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DOI:10.2214/AJR.07.3430
AJR 2009; 192:259-266
© American Roentgen Ray Society

Original Research

Cardiac MRI and Pulmonary MR Angiography of Sinus Venosus Defect and Partial Anomalous Pulmonary Venous Connection in Cause of Right Undiagnosed Ventricular Enlargement

Henryk Kafka1,2,3,4 and Raad H. Mohiaddin1,3

1 Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom.
2 Adult Congenital Heart Centre, Royal Brompton Hospital, London, United Kingdom.
3 National Heart and Lung Institute, Imperial College, London, United Kingdom.
4 Department of Radiology, Division of Cardiology, Queen's University Cardiovascular Laboratory, Kingston General Hospital, 76 Stuart St., Kingston, ON K7L 2V7, Canada.

Received November 11, 2007; accepted after revision July 1, 2008.

 
H. Kafka is a Detweiler Fellow of the Royal College of Physicians and Surgeons of Canada and has received funding support from the Department of Medicine at Queen's University, Kingston, ON, Canada.

Address correspondence to H. Kafka (kafkamd{at}usa.net).

FOR YOUR INFORMATION

A data supplement for this article can be viewed in the online version of the article at: www.ajronline.org.


Abstract
Top
 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 References
 
OBJECTIVE. Patients may be referred for cardiology assessment because of an enlarged right ventricle (RV) with no cause apparent on echocardiography. Cardiac MRI can contribute to the management of these patients by detecting sinus venosus defect or partial anomalous pulmonary venous connection (PAPVC). We sought to show how often sinus venosus defect or PAPVC was detected on MRI in patients with an enlarged RV without a previously established definite diagnosis.

MATERIALS AND METHODS. First cardiac MRI scans obtained over a 4-year period in adults with an undiagnosed cause of RV enlargement were searched for the MRI diagnosis of sinus venosus defect or PAPVC.

RESULTS. Thirty-seven patients (25 female, 12 male) met the study criteria. Nineteen patients had a cardiac MRI diagnosis of sinus venosus defect, with PAPVC being present in 95% of those patients. All PAPVCs associated with sinus venosus defect were from the right side. Eleven of the 19 patients with sinus venosus defect underwent surgery at our institution. Sinus venosus defect was confirmed in all 11 cases. Of the 37 patients, 36 had PAPVC, which was right-sided in 27 patients (75%), left-sided in seven patients (19.4%), and bilateral in two patients (5.6%). Three patients had scimitar veins. The common defects associated with PAPVC were sinus venosus defect in 18 patients (50%) and secundum atrial septal defect in six patients (17%).

CONCLUSION. This article about cardiac MRI in adults with sinus venosus defect and PAPVC shows that cardiac MRI can reliably detect and quantify these lesions when other methods have not provided a complete diagnosis for the cause of right heart enlargement.

Keywords: congenital heart disease • MR angiography • MRI • partial anomalous pulmonary venous connection • sinus venosus defect


Introduction
Top
 Abstract
 Introduction
Materials and Methods
 Results
 Discussion
 References
 
Most adult patients with an enlarged right ventricle (RV) detected on transthoracic echocardiography (TTE) will have readily apparent causes, such as an atrial septal defect (ASD). But there are situations in which no definite cause of enlarged RV is revealed on TTE. Before a diagnosis of RV cardiomyopathy or primary pulmonary hypertension is made, other less apparent causes [1, 2] must be considered, such as sinus venosus defect or partial anomalous pulmonary venous connection (PAPVC). Although TTE has not been very good at revealing these anomalies [3], good detection rates [4, 5] have been reported for transesophageal echocardiography (TEE). Despite the use of TEE, there are patients in whom the cause of RV enlargement remains unexplained or incompletely explained. Cardiac MRI has been successfully used in patients with intracardiac and extracardiac shunts [6-11], but to our knowledge there have been no systematic analyses of cardiac MRI for the detection and quantification of sinus venosus defect in the adult. In this study, we sought to show that cardiac MRI plays an important role in the detection of sinus venosus defect and PAPVC in the adult patient for whom other investigations have not provided a complete explanation for an enlarged RV.


Materials and Methods
Top
 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 References
 
Subject Selection
All first cardiac MRI scans in patients with an enlarged RV obtained between June 2002 and May 2006 were reviewed, searching for a cardiac MRI diagnosis of sinus venosus defect or PAPVC. The study group of 37 patients included only those subjects in whom there had been no definite complete diagnosis before cardiac MRI. The cardiac MRI scans were reviewed and conclusions confirmed using the criteria outlined in the following text. Notes regarding previous investigations, as well as operative notes for surgery at our institution, were reviewed.


Figure 1
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  		Fig. 1A —23-year-old woman with partial anomalous pulmonary venous connections. Phase images of through-plane velocity maps for quantifying flow in pulmonary artery and aorta in this patient with two anomalous right pulmonary venous connections and secundum atrial septum defect (patient 37). PA = pulmonary artery, Ao = aorta.

 


Figure 2
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  		Fig. 1B —23-year-old woman with partial anomalous pulmonary venous connections. Phase images of through-plane velocity maps for quantifying flow in pulmonary artery and aorta in this patient with two anomalous right pulmonary venous connections and secundum atrial septum defect (patient 37). PA = pulmonary artery, Ao = aorta.

 


Figure 3
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  		Fig. 1C —23-year-old woman with partial anomalous pulmonary venous connections. Velocity-versus-time curves for blood flow in aorta and pulmonary artery. Qp/Qs = pulmonary-to-systemic blood flow ratio.

 
Cardiac MRI and Contrast-Enhanced MR Angiography
Cardiac MRI was performed using a Sonata 1.5-T or an Avanto 1.5-T scanner (both by Siemens Medical Solutions). Multislice single-shot spinecho images using HASTE were obtained in three orthogonal planes to define the cardiac anatomy. In some patients, steady-state free precession (SSFP) multislice images were also obtained to improve definition of the blood-tissue borders. Turbo spin-echo imaging was often undertaken to delineate the edges of the sinus venosus defect. Ventricular volumes, ventricular mass, and systolic function were calculated using a stack of short-axis ECG-gated cine images. Cine phasecontrast velocity flow maps were obtained in accordance with established protocols and practice [12] to better detect the anomalous flow patterns and to calculate the pulmonary-to-systemic (Qp/Qs) blood flow ratio (Figs. 1A, 1B, and 1C and Table 1). Contrast-enhanced MR angiography (CE-MRA) was also performed in some patients. The decision to perform CE-MRA was made by the individual performing the scanning. In those cases, the coronal orientation was used during breath-hold at end-inspiration before and after the IV administration of gadopentetate dimeglumine (Magnevist, Schering) [11]. The bolus was timed to the arrival of the contrast agent in the ascending aorta. Imaging parameters are summarized in Table 2.


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  		TABLE 1 : Patient Characteristics and Cardiac MRI Findings

 

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  		TABLE 2 : Imaging Parameters

 

Specific scanning techniques used for sinus venosus defect—Sinus venosus defect is not a defect of the atrial septum. Rather, the mouth of the caval vein, usually the superior vena cava (SVC), has biatrial connections [1, 2] overriding the rim of the oval fossa, producing an extraseptal interatrial communication. It is this overriding of the caval vein across the atrial septum that forms the basis for the imaging diagnosis of the sinus venosus defect [1, 2]. Therefore, the preferred views for the sinus venosus defect are the transverse and sagittal planes that are perpendicular to the border between the SVC and the left atrium. SSFP cine imaging in these planes produces better delineation of blood-tissue borders (Figs. 2A, and 2B). Cine phase-contrast velocity flow mapping can high-light the location and size of the sinus venosus defect (Figs. 3A, 3B, 3C, 3D, 3E, and 3F and Fig. S3, AVI cine images, in supplemental data at www.ajronline.org).


Figure 4
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  		Fig. 2A —44-year-old man with sinus venosus defect. Transverse steady-state free precession cine frame (A) shows sinus venosus defect (arrow) between superior vena cava (asterisk) and left atrium (LA). Superior nature of this defect and absence of an upper rim of defect are evident in sagittal image (B). Ao = aorta, PA = pulmonary artery, RPA = right pulmonary artery, RA = right atrium.

 

Figure 5
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  		Fig. 2B —44-year-old man with sinus venosus defect. Transverse steady-state free precession cine frame (A) shows sinus venosus defect (arrow) between superior vena cava (asterisk) and left atrium (LA). Superior nature of this defect and absence of an upper rim of defect are evident in sagittal image (B). Ao = aorta, PA = pulmonary artery, RPA = right pulmonary artery, RA = right atrium.

 

Figure 6
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  		Fig. 3A —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Transverse images in 32-year-old man with sinus venosus defect (patient 24). Steady-state free precession cine image (A) shows sinus venosus defect (black arrow) between left atrium (LA) and superior vena cava (SVC) (asterisk). Corresponding in-plane velocity flow map (B) shows dark inflow from pulmonary vein (white arrow) into LA crossing sinus venosus defect and entering SVC.

 

Figure 7
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  		Fig. 3B —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Transverse images in 32-year-old man with sinus venosus defect (patient 24). Steady-state free precession cine image (A) shows sinus venosus defect (black arrow) between left atrium (LA) and superior vena cava (SVC) (asterisk). Corresponding in-plane velocity flow map (B) shows dark inflow from pulmonary vein (white arrow) into LA crossing sinus venosus defect and entering SVC.

 

Figure 8
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  		Fig. 3C —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Sagittal images in 35-year-old woman with sinus venosus defect (patient 4). Steady-state free precession cine frame (C) shows superior nature of sinus venosus defect (arrow) between LA and SVC (asterisk). Corresponding in-plane velocity flow map (D) shows dark inflow from LA across sinus venosus defect (arrow) into right atrium (RA).

 

Figure 9
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  		Fig. 3D —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Sagittal images in 35-year-old woman with sinus venosus defect (patient 4). Steady-state free precession cine frame (C) shows superior nature of sinus venosus defect (arrow) between LA and SVC (asterisk). Corresponding in-plane velocity flow map (D) shows dark inflow from LA across sinus venosus defect (arrow) into right atrium (RA).

 

Figure 10
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  		Fig. 3E —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Coronal images in 18-year-old woman with sinus venosus defect (patient 20). FLASH image (E) shows bright flow disturbance in SVC (asterisk) related to flow through sinus venosus defect. Through-plane velocity flow map (F) in same position as Eshows sinus venosus defect as dark region of flow (arrow) from LA. Ao = aorta.

 

Figure 11
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  		Fig. 3F —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Coronal images in 18-year-old woman with sinus venosus defect (patient 20). FLASH image (E) shows bright flow disturbance in SVC (asterisk) related to flow through sinus venosus defect. Through-plane velocity flow map (F) in same position as Eshows sinus venosus defect as dark region of flow (arrow) from LA. Ao = aorta.

 
Specific scanning techniques used for PAPVC— The anomalous connection of a right pulmonary vein can be to the right atrium (RA), the SVC, the SVC-RA junction, or, less commonly, the inferior vena cava (IVC). Transverse imaging is best suited to detect these connections because that plane allows cross-sectional visualization of the caval veins and the RA (Figs. 4A, 4B, 4C, and 4D). Flow from the pulmonary vein into the RA can be confirmed with cine phase-contrast velocity flow mapping (Figs. 5A, 5B, 5C, and 5D). The most common left anomalous venous connection is that of the left upper pulmonary vein to a vertical vein that drains up to the brachio cephalic vein and from there to the SVC (Figs. 6A, 6B, and 6C). CE-MRA (Figs. 6A, 6B, and 6C) has been suggested for better definition of both right and left anomalous connections [9-11].


Figure 12
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  		Fig. 4A —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Turbo spin-echo image shows connection of right upper pulmonary vein (arrow) to SVC.

 

Figure 13
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  		Fig. 4B —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Slice 20 mm caudad to A clearly shows sinus venosus defect (arrow).

 

Figure 14
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  		Fig. 4C —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Steady-state free precession cine still images at same levels as Aand Balso show sinus venosus defect (arrow, D). Arrow in C indicates anomalous connection of upper pulmonary vein to SVC.

 

Figure 15
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  		Fig. 4D —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Steady-state free precession cine still images at same levels as Aand Balso show sinus venosus defect (arrow, D). Arrow in C indicates anomalous connection of upper pulmonary vein to SVC.

 

Figure 16
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  		Fig. 5A —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Oblique axial steady-state free precession cine image (A) shows anomalous connection of right upper pulmonary vein (arrowhead). Sinus venosus defect (arrow) is also evident at this level. Bright white signal in this velocity flow map (B) confirms flow from pulmonary vein into RA.

 

Figure 17
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  		Fig. 5B —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Oblique axial steady-state free precession cine image (A) shows anomalous connection of right upper pulmonary vein (arrowhead). Sinus venosus defect (arrow) is also evident at this level. Bright white signal in this velocity flow map (B) confirms flow from pulmonary vein into RA.

 

Figure 18
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  		Fig. 5C —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Coronal steady-state free precession cine image shows connection of right pulmonary vein to RA-superior vena cava junction (arrowhead).

 

Figure 19
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  		Fig. 5D —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Dark signal in this velocity flow map shows flow (arrowhead) from anomalous pulmonary vein into RA.

 

Figure 20
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  		Fig. 6A —Contrast-enhanced MR angiography maximum-intensity-projection coronal images in three patients with partial anomalous pulmonary venous connection. Ao = aorta, RA = right atrium. 18-year-old woman with anomalous connection of right pulmonary vein (arrow) to superior vena cava (SVC) (asterisk). PA = pulmonary artery.

 

Figure 21
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  		Fig. 6B —Contrast-enhanced MR angiography maximum-intensity-projection coronal images in three patients with partial anomalous pulmonary venous connection. Ao = aorta, RA = right atrium. 63-year-old woman with anomalous connection of left upper pulmonary vein to vertical vein (arrow) that drains into brachiocephalic vein (BV) and from there into SVC (asterisk). RPA = right pulmonary artery.

 

Figure 22
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  		Fig. 6C —Contrast-enhanced MR angiography maximum-intensity-projection coronal images in three patients with partial anomalous pulmonary venous connection. Ao = aorta, RA = right atrium. 45-year-old woman with anomalous scimitar vein. Image shows that all right pulmonary veins are connected to an anomalous vein (arrow) that, in turn, drains into inferior vena cava (IVC).

 

Results
Top
 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 References
 
A total of 37 patients (25 female, 12 male; age range, 16-75 years; median age, 40 years) met the criteria for inclusion (Table 1). Twenty-five of the 37 had been referred for cardiac MRI by cardiologists from outside our institution. All 37 had undergone TTE at the referring center before cardiac MRI. The indications for TTE were dyspnea in 18, murmur in eight, palpitation or rhythm disorder in five, chest discomfort in one, and an abnormal chest radiograph in one. One patient was being followed up after previous repair of a tetralogy of Fallot, and in three patients the reason for the initial echocardiogram was not apparent from the notes. The findings before referral are summarized in Table 1.

Sinus Venosus Defect
A total of 19 patients (10 female, nine male) had a cardiac MRI diagnosis of sinus venosus defect. Qp/Qs ranged from 1.5 to 4. Of these 19 patients with sinus venosus defect, 18 (95%) had the associated finding of PAPVC. These 18 patients had a total of 30 anomalous pulmonary venous connections (1.6 per patient), all from the right side. No patient had a left-sided PAPVC or scimitar vein associated with the sinus venosus defect. Two patients with sinus venosus defect also had secundum ASD.

Of the 19 patients with sinus venosus defect, 11 have since undergone surgery at our institution (Table 3). The sinus venosus defect was confirmed in all 11 cases, as were all associated PAPVCs identified on cardiac MRI. At surgery, no other anomalous veins in addition to those documented on MRI were found. In the one case with a sinus venosus defect (patient 12) for which no PAPVC was identified on MRI, no anomalous vein was seen at surgery. The two secundum ASDs that had been diagnosed in addition to the sinus venosus defect were confirmed at surgery.


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  		TABLE 3 : Findings at Surgery in 14 Patients

 

All 19 patients with sinus venosus defect had undergone TTE before referral for cardiac MRI, and 11 had also undergone TEE. No patient had sinus venosus defect diagnosed on TTE before referral. In four cases, TEE had detected a sinus venosus defect or a possible sinus venosus defect and in those cases cardiac MRI had been requested to confirm a sinus venosus defect and to search for a possible PAPVC. In six additional patients, TTE or TEE had reported ASD or possible ASD, including one primum defect. In only one of these patients did cardiac MRI actually find an ASD in addition to the sinus venosus defect.

Partial Anomalous Pulmonary Venous Connection
A total of 60 anomalous pulmonary venous connections were shown in 36 patients. Anomalous connections were right-sided in 27 patients (75%), left-sided in seven (19.4%), and bilateral in two (5.6%). The patients with right-sided connections included three with scimitar veins [13, 14]. A single anomalous pulmonary vein connection was seen in 18 patients (50%), two anomalous connections in 12 (33.3%), and three in six patients (16.6%). Of the 60 abnormal connections, 31 (51.7%) were to the SVC, four (6.7%) were to the SVC-RA junction, 10 (16.6%) were to the RA, six (10%) were to the IVC, and nine (15%) were to a vertical vein on the left that communicated with the brachiocephalic vein. The most common associated defect was sinus venosus defect in 18 (50%) of these patients. Secundum ASD was present in six patients, including two who already had a sinus venosus defect. An intact septum was present in 14 patients (38.8%), including eight of the nine patients with a left connection and all three patients with a scimitar vein.

Twenty of these 36 patients with PAPVC underwent CE-MRA as part of their cardiac MRI examination. Although CE-MRA did provide clear delineation of the anomalous vessels and their distal aspects (Figs. 6A, 6B, and 6C), CE-MRA did not reveal any new anomalous vessels that had not already been identified on the unenhanced tomographic still and cine cardiac MRI.

Of the patients with PAPVC, 13 have undergone surgery at our institution, two with bilateral PAPVC and 11 with right PAPVC. At surgery, all the cardiac MRI findings were confirmed in 12 patients (Table 3); but in patient 6, although the secundum ASD was confirmed, the anomalous vein was not confirmed. No additional anomalous veins were identified at surgery in any of the patients.

All 36 patients with PAPVC had undergone TTE and 15 had undergone TEE before cardiac MRI. In patient 1, TTE correctly diagnosed an anomalous left pulmonary vein connection. In patient 4, TTE suggested a possible anomalous right vein that was confirmed at cardiac MRI. In patient 17, a possible anomalous left pulmonary vein was suggested by TTE and subsequently seen on cardiac MRI, but the anomalous right vein in the same patient was not detected on TTE. In the 15 patients undergoing TEE, TEE had not identified the 14 patients with anomalous right pulmonary veins or the one patient with an anomalous left pulmonary vein.


Discussion
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
References
 
Sinus Venosus Defects
Sinus venosus defects are uncommon and their clinical recognition has been difficult [3], with only 12% of sinus venosus defects confidently diagnosed on TTE [4]. Using a combination of TTE, TEE, and cardiac catheterization in a largely pediatric study of 16 patients, sinus venosus defect was confidently diagnosed in only one patient, was suspected in seven, and was not suspected in eight patients before the cardiac MRI examination that clearly imaged the sinus venosus defect in all the patients [15]. The most consistent TTE finding in patients with sinus venosus defect has been RV enlargement [4], and that was the case in our series, with RV dilatation in all 19 patients with sinus venosus defect. In the past, it has been this RV dilatation that has prompted the use of special TTE views [3] or referral for TEE [4]. Sinus venosus defect can have important long-term hemodynamic consequences, but early detection and surgical treatment are associated with low morbidity and mortality rates, with survival similar to that of a matched population [16].

TEE has been reported to be accurate for the detection of sinus venosus defect [4]. In our series, of the 11 patients with sinus venosus defect who had undergone TEE, it is not clear why TEE had detected the sinus venosus defect, or possible sinus venosus defect, in only four of the 11 patients. A previous smaller retrospective study noted that TEE missed a sinus venosus defect in two of the nine patients with sinus venosus defect documented on cardiac MRI [9]. CT has been used to detect sinus venosus defect [17], but it does not provide the same functional and flow information as cardiac MRI.

Partial Anomalous Pulmonary Venous Connection
A retrospective series of TEE in 43 adults established TEE as a reliable tool to detect PAPVC [5]. Of those 43 patients, 81% had right-sided anomalous veins. A retrospective CT study of 29 adults described a surprising predilection for left-sided anomalous veins, with 79% having left-sided veins [18], and speculated that CT may underreport right-sided veins because the anomalous right upper lobe vein is subtle on both contrast-enhanced and unenhanced CT [18]. MDCT has been proposed as a better technique to visualize anomalous pulmonary veins, with the advantage of shorter scanning times that would be of special importance for children and for patients with claustrophobia [19]. Both ECG-gated and nongated studies have been used [17-19]. MDCT does have the ongoing concerns about iodine-based contrast material and the risks associated with the radiation dose [20].

Cardiac MRI has been successful in the detection of PAPVC [7, 8]. In our series, the distribution of right-sided to left-sided lesions was similar to that reported in TEE studies [5]. Not only can cardiac MRI detect the extracardiac anomalous vessels without the need for radiation, it can also clearly delineate the intracardiac anatomy, especially the presence of sinus venosus defect [6, 15]. Furthermore, velocity flow mapping allows accurate assessment of the Qp/Qs without the need for, or the risk associated with, cardiac catheterization [21].

In patient 6, cardiac MRI diagnosed a large secundum defect, without a posterior rim, as well as an anomalous connection of the right upper PV. At surgery, the secundum ASD was confirmed, but the surgeon reported that no true anomalous connection of the right veins to the RA was seen. In 1957, Bedford et al. [22] commented on "large fossa ovalis defects in which the posterior rim is lacking or rudimentary, so that the right pulmonary veins communicate virtually with both atria," labeling such cases as having pseudoanomalous right pulmonary veins.

In studies of TEE for PAPVC diagnosis, undetected anomalous veins have been reported at surgery [4, 5]. In one study, in addition to the 37 veins identified with TEE, another 10 veins were found at surgery. These were right-sided and connected to the upper SVC [4]. No anomalous veins not detected by cardiac MRI were identified at surgery in our study or in the report by Valente et al. [15]. This is probably due to the large field of view of cardiac MRI that allows the complete imaging of the full extent of the SVC.

The use of CE-MRA in these patients with PAPVC has been advocated as a way to improve diagnosis and reduce the patient's time in the scanner [9-11]. However, recent concerns about complications associated with gadolinium contrast agents have led to recommendations that "other imaging techniques that do not require the administration of gadolinium should be taken into consideration" for at-risk patients [23]. In the 20 patients who underwent CE-MRA in addition to 2D scanning, we observed that the diagnosis of PAPVC could have been made from the 2D images alone. It must be kept in mind that these were retrospective nonblinded observations, but they do suggest that 2D cardiac MRI can be as accurate for the diagnosis and localization of PAPVC as CE-MRA.

Limitations
We have reported a highly selected group, and these results cannot be used to assess the frequency of PAPVC or sinus venosus defect. No conclusion can be drawn about the accuracy of TTE or TEE because we scanned only those patients in whom a conclusive diagnosis had not been made. We did not scan every patient with a dilated RV who underwent echocardiography. Although we can state that a cardiac MRI diagnosis of sinus venosus defect or PAPVC is quite likely to be correct, no comment can be made about the ability of CE-MRA to rule out sinus venosus defect or PAPVC because no patient with a normal scan was sent for surgical confirmation.

In conclusion, cardiac MRI can reliably detect, delineate, and quantify sinus venosus defect and PAPVC in patients for whom other methods have not provided a diagnosis for the cause of right heart dilatation and can do so without the need for gadolinium contrast agents. As a result, we recommend that cardiac MRI be performed when sinus venosus defect or PAPVC is suspected on echocardiography or clinically and in the case of patients with RV dilatation for whom no cause has been determined by other diagnostic means. The performance of cardiac MRI should be carefully considered before making a final diagnosis of idiopathic pulmonary hypertension or RV cardiomyopathy.


Acknowledgments
 
We thank Siew Yen Ho and Hideki Uemura for their expert advice on anatomic and surgical issues involved in sinus venosus defects.


References
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

  1. Al Zaghal AM, Li J, Anderson RH, Lincoln C, Shore D, Rigby ML. Anatomical criteria for the diagnosis of sinus venosus defects. Heart 1997;78:298 -303[Abstract/Free Full Text]
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