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"Quality is not an act. It is a habit."


Aristotle


Sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return

date : 18.05.2012


Teodora Monovska Monovska


Imaging Diagnostic Department; Tokuda Hospital Sofia; Vaptzarov blvd.51 B; 1407 Sofia

dori_monovska@yahoo.com


Keywords: atrial septal defect, sinus venosus type, partial anomalous pulmonary venous return, multislice computed tomography


Clinical history: 74-year-old male patient presented at the Emergency department with swelling of lower legs, tachycardia, shortness of breath. The echocardiography showed enlarged right atrium and right ventricle. A murmur was registered on auscultation.


Imaging findings:

The chest radiography demonstrates excessive cardiomegaly with prominent right atrium, right ventricle, dilated pulmonary vessels with marked hilar congestion, bilateral pleural effusion ( fig. 1 ). Additionally on the axial chest CT examination there is also compression of lung parenchyma by the pleural effusion, and significant amount of pericardial effusion ( fig. 2 , 3 , 4 , 5 ). Axial images and additional postprocessing reconstructions demonstrate abnormal communication between right upper lobe pulmonary vein and superior vena cava, and abnormal communication between right and left atria ( ASD ) ( fig.6 ). Suprisingly the septal defect is localized exactly near the junction of the superior vena cava and the right atrium.


Final diagnosis: Sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return ( abnormal communication of right upper pulmonary vein with superior vena cava ).


Discussion:

Partial anomalous pulmonary venous return ( PAPVR ) is a congenital anomaly in which one or more, but not all, of the pulmonary veins are connected to a systemic vein or to the right atrium. PAPVR’s account for 0.5 % of the congenital cardiac defects and are commonly associated with atrial septal defects. Atrial septal defects ( ASD ) are one of the commonest forms of congenital heart disease. The commonest type of ASD is ostium secundum ( 70% ) . The other forms of ASD are ostium primum type ( 20% ) , sinus venosus type ( 10% ) and coronary sinus defect ( < 1% ). About 90% of sinus venosus type of ASD present with PAPVR. Sinus venosus atrial septal defect is a rare congenital deficiency most commonly of superior interatrial septum , almost always associated with partially anomalous right pulmonary venous return to the superior vena cava or the right atrium. It occurs as a result of abnormal fusion between embryologic sinus venosus and the atrium. The location of the defect of the atrial septum in this case is very typical- in the superior part of the septum, adjacent to the superior vena cava entrance into the right atrium. The predominant hemodynamic consequence of the defect is left-to-right shunt. The PAPVR in combination with atrial septal defect is more frequently found in female population. Since the majority of patients with this anomaly are assymptomatic, complications of PAPVR result from pulmonary infection, excessive dilatation of right atrium and ventricle. The important and determinating factor is the number of pulmonary veins that communicate with the systemic circulation. The more veins that anomalously enter a systemic vessel or right atrium, the more blood returns to the right side of the heart and overloads it. It is often difficult to detect pulmonary vein confluence or the combined congenital anomaly by echocardiography and catheter based angiography. Multislice CT is an invaluable non-invasive technique that may provide useful information on anatomical abnormalities. Discovery of PAPVR from the right upper lobe can lead to the diagnosis of an atrial septal defect. This diagnosis can be significant because an undiagnosed atrial septal defect predisposes the patient to paradoxical emboli that can cause strokes and other ischemic events. Less commonly the sinus venosus defect may occur at the junction of the right atrium and inferior vena cava and be associated with anomalous connection of the right lower pulmonary vein to the inferior vena cava. A patch ( synthetic material or pericardium ) technique is used for surgical treatment of sinus venosus ASD with PAPVR. It provides redirection of blood from the right superior pulmonary vein (less commonly right lower pulmonary vein ) into the left atrium .


Differential diagnosis:

1. Ostium secundum ASD 2. Ostium primum ASD 3. Anomalous vena cava superior


Take home messages:

When an atrial septal defect is present it is important to confirm or decline other accompanying congenital anomalies. Partial anomalous pulmonary venous return is observed when a pulmonary vein communicates with a systemic vein or right atrium. Most often the anomaly is combined with sinus venosus type of atrial septal defect. The location of the defect in patients with sinus venosus type of ASD is very typical. It is located high in the interatrial septum at the junction of the superior vena cava and the right atrium. Less commonly the defect is found at the junction of inferior vena cava and right atrium. Multidetector computed tomography is an useful method for fast registration of abnormal communication of the pulmonary veins with the systemic circulation.


Bibliography:

1. H. Mai-Lan, B. Sanjeev, B. Andrew et al ; MDCT of Partial Anomalous Pulmonary Venous Return ( PAPVR ) in Adults; Journal of Thoracic Imaging; 2009; 24:89-95 2. L .Haramati, I .Moche, V.Rivera et al; Computed tomography of partial anomalous pulmonary venous connection in adults; J Comput Assist Tomogr. 2003; 27:743-9 3. H .Kasahara, R. Aeba, Y. Tanami et al ; Multislice computed tomography is useful for evaluating partial anomalous pulmonary venous connection; Journal of Cardiothoracic Surgery 2010; 5:40 4. G .Webb, M .Gatzoulis; Atrial Septal Defects in the Adult - Recent Progress and Overview; Circulation 2006, 114:1645-1653 5. T. Demos, H. Vosniak, K .Pierce et al; Venous Anomalies of the Thorax; A J Roentgenology; 2004; 182:1139-1150 6. R. D.Pascoe, J .K.Oh, C. A.Warnes et al; Diagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal Echocardiography; Circulation 1996; 94: 1049-1055 7. H. Christine J Attenhofer , H. Connolly et al ; Sinus Venosus Atrial Septal Defect:



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