Case Study

Gerrit Blignaut24 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

Decreasedvasculature

1. TGA2. Truncus arteriosis3. TAPVC4. Tricuspid atresia5. (T)Single ventricle

Cardiac enlargement?

YesNo

Ebstein anomalyPulmonic atresiaTricuspid atresia

Fallottetralogy

No cyanosis

Vasculatureincreased

Vasculature normal

Left atrialEnlarged?

No

Yes

Endocardial cushion defectAtrial septal defect

Aorta enlargement?

No = VSDAVSD/endocardial cushing defect

Yes = PDA

Aortic stenosisPulmonic stenosisCoartationIAA

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.

Hemodynamics Right Left

Atrium

Ventricle

Vasculature Normal

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%.

Hemodynamics

Right Left

Atrium

Ventricle

Vasculature Normal

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

Hemodynamics Right Left

Atrium N >

Ventricle N>

Vasculature Aorta 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