Left isomerism with tetralogy of Fallot and anomalous systemic and pulmonary venous connections

1998 ◽  
Vol 8 (1) ◽  
pp. 131-133 ◽  
Author(s):  
E. Da Cruz ◽  
L. Milella ◽  
A. Corno
Keyword(s):  
Tetralogy Of Fallot ◽  
Left Atrial ◽  
Pulmonary Veins ◽  
Right Aortic Arch ◽  
Caval Vein ◽  
Azygos Continuation ◽  
Inferior Caval Vein ◽  
Left Isomerism ◽  
The Right ◽  
Anomalous Coronary

AbstractThe association of left isomerism with interruption and azygos continuation of the inferior caval vein, anomalous connection of the right pulmonary veins to the right atrium, an interatrial commu nication of sinus venosus type, and tetralogy of Fallot with right aortic arch and anomalous coronary artery has been observed in a 6-month-old patient. As far as we are aware, the association of these congenital car diac malformations has not previously been reported in patients with isomeric left atrial appendages.

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.

Totally anomalous pulmonary venous connection through the right lung and via a “Scimitar” vein to the inferior caval vein

1993 ◽  
Vol 3 (1) ◽  
pp. 85-87
Author(s):  
Rakesh Dua ◽  
Christine McTigue ◽  
James.L Wilkinson
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Left Lung ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
The Right ◽  
Left Heart Syndrome ◽  
Anomalous Pulmonary Venous Connection

AbstractWe report a case of totally anomalous pulmonary venous connection in which the two pulmonary veins from the left lung joined to form a common vein which then passed across the midline into a hypoplastic right lung and, after receiving small veins from the right lung, passed inferiorly, exiting the lung below the hilum as a “scimitar” vein and terminating in the inferior caval vein. A separate pulmonary vein from the right lung passed inferiorly independently and joined the “scimitar” vein before it entered the inferior caval vein. There was an associated hypoplastic left heart syndrome.

Anatomic biventricular repair by intraatrial and intraventricular re-routing in patients with left isomerism

2001 ◽  
Vol 11 (1) ◽  
pp. 12-16 ◽  
Author(s):  
Hideki Uemura ◽  
Toshikatsu Yagihara ◽  
Youichi Kawahira ◽  
Yoshiro Yoshikawa
Keyword(s):  
Left Atrial ◽  
Right Ventricular Outflow Tract ◽  
Functional Results ◽  
Ventricular Outflow Tract ◽  
The Other ◽  
P Wave ◽  
Biventricular Repair ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Left Isomerism

AbstractObjective: To determine the efficacy of anatomic biventricular repair by a combination of intraatrial and intraventricular re-routing in patients with isomerism of the left atrial appendages.Methods: Anatomic biventricular repair by means of combined intraratrial and intraventricular re-routing was achieved in 5 of the 63 patients with left isomerism in whom we attempted a definitive surgical procedure. The inferior caval vein was interrupted in 3. The Mustard procedure was chosen for intraatrial redirection of blood in 4, and the Senning procedure in the other. Intraventricular re-routing was carried out via a right ventriculotomy in all patients, using an external conduit to reconstruct the morphologically right ventricular outflow tract.Results: One patient died one month after the procedure because of low cardiac output and bronchial bleeding. Obstruction across the superior caval venous channel occurred after the Senning procedure in this particular patient, related to the interrupted inferior caval vein draining via the azygous vein. The other 4 patients are currently doing well. Postoperative catheterization showed excellent cardiac performance, with no obstruction across the venous channels or the ventricular outflow tracts in these 4. No episode of significant archythmia has been noted, all patients having a regular atrial rhythm, although the P wave vector was unusual in each patient. Reoperation has been needed thus far in one patient, 128 months after the initial repair, because of obstruction of the external conduit.Conclusion: With precise recognition of the morphologic features, a combination of intraatrial and intraventricular re-routing can successfully be established in patients with isomeric left atrial appendages, with functional results in the intermediate term being reasonable.

Transcatheter therapy of anomalous systemic venous drainage

2017 ◽  
Vol 28 (3) ◽  
pp. 502-506
Author(s):  
Shahnawaz M. Amdani ◽  
Thomas J. Forbes ◽  
Daisuke Kobayashi
Keyword(s):  
Left Atrium ◽  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Three Dimensional ◽  
Venous Drainage ◽  
Azygos Vein ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Inferior Caval Vein ◽  
The Right

AbstractAnomalous drainage of the right superior caval vein into the left atrium is a rare congenital anomaly that causes cyanosis and occult infection owing to right-to-left shunting. Transcatheter management of this anomaly is unique and rarely reported. We report a 32-year-old man with a history of brain abscess, who was diagnosed with an anomalous right superior caval vein draining to the left atrium; right upper pulmonary vein and right middle pulmonary vein draining into the inferior portion of the right superior caval vein; and a left superior caval vein draining into the right atrium through the coronary sinus without a bridging vein. Pre-procedural planning was guided by three-dimensional printed model. The right superior caval vein was occluded with a 16-mm Amplatzer muscular Ventricular Septal Defect occluder inferior to the azygous vein, but superior to the entries of right upper and middle pulmonary veins. This diverted the right superior caval vein flow to the inferior caval vein system through the azygos vein in a retrograde manner and allowed the right upper pulmonary vein and right middle pulmonary vein flow to drain into the left atrium normally, achieving exclusion of right-to-left shunting and allowing normal drainage of pulmonary veins into the left atrium. At the 6-month follow-up, his saturation improved from 93 to 97% with no symptoms of superior caval vein syndrome.

Inferior-type caval vein defect – echocardiographic and surgical description of a large series of patients

2011 ◽  
Vol 22 (3) ◽  
pp. 270-278 ◽  
Author(s):  
Munesh Tomar ◽  
Sitaraman Radhakrishnan ◽  
Sunil K. Kaushal ◽  
Kulbhushan S. Dagar ◽  
Krishna S. Iyer ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Pulmonary Vein ◽  
Septal Defect ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Transoesophageal Echocardiography ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
The Right ◽  
Echocardiographic Findings

AbstractAimThis study was carried out to define the anatomical criteria for the diagnosis of inferior-type caval vein defect and compare the echocardiographic findings with surgical findings.MethodsThe records of 19 patients – 13 male and six female patients in the age group of 18 months to 27 years, who were diagnosed as inferior-type caval vein defect with or without anomalous drainage of right pulmonary vein(s) on echocardiography – were retrospectively reviewed and compared with surgical findings.ResultsSurgical diagnosis of inferior-type caval vein defect was confirmed in 17 of the 19 patients. In two patients, the surgical diagnosis was that of a large fossa ovalis atrial septal defect – confluent defect and fossa ovalis atrial septal defect with deficient inferior rim in one patient each. Surgical diagnosis of anomalous drainage of pulmonary vein(s) was based on the course of the superior rim of the defect in relation to the pulmonary veins. Our echocardiographic impression of the pulmonary veins appearing in its normal position but showing abnormal drainage to right atrium was in agreement with the surgical notes. Discrepancy was found in the number of pulmonary veins draining anomalously. The discordance was related to overdiagnosis of anomalous drainage in all except one, that is, three out of four. In one, only the right lower pulmonary vein was diagnosed to be anomalous, whereas both right upper and lower pulmonary veins were found to be anomalous.ConclusionsEchocardiography provides definite diagnosis of inferior-type caval vein defect. Inferior caval vein straddling and an intact fossa ovalis are prerequisites for diagnosis. Anomalous pulmonary venous drainage of the right pulmonary veins is very common in our series, although accurate diagnosis of the number of pulmonary veins was not possible in all cases. Multiple views on transthoracic echocardiography starting from the subxiphoid views delineate the morphology accurately. Transoesophageal echocardiography is required only in patients in whom the windows, especially the subxiphoid, are not adequate.

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.

Unusual drainage of the inferior caval vein in left atrial isomerism

1989 ◽  
Vol 24 (1) ◽  
pp. 35-39 ◽  
Author(s):  
Nathan Roguin ◽  
Simha Milo ◽  
Bernardo Vidne
Keyword(s):  
Left Atrial ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Atrial Isomerism

Clarifying the anatomy of the superior sinus venosus defect

Heart ◽  
2021 ◽  
pp. heartjnl-2021-319334
Author(s):  
Jay Relan ◽  
Saurabh Kumar Gupta ◽  
Rengarajan Rajagopal ◽  
Sivasubramanian Ramakrishnan ◽  
Gurpreet Singh Gulati ◽  
...  
Keyword(s):  
Left Atrium ◽  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Interatrial Septum ◽  
Sinus Venosus ◽  
Caval Vein ◽  
Therapeutic Implications ◽  
Superior Caval Vein ◽  
Inferior Pulmonary Vein ◽  
The Right

ObjectivesWe sought to clarify the variations in the anatomy of the superior cavoatrial junction and anomalously connected pulmonary veins in patients with superior sinus venosus defects using computed tomographic (CT) angiography.MethodsCT angiograms of 96 consecutive patients known to have superior sinus venosus defects were analysed.ResultsThe median age of the patients was 34.5 years. In seven (7%) patients, the defect showed significant caudal extension, having a supero-inferior dimension greater than 25 mm. All patients had anomalous connection of the right superior pulmonary vein. The right middle and right inferior pulmonary vein were also connected anomalously in 88 (92%) and 17 (18%) patients, respectively. Anomalous connection of the right inferior pulmonary vein was more common in those with significant caudal extension of the defect (57% vs 15%, p=0.005). Among anomalously connected pulmonary veins, the right superior, middle, and inferior pulmonary veins were committed to the left atrium in 6, 17, and 11 patients, respectively. The superior caval vein over-rode the interatrial septum in 67 (70%) patients, with greater than 50% over-ride in 3 patients.ConclusionAnomalous connection of the right-sided pulmonary veins is universal, but is not limited to the right upper lobe. Not all individuals have over-riding of superior caval vein. In a minority of patients, the defect has significant caudal extension, and anomalously connected pulmonary veins are committed to the left atrium. These findings have significant clinical and therapeutic implications.

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.

Hepatic venous connection to a persistent inferior caval vein in left isomerism

1990 ◽  
Vol 11 (9) ◽  
pp. 845-847 ◽  
Author(s):  
J. GUENTHARD ◽  
J. S. CARVALHO ◽  
R. H. ANDERSON ◽  
M. L. RlGBY
Keyword(s):  
Caval Vein ◽  
Inferior Caval Vein ◽  
Left Isomerism
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