Computed tomography in paediatric heart disease

Abstract

Cardiovascular CT (CCT) is an important imaging modality in congenital and acquired paediatric heart disease. Technological advances have resulted in marked improvements in spatial and temporal resolution of CCT with a concomitant increase in speed of data acquisition and a decrease in radiation dose. This has elevated CCT from being sparingly used to an essential diagnostic tool in the daily multimodality imaging practice alongside echocardiography, cardiovascular MR and invasive angiography. The application of CCT in paediatric congenital and acquired heart disease can be both technically and diagnostically challenging. This review highlights important considerations for current state of the art CCT across the spectrum of heart disease encountered in children.

Figure 1.

A 12-month-old infant with a failing RV after a truncus arteriosus repair referred for assessment of pulmonary blood flow. The surgically placed and purposely restrictive conduit from the RV to the PAs has mild proximal and distal calibre reduction (arrows). The presence of left to right shunting across multiple muscular ventricular septal defects (circles) supports an only mild degree of functional restriction to PA flow in the context of an unobstructed left ventricular outflow tract. A fenestrated interatrial septum shunts in the left to right direction (arrowhead). Contrast medium was administered into the left-sided chambers via a left superior vena cava to an unroofed coronary sinus, leading to higher contrast in the LA and LV compared with the RA and RV. This facilitated directional assessment of the atrial and ventricular level shunts. Note left lower lobe collapse and left pleural effusion with less severe right-sided changes. LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle.

Figure 2.

A 4-day-old neonate referred for anatomical assessment of suspected complex congenital heart disease. The heart is in the left hemithorax with the apex to the left. There is right atrial isomerism with bilateral RAAs. The atrium is effectively common due to a large interatrial communication. A single atrioventricular valve connects the atria to a single ventricle of indeterminate morphology. The single ventricle gives rise to the AO and a right-sided aortic arch that has a mirror image branching pattern. There is pulmonary atresia with an absent central pulmonary arterial confluence (circle). Both the LPA and RPA were unobstructed and independently supplied by a LPDA and RPDA, respectively. There is unobstructed supracardiac total anomalous pulmonary venous return via a common confluence (TAPVC) to a RSVC. The RSVC goes to the right-sided atrium and a LSVC goes to the left-sided atrium via an unroofed coronary sinus. A small BV is present. The IVC and HV drain to right-sided atrium. The liver was midline, the stomach was on the right, and there was bronchial situs inversus. AO, aorta; BV, bridging vein; CCT, cardiovascular CT; HV, hepatic veins; IVC, inferior vena cava; LPA, left pulmonary artery; LPDA, left patent arterial duct; LSVC, left superior vena cava; RAA, right atrial appendage; RPA, right pulmonary artery; RPDA, right patent arterial duct; RSVC, right superior vena cava.

Figure 3.

A 23-month-old toddler (A) referred for anatomical assessment prior to coarctation repair. The left-sided transverse aortic arch is hypoplastic (black square) with severe juxtaductal coarctation (arrows), post-stenotic dilation (asterisk) and collaterals formation. At the coarctation site the ligamentous arterial duct (circle) tethers the isthmic aorta towards the MPA. A 5-year-old child (B) referred for vascular, mediastinal and pulmonary assessment after presenting in heart failure with fever and raised inflammatory markers. Severe long segment narrowing is present at the aortic isthmus (arrowhead) with wall thickening and collaterals (squares). Note the severely dilated LA. Surgical biopsy established the diagnosis of Takayasu arteritis. LA, left atrium; MPA, main pulmonary artery.

Figure 4.

A 3-month-old infant with 22q11 microdeletion awaiting complete repair for tetralogy of Fallot referred for assessment of a possible left-sided patent arterial duct. An isolated LSA arises from the distal pulmonary trunk and there is a RAA. The isolated LSA is narrowed at the origin (circle). The LCCA, RCCA and right subclavian arteries arise seperately from the transverse aortic arch. The origins of the confluent branch pulmonary arteries are abnormal with a rotated and superioinferior orientation—the LPA originates above the RPA consistent with a pattern often seen in 22q11 mircodeletion. Both branch pulmonary arteries were small calibre. LCCA, left common carotid artery; LPA, left pulmonary artery; LSA, left subclavian artery; RAA, right-sided transverse aortic arch; RCCA, right common carotid artery; RPA, right pulmonary artery.

Figure 5.

An 8-day-old neonate referred for surgical planning for a known double aortic arch. There was a large aortopulmonary window (asterisk) connecting the AO to the PA. The double aortic arch has a dominant right arch (R) and a smaller left (L) transverse arch. There is severe tracheal compression from the vascular ring (insert). AO, aorta; PA, pulmonary artery.

Figure 6.

A 16-year-old teenager with prior aortic valve surgery referred for aortic root and mediastinal assessment due to new sepsis. A large aortic root abscess (asterisk) associates with extensive fat stranding and lymph node enlargement (square). The abscess has fistulous connections to the aortic sinus (arrowhead) and subvalvar left ventricular outflow tract (arrow). The RCA was proximally obstructed (circle) due to a tethered and thickened right coronary leaflet of the aortic valve. RCA, right coronary artery.

Figure 7.

A 22-month-old toddler referred for assessment of the branch pulmonary arteries with signs suggestive of high afterload on the right ventricle. The child was born with transposition of the great arteries for which an arterial switch procedure was performed, and following the Lecompte maneuver the branch pulmonary arteries (LPA and RPA) course on either side of the neo-aortic root. The neopulmonary trunk (asterisk) is unobstructed with moderate bilateral branch pulmonary artery stenosis. There is also an anomalous LCX from the proximal RCA, and the LAD arises in the space between the neopulmonary trunk (asterisk) and aortic root (square). LAD, left anterior descending coronary artery; LCX, left circumflex coronary artery; LPA, left pulmonary artery; RCA, right coronary artery; RPA, right pulmonary artery.

Figure 8.

A 15-year-old teenager referred for coronary arterial assessment following out-of-hospital cardiac arrest with ventricular fibrillation. The coronary arteries originate from a single broad ostium (asterisk) on the right coronary sinus. The LMS has long segment narrowing over a long interarterial course between the aorta and MPA. The LMS regains better calibre prior to dividing into the normal LAD and LCX. The RCA is unobstructed. Following surgical translocation of the MPA, the LMS was unobstructed (circle). LMS, left main stem; MPA, main pulmonary artery; LAD, left anterior descending coronary artery; LCX, left circumflex coronary artery; RCA, right coronary artery.

Figure 9.

A 17-year-old teenager referred for coronary arterial assessment following out-of-hospital cardiac arrest with ventricular fibrillation. A left coronary artery originates with a LMS from the MPA, and bifurcates normally into the LAD and LCX. This anomaly is called ALCAPA. The dominant and dilated RCA originated normally. The shunt from the ALCAPA had caused dilation of the left ventricle and left atrium. ALCAPA, anomalous left coronary artery from the left coronary artery; LAD, left anterior descending coronary artery; LCX, left circumflex coronary artery; LMS, left main stem; MPA, main pulmonary artery; RCA, right coronary artery.

Figure 10.

A 27-month-old toddler referred for assessment of the pulmonary arteries following repeated chest infections with suspected airways disease. There is a LPA sling in which the LPA originates from the RPA and courses behind the distal trachea towards the left hilum. There is severe proximal narrowing of the LPA sling (arrow) and mild calibre reduction of the distal trachea and proximal main bronchi (circle). LPA, left pulmonary artery; RPA, right pulmonary artery.

Figure 11.

A 27-day-old infant with a pre-natal diagnosis of pulmonary atresia referred for assessment of the pulmonary arteries and the source of pulmonary blood flow. There was pulmonary atresia with an absent connection from the RV to the central pulmonary arterial system (circle). The central pulmonary arteries were present but severely hypoplastic (arrows). There were multiple tortuous and stenosed major aortopulmonary collaterals (arrowheads) to both lungs from the DAO. These collaterals communicated with the central pulmonary arterial system. DAO, descending aorta; RV, right ventricle.

Figure 12.

An 8-year-old child (A) referred for baseline investigation of known pulmonary hypertension. There is a patent arterial duct (circle) with a restrictive segment at the MPA end. The duct was deemed suitable for percutaneous stenting in order to promote right to left shunting. Consistent with severe pulmonary hypertension the pulmonary arteries are severely dilated and there is collateral pulmonary blood supply via hypertrophied bronchial arteries (arrow). A 27-month-old toddler (B) with a dilated LA and left ventricle referred for diagnosis of potential left to right shunting lesions. A severely dilated MAPCA (dot) arises from the distal DAO to supply the left lower lobe. The left lower lobe of lung has normally draining LPV to the LA and a conventionally connected bronchial tree. The LPA, LPV and large airways of the left upper lobe iwere conventionally connected. DAO, descending aorta; LA, left atrium; LPA, left pulmonary artery; LPV, left pulmonary vein; MAPCA, major aorto pulmonary collateral artery; MPA, main pulmonary artery.

Figure 13.

A 6-month-old infant (A) referred for assessment of the optimal closure strategy for a known PDA. The PDA is suitable for percutaneous device closure. There is also moderate LUPV and mild LLPV stenosis at the LA confluence. A follow-up CCT (B) shows progressive subtotal LUPV and moderate LLPV stenosis. A 4-month-old infant (C) referred for assessment of pulmonary venous anatomy. The pulmonary venous drainage is abnormal with an ipsilateral drainage pattern of the LPVs to the LA and hemi-anomalous return of the RPVs to the RA. Consistent with this, the RPVs do not cross the plane of the interatrial septum (IAS) to go to the LA. LA, left atrium; LLPV, left lower pulmonary vein; LPV, left pulmonary vein; LUPV, left upper pulmonary vein; PDA, patent arterial duct; RA, right atrium; RPV, right pulmonary vein.

Figure 14.

A 16-year-old teenager referred for assessment of the pulmonary vasculature and lung parenchyma after new haemoptysis on a background of scimitar syndrome, hypoplastic right pulmonary artery system and prior coil embolisation of an infradiaphragmatic major aortopulmonary collateral artery to the right lung. The SV drains to the IVC with a venous collateral connection (arrows) from the small right-sided pulmonary veins to the venous plexuses around the distal oesophagus and gastric fundus (circles). Sparse distal pulmonary arterial vasculature is present due to right-sided pulmonary arterial hypoplasia. IVC, inferior vena cava; SV, scimitar vein.

Figure 15.

A 7-year-old child referred for assessment of the systemic venous anatomy prior to replacement of epicardial pacing system after a pacing lead fracture. A rudimentary RSVC drains to the RA after receiving a small azygos vein and connecting with a prominent paravertebral collateral venous plexus. A large LSVC receives a dilated hemiazygos vein, draining to the RA via a dilated CS. There is juxtaposition of the RAA that is to the left and on top of the LAA. The branch pulmonary arteries (asterisk) course around aorta following the Lecompte maneuver performed for surgical correction of transition of the great arteries. CS, coronary sinus; IVC, inferior vena cava; LAA, left atrial appendage; LSVC, left superior vena cava; RA, right atrium; RAA, right atrial appendage; RSVC, right superior vena cava;

Figure 16.

A 6-year-old child with stents in the main and branch pulmonary arteries referred for assessment of the stents, pulmonary arteries and mediastinum due to suspected endocarditis. The first CCT (A) shows extensive laminar thrombus in the LPA stent and marked mediastinal lymph node enlargement (asterisk) but no discrete collection. A subsequent CCT (B) after 5 weeks of antibiotic treatment shows complicated endocarditis with extensive pseudoaneurysms that fill with contrast medium through the stent mesh. There is also Note is made a MAPCA to left lung from the descending aorta and an incidental RIV. CCT, cardiovascular CT; LPA, left pulmonary artery; MAPCA, major aortopulmonary collateral artery; RIV, retroaortic innominate vein.

Figure 17.

A 3-year-old toddler with biventricular assist device and possible collection adjacent to the right ventricle referred for assessment of the collection and patency of the pathways. The cardiac chambers, great vessels and device components were adequately opacified and unobstructed with no thrombus. The RV and RA are compressed with the right ventricular free wall seen concave. A large pericardial collection (asterisk) was the cause of this with no acute bleeding. A repeat CCT was performed after oxygen requirements suddenly increased and there was now thrombus in the pulmonary arteries (circle). CCT, cardiovascular CT; RA, right atrium; RV right ventricle.

Figure 18.

A 6-year-old child referred for pre-surgical assessment of the retrosternal anatomy prior to replacement of a narrowed Contegra conduit that connects the RV to the MPA. The valved conduit has distal calcification (arrowhead) and narrowing (asterisk). The valve with the metallic ring sits in the mid conduit and has eroded into the thinned sternum. MPA, main pulmonary artery; RV, right ventricle.