Congenital Heart Disease - Review Day of Test Flashcards
what is the most common birth defect?
- congenital heart disease
disorder with 22q11
- truncus arteriosus
- tetralogy of Fallot
disorder with down syndrome
- ASD
- VSD
- AV septal defect
- endocardial cushion defect
disorder with congenital rubella
- septal defects
- PDA
- pulmonary artery stenosis
defect with Turner syndrome
- coarctation of aorta
defect with Marfan’s syndrome
- aortic insufficiency and dissection
defect with infant of diabetic mother
- transposition of great vessels
normal pressure gradient in the heart
- L pressure > right pressure
transition from fetal circulation
- closing of patent foramen ovale
- closing of patent ductus arteriosus
long standing left to right shunts
- increase pulmonary arterial pressure and volume
- pulmonary arteriolar hypertrophy
- reversal to right to left shunt
eisenmenger’s syndrome
- reversal to right to left shunt
- deoxygenated blood flows to body
- causes early cyanosis
acyanotic presentation
- intracardiac or vascular stenosis
- valvular regurgitations
- left to right shunts
cyanotic presentation
- poorly oxygenated blood shunted from right heart to left
- blood bypass fetal lungs
O2 sat of cyanotic presentation
- 80-85%
acyanotic conditions
- atrial septal defect
- ventricular septal defect
- patient ductus arteriosus
- congenital aortic stenosis
- pulmonic stenosis
- coarctation of aorta
cyanotic conditions
- truncus arteriosus
- transposition of the great vessels
- tricuspid atresia
- tetralogy of fallot
- total anomalous pulmonary venous return
atrial septal defects caused by
- defect in septum between right and left atria
what happens in atrial septal defects
- blood from LA moves to RA
- RV volume load and enlargement
- delayed closing of pulmonic valve
result of delayed closing of pulmonic valve
- fixed split S2 murmor at upper left and right sternal borders
atrial septal defects due to
- ostium secundum (most common)
- osmium primum
- sinus venosus
- patient foramen ovale
ostium secundum defect
- due to inadequate formation of septum secundum or excessive resorption of septum primum
ostium primum defect
- inferior portion of septum fails to fuse with endocardial cushion
sinus venosus defect
- unroofing of area between pulmonary veins and RA
patent foramen ovale
- persistence of foramen ovale
- atrial septa fail to fuse
- source of paradoxical emboli
how would a paradoxical emboli form
- DVT move from RA to LA
- go into body/brain and block arteries
symptoms of ASD
- most usually asymptomatic
adults with ASD
- may have atrial tachyarrhythmia due to right atrial enlargement
percent of membranous ventricular septal defects
- 70%
percent of muscular ventricular septal defects
- 20%
VSD due to
- defect in septum that separates right and left ventricles
initial blood flow in VSD
- result
- left to right shunt
- pulmonary hypertension
- RV hypertrophy
later blood flow in VSD
- pressure RV > LV
- right to left shunt
- Eisenmeyer’s syndrome
murmor in VSD
- harsh holosytolic murmur heart at lower left sternal border
pulmonary venous congestion due to
- left heart failure
pulmonary venous congestion symptoms
- tachypnea
- respiratory distress
- difficulty feeding
- cyanosis
- sweating
systemic venous congestion due to
- right heart failure
systemic venous congestion symptoms
- hepatosplenomegaly
- edema/asites
PDA cause
- failure of ductus arteriosus to close
blood flow in PDA
- left to right shunt between aorta and pulmonary artery ]
- decreased diastolic systemic perfusion
later result of PDA
- pulmonary hypertension reverses shunt
- leads to Eisenmeyer’s syndrome
murmor in PDA
- machine like murmur heard best at lower sternal border
PDA on CXR
- increased heart size
1st line treatment for PDA
- indomethacin
MOA of indomethacin
- prostaglandin synthesis inhibitor
symptoms of Eisenmeyer’s
- cyanosis
- clubbing
- polycythemia
aortic stenosis most common in which gender?
- males
aortic stenosis cause
- narrowing of the aorta
result of aortic stenosis
- requires lots of LV pressure to pump blood
- left ventricular hypertrophy
- high velocity jet may cause dilation of proximal aorta
cause of pulmonic stenosis
- right ventricular outflow obstruction
- abnormal formation/regression of subendocardial mesenchymal tissue
result of pulmonic stenosis
- impaired RV outflow
- increases RV pressure and RV hypertrophy
- may cause RV heart failure
coarctation of aorta due to
- narrowing of aortic lumen
theories of coarctation of aorta
- no flow no grow
- ectopic ductal tissue
no flow no grow theory
- if blood doesn’t flow to an area, that area won’t develop as well
ectopic ductal tissue theory
- ectopic muscular tissue from ductus arteriosus constricts following birth
blood flow in coarctation
- less blood flows to lower limbs because of narrowing of valve
- more blood flows to upper limbs through greater branches of the aorta
result of coarctation of aorta
- BP of upper extremity is high
- BP of lower extremity will be low
cause of persistent truncus arteriosus
- failure of truncus arteriosus to divide into pulmonary trunk and aorta
most patient with truncus arteriosus have
- accompanying VSD
symptoms of truncus arteriosus
- early cyanosis
- patients have partially deoxygenated blood flowing through systemic circuit
cause of transposition of great arteries
- aorta leaves RV
- pulmonary trunk leaves LV
what is the most common cause of neonatal cyanosis
- transposition of the great arteries
cause of TGA
- failure of aorticopulmonary septum to spiral in normal fashion
result of TGA
- 2 parallel circuits
- most infants die without a shunt placed
cause of tricuspid atresia
- absent tricuspid valve
result of tricuspid atresia
- hypoplastic RV
what does tricuspid atresia require for viability
- ASD and VSD
most common form of early cyanotic congenital heart disease
- tetralogy of fallot
things found in tetralogy of Fallot (TOF)
- pulmonic stenosis
- right ventricular hypertrophy
- overriding aorta
- VSD
PROV
pulmonic stenosis in TOF causes
- right to left shunt leading to cyanosis
murmor heard in TOF
- left upper sternal border
CXR of TOF
- boot shaped heart
Tet spell with TOF
- dyspnea with exertion
how do kids reverse TOF Tet spell?
- squat to increase pressure on aorta
- blood moves back to pulmonary artery then lungs
cause of total anomalous pulmonary venous return (TAPVR)
- pulmonary veins drain into right heart circulation instead of left heart
- going the opposite of what you would expect it to.
TAPVR associated with
- ASD +/- PDA to allow for right to left shunt to maintain cardiac output
cure for TOF
- early surgical repair