Congenital Heart Disease Flashcards
Most common congenital heart lesion in adults
atrial septal defect
shunt in atrial septal defect
left atrium to right atrium shunt
types of atrial septal defect
sinus venosus, ostium secundum, and ostium primum
what additional problems are caused by sinus venosus ASD besides the hole between the atria?
R-sided pulmonary veins drain back to the R atrium instead of the left atrium- so all the oxygenated blood coming to RA instead
Patient presents with pulmonary HTN, R ventricular failure, atrial arrhythmias. Echo shows dilated right atrium and ventricle. Increased RV impulse along LSB. You hear BOTH systolic (along LSB) and mid-diastolic ejection murmur (along apex). You also hear splitting of S2 (pulmonary valve closing a little later due to increased volume). Diagnosis?
Atrial septal defect
Problem with coronary sinus ASD
drains into LA instead of RA
What is patent foramen ovale associated with?
Cryptogenic stoke and migraine headaches
Function of foramen ovale
Hole between R and L atria that shunts blood from RA to LA to bypass lungs
shunting of blood in PFO
Right to left atrium (opposite of other atrial septal defects)
Why is PFO associated with strokes?
Because blood bypasses filtering system of lungs from RA directly into LA. Clots can get into LA from RA and into brain causing stroke
What would echocardiography show in ASD?
Dilated RV, septal defect, and saline injection can be administered
Surgical and percutaneous treatment options in ASD
Surgical- direct suture repair or patch repair. Percutaneous- catheter-implanted occlusion device- passage of catheter across atrial septal defect through femoral vein, deployed like umbrella
In which location does catheter treatment work best in ASD?
Centrally located secundum defects
Examples of percutaneous treatments for ASD
Catheter-implanted occlusion device, gore-helix, and amplatzer
shunting in VSD
left to right (pressure higher in left)
Patient presents with RV dilation and hypertrophy, LV hypertrophy, and pulmonary HTN. Displaced PMI to left. Harsh, holosystolic murmur, aortic regurgitation. Chest X ray also shows cardiac enlargement. Diagnosis?
VSD
atrioventricular defect aka
endocardial cusion defect
shunting of blood in endocardial cushion defect
shunting can occur either way
Atrioventricular defect associated with
Downs syndrome
What cardiac events is downs syndrome associated with?
Most common- atrioventricular septal defect. others include ASD, VSD, tetrology of fallot, hypoplastic left heart syndrome, and isolated elevated pulmonary artery pressure
ductus arteriosus function
in fetus, blood from pulmonary artery shunts to aorta to bypass lungs
patent ductus arteriosus shunting
shunting occurs in opposite direction of fetus- oxgenated blood goes from aorta to pulmonary artery (goes back to lungs- not as much blood getting perfused to body tissues)
Neonate presents with shortness of breath, pulmonary hypertension, volume overloaded LV. CONTINUOUS murmur (machinery-like), and bounding pulses. Diagnosis and tx?
Patent ductus arteriosus. Tx- catheter-based closure or surgical closure
Most congenital defects produce left to right shunting due to increased pressure on left side. What congenital defects cause right to left shunting?
Patent foramen ovale (ASD), and Eisenmenger syndrome, tetrology of fallot
“Blue baby” in NICU. Blueing all over body, tachypnea, grunting, flaring, weak peripheral pulses. ECG shows RAD. Large R’s in V1 and V2, and large S minimal R in V5 and V6 suggesting RVH. Normal S1, but only single S2 (do not hear closing of aortic valve). Murmum in diastole heard due to tricuspid regurgitation. Diagnosis?
Hypoplastic left ventricular syndrome
Describe shunting and defects in hypoplastic left ventricle syndrome
Atrial septal defect with left to right flow, patent ductus arteriosus with right to left shunt in systole and diastolic flow reversal
What shunts/defects left open to help in HLHS?
keep ductus arteriosus open and patent foramen ovale (so blood can flow from right to left- too much on right)
What is the main problem with transposition of the great vessels?
The RV cannot keep up with pumping through aorta to entire body
2 types of trasnposition of the great vessels
dextro and levo
dextro vs. levo
dextro- atrio-ventricular concodonance and ventriculo-arterial discordance. levo- atrio-ventricular discordonance and ventriculo-arterial discordance
repair for d-TGA
atrial inversion, ventricular inversion, or arterial switch
Which TGA is cyanotic and is a parallel circle in which there is no connection between the R and L sides of the heart?
dextro- TGA
What is coarctation of the aorta most often associated with?
bicuspid aortic valve (stenosis), also HTN in younger kids
Patient presents with diminished lower limb pulses. But radial, brachial, carotid pulses are normal. patient also has systolic murmur due to bicuspid aortic stenosis. What do you suspect?
coarctation of the aorta
Which type of repair is preferred in patients with dextro-transposition of the great vessels who have severe pulmonic stenosis?
Ventricular inversion
Child appears especially blue around nail beds and lips during episodes of crying and feeding. During auscultation, you only hear single S2. Hear systolic thrill at LSB, systolic ejection murmur. You notice dretarded growth an delayed development. Xray shows “boot-shaped heart.” ECG shows right atrial enlargement, RV hypertrophy, ventricular septal defect, overriding aorta, Right axis deviation, and RV conduction abnormlality. Diagnosis?
Tetrology of fallot
Classic “boot shaped heart” associated with…
Tetrology of fallot
What is the most common type of abnormal aortic valve?
Bicuspid aortic valve
Average age that aortic valve stenosis calcification of valves occurs/
elderly- 45 to 65 years old
4 abnormalities associated with tetrology of fallot
VSD, displacement of aorta over VSD, pulmonary artery narrowing, and RVH
Shunting of blood in tetrology of fallot
RV to LV
Shunting of blood in “pink” tetrology of fallot
LV to RV
What is Ebstein’s anomaly associated with
Septal defect of tricuspid valve is displaced into RV, so tricuspid regurgitation may occur, and also many patients also have coexistent ASD, Wolff-parkinson white syndrome
What kind of shunt would cause more symptoms of cyanosis?
Right to left because mixing of deoxy with oxy blood going to body. Left to right shunt causes less blood to get to body, but it is still oxygenated blood
What electrical abnormalities are associated with ebstein’s anomaly?
WPW syndrome, Right atrial enlargement, and right ventricular bundle branch block
Therapy for ebstein’s anomaly
Tricuspid valve replacement and ASD closure
Patient presents with cyanosis. PE shows wide splitting of S1 and S2, and also S3 and S4. Hear holosystolic murmur. Large v wave in neck veins, pulsatile liver, palpitations, peripheral edema. ECG shows Right atrial enlargement and WPW syndrome. Echo shows atrial septal defect. Diagnosis?
ebstein’s anomaly associated with ASD and TR
Cardio defects in marfan’s syndrom
mitral valve prolapse and aortic aneurysm that may dissect (dilation of aorta due to weaknening of blood vessel walls)
