Congenital Diseases

Congenital Disease

The Common Vein Copyright 2007

Aortic Valve

Normal and Thickened Aortic Valve over Time

07953c02 heart cardiac aorta aortic valve fx normal fx thickened fx bicuspid aortic valve fx calcified fx calcification fusion of the intercoronary commisures grossanatomy

Bicuspid Aortic Valve

This gray scale echo of the heart showing a short-axis aorta left atrial view, and demonstrating the aortic valve with two cusps. The patient has a diagnosis of bicuspid aortis valve which is a congenital condition. Courtesy Philips Medical Systems 33169 code cardiac heart echo aorta bicuspid aortic valve congenital imaging cardiac echo

Bicuspid Aortic Valve

72885.800 thorax thoracic aorta aortic valve fx thickened dx bicuspid aortic valve CTscan Courtesy Ashley Davidoff

D Transposition of the Great Vessels 

Transposition of the Great Vessels

This is an angiogram of the RV, showing an anteriorly placed aorta, a VSD filling the LV, and a posteriorly positioned smaller MPA. The catheter courses via the IVC into the RV. The findings are consistent with TGA and in this case a D-TGA, though it is impossible to identify the position of the aortic valve in relation to the PA in this lateral projection. An associated VSD and subpulmonary stenosis and or PS is implied by the small sized PA. Courtesy Ashley Davidoff MD 01487 code CVS heart cardiac transpoistion of the great arteries DTGA VSD subpulmonary stenosis imaging radiology angiography

DTGA

07258b02 anterior aorta posterior pulmonary artery position connection subaortic conus D TGA TGV D transposition of the great vessels D transposition of the great arteries dextro rightward MRI Davidoff MD

Corrected Transposition

LTGA
07304b01 anterior aorta posterior pulmonary artery position connection subaortic conus L TGA L TGV L transposition of the great vessels L transposition of the great arteries levo leftward MRI Davidoff MD
LTGA
28999b01 heart cardiac aorta pulmonary artery RVOT conotruncal malformation LTGA L TGV transposition of the great vessels transposition of the geat arteries corrected transposition position connection relation embryology CTscan Davidoff MD 28994 28995 28996 28997 28998 28999

Right Aortic Arch

Supravalvar Aortic Stenosis

Hx 4 year female with cocktail pesonality hearyt cardiac artery aorta supravalvular aortic stenosis supravalvar aortic stenosis Williams syndrome William’s syndrome 08233b01

Sinotubular Ectasia – Marfan’s Syndrome

72712 thoracic aorta sinus of Valsalva aortic sinuses sinotubular junction of ascending aorta sinotubular ectasia fx dilated CTscan Courtesy Ashley DAvidoff MD 72709

Marfan’s Syndrome Sinus of Valsalva Aneurysm

07974c01 heart cardiac right atrium aortic valve aortic sinus fx right atrial filling defect dx sinus of Valsalva aneurysm of the right aortic sinusprolapsing into the right atrium (RA defect) dx Marfan’s Syndrome MRI T1 weighted Davidoff MD 07973.800 07974c01 07974.800

Ascending Aorta

Aortic Valve Atresia in the Setting of Hypoplastic Left Heart Syndrome

00269b02 heart cardiac coronary artery aorta small dx aortic atresia tubular hypoplasia aortic coarctation aortic atresia PDA patent ductus arteriosus angiogram angiogaphy CHD congenital heart disease Davidoff MD 00269b01 00269b02 00269b03

Arch

Dwarf with Kyphosis and Secondary Hypertrphy of the Costal Cartilages

49838c05 50 year old female with respiratory difficulty trachea bronchi rectus abdominis muscle compression fractures kyphosis dwarf dwarfism right aortic arch tracheomalacia tracheal stenosis rectus abdominis muscle hypertrophy Davidoff MD Courtesy Ashley Davidoff MD CTscan 49838 49838c01 49838c02 49838c03 49838c04 49838c05 shape size position character growth

Right Aortic Arch

35334 Courtesy of Laura Feldman MD. code aorta arch artery right aortic arch thorax

Cervical arch

Pseudocoarctation

This series of CXRays P-A (a) lateral (b) and magnified views (c,d) showing an aberrant shape to the aortic knob which is characterised by a “3 sign” reminiscent of a coarctation segment. There is no assocated rib notching nor a pressure gradient and hence the diagnosis is a pseudocoarctation of the thoracic aorta. 35187c01 Courtesy LAura FEldman MD code CVSartery aorta thorax 3 sign peudocoarctation.

Aberrant Right Subclavian Artery

16376 aorta aberrant origin of right subclavian artery as the last vessel off the left aortic arch congenital growth position esophagus CTscan Davidoff MD

Isthmus

Patent Ductus Arteriosus

42326.800 aorta pulmonary artery finding PDA patent ductus arteriosus left to right shunt arteriovenous malformation CTscan Courtesy Ashley Davidoff MD
 

Coartcation of the Aorta

This combination of an angiogram and MRI show a focal coarctation of the aorta distal to the dilated left subclavian artery. Note the large internal mammary artery in the angiogram (a) Courtesy Ashley Davidoff MD 00254c02 CVS aorta thorax thoracic aorta coarctation

Coarctation CTscan

44146 coarctation of the aorta heart cardiac CTscan MDCT Courtesy Philips Medical

Aortic Interruption

Congenital – may be in isolation part of the HLHS or in association with other congenital heart diseases dx imaging echo sometimes MRI rx Non invasive techniques and surgical

Aortic Interruption at the Level of the Isthmus

The most obvious finding in this CXR (a) with pleuro-parenchymal changes is not the most significant. In image (b) the highlighted ribs reveal rib notching characteristic of coarctation of the aorta. The lateral examination (c) in this instance is not helpful. In the early phase of the angiogram(d), there appears to be complete interruption of the aorta with a large left subclavian artery acting as a collateral pathway. The sbsequent images e, and f, show progressive filling of the isthmus and distal thoracic aorta. The coarcatation becomes apparent characterised by a “3” sign. 35107c Courtesy Laura Feldman MD code CVS artery aorta thorax coarctation rib notching bone collateral