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170
Key Questions in CONGENITAL CARDIAC SURGERY
9. Ho S, McCarthy KP, Josen M, Rigby ML. Anatomic-echocardiographic correlates: an
introduction to normal and congenitally malformed hearts.
11.
10. Van Praagh R, Papagiannis J, Grunenfelder J, Bartram U, Martanovic P. Pathologic
anatomy of corrected transposition of the great arteries: medical and surgical
implications.
11. Anderson RH, Cook AC. Morphology of the functionally univentricular heart.
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12. Khairy P, Poirier N, Mercier LA. Univentricular heart.
12.
13. Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40-year
experience.
14. Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary
artery from the pulmonary artery: collective review of surgical therapy.
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15. Martinez RM, O’Leary PW, Anderson RH. Anatomy and echocardiography of the
normal and abnormal tricuspid valve.
16. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy
in neonates with Ebstein’s anomaly of the tricuspid valve: pathophysiologic and clinical
implications.
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Chapter 5
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Congenital cardiac imaging
Saeed Mirsadraee, Golnaz Houshmand
1 Describe the principles of computed tomography
A computed tomography (CT) scan combines a series of X-ray
images taken from different projections and the attenuation data are used to reconstruct cross-sectional images. Different tissues and organs are differentiated by their inherent
variation in attenuation; bone has high attenuation, air has the lowest attenuation and water has intermediate attenuation. Iodinated contrast agents are used to enhance visualisation of the
blood pool, such as vessels and body organs. CT plays an important role in the characterisation of congenital
cardiovascular anomalies and complications post-corrective surgery. CT exposes patients to potentially harmful ionising radiation,
especially the potential risk of cancer. The Ionising Radiation Medical Exposure Regulations (IRMER) has set out responsibilities for practitioners and operators to ensure that the benefits of the exposure to ionising radiation outweigh the risks, and that the radiation dose is kept ‘as low as reasonably practicable’ for their intended use. The main risks of exposure to iodinated contrast medium are:
171
a) allergic reaction, with anaphylactic reaction reported in 2 4 per
1000; b) renal impairment, which occurs in <2% of patients. In view of
this, screening of renal function should take place prior to CT
scanning, especially in patients with a history of kidney disease
or diabetes, and older patients. Adequate hydration is helpful
to reduce the risk from contrast-induced renal impairment.
172
Key Questions in CONGENITAL CARDIAC SURGERY
2 Describe the principles of magnetic resonance
imaging
Magnetic resonance imaging (MRI) uses a strong magnetic field and
radiofrequency waves to produce images of body tissues. The signal detected by MRI is generated from the magnetisation of
atoms with odd numbers of protons or neutrons within tissues (mainly protons). Cardiac magnetic resonance (CMR) plays an important role in the
detection of congenital anomalies and associated lesions. CMR allows non-invasive assessment of the ventricular function and shunt flow. CMR is widely used for the follow-up of left or right ventricular function, size and shunt studies in repaired and unrepaired congenital lesions and planning the timing of surgery. Although MRI is regarded as a safe investigation, there is a potential
risk of the magnetic field in patients with metal and non-conditional implantable devices. The risk of anaphylactic reaction to contrast (gadolinium) injections is
present but rare (1:10,000). Patients with renal failure are at increased risk of developing
nephrogenic systemic fibrosis (NSF) following contrast injection. This condition involves fibrosis of the skin, joints, eyes, and internal organs. The reported cases of NSF have significantly declined in recent years and patients with reduced renal function are often offered alternative contrast agents.
3 What are the principles of assessing coronary
anomalies with radiological imaging?
ECG-gated CT angiography is the non-invasive modality of choice
for the detection of the origin and assessing the course and termination of coronary artery anomalies (Figures 1 and 2), with the ability to define related structures and coronary artery fistulae (Figure
3). CT is also a reliable technique for detecting myocardial bridges
(Figure 4). In patients following repair of coronary anomalies, CT allows the
evaluation of the anastomoses and can detect the presence of any restenosis of the grafts. Cardiac magnetic resonance imaging enables assessment of
ventricular function and myocardial viability, and valvular function in patients with coronary anomalies.
5 Congenital cardiac imaging
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AB
CD
Figure 1. Gated computed tomography coronary angiography curved
multiplanar reconstruction demonstrating: A) an anomalous origin of the left main coronary artery (arrow) from the non-coronary sinus, with proximal luminal attenuation; B) anomalous origin of the left circumflex artery (arrow) from the right coronary sinus, adjacent to the origin of the right coronary artery, with a retro-aortic course and calcified atheroma in its mid segment; C) anomalous origin of a non-dominant right coronary artery (arrow head) from the left coronary sinus with an obstructive non­calcified plaque (arrow); and D) anomalous origin of a dominant right coronary artery (arrow head) from the left coronary sinus, with a non­obstructive calcified plaque (arrow).
The assessment of the coronary anomalies themselves by cardiac
MRI is challenging due to limited spatial resolution and data acquisition time. Techniques, such as navigated 3D whole heart magnetic resonance angiography, may allow adequate visualisation of coronary anomalies. Nonetheless, it can be difficult to comment on ostial narrowing due to less spatial resolution. Simple steady-state free precision (SSFP) or black blood imaging in
a routine MRI is usually able to show the origin and proximal course of the artery.
173
174
Key Questions in CONGENITAL CARDIAC SURGERY
AB
CD
Figure 2. Anomalous left coronary artery from the pulmonary artery
(ALCAPA): A) and B) computed tomography angiography volume-rendered reconstructions demonstrating a tortuous dilated right coronary artery (white arrow) and dilated collateral, which feeds the anomalous left anterior descending artery (LAD) retrogradely (black arrow); C) computed tomography short-axis double oblique multiplanar reconstruction demonstrating dilated coronary arteries and subendocardial calcification (arrow), indicating previous infarction in the LAD territory; D) cardiac magnetic resonance imaging scan demonstrating late gadolinium enhancement indicating subendocardial infarction in the anterior wall due to ischaemia related to steal phenomena.
MRI is also useful as a follow-up after repair of anomalous left
coronary artery from the pulmonary artery (ALCAPA) for evaluation of baffles used in the Takeuchi technique or direct reimplantation procedures in assessing the baffle confluency and checking the anastomotic site and reimplantation to the aortic root. Stress MRI with adenosine is helpful in the detection of ischaemia in
coronary anomalies and in a postoperative patient.
5 Congenital cardiac imaging
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AB
PA
Figure 3. Computed tomography angiography in a patient with a
coronary-pulmonary artery fistula, with the: A) 3D reconstruction demonstrating the conus branch (arrow) leading to a network of dilated tortuous vessels communicating with the pulmonary artery (PA); and B) axial image demonstrating a non-dilated pulmonary artery (25mm).
AB
175
Figure 4. Computed tomography coronary angiography demonstrating
bridging of the mid-left anterior descending coronary artery (arrow) on: A) 3D reconstruction; and B) coronal section. The bridged segment is 5mm at maximum depth and 3cm in length.
4 What are the principles of assessing an atrial septal
defect with radiological imaging?
Chest radiography in adults with a haemodynamically significant
atrial septal defect (ASD) usually reveals cardiac enlargement, right atrial dilatation, prominent central pulmonary arteries and pulmonary vascular markings.
176
Key Questions in CONGENITAL CARDIAC SURGERY
MRI is indicated in patients with poor echocardiographic acoustic
windows, for anatomical assessment of surrounding structures and associated anomalies, and where there is a need for non-invasive quantification of the shunt. MRI also allows accurate assessment of ventricular volumes and
function, with phase-contrast MRI allowing non-invasive calculation of systemic and pulmonary blood flow. CT scanning (Figure 5) plays a complementary diagnostic role in the
management of an ASD, including:
a) assessment of associated anomalies, such as anomalous
pulmonary venous return;
b) pre-operative planning, such as assessment of vascular
access; characterisation of the size, location and the shape of the defect that can be used for device selection; and assessment of coronary arteries in older patients;
c) assessment of the coronary arteries.
RV
Figure 5. ECG-gated computed tomography
(CT) angiography demonstrating a large secundum atrial septal defect (arrow), with shunting from the left to right atrium causing mixing of contrast, and a dilated hypertrophied right ventricle with flattening of the interventricular septum. Unless technically intended, shunts should be suspected when dense contrast is seen in all heart chambers on CT angiography.
5 Congenital cardiac imaging
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CT scanning may also incidentally diagnose an ASD or patent
foramen ovale (PFO). Equal opacification of the right and left heart chambers on CT may
hinder the diagnosis of a significant shunt.
5 What are the principles of assessing a ventricular
septal defect with radiological imaging?
Chest radiograph usually demonstrates dilatation of the central
pulmonary arteries, increased pulmonary vascularity and an increased cardiothoracic ratio. MRI is the modality of choice in the assessment of pulmonary and
systemic blood flow measurement, with the ratio providing an insight into the haemodynamic significance of the septal defects. MRI is also the gold standard for assessing LV function and size and
therefore can help in the decision making for VSD closure. In patients who develop arrhythmias following VSD repair, late
gadolinium MRI can localise the scar, which can be subsequently ablated. Although CT scanning (Figure 6) is not the primary imaging modality
for VSDs, it can provide a complementary assessment of the type, location, size and number of the VSD(s), as well as the chamber sizes, right or left ventricular outflow tract obstruction, and coexisting cardiac abnormalities, such as tetralogy of Fallot.
177
AB
RV
PA
Figure 6. ECG-gated computed tomography angiography
demonstrating: A) a membranous ventricular septal defect (VSD), with contrast entering from the left ventricle to a relatively normal sized right ventricle (RV); despite B) significant pulmonary artery dilatation.
178
Key Questions in CONGENITAL CARDIAC SURGERY
CT can be helpful in the assessment of coronary arteries and for
surgical planning (Figure 7), as it allows assessment of the proximity of the VSD to the surrounding valves and the atrioventricular conduction axis.
AB
Figure 7. ECG-gated computed tomography angiography demonstrating
a membranous ventricular septal defect (arrow): A) pre-operatively; and B) following implantation of a closure device.
A VSD is sometimes identified incidentally on CT.
6 What are the principles of assessing an atrioventricular
septal defect (AVSD) with radiological imaging?
Chest radiography usually shows cardiomegaly, as well as large
proximal and small peripheral pulmonary arteries (peripheral pruning). Pulmonary venous markings are more prominent in patients with significant common AV valve regurgitation. CT may provide further anatomical information (Figure 8), especially
when echocardiography is inconclusive. It is also helpful in assessing outflow tract obstruction and defining coexisting anomalies, such as coarctation. In unbalanced AVSD and heterotaxy syndrome, CT scanning can be
useful in defining associated anomalies. Similar to CT, MRI may also allow further anatomical evaluation and
can be used to follow up operated patients, as it is helpful in the quantification of mitral regurgitation, LV size and function, and haemodynamic evaluation of any residual shunt.
5 Congenital cardiac imaging
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Figure 8. ECG-gated computed tomography
axial image demonstrating a complete atrioventricular septal defect, associated with significant right ventricular enlargement and Eisenmenger syndrome.
179
7 What are the principles of assessing tetralogy of Fallot
with radiological imaging?
CT is a fast and convenient method in both the paediatric and adult
populations for assessing the morphology of the right ventricular outflow tract, pulmonary arteries and aorta (Figure 9). Other coexisting anomalies, such as an ASD, patent ductus
arteriosus and aortopulmonary collaterals, can also be detected (Figure 10). CT is particularly useful in planning for re-do surgery, to evaluate the
patency of coronary arteries, and to note the relation of the heart and large vessels to the sternum (Figure 11). MRI is used to delineate anatomy, function and haemodynamic
status. It can be used in neonates with tetralogy of Fallot where there is a facility for adequate sedation, but it is particularly helpful in the follow-up of older children and adults with total correction of tetralogy of Fallot. CMR allows assessment of ventricular size and function, and quantification of pulmonary regurgitation. CMR and flow velocity mapping can also detect and quantify any residual VSD, if present. Phase contrast and cine MRI are the preferred modalities to quantify