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9/17/2013 Outflow Tract View : Normal and Abnormal Anitha Parthiban MD, FAAP Director , Pediatric Echocardiography Children’s Mercy Hospitals & Clinics ©2013 Children's ©2013 Mercy Children's Hospitals Mercyand Hospitals Clinics.and All Clinics. Rights Reserved. All Rights Reserved. 03/13 03/13 Outline Review anatomy of normal outflow tracts Imaging of the outflow tracts in the fetal heart Differential diagnosis of abnormal outflow tract view Brief review of Conotruncal defects 2 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 1 9/17/2013 Conotruncal Defects Defects of the outflow tracts , constitute 15-20% of CHD Key events in formation of outflow tracts Leftward shift of the conotruncus to override the muscular septum Septation of the contruncus Spiraling of the outflow tracts 3 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Outflow Tracts- Checklist Are there two outflow tracts? Ventriculoarterial concordance ? Great arteries cross each other? Caliber of pulmonary trunk vs ascending aorta Excursion /size of aortic and pulmonic valves and sub valvar area 4 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 2 9/17/2013 Short axis view- basal 5 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Case 1 6 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 3 9/17/2013 Case 1 7 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Case 1 8 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 4 9/17/2013 Truncus Arteriosus Single arterial trunk arises from the heart giving rise to aorta , pulmonary arteries and the coronary arteries 1.2% of CHD 33% of patients have Di-George syndrome (Deletion in chromosome 22q11) 9 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Truncus Arteriosus Truncal valve overrides the large ventricular septal defect Truncal valve is dysplastic and often insufficient (50%), stenotic in about 33% of patients Neonatal congestive heart failure necessitating repair 10 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 5 9/17/2013 Case 2 11 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Case 2 12 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 6 9/17/2013 Case 2 13 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 14 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 7 9/17/2013 Transposition of Great Arteries (d-TGA) 4.7% CHD VSD may be present (40%) or intact ventricular septum (IVS) Pulmonary/ sub pulmonic stenosis may be present 15 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Transposition of Great Arteries (d-TGA) With TGA/IVS, severe cyanosis is present Patency of the foramen ovale and ductus arteriosus for mixing 16 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 8 9/17/2013 Case 3 17 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 9 9/17/2013 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 Tetralogy of Fallot Large ventricular septal defect (VSD) Aorta is large and overrides VSD Size discrepancy in 3 vessel view (pumonary trunk smaller than aorta) 20 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 10 9/17/2013 Tetralogy of Fallot Ductus arteriosus frequently small Absent pulmonary valve – aneurysmal dilation of the pulmonary arteries Pulmonary atresia (valve does not form) Reversal of flow in the ductus arteriosus indicates critical pulmonary stenosis ( ductal dependant) 21 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Case 4 22 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 11 9/17/2013 Case 4 23 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 12 9/17/2013 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 Critical Aortic Stenosis Severely obstructive aortic valve- thick, doming Dilated poorly functioning left ventricle Restricted mitral valve opening Mitral regurgitation Aortic valve velocity may be normal 26 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 13 9/17/2013 Critical Aortic Stenosis Reversal of flow in the aortic arch and foramen ovale (FO) Restrictive FO – cardiomegaly and left atrial dilation Mid gestation severe aortic stenosis may evolve to hypoplastic left heart syndrome Role for fetal aortic valvuloplasty 27 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 28 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 14 9/17/2013 29 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Case 5 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 15 9/17/2013 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 ©2013 Children's Mercy Hospitals and Clinics. All Rights Reserved. 01/13 03/13 16 9/17/2013 Critical Pulmonary Stenosis Mild to moderate pulmonary stenosis (PS) difficult to diagnose Severe PS – right ventricular hypertrophy Right to left shunt at the FO- left heart enlargement Tricuspid regurgitation 33 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 Critical Pulmonary Stenosis Retrograde flow in the ductus arteriosus Treatment- balloon valvuloplasty, radiofrequency perforation, surgical 34 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 17 9/17/2013 Outflow tract view Outflow tract evaluation is an essential aspect of fetal cardiac evaluation Normal 4 chamber view does not rule out critical congenital heart disease Systematic imaging key for accurate diagnosis 35 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 References Echocardiographic Anatomy in the Fetus : Chiappa EM, Cook CC, Botta G, Silverman NH www.aium.org 36 ©2013©2013 Children's Children's MercyMercy Hospitals Hospitals and Clinics. and Clinics. All Rights All Rights Reserved. Reserved. 03/13 03/13 18