atrial communication associated with scimitar syndrome

2021 ◽  
pp. 1-3
Author(s):  
Asish R. Mohakud ◽  
Sangamesh Bawage ◽  
Kothandam Sivakumar
Keyword(s):  
Pulmonary Artery ◽  
Blood Supply ◽  
Left Atrial ◽  
Venous Drainage ◽  
Scimitar Syndrome ◽  
Rare Association ◽  
Different Types ◽  
Right Pulmonary Artery ◽  
The Right

Abstract Right pulmonary artery to left atrial fistula is classified based on the right pulmonary artery branching, individual right pulmonary venous drainage, and presence of an aneurysmal segment. A rare association with scimitar syndrome and right lung devoid of blood supply from right pulmonary artery is described in this report. The anatomical and management differences between the different types are highlighted.

Unilateral unidirectional superior cavopulmonary anastomosis in a patient with bilateral superior caval veins and atretic left pulmonary artery

2018 ◽  
Vol 29 (3) ◽  
pp. 416-418
Author(s):  
Sachin Talwar ◽  
Mayank Yadav ◽  
Shiv Kumar Choudhary
Keyword(s):  
Pulmonary Artery ◽  
Left Pulmonary Artery ◽  
Prosthetic Material ◽  
Univentricular Heart ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Cavopulmonary Anastomosis ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Patent Arterial Duct

AbstractA unidirectional superior cavopulmonary anastomosis was performed on the right side in a patient with a functionally univentricular heart, atresia of main and left pulmonary artery, bilateral superior caval veins, and a patent arterial duct in the right pulmonary artery. Anastomosis of the left superior caval vein to the right superior caval vein created a neo-innominate vein without using prosthetic material.

Unusual variant of tetralogy of Fallot with pulmonary atresia: a right pulmonary artery from the ascending aorta and a coronary-to-left pulmonary artery collateral

2014 ◽  
Vol 25 (3) ◽  
pp. 554-556
Author(s):  
Betul Yilmaz ◽  
Matthew A. Crystal ◽  
Brett R. Anderson
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Ascending Aorta ◽  
Unusual Combination ◽  
Right Pulmonary Artery ◽  
Unusual Variant ◽  
Aortopulmonary Collateral ◽  
The Right

AbstractWe present the case of a newborn with tetralogy of Fallot and pulmonary atresia, with a right pulmonary artery from the ascending aorta, and a left pulmonary artery arising from the right coronary artery via an indirect aortopulmonary collateral. The embryogenesis of this unusual combination of pulmonary blood supply has significant implications when considering normal migration of the aortopulmonary septum.

Meandering pulmonary veins mimicking scimitar syndrome

2018 ◽  
Vol 28 (10) ◽  
pp. 1171-1173
Author(s):  
Ravindra S. Pawar ◽  
Vimal Raj ◽  
Suresh Pujar V
Keyword(s):  
Rare Case ◽  
Pulmonary Veins ◽  
Lower Lobe ◽  
Venous Drainage ◽  
Scimitar Syndrome ◽  
Caval Vein ◽  
Descending Aorta ◽  
Inferior Caval Vein ◽  
The Right ◽  
Systemic Arterial Supply

AbstractScimitar or pulmonary venolobar syndrome, a rare pulmonary anomaly, consists basically of anomalous pulmonary venous drainage of the right lung to the inferior caval vein, anomalous systemic arterial supply to the right lower lobe from the descending aorta, hypoplasia of the right lung, and dextroposed heart. We present a rare case with constellation of all these findings of scimitar syndrome, but with the aberrant pulmonary vein draining into the left atrium.

Role of caval venous manipulation in treatment of protein-losing enteropathy

2008 ◽  
Vol 18 (3) ◽  
pp. 275-281 ◽  
Author(s):  
Shaji Menon ◽  
Donald Hagler ◽  
Frank Cetta ◽  
Peter Gloviczki ◽  
David Driscoll
Keyword(s):  
Cardiac Disease ◽  
Fontan Operation ◽  
Restrictive Cardiomyopathy ◽  
Venous Occlusion ◽  
Caval Vein ◽  
Protein Losing Enteropathy ◽  
Inferior Caval Vein ◽  
Extracardiac Conduit ◽  
Right Pulmonary Artery ◽  
The Right

AbstractProtein-losing enteropathy has been associated with the Fontan operation, restrictive cardiomyopathy, constrictive pericarditis, tricuspid valvar stenosis and insufficiency, and superior caval venous obstruction. The mechanism of development of this complication of cardiac disease likely is multifactorial. We report here our experience with 3 patients with protein-losing enteropathy. Two had superior caval venous occlusion. The third patient had undergone an extracardiac Fontan operation, with stenosis of the extracardiac conduit placed to the right pulmonary artery. In all three cases, the complication was resolved by restoring unobstructed flow of blood in the superior or inferior caval vein. Since the aetiology of protein-losing enteropathy associated with cardiac disease is multifactorial, reduction of pressure in the tributaries of the superior caval vein may, in some cases, be curative. It is more difficult to cure the problem when it is associated with the Fontan operation.

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