Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

Crystal, Matthew A.; Najashi, Khaled Al; Williams, William G.; Redington, Andrew N.; Anderson, Robert H.

doi:10.1016/j.jtcvs.2008.12.010

Cited by 33 publications

(40 citation statements)

References 16 publications

(19 reference statements)

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“…[4][5][6] It has been reported with an intact atrial septum [4] and in association with an interatrial communication; this can be an ostium secundum defect [5] or a sinus venosus defect. [6] In the latter case, total [7] or partial [8] anomalous drainage of pulmonary veins to the IVC can be associated. Also, in this case, there is frequently overriding of the intact muscular border of the oval fossa by the mouth of the IVC.…”

Section: Discussionmentioning

confidence: 99%

Inferior vena cava return in left atrium: a rare presentation with severe mitral regurgitation

Chibane¹

2016

Turk Gogus Kalp Dama

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ÖZBu yazıda, klinik tabloyu değiştiren şiddetli mitral yetersizlik ile ilişkili interatriyal iletişimli sol atriyumda inferior vena kavanın anormal bağlantısı olan nadir bir olgu sunuldu. Mitral kapak tamiri ve inferior cava akımının yeniden sağ atriyuma yönlendirilmesinin ardından başarılı bir düzeltme sağlandı.Anah tar söz cük ler: Kardiyak septal defekt; mitral kapak yetersizliği; sinüs venozus atriyal septal defekt; sistemik venöz dönüş. ABSTRACTHerein, we present a rare case of abnormal connection of the inferior vena cava to the left atrium with an interatrial communication associated with severe mitral regurgitation which altered clinical presentation. Successful correction was achieved after mitral valve repair and re-routing of the inferior cava flow to the right atrium.Keywords: Cardiac septal defect; mitral valve insufficiency; sinus venosus atrial septal defect; systemic venous return.Inferior vena cava (IVC) draining into the left atrium is a rare anomaly of systemic venous return. [1][2][3] In the literature, several cases with various anatomical associations have been published. [1][2][3][4][5][6] Herein, we report a female case with a Marfanoid morphotype, presenting with an abnormal return of the IVC into the left atrium associated with an interatrial communication and severe mitral regurgitation. CASE REPORTA 16-year-old schoolgirl was referred for surgical treatment of severe mitral regurgitation associated with an interatrial communication. Physical examination revealed a good overall condition with dyspnea New York Heart Association (NYHA) Class II without cyanosis. Blood pressure was 107/65 mmHg, oxygen saturation 96%, weight 42 kg and height 166 cm. The patient suffered from several phenotypic features mimicking Marfan syndrome; however, she did not fulfill the Ghent's criteria for the definite diagnosis of Marfan syndrome. She presented with skeletal malformations including moderate pectus excavatum, arachnodactyly, joint hypermobility, an arm span to height ratio greater than 1.05; and a decrease in visual acuity. The cardiac examination showed a 3/6 systolic ejection murmur at the apex, split and fixed S2. There was no sign of the right heart failure and all findings were non-specific. Electrocardiography showed a regular sinus rhythm at 8/bpm and biatrial hypertrophy. Chest radiography revealed a cardiothoracic ratio of 0.5 with a prominent pulmonary artery segment and increased pulmonary vascular markings.Echocardiography demonstrated a moderately dilated left ventricle with normal systolic functions and dilated right cavities; an aortic root diameter of 20 mm without regurgitation, Grade 3 mitral regurgitation by A2 and P2 prolapse and trivial tricuspid regurgitation. A low-situated interatrial communication was also diagnosed with significant left to right shunting (QP/QS>2). Computed tomography scan demonstrated a large IVC connecting directly to the floor of left atrium (Figure 1).The patient was operated on by median sternotomy under cardiopulmonary bypass and aortic

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Section: Discussionmentioning

confidence: 99%

Inferior vena cava return in left atrium: a rare presentation with severe mitral regurgitation

Chibane¹

2016

Turk Gogus Kalp Dama

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“…Different approaches for the treatment of inferior SVDs have been successfully reported, including a suture closure with8 and without9 a patch, and reimplantation of the anomalous pulmonary vein 10. We need to look for the entry point of the IVC cannula into the IVC and suturing the patch beneath the cannula not to mistake the Eustachian valve for the inferior margin of ASD.…”

Section: Discussionmentioning

confidence: 99%

A case of residual inferior sinus venosus defect after ineffective surgical closure

et al. 2014

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A 38-year-old woman presented with cyanosis and heart failure 34 years after patch closure of an atrial septal defect and partial anomalous pulmonary venous connection. CT and cardiac catheterisation showed a residual defect that caused right-to-left shunting. The patch almost blocked the inferior vena cava from the right atrium, resulting in uncommon drainage of the inferior vena cava into the left atrium. Other anomalies included the coronary-to-pulmonary artery fistula and duplicate inferior vena cava with dilated azygos venous system. A second surgery was performed, and we confirmed an inferior sinus venosus defect, which is rare and can be misdiagnosed. The ineffective patch closure had caused a haemodynamic status that rarely occurs. We describe the diagnostic process and emphasise the importance of correctly understanding the entity.

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“…the fossa ovalis and its surrounding muscular rim), they are placed in the same physiological category as atrial septal defects (ASD) and account for up to 11% of interatrial communications (1–4). SVDs of the inferior vena cava (IVC) type, or inferior SVDs, involve a deficiency in postero-inferior aspects of the atrial septum, resulting in a communication between the right and left atrial chambers through the mouth of the inferior vena cava (IVC) (2, 5, 6). An intact fossa ovalis and the IVC straddling over the defect are considered as additional prerequisites for diagnosing inferior SVDs.…”

Section: Introductionmentioning

confidence: 99%

“…An intact fossa ovalis and the IVC straddling over the defect are considered as additional prerequisites for diagnosing inferior SVDs. Anomalous pulmonary venous drainage to the IVC is a common finding, and has been regarded by some as an anatomical criterion for the diagnosis of inferior SVDs (2, 5, 6), whereas, others have not included this feature as a diagnostic criterion (1) because it is not present consistently.…”

Section: Introductionmentioning

confidence: 99%

“…The presence of an inferior SVD may also influence the specific placement of the IVC cannula prior to cardiopulmonary bypass (1). Previous studies have reported challenges with accurate preoperative diagnosis of inferior SVDs, with the defects often being mistaken for large secundum ASDs with inferior extension (5–7). While combination defects within the atria may be present, defining the posteroinferior rim anatomy itself has profound implications upon treatment strategy and overall hospital course.…”

Section: Introductionmentioning

confidence: 99%

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The Parasternal Short-Axis View Improves Diagnostic Accuracy for Inferior Sinus Venosus Type of Atrial Septal Defects by Transthoracic Echocardiography

Snarr

Liu

Zuckerberg

et al. 2017

Journal of the American Society of Echocardiography

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Background-Sinus venosus defects (SVD) of the inferior vena cava (IVC) type, or inferior SVDs, are an uncommon form of atrial communication located outside the confines of the fossa ovalis and involve override of the IVC. Despite numerous studies describing the anatomical and echocardiographic features of the inferior SVD, distinguishing this defect from a large secundum atrial septal defect (ASD) by echocardiography is often challenging. Accurate diagnosis of an inferior SVD and correct differentiation from a secundum ASD is essential for appropriate presurgical planning. Absence of the posterior rim in the parasternal short axis views may serve as a useful clue in diagnosing inferior SVDs. We sought to determine the utility of using the presence or absence of a posterior atrial rim in the parasternal short axis view to help distinguish an inferior SVD from a secundum ASD. This sign may help clinch the diagnosis when subcostal imaging is suboptimal.

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Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

Abstract: Transthoracic echocardiographic analysis should remain the modality of choice for diagnosis of the inferior sinus venosus defect. We report excellent surgical results with a patch or baffle, correctly redirecting the anomalous venoatrial connections.

Cited by 33 publications

References 16 publications

Inferior vena cava return in left atrium: a rare presentation with severe mitral regurgitation

Inferior vena cava return in left atrium: a rare presentation with severe mitral regurgitation

A case of residual inferior sinus venosus defect after ineffective surgical closure

The Parasternal Short-Axis View Improves Diagnostic Accuracy for Inferior Sinus Venosus Type of Atrial Septal Defects by Transthoracic Echocardiography

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