Septum primum malposition defect and inferior sinus venosus defect: a rare association

2014 ◽  
Vol 25 (7) ◽  
pp. 1389-1392 ◽  
Author(s):  
Deepa Prasad ◽  
Christopher Snyder ◽  
Ravi Ashwath
Keyword(s):  
Respiratory Distress ◽  
Pulmonary Veins ◽  
Atrial Septum ◽  
Sinus Venosus ◽  
Rare Association ◽  
Septum Primum ◽  
Sinus Venosus Defect ◽  
The Right ◽  
Inferior Sinus Venosus Defect ◽  
Septum Secundum

AbstractWe present a case of a 2-year-old girl who presented with respiratory distress and umbilical abscess, and was found to have an inferior sinus venosus defect, malposition of the atrial septum primum, absent septum secundum, and anomalous drainage of the right upper and lower pulmonary veins to the right atrium.

Tetralogy of Fallot With Interatrial Communication of the Inferior Sinus Venosus Type: A Rare Association Causing Post-Operative Desaturation

2019 ◽  
Vol 10 (6) ◽  
pp. 801-802
Author(s):  
Gaurav Agrawal ◽  
Anupam Das
Keyword(s):  
Case Report ◽  
Tetralogy Of Fallot ◽  
Sinus Venosus ◽  
Transthoracic Echocardiogram ◽  
Interatrial Communication ◽  
Postoperative Course ◽  
Rare Association ◽  
Intracardiac Repair ◽  
Sinus Venosus Defect ◽  
Inferior Sinus Venosus Defect

This case report describes a rare association of tetralogy of Fallot with interatrial communication of the inferior sinus venosus type (inferior sinus venosus defect). The patient underwent intracardiac repair for tetralogy of Fallot at 8 months of age. His postoperative course was complicated by desaturation. On reexploration, interatrial communication of the inferior sinus venosus type was revealed which went unnoticed on transthoracic echocardiogram as well as on computerized tomogram.

Echocardiographic diagnosis of inferior sinus venosus defects

1992 ◽  
Vol 2 (4) ◽  
pp. 338-341 ◽  
Author(s):  
José A. Ettedgui ◽  
Ralph D. Siewers ◽  
James R. Zuberbuhler ◽  
Robert H. Anderson
Keyword(s):  
Correct Diagnosis ◽  
Pulmonary Veins ◽  
Sinus Venosus ◽  
Caval Vein ◽  
Diagnostic Features ◽  
Cross Sectional ◽  
Interatrial Communication ◽  
Inferior Caval Vein ◽  
Sinus Venosus Defect ◽  
Inferior Sinus Venosus Defect

AbstractSinus venosus defects are relatively uncommon forms of interatrial communication in which one of the caval veins has a biatrial connection, and partial anomalous pulmonary venous connections are the rule. The variety related to the inferior caval vein is much the rarer of the two forms, and here the defect is posterior and inferiorly placed. To determine whether an inferior sinus venosus defect could be identified preoperatively, we reviewed retrospectively the available echocardiograms and cineangiograms of the five patients with an inferior sinus venosus defect who underwent surgical repair between January 1983 and December 1991. A cross-sectional echocardiogram had been performed preoperatively in four of these patients, but the correct diagnosis had been made in only one case. Retrospective study, however, showed that the extent of the true atrial septum and the nature of the junction of the inferior caval vein with the atriums could be evaluated from the subcostal position in three of these four patients. Each of the three had an interatrial communication located posteriorly and inferiorly adjacent to the atrial connection of the inferior caval vein, along with abnormally connected right pulmonary veins. The oval fossa was intact and the coronary sinus was normal in all. Cardiac catheterization had been performed in three of the five patients. Anomalous return of right pulmonary veins was noted in each, and the posterior and inferior location of the interatrial communication was evident in two. An interatrial communication adjacent to the atrial connection of the inferior caval vein, along with anomalous connection of the right-sided pulmonary veins, are the diagnostic features of the inferior variety of sinus venosus defects. These features can be demonstrated by cross-sectional echocardiography from the subcostal position.

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.

Abstract 14310: Elephant Sitting on My Chest: A Tale of Two Vessels

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Anudeep K Dodeja ◽  
Patrick I McConnell ◽  
Elisa A Bradley ◽  
Curt Daniels ◽  
Laxmi S Mehta ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Symptomatic Relief ◽  
Pulmonary Veins ◽  
Main Pulmonary Artery ◽  
Laboratory Data ◽  
Venous Drainage ◽  
Sinus Venosus ◽  
Exercise Intolerance ◽  
Extrinsic Compression ◽  
Sinus Venosus Defect

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

scholarly journals The Vein Of Galen Aneurysmal Malformation Associated With Sinus Venosus Defect And Anomalous Pulmonary Veins

2020 ◽  
Vol 5 (2) ◽  
pp. 1-4
Author(s):  
Merjema Karavdic ◽  
Keyword(s):  
Neonatal Period ◽  
Pulmonary Veins ◽  
Arteriovenous Shunt ◽  
Sinus Venosus ◽  
Vein Of Galen ◽  
Cardiac Defect ◽  
Congenital Cardiac Defect ◽  
Sinus Venosus Defect

he Vein of Galen Aneurysmal Malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM is safer in infancy, even it may be undertaken in the neonatal period if necessary.

scholarly journals Echocardiographic diagnosis and repair of an inferior sinus venosus defect

2008 ◽  
Vol 24 (1) ◽  
pp. 67 ◽  
Author(s):  
Daniel R. Wong ◽  
Camille L. Hancock Friesen ◽  
Andrew E. Warren ◽  
Stacy B. O’Blenes
Keyword(s):  
Sinus Venosus ◽  
Sinus Venosus Defect ◽  
Inferior Sinus Venosus Defect

scholarly journals P648 Right heart volume overload after surgical correction of atrial septal defect sinus venosus type with partial anomalous pulmonary veins drainage

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Ruiz-Zamora ◽  
L Alvarez-Roy ◽  
G Pinillos-Francia ◽  
A Gutierrez-Fernandez ◽  
M Gomez-Llorente ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Vein ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Sinus Venosus ◽  
The Right

Abstract 40 year-old male with history of congenital heart disease. Atrial septal defect (ASD) sinus venosus type associated with partial anomalous pulmonary venous drainage (PAPVD) was diagnosed during childhood and surgically repaired at the age of three. Since then the patient was asymptomatic and he was lost to follow up when reached adulthood. A transthorathic echocardiogram was performed during a hospitalization because of a complicated pneumonia. A severe dilatation of right chambers and main pulmonary artery was observed. The estimated Qp:Qs by this technique was 2.0 but the atrial septum seemed to be intact. Agitated saline was administered in this procedure and no passage of microbubbles was observed. A cardiac MRI was performed because of suspicion of anomalous pulmonary venous drainage. The findings observed in the echocardiogram were confirmed and an uncommon PAPVD was demonstrated by this technique: the superior left pulmonary vein drainaged into a dilated innominated vein. Also, a small pulmonary vein from the right upper lobe emptied into a "venous conduct" (yellow circle) located posterior to the superior cava vein (SCV) that later drainaged into the right atrium under the mouth of the SVC, posterior to the interatrial septum. Surgically correctioin of the PAPVD was performed. Discussion PAPVD is a congenital heart disease characterized by the drainage of one or several pulmonary veins –but not all of them- into the right atrium or systemic veins, which leads to left-to-right shunting. The estimated incidence of this disease ranges between 0.1 and 0.2% of the general population. The right superior pulmonary vein is the most frequently involved and is commonly associated to ASD sinus venosus type. Bilateral anomalous drainage, as occurred in this case, is exceptional. Furhermore, this case highlights the importance of long-term follow-up in patients with congenital heart disease due to the fact that, even when the disease is considered to be cured, long term complications could appear compromising the prognosis of the patient. Abstract P648 Figure. Unusual PAPVD

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