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Year : 2014  |  Volume : 7  |  Issue : 2  |  Page : 160-162
The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect


1 Department of Cardiology, Faculty of Medicine, The University of Sydney, The Children's Hospital at Westmead, Sydney, New South Wales, Australia
2 Department of Cardiology, Faculty of Medicine, The University of Sydney, Westmead Hospital, Sydney, New South Wales, Australia
3 Department of Cardiology, Faculty of Medicine, The University of Sydney, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

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Date of Web Publication14-May-2014
 

   Abstract 

Sinus venosus atrial septal defects (SV-ASDs) are inter-atrial communications caused by a deficiency of the common wall between the superior or inferior vena cava and the right-sided pulmonary veins. The diagnosis can be challenging, especially in adults with delayed presentation. We present images that illustrate an example of the role of cardiac magnetic resonance imaging (CMRI) in the diagnosis and follow-up of a patient with SV-ASD.

Keywords: Atrial septal defect, adult congenital heart disease, cardiac MRI

How to cite this article:
Ganigara M, Tanous D, Celermajer D, Puranik R. The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect. Ann Pediatr Card 2014;7:160-2

How to cite this URL:
Ganigara M, Tanous D, Celermajer D, Puranik R. The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect. Ann Pediatr Card [serial online] 2014 [cited 2019 Oct 14];7:160-2. Available from: http://www.annalspc.com/text.asp?2014/7/2/160/132509



   Introduction Top


Sinus venosus atrial septal defects (SV-ASDs) are inter-atrial communications caused by a deficiency of the common wall between the superior or inferior vena cava and the right-sided pulmonary veins, and are typically associated with partial anomalous right pulmonary venous drainage (PAPVD). They account for 10-15% of all atrial septal defects. As with all ASDs, many patients with this type of defect can be free of symptoms even well into adulthood. This makes early diagnosis challenging and often difficult. Surgical repair is the only treatment for SV-ASD, and the most appropriate surgical strategy varies according to the configuration of the pulmonary veins. Late diagnosis and thus delayed surgical repair carries an increased risk of morbidity and mortality. We illustrate an example of the role of cardiac magnetic resonance imaging (CMRI) in the diagnosis and follow-up of a patient with SV-ASD.


   Clinical Summary Top


A 25-year-old asymptomatic male was incidentally detected to have a murmur. Clinical examination revealed a prominent right ventricular (RV) impulse, a wide and fixed split of the second heart sound with a soft pulmonary component and an ejection systolic murmur at the left upper sternal border. Electrocardiogram (ECG) showed sinus rhythm with partial right bundle branch block pattern. Chest X-ray showed an enlarged right heart with increased pulmonary vascularity. A transthoracic echocardiogram (TTE) demonstrated a moderately dilated RV and right atrium with normal systolic ventricular function. There was trivial tricuspid valve regurgitation with a measured peak regurgitant gradient of 22 mmHg on Doppler evaluation. To further delineate a suspected ASD, a transesophageal echocardiogram (TEE) was performed. TEE demonstrated a SV-ASD with the right upper pulmonary vein connecting to the superior vena cava (SVC); however, the complete pulmonary venous anatomy was not adequately defined.

Hence, CMR examination (1.5 T) was performed. This included standard steady-state free precession (SSFP) cine imaging, breath-held fat suppressed three-dimensional SSFP pulse sequence, gadolinium-enhanced three-dimensional gradient-echo sequences for MR angiography (MRA), and velocity-encoded phase-contrast imaging for flow through the great vessels and shunt calculations (ratio of the net pulmonary flow/net aortic flow). Cine stacks using contiguous slices were obtained in the short axis of the heart and in a trans-axial plane through the entire cardiac mass.

CMRI confirmed a superior SV-ASD measuring 25 mm with anomalous drainage of the right upper pulmonary vein into a single right-sided SVC [Figure 1]. The other pulmonary veins were noted to be draining normally. The right-sided cardiac chambers were dilated with normal RV systolic function. The RV was not hypertrophied and the ventricular septal motion was normal. The indexed RV end-diastolic volume measured 198 ml/m 2 (normal range 55-105 ml/m 2 ). The measured pulmonary to systemic blood flow (Qp:Qs) ratio was 2.1. The left ventricular size and function was normal.
Figure 1: (a) Breath-held fat suppressed three-dimensional steady-state free precession (SSFP) pulse sequence in diastole in the sagittal view demonstrating sinus venosus atrial septal defect (SV-ASD) (arrow) between superior vena cava (SVC) and left atrium (LA). (b) Breath-held fat suppressed three-dimensional SSFP pulse sequence in diastole in the axial view demonstrating SV-ASD (arrow) between SVC and LA. (c) Turbo spin-echo black blood image in the same axial plane as ure 1b demonstrating SV-ASD (arrow) between SVC and LA. (d) SSFP image showing the dilated right ventricle (RV) and left ventricle (LV)

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The patient underwent surgery where a pericardial patch was used to redirect the anomalously draining right upper pulmonary vein to the left atrium and close the defect. Intraoperative TEE confirmed a good surgical result. The patient made a good recovery postoperatively.

At 1-year follow-up, he continued to be asymptomatic. The clinical findings of RV volume overload were no longer evident clinically. TTE showed persisting dilatation of the right heart chambers. CMRI showed no residual ASD with unobstructed and normally draining systemic and pulmonary venous flows. The indexed RV end diastolic volume measured 134 ml/m 2 (normal range 55-105 ml/m 2 ), suggesting persisting moderate dilatation. The RV systolic function was preserved. There was normal size and function of the left ventricle.


   Discussion Top


This case highlights the increasingly important role played by CMRI in the diagnosis and management of SV-ASDs, especially in adults. This pathology can easily be 'missed' on TTE. [1] TEE is a sensitive and cost-effective modality in diagnosing SV-ASDs. However, it is an invasive procedure and may not always provide adequate anatomical and functional information. [2],[3] Importantly, both TTE and TEE can reliably estimate the right-heart pressure, a key pre-surgical criteria. Cardiac catheterization and contrast-enhanced computed tomography (CT) scan are other modalities that can be used; however, they involve exposure to ionizing radiation, and while the former is accompanied by the risks of an invasive procedure the latter does not always provide adequate functional information. [4]

CMRI has inherent advantages over the traditionally used methods. CMRI can accurately quantify ventricular volumes and the magnitude of shunting. [5] Muthurangu et al., have noted that in certain situations, phase contrast MRI is more accurate than invasive cardiac catheterization in quantifying flow and shunt size. [6] Gadolinium enhanced MRA is a sensitive method in detecting abnormalities of pulmonary venous return and can act as a surrogate for PA pressures along with detailed flow data. [7] Additionally; CMRI is noninvasive, has high spatial resolution with large fields of view, and does not involve exposure to ionizing radiation. This allows for serial studies and documentation of normalization of the ventricular volumes, especially relevant in cases such as ours where, late diagnosis and repair is often associated with persisting cardiac dilatation and increased morbidity. [8] These characteristics make CMRI an ideal imaging modality in the follow up of SV-ASDs.

 
   References Top

1.McDonald RW, Rice MJ, Reller MD, Marcella CP, Sahn DJ. Echocardiographic imaging techniques with subcostal and right parasternal longitudinal views in detecting sinus venosus atrial septal defects. J Am Soc Echocardiogr 1996;9:195-8.  Back to cited text no. 1
    
2.Pascoe RD, Oh JK, Warnes CA, Danielson GK, Tajik AJ, Seward JB. Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography. Circulation 1996;94:1049-55.  Back to cited text no. 2
    
3.Ferrari VA, Scott CH, Holland GA, Axel L, Sutton MS. Ultrafast three-dimensional contrast enhanced magnetic resonance angiography and imaging in the diagnosis of partial anomalous pulmonary venous drainage. J Am Coll Cardiol 2001;37:1120-8.  Back to cited text no. 3
    
4.Lembcke A, Razek V, Kivelitz D, Rogalla N, Rogalla P. Sinus venous atrial septal defect with partial anomalous pulmonary venous return: Diagnosis with 64-slice spiral computed tomography at low radiation dose. J Pediatr Surg 2008;43:410-1.  Back to cited text no. 4
    
5.Prompona M, Muehling O, Naebauer M, Schoenberg SO, Reiser M, Huber A. MRI for detection of anomalous pulmonary venous drainage in patients with sinus venosus atrial septal defects. Int J Cardiovasc Imaging 2011;27:403-12.  Back to cited text no. 5
    
6.Muthurangu V, Taylor A, Andriantsimiavona R, Hegde S, Miquel ME, Tulloh R, et al. Novel method of quantifying pulmonary vascular resistance by use of simultaneous invasive pressure monitoring and phase-contrast magnetic resonance flow. Circulation 2004;110:826-34.  Back to cited text no. 6
    
7.Puvaneswary M, Leitch J, Chard RB. MRI of partial anomalous pulmonary venous return (scimitar syndrome). Australas Radiol 2003;47:92-3.  Back to cited text no. 7
    
8.de Koning WB, van Osch-Gevers LM, Robbers-Visser D, van Domburg RT, Bogers AJ, Helbing WA. Enlarged right ventricular size at 11 years' follow-up after closure of secundum-type atrial septal defect in children. Cardiol Young 2013;23:7-13.  Back to cited text no. 8
    

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Correspondence Address:
Dr. Madhusudan Ganigara
Department of Cardiology, The Children's Hospital at Westmead, Westmead, New South Wales-2145
Australia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.132509

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    Figures

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