In patients scheduled for reoperation, CMR [or computed tomography CT ] provides information to assess the relationship between vascular structures, the heart, and the sternum. Limitations include availability, higher cost, artefacts from stainless steel implants, and relative contraindication in patients with pacemakers or implantable cardioverter-defibrillators ICDs.
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Magnetic resonance-compatible devices are increasingly used and are desirable for ACHD patients; selected patients with in situ conventional devices may safely undergo CMR with appropriate local protocol. Siting the device on the opposite side of the chest from the position of the heart may be preferable in ACHD patients. Computed tomography may also have added value in younger patients, for instance to assess aberrant coronary anatomy. Pulmonary parenchyma imaging is also provided which is highly relevant for patients with pulmonary hypertension. Furthermore, CT complements assessment of mechanical heart valve dysfunction and allows 3D visualization of abscess formation in endocarditis.
Computed tomography is an alternative to selective coronary angiography in older patients referred for ACHD surgery. However, CT exposes the patient to ionizing radiation and iodinated contrast agents and does not provide information on haemodynamics, flow rate, or velocity. Computed tomography can be used to acquire ventricular volumes and function but with lower temporal resolution than CMR or echocardiography and at the expense of additional radiation exposure. It tends to overestimate ventricular volumes and is clearly unattractive for serial measurements because of radiation.
Anomalous coronary artery from the pulmonary artery Ai and Aii studied with computed tomography preoperatively. Post-operative computed tomography Bi and Bii shows patent left internal mammary graft to mid left anterior descending artery images courtesy of Dr Mike Rubens. Nuclear scintigraphy has been reserved for selected patients only, for example for myocardial stress perfusion imaging or differential pulmonary blood flow quantification when CMR is not available.
Diagnostic cardiac catheterization and angiography are less frequently performed in ACHD nowadays; they are reserved for specific clinical indications such as calculation of pulmonary vascular resistance or where the diagnosis remains uncertain after non-invasive imaging or for percutaneous interventions. Cardiac imaging goals in selected adult congenital heart diseases with strengths and weaknesses of different imaging modalities. Echocardiography is used to assess LV outflow tract LVOT obstruction for example due to subaortic ridge, aortic valvar, and supravalvar stenosis plus aortic coarctation or re-coarctation; Doppler-derived diastolic tail in the descending thoracic aorta and continuous abdominal aortic flow indicate significant coarctation.
Aortopathy involving the aortic root or proximal ascending aorta should be sought on 2D echocardiography. Echo Doppler may reveal non-specific high-flow velocities across the isthmus in patients after coarctation repair given the fact that elasticity of the aortic wall is lost in this trajectory, causing flow velocities to increase even in the absence of stenosis. Cardiovascular magnetic resonance is the gold standard for assessing LV mass and can be useful to assess multi-level LVOT obstruction, aortic valve morphology, calibre of the entire aorta, and collateral flow.
All adults with aortic coarctation should undergo cross-sectional imaging usually CMR at least once.
Contrast-enhanced cardiovascular magnetic resonance angiography CE-CMRA in an adult presenting with systemic hypertension due to severe aortic coarctation A. Computed tomography imaging following endovascular stenting B. Computed tomography and CMR are especially useful for detecting associated anomalous pulmonary veins that insert into the superior vena cava above the level of the azygous vein.
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Cardiovascular magnetic resonance and CT give information on the distance of the anomalous pulmonary vein from the cardiac mass, which in turn is important for planning the surgical approach to pulmonary venous redirection. Cardiovascular magnetic resonance or computed tomography may be useful in the diagnosis of sinus venosus defects, which can be at the orifice of the superior or less commonly inferior caval veins and to delineate anomalous pulmonary venous drainage.
Cardiovascular magnetic resonance images showing sinus venosus atrial septal defect asterisk in A and the anomalous drainage of the right upper pulmonary vein to superior vena cava B. Patients with atrioventricular septal canal defects have a common atrioventricular junction with a spectrum of lesions with potential for shunting at atrial level ostium primum defects , ventricular level, or both atrial and ventricular level.
The most common adult complication for repaired patients is left atrioventricular valvar regurgitation of note this valve has abnormal tri-leaflet morphology. Imaging must also assess for other potential complications including pulmonary hypertension and LVOT obstruction. Transthoracic echocardiography is usually able to address the majority of concerns and TOE used to assess for suitability for repair vs. Residual haemodynamic and electrophysiological abnormalities contribute to increasing morbidity and mortality rates arising in adulthood.
These may be difficult to assess with TTE alone due to their anterior location. A multimodality imaging approach is often utilized. Aortic root dilation is also common in patients with repaired TOF in some patients associated with significant aortic regurgitation. Pulmonary regurgitation can be assessed with echocardiography 18 although the gold standard for its quantification is CMR. Quantification of right atrial size is helpful as large right atrial area has been associated with sustained atrial tachyarrhythmias in these patients.
Diastolic still image from cardiovascular magnetic resonance cine pre-pulmonary A and post-pulmonary B valve replacement for pulmonary regurgitation status post repaired tetralogy of Fallot. D Derived from 3D cardiovascular magnetic resonance acquisition after segmentation of chambers, outflows, and scar using Mimics, Materialise NV courtesy of collaboration with Drs Veronica Spadotto and Jennifer Keegan.
LV, left ventricle; RV, right ventricle. Focal RV fibrosis on late gadolinium enhancement CMR imaging is associated with adverse clinical prognosticators in adults with repaired TOF; prospective studies are required. Furthermore, cardiac CT provides information on the extent of conduit calcification for stent deployment. Cardiac computed tomography in a patient with RV—PA conduit A , showing virtually single origin coronary arteries passing between the aorta and narrow segment of conduit B — D images courtesy of Dr Mike Rubens.
Many surviving adults with transposition of the great arteries TGA would have had atrial switch surgery Mustard or Senning operation. Systemic RV dysfunction is a determining factor for late morbidity and mortality. Total isovolumic time and peak systolic strain measures have prognostic value. Systemic atrioventricular tricuspid valve regurgitation usually reflects progressive RV dilatation and dysfunction and is most sensitively assessed by echocardiography, as is the presence of pulmonary hypertension or baffle leaks better delineated with the use of contrast.
Associated lesions such as pulmonary stenosis and ventricular septal defect can also be assessed. Pulmonary hypertension can be difficult to diagnose when there is no mitral regurgitation but equal LV and RV size on apical four-chamber view is suggestive of pulmonary hypertension or significant baffle leak. Cardiovascular magnetic resonance in addition to echocardiography is often helpful for assessment of baffle patency. The spectrum of clinical presentation is wide and depends on associated lesions; presentation in adulthood is possible.
The tricuspid valve in ccTGA is often intrinsically abnormal Ebstein type and may be underdiagnosed and results in tricuspid regurgitation; the latter may also be secondary to RV dysfunction and annular dilatation. Echocardiography can be used to assess RV dysfunction and tricuspid regurgitation. Diastolic dysfunction can be assessed by Doppler flow of RV filling pattern. Cardiovascular magnetic resonance allows gold standard quantification of systemic RV ejection fraction. This may be used to enable clinical decision-making with regard to tricuspid valve surgical replacement.
Computed tomography may be of value in the setting of systemic RV dysfunction with wide QRS when cardiac resynchronization is contemplated to assess the coronary sinus anatomy. We have recently shown that systemic RV late gadolinium enhancement correlates with histological fibrosis, is associated with clinical disease progression, and predicts outcomes, 29 justifying its periodic use.
Introduction
Arterial switch procedure, i. Supravalvar pulmonary stenosis, neo-aortic root dilatation, aortic valve regurgitation, LV dysfunction, and coronary occlusion are relatively common complications. Echocardiography with Doppler can assess aortic valve and LV function and RV pressures, whereas the main and branch pulmonary arteries are often difficult to image.
Computed tomography is particularly suited to image the proximal coronary arteries, reimplanted during repair. Cardiovascular magnetic resonance evaluation of coronary origins is also excellent, although CT is superior in excluding coronary stenoses. The superior vena cava is connected end to side to the top of the right PA, whereas the inferior vena cava is channelled by a patch, flap, or conduit up one side of the right atrium to the PA. Restrictive ventricular septal defect in the setting of ventricular arterial discordance is unfavourable, causing subaortic stenosis.
Ventricular systolic and diastolic dysfunction determine outcome; echocardiography can assess both. A and B Patent Fontan pathways status post-atriopulmonary Fontan. In patient C , thrombus has formed arrows due to sluggish flow in the dilated right atrium. In D , late gadolinium enhancement cardiovascular magnetic resonance evidence of rudimentary endocardial right ventricular fibrosis is seen arrows.
E and F Patent total cavopulmonary pathways asterisks. Imaging is fundamental to the lifelong care of ACHD patients. Cross-sectional imaging with CMR or CT provides complementary and invaluable information on cardiac and vascular anatomy and other intra-thoracic structures. Cardiac catheterization is, mostly reserved for assessment of pulmonary vascular resistance, ventricular end-diastolic pressure, and percutaneous interventions.
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Use of different imaging modalities in lifelong follow-up. Imaging goals in specific congenital heart diseases. Imaging of congenital heart disease in adults Sonya V. Abstract Imaging is fundamental to the lifelong care of adult congenital heart disease ACHD patients.
Congenital heart disease , Imaging , Echocardiography , Magnetic resonance imaging , Computed tomography , Chest X-ray. Time-resolved CE MR angiogram is used for sequential assessment as the contrast passes through the vasculature and to assess for collaterals and pulmonary arteriovenous malformations. Shunt fraction can be calculated by assessing forward flow in the main pulmonary Qp and ascending aorta Qs.
Imaging of congenital heart disease in adults | European Heart Journal | Oxford Academic
This technique is a balanced SSFP acquisition used to obtain anatomical information. A T2 preparation increases signal of the blood compared to myocardium. The sequence is triggered to end diastole to avoid cardiac motion. A navigator is used to monitor diaphragmatic movement and avoid respiratory motion artefact. The scan may be performed without contrast, however administration of Gd-based contrast improves the SNR and CNR contrast to noise ratio.
Often it is used to assess the origins and proximal course of the coronary arteries. These sequences are used for assessment of fibrosis or infarction. This is normally performed minutes following administration of 0. The LGE sequences used are the segmented double inversion recovery sequence; the phase sensitive inversion recovery PSIR , or the single shot acquisition. An initial inversion pulse is used to null the myocardium TI time.
Fibrosis may act as a substrate for arrhythmia. Enhancement may be seen if there has been surgical patch repair, scar from previous surgery or postsurgical infarction. Atrial septal defects constitute the most common shunt lesion detected de novo in adulthood.
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Cardiac MRI plays an important role in assessing and quantifying right ventricular overload, visualizing the defect and calculating the shunt fraction. En face phase contrast cine imaging allows measurement of the defect. Following ASD closure, cardiac MRI is important to assess for any residual shunting, the degree of reduction in right ventricular volumes and for assessment of complications such as device dislodgement.
They may be muscular, inlet, outlet or membranous VSD. The septal leaflet of the tricuspid valve may close the defect or the aortic cusp may prolapse into the defect. Cardiac MRI has a vital role in assessing the defect, quantifying ventricular volumes and the shunt fraction. Coarctation occurs at the junction of the distal aortic arch with the descending thoracic aorta.
It can occur due to protrusion of tissue extending from the posterior aspect of the aortic wall to the opposite wall, contiguous with the ductus arteriosum. Surgical management includes resection and end-to-end repair, subclavian flap repair, prosthetic patch aortoplasty, interposition graft or extra-anatomic bypass graft. Collateral flow can be assessed by quantifying flow just proximal to the coarctation and in the descending aorta at the level of the hiatus.
Aortic angiogram is obtained to assess the coarctation site, collaterals and remainder of the thoracic aorta 17 Figures 4, 5. The aortic arch may be hypoplastic. Left ventricular hypertrophy 16 due to coarctation or aortic valve stenosis may be visualized on the SSFP cine sequences. Infundibulectomy is performed when there is moderate RVOT obstruction in the presence of a normal pulmonary valve annulus. Contrast-enhanced MR angiogram can be used to assess dimensions of the pulmonary arteries and thoracic aorta.
Flow analysis of the valves and across conduit and branch pulmonary arteries can be performed with VE PC cine imaging. LGE sequences will show enhancement around transannular patch repair due to formation of fibrosis. The aorta arises from the right ventricle via a subaortic infundibulum while the pulmonary artery arises directly from the left ventricle. Arterial switch is performed when there is an intact ventricular septum. This was first performed by Jatene.
The pulmonary trunk is moved forward into a new position anterior to the aorta and the great arteries are sutured in place. The coronary arteries are sutured into the neo-aorta. The aorta and pulmonary artery are transected above the level of the valve sinuses. Coronary arteries are sutured into the neoaorta. Long-term complications include supravalvular pulmonary outflow obstruction, neo-aortic regurgitation and arrhythmias.
The pulmonary valve is oversown and a conduit placed between the right ventricle and pulmonary artery. Venous baffles direct deoxygenated venous blood to the pulmonary artery via the left ventricle. The pulmonary venous baffles allow circulation of oxygenated blood through the right ventricle to the aorta. These are seen more easily with transoesophageal echocardiogram rather than with cardiac MRI 23 Figure 8. Right heart dysfunction and tricuspid regurgitation may develop.
Neo-aortic dilatation and regurgitation can be assessed. Baffle leaks and obstruction can be detected.
MRI in the assessment of congenital heart disease
The oblique coronal view is used to assess systemic baffles while the oblique axial plane is used to assess pulmonary venous baffle. In baffle leaks, Qp: Qs can be measured with phase contrast cine imaging to calculate shunt fraction. To assess stenosis, an SSFP gradient echo sequence will demonstrate the dephasing jet through the site of obstruction.
VEPC cine imaging can be performed to assess peak velocity and regurgitant fraction. It allows accurate anatomical and functional assessment. The workup for potential surgery or percutaneous procedures may also be performed with the help of cardiac MRI. This article has highlighted some of the commonest noncyanotic and cyanotic conditions requiring regular follow up with cardiac MRI. MRI in the assessment of congenital heart disease.