Case Study Gerrit Blignaut 24 February 2012

Patient 1: Cyanotic Give the diagnosis and specific radiological sign

Patient 2 Cyanotic

Patient 3 Cyanotic

Patient 4 Acyanotic

Types of cardiac lesions 1:Acyanotic with increase pulmonary vasculature L>R shunt: VSD, ASD, PDA 2:Acyanotic with normal pulmonary vasculature Outflow tract obstruction: AS, PS, CoA 3:Cyanotic with increase vasculature Mixture of systemic and pulmonary blood 4:Cyanotic with decrease vasculature Obstruction pulmonary flow R>L shunt

Cyanotic: Increased vasculature Decreased vasculature 1.TGA 2.Truncus arteriosis 3.TAPVC 4.Tricuspid atresia 5.(T)Single ventricle Cardiac enlargement? Yes No Ebstein anomaly Pulmonic atresia Tricuspid atresia Fallot tetralogy

No cyanosis Vasculature increased Vasculature normal Left atrial Enlarged? No Yes Endocardial cushion defect Atrial septal defect Aorta enlargement? No = VSD AVSD/endocardial cushing defect Yes = PDA Aortic stenosis Pulmonic stenosis Coartation IAA

Patient 1:

Tetralogy of Fallot 4 Components VSD RVOT obstruction Overriding aorta, Right ventricular hypertrophy. 10% of congenital cardiac lesions. 25% has right sided aortic arch Associated with TOF, rib anomallies and scoliosis.

HemodynamicsRightLeft Atrium Ventricle VasculatureNormal

Patient 2

Ebsteins anomaly Downward displacement of the tricuspid valve into the inflow portion of the right ventricle. Forms a common right ventriculoatrial chamber and causes TI. TI: Dilatation of the RVOT and all proximal right heart structures. Right atrium becomes enlarged, and a R>L shunt (through a patent foramen ovale or ASD.) Cyanosis

Associations: Maternal lithium usuage. Patent foramen ovale/ ASD 80%. HemodynamicsRightLeft Atrium Ventricle VasculatureNormal

Patient 3

Transposition of great vessels Infants of diabetic mothers. Isolated in 90%, rarely associated with a syndrome Pulmonary artery to the right of its normal location. Obscured by the aorta on PA CXR. Cardiovascular silhouette varies from normal to enlarged and globular. Causes for narrowing superior mediastinum CXR TGA Thymic atrophy Hyperinflated lungs

HemodynamicsRightLeft AtriumN > VentricleN> VasculatureAorta normal

Patient 4

Coarctation of aorta Posterior infolding of the aortic lumen. Junction of the aortic arch and the descending thoracic aorta. Narrowing of the lumen at insertion of ductus or ligamentum arteriosus. Cause obstruction outflow from LV. 7% of congenital cardiac lesions. Clinical manifestations CCF infancy -- hypertension with difference upper lower limbs.

Coarctation syndrome: Coarctation PDA VSD Associations Turner syndrome CoW aneurysms PDA aneurysm

Localized coarctation Left subclavian artery or the ligamentum arteriosum. Dilatation of the left subclavian artery. Collateral vessels forms to bypass the coarctation. Rib notching 3/4 -8 (Desc aorta) Costocervical trunk supply ½ intercostal arteries (usually spared).

Tubular hypoplasia (Preductal coarctation) Narrowing beyond the origin of innominate artery. Combined focal constriction before the level of the ductus arteriosus. Associated with a deformed or bicuspid aortic valve. R>L shunt via PDA or VSD

PET

Reference: Classic Imaging Signs of Congenital Cardiovascular Abnormalities RadioGraphics 2007 ; 27:1323–1334 Primer p 117 – 140. Universitas iSite