Understanding the spectrum of sinus venosus interatrial communications

2016 ◽  
Vol 27 (3) ◽  
pp. 418-426 ◽  
Author(s):  
Justin T. Tretter ◽  
Sathish Chikkabyrappa ◽  
Diane E. Spicer ◽  
Carl L. Backer ◽  
Ralph S. Mosca ◽  
...  
Keyword(s):  
Cardiac Development ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Interatrial Septum ◽  
Sinus Venosus ◽  
Group 13 ◽  
Common Wall ◽  
Sinus Venosus Defect ◽  
Autopsy Specimens ◽  
Anomalous Pulmonary Venous Connections

AbstractBackgroundIt is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects.MethodsWe reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens.ResultsWe evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases.ConclusionAppreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.

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.

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.

scholarly journals The morphogenesis and associated anomalous pulmonary venous drainage in sinus venosus defect

2020 ◽  
Vol 61 (1) ◽  
pp. 92-99
Author(s):  
Hsin-Mao Hsu ◽  
Ya-Ting Chang ◽  
Wen-Jen Su ◽  
Jaw-Ji Chu ◽  
Yu-Sheng Chang ◽  
...  
Keyword(s):  
Venous Drainage ◽  
Sinus Venosus ◽  
Anomalous Pulmonary Venous Drainage ◽  
Sinus Venosus Defect

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 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

scholarly journals Veno-venous bridges: the forerunners of the sinus venosus defect

2011 ◽  
Vol 21 (6) ◽  
pp. 623-630 ◽  
Author(s):  
Ryan J. Butts ◽  
Andrew M. Crean ◽  
Anthony M. Hlavacek ◽  
Diane E. Spicer ◽  
Andrew C. Cook ◽  
...  
Keyword(s):  
Septal Defect ◽  
Clinical Information ◽  
Sinus Venosus ◽  
Morphological Evidence ◽  
Radiological Findings ◽  
Sinus Venosus Defect ◽  
Anomalous Pulmonary Venous Connections

AbstractBackgroundDifferentiation of the so-called sinus venosus defect from other defects permitting shunting between the atrial chambers remains problematic. The lesion is not a true septal defect, and current theories to explain the existence of the sinus venosus defect fall short. The presence of persistent systemic to pulmonary venous connections has been proposed to explain the existence of the sinus venosus defect.MethodsClinical histories and radiological findings of six patients are reviewed. Three patients have veno-venous bridges, two have partial anomalous pulmonary venous connections, and one patient has a sinus venosus defect. The clinical information is reviewed, along with current developmental and morphological considerations.DiscussionWe provide radiographic, developmental, and morphological evidence to support the theory that a so-called sinus venosus defect is the consequence of persistence of foetal systemic to pulmonary veno-venous bridges, rather than of deficiencies in atrial septation.

DEVELOPMENT OF THE PULMONARY VEINS

PEDIATRICS ◽  
1956 ◽  
Vol 18 (6) ◽  
pp. 880-887
Author(s):  
Catherine A. Neill
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Atrial Septum ◽  
Sinus Venosus ◽  
Human Embryos ◽  
Left Auricle ◽  
Serial Sections ◽  
The Common ◽  
Common Pulmonary Vein

The examination of serial sections of human embryos between 24 and 34 days (3 to 11 mm) and the use of plastic reconstructions, showed that the common pulmonary vein develops as an outgrowth from the medial superior wall of the left auricle and unites with the angioblastic plexus of the developing lung bud. No evidence was found that the vein connects directly with the sinus venosus in the early stages, and later shifts in position as the atrial septum grows. Anomalous pulmonary venous drainage is classified in four main types, and theories of development are briefly discussed.

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.

Repair of Sinus Venosus Defect and Anomalous Pulmonary Veins Associated With Vein of Galen Malformation

2018 ◽  
Vol 11 (4) ◽  
pp. NP151-NP154
Author(s):  
Heather N. Anderson ◽  
Sameh M. Said ◽  
Frank Cetta
Keyword(s):  
Pulmonary Veins ◽  
Sinus Venosus ◽  
Surgical Removal ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Unique Challenge ◽  
Percutaneous Device ◽  
Embolization Treatment ◽  
Sinus Venosus Defect ◽  
Cardiac Enlargement

Sinus venosus defect (SVD) is a deficiency in the sinus venosus portion of the atrium, often associated with partial anomalous pulmonary venous connections. In patients with vein of Galen malformation (VGAM), SVD provides a unique challenge with respect to embolization treatment. In our case, the child had percutaneous device closure of the SVD prior to VGAM embolization. Nine years later, he required surgical removal of the SVD device and Warden procedure due to progressive right-sided cardiac enlargement.

Long-Term Outcomes of the Double-Barrel Technique for Superior Sinus Venosus Defect With Partially Anomalous Pulmonary Venous Connection

2020 ◽  
Vol 11 (6) ◽  
pp. 733-741
Author(s):  
Ujjwal Kumar Chowdhury ◽  
Srikant Sharma ◽  
Lakshmi Kumari Sankhyan ◽  
Niwin George ◽  
Sukhjeet Singh ◽  
...  
Keyword(s):  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Growth Potential ◽  
Sinus Venosus ◽  
Long Term Outcomes ◽  
Sinus Venosus Defect ◽  
Anomalous Pulmonary Venous Connection

Background: Repair of superior sinus venosus defect with high partially anomalous pulmonary venous connection (PAPVC) using an intracardiac baffle may be complicated by systemic or pulmonary venous pathway obstruction and sinus nodal dysfunction (SND). Our surgical strategy for repair of all types of superior sinus venosus defect has evolved chiefly to avoid the abovementioned complications and preserving the growth potential of the superior cavoatrial junction. Methods: Between 2007 and 2019, fifty consecutive patients aged 2 to 60 (mean, 17.6±16.7) years underwent repair of superior sinus venosus defect using the double-barrel technique as described. The anomalous pulmonary veins drained into the superior cavoatrial junction in 17 patients and more than 2 cm above the cavoatrial junction in 33 patients. Results: There were no early or late deaths and no reoperations. At a mean follow-up of 103.9 (±50.2) months, all survived the operation, and actuarial freedom from SND was 97.9% (±standard error, 0.02%; 95% CI: 0.86-0.99). No patient had systemic or pulmonary venous pathway obstruction. A permanent pacemaker was required in one (2%) patient for sick sinus syndrome. Conclusions: The double-barrel method is an expedient, safe, and effective technique in superior sinus venosus defect. It provides dual drainage of superior vena cava preserving the superior cavoatrial junction without causing systemic or pulmonary venous pathway obstruction and can be utilized in all cases including those with high PAPVC. Preservation of the cavoatrial junction and use of autogenous atrial tissue for systemic venous pathway avoids SND and preserves growth potential.

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