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.

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.

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.

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.

scholarly journals P649 Before and after: sinus venosus atrial septal defect

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Marco Clement ◽  
R Eiros ◽  
R Dalmau ◽  
T Lopez ◽  
G Guzman ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Left Atrium ◽  
Atrial Flutter ◽  
Superior Vena Cava ◽  
Right Atrium ◽  
Septal Defect ◽  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
The Right

Abstract Introduction The diagnosis of sinus venosus atrial septal defect (SVASD) is complex and requires special imaging. Surgery is the conventional treatment; however, transcatheter repair may become an attractive option. Case report A 60 year-old woman was admitted to the cardiology department with several episodes of paroxysmal atrial flutter, atrial fibrillation and atrioventricular nodal reentrant tachycardia. She reported a 10-year history of occasional palpitations which had not been studied. A transthoracic echocardiography revealed severe right ventricle dilatation and moderate dysfunction. Right volume overload appeared to be secondary to a superior SVASD with partial anomalous pulmonary venous drainage. A transesophageal echocardiography confirmed the diagnosis revealing a large SVASD of 16x12 mm (Figure A) with left-right shunt (Qp/Qs 2,2) and two right pulmonary veins draining into the right superior vena cava. Additionally, it demonstrated coronary sinus dilatation secondary to persistent left superior vena cava. CMR and cardiac CT showed right superior and middle pulmonary veins draining into the right superior vena cava 18 mm above the septal defect (Figures B and C). After discussion in clinical session, a percutaneous approach was planned to correct the septal defect and anomalous pulmonary drainage. For this purpose, anatomical data obtained from CMR and CT was needed to plan the procedure. During the intervention two stents graft were deployed in the right superior vena cava. The distal stent was flared at the septal defect level so as to occlude it while redirecting the anomalous pulmonary venous flow to the left atrium (Figure D). Control CT confirmed the complete occlusion of the SVASD without residual communication from pulmonary veins to the right superior vena cava or the right atrium (Figure E). Anomalous right superior and middle pulmonary veins drained into the left atrium below the stents. Transthoracic echocardiographies showed progressive reduction of right atrium and ventricle dilatation. The patient also underwent successful ablation of atrial flutter and intranodal tachycardia. She is currently asymptomatic, without dyspnea or arrhythmic recurrences. Conclusions In this case, multimodality imaging played a key role in every stage of the clinical process. First, it provided the diagnosis and enabled an accurate understanding of the patient’s anatomy, particularly of the anomalous pulmonary venous connections. Secondly, it allowed a transcatheter approach by supplying essential information to guide the procedure. Finally, it assessed the effectiveness of the intervention and the improvement in cardiac hemodynamics during follow-up. Abstract P649 Figure.

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.

Abnormalities of Pulmonary Venous Return

Chest Imaging ◽  
2019 ◽  
pp. 533-537
Author(s):  
Kristopher W. Cummings
Keyword(s):  
Atrial Septal Defect ◽  
Septal Defect ◽  
Venous Return ◽  
Pulmonary Veins ◽  
Lower Lobe ◽  
Scimitar Syndrome ◽  
Systemic Vein ◽  
Anomalous Pulmonary Venous Return ◽  
The Right

Abnormalities of pulmonary venous return in adults result from anomalous drainage of one or more pulmonary veins into a systemic vein, resulting in a left-to-right shunt. Partial anomalous pulmonary venous return (PAPVR) is most commonly encountered in adults in the upper lobes. PAPVR in the right upper lobe is commonly associated with a sinus venous atrial septal defect, whereas in the right lower lobe it is commonly encountered in association with other anomalies in Scimitar syndrome. Left upper lobe PAPVR is usually isolated. In some instances, patients can develop pulmonary over-circulation and hypertension, necessitating intervention. This chapter emphasizes CT and MR features key to recognizing and diagnosing these anomalies.

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.

Biatrial drainage of right superior caval vein with preferential streaming into the left atrium

2019 ◽  
Vol 29 (7) ◽  
pp. 996-998
Author(s):  
Omar Abu-Anza ◽  
Ravi Ashwath
Keyword(s):  
Atrial Septal Defect ◽  
Left Atrium ◽  
Septal Defect ◽  
Venous Return ◽  
Sinus Venosus ◽  
Cardiac Anomaly ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Anomalous Pulmonary Venous Return ◽  
The Right

AbstractBiatrial drainage of the right superior caval vein is an extremely rare cardiac anomaly that generally presents in childhood. We present a case of anomalous connection of the right superior caval vein with superior sinus venosus atrial septal defect and partial anomalous pulmonary venous return in a 5-month-old male presenting with unexplained cyanosis and hypoxia.

Hybrid closure of a large atrial septal defect using Occlutech Flex II septal occluder in a patient with interrupted inferior caval vein

2020 ◽  
Vol 30 (6) ◽  
pp. 880-882
Author(s):  
Amjad Bani Hani ◽  
Mai Abdullattif ◽  
Iyad AL-Ammouri
Keyword(s):  
Cardiopulmonary Bypass ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Inferior Caval Vein ◽  
Thoracotomy Incision

AbstractWe present a case of a 31-year-old male with a large atrial septal defect, who was found to have interrupted inferior caval vein with azygous continuation to the superior caval vein, which precluded transcutaneous closure by device. The defect was successfully closed with a 33 mm Occlutech Figula septal occluder using a sub-mammary small thoracotomy incision and per-atrial approach without using cardiopulmonary bypass. The patient was discharged home after 48 hours of procedure.

Surgical repair of total anomalous pulmonary venous connection: Lateral approach

2021 ◽  
Keyword(s):  
Cardiopulmonary Bypass ◽  
Atrial Septal Defect ◽  
Left Atrium ◽  
Septal Defect ◽  
Surgical Repair ◽  
Pleural Cavity ◽  
Pulmonary Veins ◽  
Lateral Approach ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

We describe the lateral approach to the surgical repair of a total anomalous pulmonary venous connection in this video tutorial. The goal of the operation is to create an unobstructed anastomosis between the pulmonary confluence and the left atrium, ligate the systemic venous connections, and close the atrial septal defect. After a median sternotomy and initial dissection of the structures surrounding the heart, cardiopulmonary bypass is initiated by aortobicaval cannulation. The patient is then cooled to attain mild hypothermia (30°C). The heart is arrested by a dose of antegrade cold cardioplegia. The right pleural cavity is opened widely. The heart is retracted and pushed into the right pleural cavity. The vertical vein is ligated near its connection with the innominate vein. An incision is made along the length of the confluence, stopping short of the individual pulmonary veins. The left atrial appendage is retracted, and the left atrium is opened in alignment with the opening in the confluence. The left atrium and the pulmonary confluence are anastomosed widely with 7-0 polypropylene suture material. The heart is put back into the mediastinum. Rewarming is started. The atrial septal defect is closed through the right atrium using a large untreated autologous pericardium patch. The patient is then weaned off cardiopulmonary bypass.

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