9/8/2009 < 1 1,2 3,4 5,6 7,8 9,10 11,12 13,14 15,16 17,18 > 18. Tetralogy of Fallot. Complex Congenital Heart Disease.

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1 Current Indications for Pediatric CTA S Bruce Greenberg Professor of Radiology Arkansas Children s Hospital University of Arkansas for Medical Sciences Age distribution Cardiac CTA < 1 1,2 3,4 5,6 7,8 9,10 11,12 13,14 15,16 17,18 > 18 Common cardiac CTA indications Pulm vein Coarctation anom TOF Tetralogy of Fallot Complex congenital heart disease Vascular ring/sling 2008 Arkansas Children s Hospital Cardiovascular MDCT exams Infants (< 1 year) 32% Newborns (< 1 month) 9% HLHS Ring/sling Complex CHD Hypoplastic left heart syndrome Coarctation / interruption of aortic arch Pulmonary vein anomaly 2008 Arkansas Children s Hospital Cardiovascular MDCT exams Vascular anomalies with respiratory distress Post-operative operative Congenital Heart Disease Vascular anomalies with respiratory distress Complex arch anomaly Vascular ring Isolated subclavian artery Pulmonary sling Williams syndrome & Williams-like like syndrome Miscellaneous 1

2 9/8/2009 Vascular anomaly with respiratory distress: complex arch anomaly Infant with vascular anomalies Patent ductus arteriosus Patent ductus arteriosus and complex aortic arch Pulmonary sling Effective dose = 0.6 msv 1 year old with William Syndrome Thorax and abdomen (cardiac gated) Effective dose 5.2 msv 1 year old with William Syndrome Normal renal arteries Supravalvar aortic stenosis (blue arrow) Branch pulmonary artery stenoses (white arrows 2

3 Infant with Scimitar syndrome Multiple anomalies Not easily characterized by echocardiography Multiple organ systems Vascular Airway Abdomen viscera White arrow points to scimitar vein and a pulmonary sequestration : multiple congenital anomalies Yellow arrow indicates arterial supply of sequestered lung from right renal artery Atrioventricular septal defects & pulmonary atresia Bilateral patent ducti arterosi supply non-confluent branch pulmonary arteries Total anomalous venous return below the diaphragm 3

4 : Not easily characterized by echo Bilateral trilobed lungs and situs inversus abdominus Upstairs downstairs heart In-flow & Out-flow Post-operative operative Congenital Heart Disease Palliative procedures Hypoplastic left heart syndrome Right heart obstruction Corrective procedures Complex tetralogy of Fallot Atrioventricular septal defects Anomalous venous return Hypoplastic left heart syndrome Post-operative operative Congenital Heart Disease: Hypoplastic left heart - hybrid procedure Right Left Thrombosed Sano shunt Patent ductus arteriosus stent 4

5 Hybrid procedure: PDA stent and native aorta measurements Hybrid procedure: branch pulmonary artery banding Tetralogy of Fallot with absent pulmonary valve Left bronchus narrowing despite LeCompte maneuver LeCompte Maneuver Pericardial mono-cusp valve Summary Echocardiography the initial imaging tool for all congenital heart disease CTA role for infants and small children Respiratory distress Complex congenital heart disease Post operative imaging Adult Congenital Heart Disease Prevalence 4-6 per 1000 live births 85% live into adulthood 5% per year complex congenital heart disease > 1,000,000 Americans Adult : Pediatric Ratio : :40 5

6 Corrections or palliations? Long term complications Hospitalizations Mortality Complications Arrhythmias Vascular lesions Heart failure Imaging teenagers and adults with congenital heart disease Echocardiography - limited windows in adults Magnetic resonance angiography Availability equipment and personnel Contraindications and limitations Computed tomography angiography Large field of view Higher spatial resolution than MRI Morphology and some function Indications for CTA in teenagers and adults with CHD CT rarely used for primary diagnosis of congenital heart disease in adults Echocardiography inadequate and MRI not available or contraindicated Primary diagnosis Post operative Red arrow coronary sinus Yellow arrow - ASD CTA preferred to MRI Acutely ill patient Cardiopulmonary disease Ferromagnetic implants likely to cause artifact Clips and coils Vascular stents Evaluation metal stents and prosthetic valves High resolution imaging Coronary arteries Cardiopulmonary disease & Acutely ill patient 25 year old with unrepaired ASD, Eisenmenger physiology and increasing dyspnea 6

7 9/8/2009 Pulmonary artery thrombus Metal stent evaluation Posterior view Right pulmonary artery stent (status post double outlet right ventricle with pulmonary artery hypoplasia) Occlusion of left pulmonary artery by thrombus High resolution imaging Compression of left coronary artery by right pulmonary artery Left sinus of Valsalva aneurysm Anatomy better illustrated than by catheter angiography or MRA Summary: CTA of Adult Congenital Heart Disease 19 year old tricuspid atresia & cyanosis MDCTA indications Easier than MRI High resolution imaging Best to complement echocardiography when MRI not possible or practical Great for Metal stents Associated coronary artery anomalies Associated pulmonary disease Classic Fontan palliation Veno--veno shunt: left upper extremity vein Veno preferential flow to left upper pulmonary vein 7

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