Echocardiographic diagnosis of totally anomalous pulmonary venous connection to the azygos vein

1999 ◽  
Vol 9 (3) ◽  
pp. 305-309 ◽  
Author(s):  
Anna Maria Musolino ◽  
Giuseppe Santoro ◽  
Bruno Marino ◽  
Roberto Formigari ◽  
Paolo Guccione ◽  
...  
Keyword(s):  
Venous Blood ◽  
Azygos Vein ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Heart Malformation ◽  
Precise Diagnosis ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection ◽  
Axis View

AbstractTotally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood returns anomalously to the azygos vein. Among 111 consecutive patients with totally anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation was present in seven cases. By echocardiography, using a subxyphoid short-axis view at the atrial level and a modified suprasternal sagittal view, the malformation was diagnosed when the pulmonary venous confluence was traced posteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the first two patients, both by echocardiography and angiocardiography. In the subsequent five patients, a precise diagnosis was obtained by echocardiography. Echocardiography, therefore, can be considered an accurate diagnostic tool permitting recognition of totally anomalous pulmonary venous connection to the azygos vein, and permitting corrective surgery without recourse to catheterization and angiography.

Partially Anomalous Pulmonary Venous Connection to Solitary and Persistent Left Superior Caval Vein in Usual Visceroatrial Arrangement

2017 ◽  
Vol 11 (4) ◽  
pp. NP88-NP90
Author(s):  
Anoop Ayyappan ◽  
Arun Gopalakrishnan ◽  
Kapilamoorthy Tirur Raman
Keyword(s):  
Pulmonary Veins ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Although the occurrence of bilateral superior caval veins (SCVs) is not unusual, persistence of the left SCV with atretic right SCV is extremely uncommon in the setting of normal visceroatrial arrangement. We report such a case that was also associated with anomalous pulmonary venous connection of the right pulmonary veins to the solitary left SCV.

Intracardiac or extracardiac conduit modification of the Fontan procedure in hearts with univentricular atrioventricular connection and left superior caval vein draining to coronary sinus

1997 ◽  
Vol 7 (2) ◽  
pp. 215-219
Author(s):  
Jacques A.M. van Son ◽  
Volkmar Falk ◽  
Friedrich W. Mohr
Keyword(s):  
Coronary Sinus ◽  
Venous Blood ◽  
Azygos Vein ◽  
Caval Vein ◽  
Arterial Circulation ◽  
Superior Caval Vein ◽  
Inferior Caval Vein ◽  
Extracardiac Conduit ◽  
Hepatic Venous Blood ◽  
Pulmonary Arterial

AbstractIn 3 patients with isomeric morphologically left atrial appendages, univentricular atrioventricular connection, concordant ventriculoarterial connections, bilateral superior caval veins, with the left one draining via the coronary sinus, together with absence of any communicating vein, interruption of inferior caval vein with drainage via a right-sided (n=2) or left-sided (n=l) azygos vein, the hepatic venous blood was rerouted via the large coronary sinus into the pulmonary arterial circulation. In a fourth patient with similar pathology, having interruption of the left-sided inferior caval vein with drainage to the left-sided superior caval vein via a left-sided azygos vein and a large communicating vein, the pathway from the left superior caval vein to the coronary sinus was correspondingly small. An extracardiac conduit was therefore constructed between the hepatic veins and the left pulmonary artery so as to reroute the hepatic venous blood into the pulmonary arterial circulation. At a mean follow-up of 8.5 months, all patients are clinically well and none of them have developed pulmonary arteriovenous malformations. To avoid the latter complication in Fontan physiology, especially in the setting of an interrupted inferior caval vein with drainage via the azygos vein, we believe that it is preferable to reroute the hepatic venous blood into the pulmonary circulation.

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.

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.

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.

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.

Spontaneous closure of arterio-venous pulmonary fistulas by redirection of hepatic venous blood 9 years after Glenn anastomosis in a 12-year-old girl

2019 ◽  
Vol 29 (10) ◽  
pp. 1287-1289
Author(s):  
Katarzyna Gendera ◽  
Andreas Eicken ◽  
Peter Ewert
Keyword(s):  
Pulmonary Artery ◽  
Venous Blood ◽  
Covered Stent ◽  
Pulmonary Trunk ◽  
Surgical Ligation ◽  
Hepatic Venous Blood ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Radiofrequency Perforation ◽  
Glenn Procedure

AbstractWe report on a 12-year-old girl with Ebstein’s anomaly after a unidirectional Glenn procedure with surgical ligation of the proximal right pulmonary artery, who suffered from significant central cyanosis caused by multiple arterio-venous fistulas in the right lung. The continuity between the right pulmonary artery and the pulmonary trunk was restored with the use of radiofrequency perforation and consecutive covered stent implantation.

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.

Cavo-pulmonary anastomosis excluding the portal venous return: an alternative for patients with protein-losing enteropathy secondary to the Fontan procedure

1998 ◽  
Vol 8 (2) ◽  
pp. 221-227 ◽  
Author(s):  
Miguel Barbero-Marcial ◽  
Arlindo Riso ◽  
Edmar Atik ◽  
Carla Tanamati ◽  
Humberto Reis ◽  
...  
Keyword(s):  
New York ◽  
Fontan Procedure ◽  
Heart Association ◽  
Functional Class ◽  
Caval Vein ◽  
Venous Flow ◽  
Protein Losing Enteropathy ◽  
Right Pulmonary Artery ◽  
New York Heart Association ◽  
The Right

AbstractSevere protein losing enteropathy occurred in 3 of our 128 patients undergoing the Fontan procedure in the period of 1982 to 1994. The complication occurred 4, 4.5 and 5 years after the procedure, respectively. One patient had double inlet left ventricle while the other two had tricuspid atresia. All had initially undergone an atrio-pulmonary connection, and the duration of the enteropathy was 12, 13,5 and 15 months. All patients were in functional class IV of the New York Heart Association. The presence of protein losing enteropathy was confirmed by determining levels of fecal alfa-1 antitrypsin, enteric clearance of 51 Cromo labeled albumin, and endoscopy with histopathological study of the small bowel.They underwent further surgery to convert the atrio-pulmonary to a cavo-pulmonary anastomosis which excluded all the hepatic veins. A bovine pericardial baffle was sutured in the inferior caval vein so as to leave the hepatic venous flow draining to the right atrium and thence through an atrial septal defect to the left atrium and systemic circulation. The inferior caval venous flow was directed through an intra-atrial tunnel to the right pulmonary artery, and the superior caval vein was anastomosed to the superior aspect of the right pulmonary artery, thus completing the total cavo-pulmonary anastomosis with exclusion of the portal venous flow.Postoperatively, there was total regression of the clinical and laboratory findings. All patients are now in functional class I or II of the New York Heart Association. They have mild to moderate cyanosis up to three and a half years from the reoperation. We recommend the technique as an alternative for patients with severe protein losing enteropathy occurring after the Fontan procedure.

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