British Journal of Radiology 74 (2001),280-282 © 2001 The British Institute of Radiology
MRI diagnosis of a previously undiagnosed large trabecular ventricular septal defect in an adult after multiple catheterizations and angiocardiograms
N I Stauder, MD1,
S Miller, MD1,
A M Scheule, MD2,
G Ziemer, MD2 and
C D Claussen, MD1
1 Departments of Diagnostic Radiology
2 Thoracic, Heart and Vascular Surgery, University of Tuebingen, Hoppe-Seyler-Str. 3, 72076 Tuebingen, Germany
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Abstract
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Ventricular septal defect (VSD) is the most frequent congenital heart disease (25–30%). The diagnosis of VSD is usually made echocardiographically by means of colour Doppler ultrasound, and is confirmed by angiocardiography in most cases. We describe a case in which an additional large trabecular VSD was demonstrated by MRI after previous cardiac catheterizations and angiocardiography in various hospitals. MRI allows an exact presentation of the anatomy, including areas that are difficult to assess, for instance the apical septum. Determination of cardiac output, regurgitation volume and the Qp/Qs quotient in patients with shunt volume in heart defects is also possible with one examination.
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Introduction
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Ventricular septal defect (VSD) is the most frequent congenital heart disease (25–30%). Depending on the size and haemodynamic effect, patients undergo either conservative treatment or surgical intervention. MRI has been used increasingly during recent years for diagnosis and follow-up [1]. We describe a case in which an additional large trabecular VSD was discovered by means of MRI after previous cardiac catheterizations and angiocardiography.
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Case report
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A cardiac murmur and cyanosis were detected from birth in a female patient who is now 25 years old. At that time, cardiac catheterization showed a perimembraneous VSD, an open foramen ovale, hypertrophy of the outflow channel relating to the ventricle and hypoplasia of the ascending aorta. A left-to-right shunt of 54% and a right-to-left shunt of 13% were detected at ventricular level based on the Fick principle. A left-to-right shunt was found at atrial level. Owing to pulmonary hypertension with mixed shunting, surgical intervention was not feasible at the time of diagnosis. Catheterization also showed mitral stenosis with a parachute mitral valve, secondary tricuspid regurgitation caused by dilatation of the right ventricle, and an atrial septal defect of ostium secundum type. At the age of 23 years, the patient had increasing syncopal attacks and progressively decreasing physical ability. She underwent further catheterization at the age of 24 years. The quotient between flow in the pulmonary circulation and flow in the systemic circulation (Qp/Qs) at that time was 3 without inhalation of oxygen. Surgical correction was planned because of these findings. Pre-operative echocardiography showed no additional findings to the previous ones. Pre-operative MRI with a 1.5 T Magnetom Vision (Siemens, Erlangen, Germany) was performed. A phased array body coil was used. After axial T1 weighted turbo spin echo sequences, which were triggered by the ECG (TR=RR; TE=12 ms; slice thickness=5 mm; field of view (FOV)=320 mm) in the area above the mediastinum, flow sensitive FLASH 2D sequences adjusted to the findings were used (TReff 
11 ms; TE=4.8 ms; flip angle=25
; FOV=320 mm; measurement during respiratory breath-hold; 1 acquisition). To determine shunt volume, flow measurements were carried out during normal breathing with phase contrast FLASH 2D technique (TR=25 ms; TE=5 ms; pixel=0.98 mmx0.98 mm; velocity encoding value=250 cm s-1 in the ascending aorta and 75 cm s-1 in the pulmonary trunk) in the ascending aorta, proximal to the brachiocephalic trunk and orthogonal to the pulmonary trunk.
The atrial septal defect (ASD) of ostium secundum type was confirmed on short and longitudinal axis images (Figure 1a
). A VSD with a diameter of 1.5 cm was detected in the area of the membraneous septum, 1.5 cm proximal to the pulmonary valve (Figure 1b). Cine images showed mitral stenosis with a typical obstruction of the aperture of the anterior mitral leaflet, concordant with the finding of a parachute mitral valve. There was also a defect in the area of the apical muscular septum, with flow phenomena that were dependent on the heart cycle. This led to the asumption of an additional trabecular VSD with a diameter of 1.5 cm (Figure 1c). There was a mean flow of 183 ml s-1 in the pulmonary circulation. In comparison, there was a systemic mean flow of 68 ml s-1 in the ascending aorta and a Qp/Qs of 2.7.
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Figure 1. (a) 9-fold k-space segmented FLASH 2D cine MRI of the heart in four chamber view: an atrial septal defect of secundum type is demonstrated in the area of the atrial septal wall (arrow). (b) FLASH 2D cine MRI of the heart, short axis view: perimembraneous ventricular septal defect (VSD) (arrow). (c) FLASH 2D cine MRI of the heart in four chamber view: a trabecular VSD can be seen in the apical part of the ventricular septum (arrow).
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Surgery consisted of direct ASD closure as well as patent ductus arteriosus closure. The perimembraneous VSD was closed with a Dacron patch. Mitral valve repair followed. The apical trabecular VSD of muscular type was confirmed transatrially and was closed with a Dacron patch (Figure 2). The post-operative course was uneventful. On the 14th day after surgery, the patient underwent follow-up MRI. The pre-operative diameter of the right ventricle was 6.4x5.1 cm2 and the post-operative diameter was 5.8x4.9 cm2. The right atrium had a pre- and post-operative diameter of 6x4.8 cm2. The diameter of the dilatated left ventricle pre-operatively was 8.4x4 cm2 and post-operatively it was 7.4x3.5 cm2. There was no relevant change of the functional parameters of the heart between the two measurements. The left ventricular ejection fraction pre-operatively was 62% and post-operatively it was 60%. As a sign of a residual defect in the perimembraneous septum, there was still a minor systolic jet in the left ventricular outflow tract. The Qp/Qs quotient was 1.
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Figure 2. Post-operative cine MRI of the heart in four chamber view: closure of the apical ventricular septal defect with Dacron patch (arrow).
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Discussion
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VSD is the most common congenital heart deformity, affecting 2–4% of newborn infants [2]. It can occur as a single malformation or as part of a multiple malformation. 70–80% of cases are minor whilst 20–30% of cases are more complex defects [3]. The VSD is located in the perimembraneous septum in approximately 80% of cases. In rare cases (10–20%), the VSD is located in the apical area of the trabecular septum [2, 3]. Muscular defects of the septum are often minor and either close spontaneously or remain haemodynamically insignificant [2]. When such defects are haemodynamically relevant, there is increased pulmonary blood flow with right ventricular hypertrophy and secondary tricuspid regurgitation as well as dilatation of the left atrium and ventricle. Eisenmenger's reaction with reversed shunt develops in 8–10% of untreated cases [3]. Decisive criteria for considering surgery beyond childhood is the relation between flow in the pulmonary circulation and that in the systemic circulation (Qp/Qs) and oxygen reactivity of the pulmonary vessels. In patients with Qp/Qs>1.5, as in the case described above, early closure of the intracardial shunt is recommendend. A Qp/Qs<1.5 is usually not an indicator for surgery [4].
Diagnosis is usually made echocardiographically by colour Doppler ultrasound [5]. In most cases the diagnosis is confirmed by angiocardiography [6]. MRI has recently become important in the diagnosis of congential heart disease. It allows exact delineation of the anatomy, including areas that are difficult to assess, for instance the apical septum. In respect to this patient, MRI gave additional relevant information. Several echocardiographic and catheterization procedures had failed to diagnose an apical VSD in this patient over 20 years. In addition to exact presentation of the anatomy, determination of cardiac output and regurgitation volume in patients with cardiac volume or shunt volume in heart defects is also possible within the framework of an examination [7]. A comparison between invasive methods of measurement and flow measurements with the MR phase contrast technique during normal breathing have shown that MRI can reliably determine the extent of the shunt volume and the Qp/Qs quotient [8, 9].
Received for publication July 17, 2000.
Revision received November 13, 2000.
Accepted for publication November 27, 2000.
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Abstract
Introduction
