A rare cause of abnormal pulmonary venous drainage: septum primum malposition

2020 ◽  
Vol 30 (11) ◽  
pp. 1716-1721
Author(s):  
Pelin Ayyıldız ◽  
Erkut Öztürk ◽  
Taner Kasar ◽  
Aysel Türkvatan ◽  
İsmihan Selen Onan ◽  
...  
Keyword(s):  
Transthoracic Echocardiography ◽  
Septal Defect ◽  
Multidetector Ct ◽  
Venous Drainage ◽  
Double Outlet Right Ventricle ◽  
Caval Vein ◽  
Segmental Analysis ◽  
Superior Caval Vein ◽  
Surgical Data ◽  
Septum Primum

AbstractObjectives:This study aimed to evaluate the clinical features of patients with septum primum malposition, imaging tools used for diagnosis, and their effects on the surgical approach.Materials and methods:Patients diagnosed with septum primum malposition in our paediatric cardiac centre between 1 January, 2015 and 1 January, 2019 were included in the study. In all patients, the age, reason for admission, transthoracic echocardiography, cardiac multidetector CT angiography findings, and subsequent surgical data were evaluated.Results:Fifteen patients were diagnosed with septum primum malposition during the study period. The median age was 12 months (2 months–10 years). Six patients were left isomeric, and the rest were situs solitus; 80% of the patients (n = 12) had additional secundum atrial septal defect. There was cardiac pathology in 46% of the patients (n = 7) in addition to the abnormal pulmonary venous drainage, ventricular septal defect (n = 3), left ventricularhypoplasia (n = 2), cortriatriatum sinister (n = 2), double outlet right ventricle (n = 1), and atrioventricular septal defect (n = 1). There was bilateral superior caval vein in three patients, right-sided superior caval vein in 11 patients, and left-sided superior caval vein in one patient. All three patients with total abnormal pulmonary venous drainage were left atrial isomeric. There were differences between the results of transthoracic echocardiography and CT angiographies in two patients. The surgical strategy was changed in three patients after the preoperative diagnosis of septum primum malposition.Conclusion:Septum primum malposition should be kept in mind during the imaging of complex CHDs specifically during the segmental analysis of the pathologies with heterotaxy syndromes; it should be differentiated from other aetiologies of abnormal pulmonary venous drainage as accurate diagnosis would facilitate the ideal surgery in these complex pathologies requiring a detailed preoperative preparation.

Horseshoe lung associated with anomalous pulmonary venous connection without pulmonary hypoplasia

1995 ◽  
Vol 5 (1) ◽  
pp. 91-93 ◽  
Author(s):  
Antonio Corno ◽  
Luca Rosti ◽  
Ivan Machado
Keyword(s):  
Ventricular Septal Defect ◽  
Congenital Malformation ◽  
Septal Defect ◽  
Pulmonary Hypoplasia ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Horseshoe Lung ◽  
Anomalous Pulmonary Venous Connection

SummaryHorseshoe lung is an exceedingly rare congenital malformation, characterized by unilateral pulmonary hypoplasia together with a midline isthmus producing fusion of the tissues of the lower lobes. It is frequently associated with other cardiac and extracardiac anomalies. We report an infant with a variant of the horseshoe lung with partial anomalous venous connection of intracardiac type, ventricular septal defect, and persistent left superior caval vein. There was no pulmonary hypoplasia.

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.

Divided left atrium associated with supravalvar mitral ring

1999 ◽  
Vol 9 (4) ◽  
pp. 423-426 ◽  
Author(s):  
Silvia Álvares ◽  
António Sá Melo ◽  
Manuel Antunes
Keyword(s):  
Pulmonary Hypertension ◽  
Left Atrium ◽  
Septal Defect ◽  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Caval Vein ◽  
Interatrial Communication ◽  
Superior Caval Vein ◽  
Rare Association ◽  
Echocardiographic Examination

AbstractReported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.

Multimodality imaging in delineation of complex sinus venous defects and treatment outcomes over the last decade

2021 ◽  
pp. 1-9
Author(s):  
Li Y. Ng ◽  
Lars Nolke ◽  
Adam James ◽  
Brian Grant ◽  
Orla Franklin ◽  
...  
Keyword(s):  
Pulmonary Vein ◽  
Multimodality Imaging ◽  
Venous Drainage ◽  
Final Diagnosis ◽  
Sinus Venosus ◽  
Patch Repair ◽  
Pulmonary Venous Obstruction ◽  
Caval Vein ◽  
Residual Shunt ◽  
Superior Caval Vein

Abstract Background: Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging. Methods: Retrospective review of all patients from February 2008 to January 2019. Results: Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5−15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6−15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter. Conclusion: Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

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.

Right-sided abnormalities

2018 ◽  
Author(s):  
Nick Archer ◽  
Nicky Manning
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Pulmonary Atresia ◽  
Venous Drainage ◽  
Double Outlet Right Ventricle ◽  
Absent Pulmonary Valve ◽  
Atrioventricular Junction

This chapter explores right-sided abnormalities, discussing the venoatrial junction (including both azygous/hemiazygous connections and abnormal systemic venous drainage), the atrioventricular junction (including tricuspid atresia and Ebstein’s anomaly/tricuspid valve dysplasia), and the ventriculoarterial junction (including pulmonary stenosis, pulmonary atresia with intact septum, tetralogy of Fallot, tetralogy of Fallot with absent pulmonary valve, pulmonary atresia with ventricular septal defect, and double outlet right ventricle), and arterial abnormalities.

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