Korean J Radiol.  2001 Sep;2(3):121-131. 10.3348/kjr.2001.2.3.121.

MR Imaging of Congenital Heart Diseases in Adolescents and Adults

• Choe YH
• Kang IS
• Park SW
• Lee HJ

• KMID: 754096
• DOI: http://doi.org/10.3348/kjr.2001.2.3.121

Abstract

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

Keyword

Heart, MR; Heart, diseases; Heart, congenital anomalies

MeSH Terms

Adolescent
Adult
Heart Defects, Congenital/*diagnosis
Human
*Magnetic Resonance Imaging
*Magnetic Resonance Imaging, Cine
Support, Non-U.S. Gov't

Figure

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  Fig. 1 Diastolic phase short-axial image obtained using the FIESTA technique shows excellent blood-tissue contrast.

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  Fig. 2 Calculation of pulmonary-to-systemic arterial flow ratio (Qp/Qs) using velocity-encoded phase-contrast cine MRI in a 24-year-old female with a secundum atrial septal defect. A. An imaging plane perpendicular to the long axes of the aorta and main pulmonary artery is prescribed. B, C. ROIs were drawn on the pulmonary artery and aorta on the cine images (magnitude images on the left plots of B, C). Curves on the right plots show flow volume versus ECG-trigger delay time. Flow volume of the pulmonary artery and aorta was 8,498 ml/min and 5,833 ml/min, respectively, with a Qp/Qs of 1.5. In this patient, the Qp/Qs determined by radioisotope study was 1.7.

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  Fig. 3 41-year-old female with partial anomalous pulmonary venous connection of right pulmonary veins to the right atrium and two atrial septal defects. A, B. Coronal source images obtained by contrast-enhanced MR angiography clearly show right upper and middle pulmonary veins (solid arrows) connected with the right atrium. Two atrial septal defects (open arrows) are also visible. C. Axial double inversion-recovery images show the right lower pulmonary vein (arrow) connected with the right atrium.

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  Fig. 4 Small, tricky, atrial septal defect in a 44-year-old male. Transthoracic and transesophageal echocardiography failed to depict the atrial septal defect in spite of clinical suspicion of a left-to-right shunt. A. Axial spin-echo MR image shows intact interatrial septum at the level of the aortic valve. B. Spin-echo image cephalad to A shows a small defect (arrow) in the periphery of the interatrial septum near the junction with the superior vena cava.

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  Fig. 5 Tetralogy of Fallot in a 37-year-old female. MRI rather than catheter angiography was performed prior to surgery. A. Axial spin-echo MR image shows severe infundibular stenosis (arrow). B. Three-dimensional contrast-enhanced MR angiography shows small right pulmonary artery (solid arrow) and a ductus diverticulum (open arrow). Note, too, the right-sided aortic arch with mirror image branching.

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  Fig. 6 Pulmonary atresia with ventricular septal defect in a 14-year-old female. MRI was performed to evaluate the size and number of aortopulmonary collateral arteries and to verify the presence of central pulmonary arteries (A, spin-echo image; B and C, three-dimensional images obtained by contrast-enhanced MR angiography with partial volume reconstruction). A major collateral aortopulmonary artery (large solid arrow in A, B) arises from the descending thoracic aorta. Note, too, the presence of another collateral artery (small solid arrow in B) just distal to the aortic arch and small collateral arteries (open arrows in A, B and solid arrow in C). Small central pulmonary arteries (small arrow in A, open arrow in C) with stenosis in the confluent segment are demonstrated by spin-echo imaging and three-dimensional MR angiography.

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  Fig. 7 Tubular hypoplasia of the aortic arch and coarctation in a 17-year-old female. Maximal intensity projection image obtained by contrast-enhanced MR angiography clearly shows arch hypoplasia and coarctation (arrow). This patient also suffered intracerebral hemorrhage, probably associated with coarctation.

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  Fig. 8 Subaortic stenosis associated with coarctation of the aorta in a 14-year-old male. Short-axis spin-echo MR image demonstrates subaortic stenosis (SA) due to hypertrophied anterolateral muscle (M).

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  Fig. 9 Aneurysm of the ductus arteriosus in a 31-year-old male. A. Axial spin-echo MR image shows a large aneurysm (arrow) with slow flow within. Spin-echo images do not clearly indicate the origin of this aneurysm. B. Three-dimensional contrast-enhanced MR angiography depicts a bell-shaped aneurysm (A) connected with the aorta. Poor opacification of the hypoplastic left pulmonary artery (arrow) suggests closure of the pulmonary arterial end of the ductus arteriosus.

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  Fig. 10 Rupture of an aneurysm of the sinus of Valsalva in a 42-year-old male. A. Cine MR image in the oblique coronal plane. Turbulent flow (open arrow) is demonstrated as signal voids in the right atrium emanating from the tip of the 'windsock' (solid arrow). B. Conventional cine angiography performed after MRI also shows the ruptured aneurysm of the coronary sinus. Due to low image contrast between the cardiac chambers and aneurysm, the 'windsock' is barely perceptible, however. Surgical findings confirmed the presence of a 4-cm long aneurysm of the sinus of Valsalva and a 0.5-cm-sized tear at the tip of the aneurysm.

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  Fig. 11 Ebstein's anomaly of the tricuspid valve in a 17-year-old female. A. Oblique axial spin-echo image shows displaced attachment (thick arrow) of the posterior leaflet (thin arrows). B. Conventional cine MR image in the oblique coronal plane shows a large signal void area due to severe tricuspid regurgitation.

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  Fig. 12 Unroofed coronary sinus in a 24-year-old male. A. Axial spin-echo MR image shows an enlarged coronary sinus (arrow). B. Oblique sagittal planes were selected for imaging of the coronary sinus and left atrium. C. Coronary sinus view, as prescribed in B, shows a large defect (arrow) in the wall between the coronary sinus (CS) and left atrium (LA).

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  Fig. 13 Corrected transposition in a 14-year-old male. A. Axial spin-echo image shows the left-sided aorta (A) alongside the hypoplastic pulmonary artery (P). B, C. Coronal spin-echo images show a large ventricular septal defect (V) and aorta (A) arising from the left-sided and superiorly located hypoplastic right ventricle (RV). LV, left ventricle

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  Fig. 14 Biventricular function evaluation using fast cine MR imaging and semi-automated ventricular function analysis software. This 22-year-old male has undergone surgical repair for tetralogy of Fallot and now presents with decreased biventricular function and pulmonary regurgitation. The figure shows three-dimensional images of the right (yellow) and left (red) ventricles in diastolic (left image) and systolic (right image) phases. The three-dimensional volume ejection fraction calculated by this technique was 20.1% and 20.0% for right and left ventricles, respectively.

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  Fig. 15 Estimation of the severity of pulmonary regurgitation after repair of tetralogy of Fallot using velocity-encoded cine MRI. The curve on the right plots pulmonary arterial flow against ECG-trigger delay time. The area between the baseline and the curve below the baseline represents regurgitant volume.

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