Cardiac Defects Flashcards
Most common defect in Down’s
AV septal defect
Most common defect in DiGeorge
- Tetralogy of Fallot
2. Truncus arteriosus
DiGeorge Symptoms
- Facial phenotype
- Developmental delay
- Parathyroid hypoplasia - hypocalcemia
- Thyroid hypoplasia - immune deficiency
Tetralogy of Fallot Types
Pink TOF - less severe RVOT obstruction = more BF to lungs
Cyanotic TOF - more severe RVOT obstruction
Signs and symptoms of Tetralogy of Fallot
- LUSB systolic murmur - PS
- +/- cyanosis
- +/- tachypnea = increased BF = increased tachypnea
Treatment of Tetralogy of Fallot
- PGE2 = to maintain PDA and lower BF to lungs
- Surgery = at 3-6 months of age
Ventriculotomy, transannular patch repair, and RV to pulmonary artery circuit
Tet spells; who they happen in and how to treat
Tetralogy of fallot
Tx:
First line = calm down, O2, and knee to chest
IV line = morphine, saline, ketamine, phenyleprine
Beta-blocker
Types of AV septal defects
Partial/Transitional = like ASDs
Complete/Intermediate = like ASD + VSD
Most common defect w/ a diabetic mother
Truncus arteriosus
Blood flow in truncus arteriosus; how does it change over time
As pulmonary vascular resistance decreases after birth = more BF to lungs = heart failure and pulmonary overcirculation/HTN
Types of Truncus arteriosus and mortality
Moratilty - 5 weeks of age w/out intervention
Depend on pulmonary
BF:
Increased BF Decreased BF (rare)
Symptoms of increased pulmonary BF in truncus arteriosus
Mild cyanosis Increased pulse pressure Hyperdynamic precordium Normal S1 and loud single S2 Pansystolic murmur at LLSB, systolic murmur at USB Diastolic murm
Tachypnea, hepatomegaly, poor feeding, diaphoresis
Sympotms of decreased pulmonary BF w/ out pulmonary regurgitation
Due to PA stenosis or pulmonary vascular disease
Moderate to severe cyanosis
Normal pulses and pulse pressure
Systolic murmur - PA stenosis
Truncus arteriosus common valve abnormalitites
3 cusps = most common
Can be stenotic or insufficient
Truncus arteriosus surgery
Repair VSD so LV pumps to aorta
RV to PA conduit
Aortic Valve precursor
Subendocardial mesenchyme
Effects of aortic valve stenosis
Aortic root dilation
Leaflet destruction
Ventricular hypertrophy
Aortic stenosis treatment
Maintain PDA
Valvuloplasty
Surgical commissurotomy
Effect of aortic stenosis on fetal circulation
Lower blood O2 content to the brain and coronary
Effect of coarctation on fetal circulation
Not much effect; babies without an aortic isthmus still get good perfusion to all tissues
Born healthy and of normal birth weight
How does coartication develop?
Hemodynamic theory - disturbances cause reduced blood flow and volume in fetal aortic arch
Ductal tissue theory - migration of ductal arteriosus smooth muscle into the aorta = causes narrowing and constrictuion
Genetic causes - Turner’s syndrome
Most common Turner’s syndrome anomalies
- Bicuspid valve
2. aortic coartication
Aortic coartication presentation
- Infant w/ CHF - complex coarticatoin
- Child/adolescent w/ systolic hypertension
- murmur; continous chest murmur due to collaterals, mitral stenosis/VSD murmurs
CXR signs of aortic coartication
Aortic knob
Aortic indentation at LSB w/ opposing esophageal indentation (barium swallow)
Aortic coartication therapy
- End to End anastomosis; no circumferential suture = less recurrence
- Prosthetic patch aortoplasty; high incidence of late aneurysms
- Subclavian flap aortoplasty; sacrifices left subclavian artery (has growth potential)
- Angioplasty/stenting
Treatment of choice for infants w/ aortic coartication
Subclavian flap aortoplasty
Persistent PDA causes what?
Heart failure and poor growth in infants and children
Hypoplastic Left Heart Syndrome; what shunts must remain for fetus to be viable
ASD - L to R
PDA - R to L
Hypoplastic Right Heart Syndrome; what shunts must remain for fetus to be viable
ASD - R to L
PDA - L to R
When is a PDA needed?
Systemic blood flow when there is severe aortic flow obstruction (R to L)
Pulmonary BF when there is severe obstruction to pulmonary flow (L to R)
What is transposition?
Ventricular-Arterial discordance
Gender and transposition
M>F
Transposition is seen commonly in what infants
Infants of diabetic mothers
Embryological cause of transposition
Abnormal spiraling/conotruncal rotation during septation with aorticopulmonary septum
How to create intercirculatory mixing in transposition?
Atrial level - ASD
Ventricular level - VSD (common in 40-45%)
Arterial level - PDA (use PGE1)
Transposition; associated lesions
50% - TGA w/ PFO or PDA
50% - TGA w/ other anomalies = VSD (40-45%), LVOT (5%), coartication
Transposition w/ VSD treatment
Does not require PGE1
The effect of transposition on coronary circulation
Provides deoxygenated blood to heart; many variants in coronary artery branches - ie circumflex from RCA, single RCA or LCA, etc
Clinical features of transposition
Early cyanosis
Loud S2 (due to anterior location of aorta)
Hyperoxia - excess blood oxygen; due to separation of circulation
CXR appearance of transposition
“Egg on a string” - egg shaped heart w/ a narrow mediastinum
Cardiomegaly
Management of transposition
Intercirculatory mixing
- Create ASD w/ balloon atrial septostomy
- PGE1
Oxygen
Volume - IV fluids
Correct metabolic abnormalities
What is the Senning operation?
Surgical correction of transposition- Pulmonary veins into RA, atrial baffle (shunt) for SVC/IVC to LV
Complications of Senning
RV dysfunction, arrhythmias
What is the Jatene arterial switch?
Transposition surgery
Risks of Jatene switch
Coronary issues
Aortic root dilation
Aortic insufficiency
PA stenosis
VSD - locations
Outlet/subarterial (type 1) - near LV outlet
Perimembranous (type 2) - most common
Inlet (type 3)- near RV inlet
Muscular (type 4)
VSD complications
CHF due to large volume - L to R shunting
Pulmonary HTN leading to shunt reversal - Eisenmenger’s syndrome
LV dysfunction due to chronic volume overload
Bacterial endocarditis
LATE:
Aortic regurgitation
Arrhythmias
Double chamber RV
What determines VSD magnitude
Size of VSD
Pulmonary vascular resistance
VSD survival
Depends on VSD size and location and development of pulmonary HTN
