Electronic ISSN 2287-0237

VOLUME

THE SHORT CUT STRATEGIES IN DIAGNOSTIC APPROACH FOR RARE CONGENITAL HEART DISEASES USING MRI , DEMONSTRATED BY TEN SELECTED CASES OF OUR EXPERIENCE AT BANGKOK HEART HOSPITAL.

SEPTEMBER 2016 - VOL.12 | CASE REPORT

Almost congenital heart diseases (CHD) are difficult to diagnose especially in rare diseases. Occasionally, the diagnosis of a rare congenital heart disease must occur on first sight. Although there are many superb diagnostic tools such as 3D echocardiography, Multi-Detector Computed Tomography (MDCT) and Magnetic Resonance Imaging (MRI) which can provide more information on CHD, the lack of experience of the cardiac imaging operator observing some rare congenital heart diseases may cause an incomplete or a missing diagnosis. This article will share the experiences of conceptual strategies for protocol preparation to obtain the key images to diagnose rare congenital heart disease that can be practiced for routine work or even in the first recognition using MRI through the demonstration of ten selected cases of our experience at Bangkok Heart Hospital.

 

Keywords:

coronary sinus ASD, sinus venosus ASD, coronary arterio-venous fistula, Ebstein’s anomaly, pulmonary atresia, scimitar PAPVR, left atrial appendage aneurysm, truncus, gerbode defect, persistent left SVC connect with the coronary sinus, MRI

MEDIA
Figure 1.1:
: Coronary Sinus ASD case: The scout images of the gradient echo CINE MRI on axial view demonstrate the contour of all cardiac chambers and also display the communicating defect between the left atrium and coronary sinus (black arrow sign ) , CS= coronary sinus , SVC= Superior Vena Cava , IVC= Inferior Vena Cava.
Figure 1.2:
Coronary sinus ASD case: The gradient echo CINE MRI on short axis view of the atria on the slice cut parallel through the coronary sinus course demonstrates the communicating defect of the coronary sinus and the left atrium
Figure 1.3:
Coronary sinus ASD case: The gradient echo CINE MRI on axial view scout image demonstrates the persistent left SVC (PLSVC) at the left lateral side of the aortic arch.
Figure 1.4:
Demonstrating diagram to show pathologic defect of si- nus venosus ASD and coronary sinus ASD.
Figure 2.1:
Sinus venosus ASD case: The gradient echo CINE MRI images demonstrate the cardiac chamber contour and also display the communicating defect between the left atrium and the SVC through the defect inter-atrium septum (red arrow sign). RA= right atrium , LA= left atrium , RV= right ventricle, LV = left ventricle.
Figure 2.2:
Sinus venosus ASD case: The gradient echo CINE MRI on the axial plane demonstrates the drainage of the blood flow from the right upper lung pulmonary veins into the right SVC (PAPVR).
Figure 2.3:
Sinus venosus ASD case: The MRA (angiography) with gadolinium contrast injection of great vessels reveals the course of drainage of the right upper lung pulmonary veins into the emptying portion of the right SVC.
Figure 3.1:
Atrial appendage aneurysm case: The gradient echo MRI on axial view shows the huge LAA with some blood clots (Hypo-signal mass, white arrow sign). LAA = left atrial appendage, RAA= right atrial appendage.
Figure 3.2:
Atrial appendage aneurysm case: The MR with delayed contrast enhancement study confirms the LAA aneurysm with clot (dashed circle).
Figure 3.3:
A; Demonstrating diagram on short axis view of both atria to show the pathologic left atrial appendage aneurysm. B; LAA aneurysm is shown on coronal view of the heart. AV=aortic value, PV=pulmonic valve, LA=left atrium, RA=right atrium, LAA=left atrial appendage, IVC=inferior ena cava.
Figure 4.1:
Coronary venous fistula case: The gradient echo CINE MRI images on coronal plane demonstrate the running course of the anomalous LCX coronary that originates from the left aortic cusp and running above the roof of the left atrium (LA) and emptying into the RA (black arrow). RA= right atrium, LA= left atrium, LV= left ventricle.
Figure 4.2:
Coronary venous fistula case: The gradient echo CINE MRI images on coronal plane demonstrate the running course of the anomalous LCX coronary (red arrow sign) that originates from the left aortic cusp and running above the roof of the left atrium (LA) and emptying into the RA (black arrow), RA= right atrium, LA= left atrium , LV= left ventricle.
Figure 4.3:
The demonstrating diagram of left circumflex coronary fistula (arteriovenous fistula) with emptying into the RA. AO=aorta, MPA=main pulmonary artery, RA=right atrium, LV=left ventricle, SVC=superior vena cava, IVC=inferior vena cava, LAD=left anterior descending cornary artery, LCX=left circumflex coronary artery.
Figure 5.1:
Ebstein’s anomaly case: The gradient echo CINE MRI on four-chamber view demonstrates the downward displacement of the septal and posterior leaflets (black arrow) in relation to the anterior mitral valve leaflet insertion (white arrow ) is >8 mm/m2 body surface area of Ebstein’s anomaly.
Figure 5.2:
The demonstrating diagram of the ebstein’s anomaly that shows the low lying or the downward displacement of the tricuspid valve leaflets. The ebstein’s anomaly will be diagnosed if the downward displacement of the septal and posterior leaflets in relation to the anterior mitral valve leaflet id > 8mm/m2 body surface area. SVC= superior vena cava, IVC= inferior vena cava , LA= left atrium, RA= right atrium , LV= left ventricle , RV= right ventricle , TV= tricuspid valve.
Figure 6.1:
Gerbode defect case: The gradient echo CINE MRI on the axial plane images demonstrate a high possible left to right membranous VSD jet flow in a suspicious case of Gerbode defect. Ao=Aorta, LA=left atrium, LV=left ventricle. It has to be confirmed that the left to right VSD flow is going to the RA directly or through the defect of the septal leaflet of the tricuspid valve.
Figure 6.2:
Gerbode defect case: The gradient echo CINE MRI on a four-chamber plane image shows the left to right shunt flow from the left ventricle to the right atrium.
Figure 6.3:
Gerbode defect case: The gradient echo CINE MRI on coronal view confirms the left to right flow from the membranous VSD to the RA (*). RA = right atrium, LA= left atrium , LV= left ventricle, TV=tricuspid valve.
Figure 6.4:
The demonstrating diagram of the Gerbode defect or congenital left ventricular to right atrial (LV-RA) shunt. The left to right shunt flow from the left ventricle (LV) to right atrium (RA) is running through the small area of the membranous septum. The potential communicating defect is above the septal commissure of the tricuspid valve and below the commissure of the mitral valve. IAS=inter-atrial septum, MS=membranous septum, IVS=inter ventricular septum.
Figure 7.1:
Persistent left SVC (PLSVC) emptying into the coronary sinus case: The serial images (1-6) of the gradient echo CINE MRI on axial plane reveal the running course from the origin (left lateral to the aortic arch) to the termination site which is coronary sinus. Arch=aortic arch, SVC= superior vena cava, CS= coronary sinus, LA= left atrium.
Figure 7.2:
Persistent left SVC emptying into the coronary sinus case: The serial images of the MR angiography with gadolinium contrast injection confirm the existence of the left SVC and its termination site is coronary sinus.CS= coronary sinus , LA= left atrium, RA= right atrium , LSVC =left superior vena cava.
Figure 7.3:
Demonstrating diagram of the persistent left superior vena cava (PLSVC) that is emptying into the coronary sinus. The coronary sinus is located at the posterior surface of the heart amd is empting directly into the RA. MPA=main pulmonary artery vessel, CS=coronary sinus.
Figure 8.1:
Pulmonary artery atresia case: The gradient echo CINE MRI images on the axial plane are used as scout images. They show prominent aorta and membranous VSD with absence of the main pulmonary artery and the very small right pulmonary artery (RPA) and left pulmonary artery (LPA). Pulmonary atresia with membranous VSD is suggested. * = aorta
Figure 8.2:
Pulmonary artery atresia case: The MRA with gadolinium contrast injection reveals the small right and left pulmonary artery (RPA/LPA) with the absence of the main pulmonary artery (MPA). The sizeable LV apical clot (white dashed circle) is observed.
Figure 8.3:
Pulmonary artery atresia case: The MRA with adolinium contrast injection reveals the enlarged right pulmonary veins and the collaterals from the aorta (white arrow).
Figure 8.4:
Demonstrating diagram of the pulmonary artery atresia shows a complete disruption between the right ventricular outflow tract (RVOT) and the main pulmonary artery (pulmonic valve is not formed) and the ventricular septal defect (VSD). RPA and LPA=right and left pulmonary artery respectively.
Figure 9.1:
Scimitar PAPVR case: The multi-slice images with whole heart and great vessels coverage of the gradient echo CINE MRI on axial plane are used as scout images. The images reveal scimitar syndrome (dextro-cardia) hypoplasia of the right pulmonary artery and the right lung and anomalous arterial supply of the right lower lobe from the abdominal aorta (the right sided PVs emptying into the IVC at the right atrial–IVC junction above the diaphragm). The vessel branch joining with the IVC at the region of emptying port into the RA is a suspicious clue for Scimitar PAPVR.
Figure 9.2:
Scimitar PAPVR case: The images of the gradient echo CINE MRI on the axial plane show a slightly smaller RPA.
Figure 9.3:
Scimitar PAPVR case: The MRA with gadolinium contrast injection reveals Scimitar PAPVR of the mid right pulmonary veins (arrow sign) drainage into the IVC. AV= aortic valve, IVC= inferior vena cava, RA= right atrium, RV =right ventricle, RPV= right pulmonary vein, LPV = left pulmonary vein.
Figure 9.4:
Demonstrating diagram of the Scimitar PAPVR that is caused by the blood drainage from the right pulmonary veins into the IVC which produces a Turkish sword (Scimitar)-liked curve shadow on chest x-ray along the right cardiac border (black arrow sign).
Figure 10.1:
Truncus case: The gradient echo CINE MRI on the axial plane images are used as scout images and they reveal a huge common arterial trunk (aorta) and its pulmonary artery branches. The overriding of the common arterial trunk (LVOT and RVOT pump blood flow to the common trunk) including the persistent left SVC (LSVC) which is emptying into the coronary sinus. AO= aorta, RPA= right pulmonary artery , LV = left ventricle , RV= right ventricle.
Figure 10.2:
: Truncus case: The gradient echo CINE MRI image on sagittal view reveals the common arterial (aorta) trunk structure with its pulmonary artery branch and the PDA (patent ductus arteriosus) that forms the connection between the proximal thoracic descending aorta at the ductal region and the left pulmonary artery branch.
Figure 10.3:
Truncus case: MRA with gadolinium contrast injection image demonstrates the common arterial (aorta) trunk structure with its pulmonary artery branch and the PDA that forms the connection between the proximal thoracic descending aorta at the ductal region and the left pulmonary artery branch. PA= pulmonary artery
Figure 10.4:
Demonstrating diagram of the Truncus arteriosus that is composed of a single common arterial trunk as a stem (arising from the normal ventricle) and forms the branches by the arising of the pulmonary arteries. The associated disease such as PDA (patent ductus arteriosus) and sub-valvular VSD and ASD (atrial septal defect) are also shown.
SEARCH VOLUME & NUMBER
GO
RECENT VOLUME & NUMBER