Diagnosis, management, and follow-up of systemic venous drainage via a single left superior caval vein into the left atrium

2006 ◽  
Vol 16 (6) ◽  
pp. 590-592 ◽  
Author(s):  
Jean-Marc Schleich ◽  
Ons Azzabi ◽  
Claude Almange
Keyword(s):  
Computed Tomography ◽  
Left Atrium ◽  
Right Atrium ◽  
Contrast Echocardiography ◽  
Venous Drainage ◽  
Surgical Correction ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
The Right

A 15-month-old boy presented with asymptomatic hypoxaemia due to right-to-left venous shunting via a left superior caval vein emptying into the left atrium, in absence of right superior caval vein. The diagnosis, suspected by contrast echocardiography, was confirmed by computed tomography and angiography. The child underwent surgical correction of the systemic anomalous return by tunnelling the left superior caval vein towards the right atrium. An asymptomatic narrowing inside the intra-atrial baffle developed 6 months later.

Contrast echocardiography for diagnosis of pulmonary arteriovenous fistulas late after construction of a Glenn anastomosis

1998 ◽  
Vol 8 (2) ◽  
pp. 228-236
Author(s):  
Gül Sagin-Saylam ◽  
Jane Somerville
Keyword(s):  
Right Atrium ◽  
Contrast Echocardiography ◽  
Cardiac Catheterisation ◽  
Arterial Oxygen ◽  
Caval Vein ◽  
Glenn Shunt ◽  
Arteriovenous Fistulas ◽  
Superior Caval Vein ◽  
The Right ◽  
Pulmonary Arteriovenous Fistulas

AbstractTo demonstrate the use of transthoracic contrast echocardiography in the detection of pulmonary arteriovenous fistulas in patients with a previously constructed anastomosis between the superior caval vein and the right pulmonary artery (Glenn shunt), and to examine their prevalence in this special population, we evaluated prospectively all patients followed up in the Grown-Up Congenital Heart Unit subsequent to construction of a classical or bi-directional Glenn shunt. We studied 12 patients, aged from 21 to 38 (mean 28 ± 4.8) years who had had a previous cavopulmonary shunt in place for a period of 4 to 33 years (mean 24±9 years). All were examined with cross-sectional contrast echocardiography, 11 patients had cardiac catheterisation and angiography, and 6 patients had magnetic resonance imaging. Systemic arterial oxygen saturations at rest, and during exercise using the modified Bruce protocol, were measured in all patients. Contrast echocardiography showed evidence of pulmonary arteriovenous fistulas in 7 of the 12 patients, with appearance of echo contrast in the left atrium 1–8 seconds after peripheral venous injection in the arm. Simultaneous appearance of microbubbles in the right atrium revealed a residual communication between the superior caval vein and the right atrium in 2 patients, and presence of collaterals between the superior and inferior caval veins in one. Cardiac catheterisation and angiography showed obvious fistulas in 4 patients, and revealed suggestive findings in 2. In patients deemed to have pulmonary arteriovenous fistulas on contrast echocardiography, arterial oxygen saturations at rest (51–94%, mean 75±15.3%) and on exercise (23–91%, mean 53±24.2%) were significantly lower compared to patients judged to be without fistulas (p<0.005). Pulmonary hypertension in the contralateral lung was more common in patients with fistulas (mean left pulmonary arterial pressure 22–110 mm Hg, p=0.014). In patients with cavopulmonary anastomoses, pulmonary arteriovenous fistulas occur frequently in the long term (10–33, mean 25.7±8 years), and are associated with worsening systemic arterial desaturation. Contrast echocardiography should be included in the regular evaluation of these patients as a simple and sensitive technique for the detection of pulmonary arteriovenous fistulas, particularly with the devel opment of increasing cyanosis.

A Review of the Surgical Management of Anomalous Connection of the Right Superior Caval Vein to the Morphologically Left Atrium and Biatrial Drainage of Right Superior Caval Vein

2020 ◽  
Vol 11 (4) ◽  
pp. 466-484
Author(s):  
Ujjwal Kumar Chowdhury ◽  
Robert H. Anderson ◽  
Niwin George ◽  
Sukhjeet Singh ◽  
Lakshmi Kumari Sankhyan ◽  
...  
Keyword(s):  
Left Atrium ◽  
Surgical Management ◽  
Clinical Presentation ◽  
Contrast Echocardiography ◽  
Surgical Techniques ◽  
Caval Vein ◽  
Radiographic Findings ◽  
Superior Caval Vein ◽  
Disease Entities ◽  
The Right

The present perspective is a synthesis of published investigations in the setting of anomalous connection of the right superior caval vein to the morphologically left atrium or biatrial drainage of the right caval vein. We identified 57 suitable cases from 97 investigations, reviewing the clinical presentation, diagnostic modalities utilized, surgical techniques used, and their outcomes. Clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, radionuclide perfusion scan, magnetic resonance imaging, and angiocardiography provided the diagnostic information and were used to define the disease entities before surgery. We have also addressed several issues concerning the influence of the so-called heterotaxy: the establishment of the diagnosis, the variation in clinical presentation, and subsequent management. For the overall group of patients undergoing either surgical intervention or transcatheter treatment with an Amplatzer vascular plug, the operative mortality remains high at 9.5%. We submit that an increased appreciation of these disease entities will contribute to improved future surgical management.

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.

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.

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.

Right superior caval vein draining into the left atrium—diagnosis by color flow mapping

1995 ◽  
Vol 5 (4) ◽  
pp. 345-349 ◽  
Author(s):  
Luis E. Alday ◽  
Hector Maisuls ◽  
Roberto De Rossi
Keyword(s):  
Left Atrium ◽  
Clinical Findings ◽  
The Other ◽  
Flow Mapping ◽  
Caval Vein ◽  
Color Flow ◽  
Superior Caval Vein ◽  
Color Flow Mapping ◽  
Excellent Method ◽  
The Right

AbstractWe report two female patients, one aged four years and the other a newborn, referred for evaluation of cyanosis with otherwise normal cardiovascular findings, who proved to have the right superior caval vein draining into the morphologically left atrium. In both patients, the diagnosis was made by color flow mapping. The older child underwent catheterization and subsequent successful surgical correction. A right superior caval vein draining into the left atrium, although very rare, should always be considered a diagnostic possibility in the presence of cyanosis and normal clinical findings. Color flow mapping is an excellent method with which to make the diagnosis.

scholarly journals P1697 A rare case: primary cardiac sarcoma in the left and right atrium presenting with dispnea due to mitral valvular obstruction

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Akturk ◽  
T S Tan ◽  
M Mammadov ◽  
I Dincer ◽  
C Erol
Keyword(s):  
Computed Tomography ◽  
Left Atrium ◽  
Right Atrium ◽  
Bone Lesions ◽  
Cardiac Tumors ◽  
Iliac Wing ◽  
Left And Right ◽  
Primary Cardiac Sarcoma ◽  
The Right ◽  
Primary Cardiac Tumors

Abstract Introduction Primary cardiac tumors are extremely rare; the incidence is approximately 0.001% to 0.03%. Malignant tumors account for 25% of primary cardiac tumors, and among those, sarcomas are the most prevalent. We report a case of primary cardiac sarcoma presenting with dyspnea due to mitral valvuler obstruction. Case Report A 41-year-old woman was admitted to the hospital with dyspnea for 8 months without any obvious causes. She was healthy previous and without family history. Physical examination was normal. Thorax computed tomography (CT) scan was performed with the doubt of pulmonary disease. A 1x1 cm hypodense nodule was detected in superior segment of the right lower lobe lung. Positron emission tomography-computed tomography (PET-CT) revealed a 1*1 cm nodule in the lung. And also increased 18F-fluorodeoxyglucose uptake was observed in the left scapula, left iliac wing and right 4th rib, likely due to metastasis. Biopsy from the lesion of iliac wing was performed. Pathologic examination was primarily compatible with the malign mesenchymal tumor and sarcoma infiltration; but malignant epithelial tumor and metastasis of carcinoma could not be ruled out. Magnetic resonance imaging of the lung was performed to find the origin of the metastatic tumor. A 8,6 x 5,3 x 5,1 cm mass filling the right and left atria was detected.It was extending from the right atrium to the superior vena cava and also from the left atrium to the right inferior and superior pulmonary venules. The image of mass was compatible with sarcoma. Transthoracic echocardiography was performed. A mass in the left and right atrium was detected. It was filling the left atrium. Due to the mass, the maximum mitral gradient was 21 mm Hg and the mean mitral gradient was 10 mm Hg, as if there was mitral stenosis.Systolic pulmonary artery pressure was 40 mmHg. Mild mitral insufficiency was detected. The patient was evaluated with the department of oncology and cardiovascular surgery. It was decided that the mass was inoperable. So transesophageal echocardiography was not performed. It was thought that, the mass was primary cardiac sarcoma, pulmonary nodule and bone lesions were metastasis of this primary cardiac sarcoma. So chemotherapy protocol (ifosfamide,adriamycin,mesna) was started. Conclusion Cardiac tumors have many clinical presentations. Early stages of the disease are often asymptomatic. In advanced stages, patients present with symptoms of the classic triad (intracardiac obstruction, systemic embolization, constitutional symptoms) Symptoms are often non-spesific; so diagnostic suspicion is very important. Although cardiac sarcoma therapy includes complete surgical excision, followed by radiotherapy and chemotherapy, prognosis is still poor. Surgery can offer dramatic palliation of symptoms in cases of valvular obstruction. In conclusion, it remains clear that early diagnosis and treatment are extremely important because of their prognostic and therapeutic value. Abstract P1697 Figure.

Anatomical considerations for the management of a left-sided superior caval vein draining to the roof of the left atrium

2016 ◽  
Vol 27 (5) ◽  
pp. 846-850
Author(s):  
Jelena Saundankar ◽  
Andrew B. Ho ◽  
Anthony P. Salmon ◽  
Robert H. Anderson ◽  
Alan G. Magee
Keyword(s):  
Left Atrium ◽  
Coronary Sinus ◽  
Right Atrial ◽  
Caval Vein ◽  
Cross Sectional ◽  
Interatrial Communication ◽  
Unroofed Coronary Sinus ◽  
Superior Caval Vein ◽  
Coronary Sinus Ostium ◽  
The Right

AbstractAimsThe pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management.Methods and ResultsBoth patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug.ConclusionsPersistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Endocarditis of a congenital coronary fistula in a child

2017 ◽  
Vol 28 (2) ◽  
pp. 334-337 ◽  
Author(s):  
Thomas Krasemann ◽  
Ingrid M. van Beynum ◽  
Ingrid M. E. Frohn-Mulder ◽  
Michiel Dalinghaus
Keyword(s):  
Coronary Artery ◽  
Right Coronary Artery ◽  
Right Atrium ◽  
Age Group ◽  
Coronary Fistula ◽  
Vascular Plug ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Paediatric Age ◽  
The Right

AbstractEndocarditis of congenital coronary fistulas in the cardiac chambers is rare, especially in the paediatric age group. We describe the case of a 9-year-old boy with a fistula from the dilated right coronary artery to the junction of the superior caval vein to the right atrium, complicated by endocarditis. Treatment consisted of 6 weeks of antibiotics and interventional closure of the fistula 3 months later with an Amplatzer vascular plug.

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.

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