Cardiology 47, 48 Flashcards
When to suspect CHD? congenital heart defect
- Positive family history
- chromosome abnormalities
- heart murmur
- failure to thrive
- abnormal skin color
- tachypnea/dyspnea
- easily exhausted
- pronounces sweating during feeding
- active precordial pulsations
- abnormal peripheral pulses
Prenatal diagnosis of congenital heart defect
- General screening by obstetrician in every trimester
- Fetal echocardiography by pediatric cardiologist
(if suspicious finding,
developmental abnormality,
multiple pregnancies,
family history of CHD,
>40 maternal age, maternal IDDM)
Should be done week 18-22 so that termination of pregnancy is still an option
when should fetal ECHO be performed and why
Should be done week 18-22 so that termination of pregnancy is still an option
newborn diagnosis in congenital heart defect
Pulse oximetry screening
Classification of CHD:
Cyanotic heart defects
Non-cyanotic heart defect
Cyanotic congenital heart defects
types
- Right-to-left shunt:
*Tetralogy of Fallot - Separate circulations:
*Transposition of the great arteries - Complete mixing:
*Tricuspid atresia
*Truncus arteriosus
*Hypoplastic left heart syndrome
*TAPVR
right to left shunt cyanotic heart defect
Tetralogy of Fallot
Complete mixing type of cyanotic heart defect
Tricuspid atresia
Truncus arteriosus
Hypoplastic left heart syndrome
TAPVR
Tetralogy of fallot characterized by
1) pulmonary stenosis
2) VSD
3) misplaced aorta
4) right ventricular hypertrophy
what disease have seperate circulations in cyanotic heart defect
transposition of great vessel
Tetralogy of fallot results in which type of shunt
Right to left shunt
Tetralogy of fallot what determine extent of shunting
Degree of stenosis determines extent of shunting + cyanosis
cyanosis
bluish color of the skin and mucus membranes, arterial hypoxemia (arterial sat of < 95%)
Perioral (around mouth) cyanosis or acrocyanosis (hands/feet) —> NOT a sign of cyanotic heart defect, but a common physiological sign of cold/decreased circulation
clinical features of tetralogy of fallot
- Boot-shaped heart on x-ray
- Patients learn to squat in response to cyanosis —> increase systemic resistance —> more blood flow through stenotic pulmonary arteries to lungs
tetralogy of fallot treatment
surgery
Transposition of the great vessels characterized by
Characterized by
pulmonary a. arising from LV
+ aorta arising from RV
Associated with maternal diabetes
Clinical features of transposition of the great vessels
Early cyanosis
—> pulmonary + systemic circuits do not mix
treatment of transposition of the great vessel
Creation of shunt (ASD) —> required for survival
PGE administered to maintain PDA until surgery
cyanotic heart defects
- Tetralogy of fallot
- Transposition of the great vessels
- Tricuspid atresia
- Truncus arteriosus
- Hypoplastic left heart syndrome
- Total anomalous pulmonary venous return (TAPVR)
Non-cyanotic congenital heart defects
Left-to-right- shunt: ASD, VSD, AVSD, PDA
Obstructive lesions: AS, PS, CoA
Tricuspid atresia
- Absent or rudimentary tricuspid valve resulting in no blood flow between RA and RV
- Patient survival is only possible if there are interatrial or interventricular communications (ASD, VSD)
- Accompanied by RV hypoplasia and RA dilation due to volume overload
clinical features of tricuspid atresia
central cyanosis
holosystolic murmur at lower left sternal border
diminished peripheral pulses
treatment of tricuspid atresia
surgery
Truncus arteriosus
- Underdevelopment of aorticopulmonary septum
—> failure of truncus arteriosus to divide into the aorta and pulmonary trunk
—> instead a single trunk that receives output from both ventricles
*Truncus arteriosus:
single arterial trunk that originates from both ventricles of the embryonic heart and give rise to ascending aorta and pulmonary trunk
*Truncus arteriosus:
single arterial trunk that originates from both ventricles of the embryonic heart and give rise to ascending aorta and pulmonary trunk
truncus arteriosus is associated with what disease
DiGeorge syndrome
Truncus arteriosus
effect of oxygenated blood
Results in the mixture of oxygenated and deoxygenated blood
clinical features of truncus arteriosus
cyanosis
respiratory distress
failure to thrive,
harsh systolic murmur
bounding peripheral pulses
truncus arteriosus treatment
surgery
Hypoplastic left heart syndrome
Spectrum of disease consisting of severe hypoplasia of left ventricle with possible
and/or atresia of the mitral valve, aortic valve, or aortic arch
Hypoplastic left heart syndrome associated with which diseases
Associated with
Patau syndrome,
Edward’s syndrome,
Turner syndrome
prognosis and treatment of hypoplastic left heart syndrome
bad prognosis
treatment surgery
Total anomalous pulmonary venous return (TAPVR)
All four pulmonary veins drain into systemic venous circulation instead of left ventricle
—> oxygenated blood returns back to right atrium —> pulmonary edema
clinical features of Total anomalous pulmonary venous return (TAPVR)
cyanosis,
respiratory failure,
failure to thrive,
hepatomegaly
Total anomalous pulmonary venous return (TAPVR) treatment
surgery
Non-cyanotic congenital heart defects
Types* :
Left-to-right- shunt: ASD, VSD, AVSD, PDA
Obstructive lesions: AS, PS, CoA
Atrial septal defect (ASD)
Defect in septum btw atria
ASD associated with
Down syndrome, fetal alcohol syndrome
ASD results in what shunt
left to right shunt
auscultation of ASD
split S2 (increased blood in right heart —> delays closure of pulmonary valve
—> 2 sounds
Paradoxical emboli:
emboli produced in systemic circulation normally go to lungs but can now travel to left heart —> brain)
associated with ASD
Most common congenital defect of heart
Ventricular septal defect (VSD)
VSD associated with
Down syndrome,
intrauterine infections (TORCH),
maternal diabetes
Ventricular septal defect (VSD) what shunt
Results in left-to-right shunt
—> dilation of LV since increased preload (blood goes through shunt instead of the periphery —> RV —> lungs —> back to LV)
Size of shunt determines
extent of shunting
+ age of presentation
Small VSD defect
—> asymptomatic
Large VSD defect —>
can lead to Eisenmenger syndrome (reversal of shunt)
treatment of VSD
loop diuretics
ACEi,
surgery
Atrioventricular septal defect (AVSD) associated with
down syndrome
AVSD shunt type
Defect in septum btw atria and ventricles of right and left heart
Results in left-to-right shunt —> dilation of heart —> HF + pulmonary HTN
treatment: surgery
Patent ductus arteriosus (PDA)
ure of ductus arteriosus (connects aorta + pulmonary a.) to close
Patent ductus arteriosus (PDA) associated with
Associated with congenital rubella
PDA shunt type
Results in left-to-right shunt *
- In utero DA shunts blood in other direction (right-to-left) to bypass lungs
- Systemic circulation increases after birth
- If DA stays open —> blood shunt the other direction (left-to-right)
If DA stays open —>
blood shunt the other direction (left-to-right)
in utero Ductus arteriosus function
In utero DA shunts blood in other direction (right-to-left) to bypass lungs
PDA has hemodynamically similar effects as
vsd
complication of PDA
Eisenmenger syndrome with increasing pulmonary pressure
treatment of PDA
indomethacin —> decrease PGE —> close PDA (kept open in utero by placental PGE)
Coarctation of aorta (CoA)
Narrowing of aorta
* infantile form
* adult form
Coarctation of aorta (CoA)- infantile form
Associated with PDA
* Coarctation lies distal to aortic arch but before PDA
- Presents as lower extremity cyanosis
Adult form CoA
- Coarctation lies distal to aortic arch ‣
- Presents as hypertension in upper extremities + hypotension in lower extremities
- Not associated with PDA
which form of CoA is associated with PDA
infantile form
Coarctation lies distal to aortic arch but before PDA
Presents as lower extremity cyanosis
coarctation position in infantile vs adult form of CoA
Coarctation lies distal to aortic arch but before PDA –> lower extremity cyanosis
Coarctation lies distal to aortic arch ‣
Presents as hypertension in upper extremities + hypotension in lower extremities
what is used for diagnosis of CoA
4 limb pulsoximetry:
crucial to check both upper (brachial) and lower (femoral) extremity pulses!
—> if decreased in legs: possible coarctation that need to be double checked with saturation and blood pressure
