Pathoma - Cardiac congenital defects Flashcards
Describe Eisenmenger Syndrome
Reversal of a shunt from L–>R to R–>L
L to R shunt causes increased flow through pulmonary circulation which then leads to hypertrophy of the pulmonary vessels and pulmonary
HTN Increased pulmonary resistance results in reversal of the shunt and late cyanosis
What is the most common congenital heart defect
Ventricular septal defect
Describe the presentation of ventricular septal defect
L to R shunt
Small shunts often asymptomatic
Large defects can lead to Eisenmenger syndrome
Common association of VSD
Fetal alcohol syndrome
Describe the presentation of atrial septal defect
L to R shunt with split S2 on auscultation (increased blood in R heart delays closure of pulmonary valve)
Complication of ASD
Paradoxical emboli (venous emboli that travel from RA to LA to arterial system)
Disease associate with patent ductus arteriosus
Congenital rubella
Presentation of PDA
L to R shunt between aorta and pulmonary artery
May lead to Eisenmenger syndrome
Describe the pattern of cyanosis in PDA
Lower extremity cyanosis (ductus arteriosus occurs after the upper extremities already branch off)
Treatment of PDA
Indomethacin (decreases PGE, which is what keeps the PDA open)
What are the 4 defects in tetralogy of fallot?
(1) Pulmonic artery stenosis
(2) RV hypertrophy
(3) VSD
(4) Displacement of aorta over the VSD
Presentation of Tetralogy of fallot
R to L shunt (due to pulmonic stenosis) causes early cyanosis
Patients squat in response to cyanotic spell
‘Boot-shaped’ heart on X-ray
Describe the defect in Transposition of the great vessels
Pulmonary artery arises from the LV and aorta arises from the RV
Results in two independent circuits that do not mix
Disease associated with transposition of the great vessels
Maternal diabetes
Treatment of Transposition of the great vessels
Creation of a shunt is necessary for survival
PGE can be given to maintain PDA until definitive surgical repair is performed
Describe the defect in Truncus Arteriosus
Single large vessel arising from both ventricles
Presentation of Truncus arteriosis
Early cyansosis because deoxygenated blood from RV mixes with oxygenated blood from LV before pulmonary and aortic circulations separate
How is the RV affected by tricuspid atresia
RV is hypoplastic
What other defect is associated with tricuspid atresia?
ASD, resulting in R to L shunt
Presents with early cyanosis
Where is the location of infantile aortic coarctation and describe the presentation
Coarctation lies after (distal to) the aortic arch, but proximal to the PDA
Associated with PDA
There will be a R to L shunt at the PDA (after the coarct), leading to lower extremity cyanosis
What disease is associated with infantile aortic coarctation
Turner syndrome
Where is the location of adult aortic coarctation and describe the presentation
Coarctation lies distal to the aortic arch with NO patent ductus arteriosis
This lead to shunting of the blood to the vessels of the upper extremities (HTN of UE and hypotension and weak pulses of LE)
Describe X-ray findings of adult aortic coarctation
Collateral vessels will form proximal to the coarct
Collaterals will develop across intercostal arteries, and engorged arteries will cause ‘notching’ of the ribs on x-ray
What other defect is associated with adult aortic coarctation
Bicuspid aortic valve
