Cardiac Congenital Disorders Flashcards
Eisenmenger’s syndrome features
Severe pulmonary arterial hypertension due to elevated pulmonary vascular resistance
Causes right to left intracardiac shunt or greater artery shunting
Systemic arterial desaturation
Features of TOF
- VSD
- Right ventricular outflow obstruction
- Overriding aorta
- Right ventricular hypertrophy
Features of coarctation of aorta
Narrowing of aorta at aortic isthmus, or rarely, in descending thoracic or abdominal aorta
Associated with Turner’s syndrome
Systolic overload i.e. LVH
Chronic upper body systemic hypertension
Difference in pulses above and below coarctation of aorta
Aneurysms, pseudoaneurysm, dissection
Features of ASD
Left to right intracardiac shunt
Right heart enlargement
Pulmonary arterial hypertension (in minority of patients)
Systolic ejection murmur over 2nd left ICS sternal border
Wide and fixed splitting of second heart sound
Prior to surgery of ASD, what is important to evaluate?
Severe PAH - closure is contraindicated
When is ASD closure indicated?
When pulmonary-to-systemic blood flow (shunt) ratio (Qp/Qs) > 1.5:1
VSD features
Harsh holosystolic murmur, best heard over left sternal border
Palpable thrill
Evidence of LVH and RVH
When is closure of VSD indicated?
Left to right shunt is moderate to large
Pulmonary-to-systemic flow > 1.5:1
Contraindicated in Eisenmenger’s syndrome and severe PAH
Cardiac features of Marfan’s syndrome
Progressive aortic root dilatation and aortic dissection
Serial echos required
Features of PDA
Signs and symptoms of pulmonary hypertension and heart failure
Main locations for ASD
- Fossa ovalis (ostium secundum)
- Inferior portion of atrial septum (Ostium primum)
- Superior portion of atrial septum (sinus venosus)
Associated conditions for TOF
DiGeorge syndrome
Down syndrome
Clinical findings of TOF
Cyanosis - depending on severity of RVOTO
Tet spells
Relief of symptoms when squatting (increased SVR)
Harsh systolic ejection murmur (caused by RVOTO)
Single S2
RV heave and systolic thrill
Features of heart failure
X-ray features of TOF
Boot shaped heart
Normal or decreased pulmonary vascular markings
Features of transposition of great vessels
Anatomical reversal of aorta and pulmonary artery
Clinical findings of transposition of great vessels
Cyanosis - not affected by exertion or supplemental oxygen
Tachypnoea
Single, loud S2
No murmur
Diminished femoral pulses
CXR findigns of TGV
“Egg on a string” appearance
Increased pulmonary vascular markings
Features of tricuspid valve atresia
Absent or rudimentary tricuspid valve, results in no blood flow between RA and RV
ASD, VSD - only way for patient to have intertrial and interventricular communications
RV hypoplasia
Clinical features of tricuspid valve atresia
Central cyanosis
Tachypnoea
Holosystolic murmur at lower left sternal border
Single S2
Jugular venous distension with prominent A wave
Diminished peripheral pulses
Features of Ebstein anomaly
Malformed tricuspid valve leaflets that are displaced into right ventricle with subsequent tricuspid valve regurgitation right heart enlargement
Interatrial communication
Conduction disorders
Clinical findings of Ebstein anomaly
Depending on severity of abnormality
Mild apical displacement –> asymptomatic presentation throughout adulthood
Moderate –> cyanosis or heart failure in infancy or childhood
Severe –> in utero heart failure –> death
Loud S1
Widely split S1 and S2
Holosystolic murmur at left sternal border
Digital clubbing
ECG and CXR findings of ASD
ECG - RAD, RBBB, RVH
CXR features of enlarged R atrium and ventricle, increased pulmonary vasculature, dilated main pulmonary artery, small aortic knob
Features of PFO
Variant of cardiac anatomy in which foramen ovale remains patent beyond 1 year of age
Results in embolisms
Features of patent ductus arteriosus
Failure of ductus arteriosus to completely close postnatally
Causes of persistent communication between aorta and pulmonary artery –> left to right shunt –> volume overload of pulmonary vessels
Clinical findings of PDA
Bounding peripheral pulses
Wide pulse pressure
Heaving, laterally displaced apical impulse
Loud continuous murmur best heart in left infraclavicular region and loudest at S2 - if large PDA
Investigation findings of coarctation of aorta
ECG - signs of LVH
X-ray - cardiomegaly and increase pulmonary vascular markings
Figure of 3 sign
Rib notching
Features of pulmonary valve stenosis
Obstruction of blood outflow from right ventricle into pulmonary arteries during systole
Causes RV outflow obstruction –> RVH
Clinical findings of pulmonary valve stenosis
Systolic murmur best heard over second left ICS at sternal border
S2 wide splitting
