Congenital Heart Defect Flashcards
diagnostics for VSD
- CXR - Unreliable; may indicate LAE, RV hypertrophy, LV hypertrophy, or pulmonary artery enlargement
- Echo - Determines location and size
- MRI - Use if echo does not diagnose
- Cardiac cath : Used if echo/MRI did not diagnose, but still has pulmonary HTN
- ECG - LV hypertrophy; basically whole heart is enlarged
aycanotic conditions
- ASD
- VSD
- PDA
- PV Stenosis
- Coarctation of the Aorta
- Aortic Stenosis
cyanotic conditions
- Tetralogy of Fallot
- Pulmonary Atresia
- Tricuspid Atresia
- Hypoplastic Left Heart Syndrome Transposition of the Great Arteries
- Total Anomalous Pulmonary Venous Return
acyanotic heart disease pathology and cause?
- Caused by fetal heart malformation, can lead to HF
- ASD, PDA, and VSD
- All three cause L-to-R shunt
- oxygenated blood flows redundantly through pulmonary circulation
- becomes Eisenmenger syndrome over time
s/s of acyanotic heart disease
Sometimes asx, but can lead to heart failure, Eisenmenger syndrome
- Heart failure - poor feeding/failure to thrive, fluid retention, pulmonary congestion, hepatomegaly, rsp distress, elevated jugular venous pressure
- Eisenmenger syndrome - With exertion: cyanosis, palpitations dyspnea, chest pain, syncope
Left-to-Right Shunt Lesions
- atrial septal defect (ASD)
- ventricular septal defect (VSD)
- atrioventricular septal defect (AV canal)
- patent ductus arteriosus (PDA)
- outflow obstruction
- pulmonary stenosis
- aorti stenosis
- coarctation of aorta
A hole in the heart wall dividing left/right atria (left-to-right shunt)
atrial septal defect
- at the site of the foramen ovale and ostium secundum
- MC ASD - accounts for 10-15 % of congenital heart defects
- Associated with fetal alcohol syndrome
which ASD type?
Ostium secundum
- at the level of the TV and MV, “endocardial cushion defect
- found in 25 % of Down’s Syndrome; still fwer cases for for congenital heart defects
Ostium primum
s/s of ASD
- Fixed, split S2 and pulmonic ejection murmur (louder with age)
- Infants and children :Respiratory infections, Failure to thrive
- Adults (before 40): Palpitations, exercise intolerance, dyspnea, fatigue
dx for ASD
- CXR
- R heart dilation
- prominent pulmonary vascularity - TEE
- visualize size and location accurately - Right heart catheterization
ASD - Increased oxygen saturation in which parts of the heart with R heart cath?
R atrium
R ventricle
pulmonary artery
tx ASD
Percutaneous surgical closure
- asx child with a large hemodynamically “significant” defect - elective closure at 1-3 yrs before late complications occur (RV dysfunction and dysrhythmias)
- Or closure of a mod-large defect when the child is 4-6 y/o (defects >8 mm unlikely to close on their own)
- Adults: surgery in cases of RVE, paradoxical embolism, R-to-L shunt
hole in the septum dividing the two lower chambers of the heart (ventricles)
ventricular septal defect (VSD)
VSD - More blood pumped into the lung and pulmonary artery, causing what conditions
- Heart failure
- Pulmonary HTN
- Arrhythmias
- Stroke
2 main VSD types
- Membranous - upper septum (most common)
- Muscular - lower septum
- Inlet - in the posterior portion of the V septum beneath the TV
Most common congenital heart disease, accounts of 25% of CHD
VSD
sizes of VSD
- Small - moderate VSD : 3-6mm; asx and 50% will close spontaneously by age 2yrs.
- Moderate – large VSD, almost always have sx and will require surgical repair.
s/s VSD
- asx
- At birth: Holosystolic murmur (loud, high-pitched) located at LLSB
- Size of defect
- Small: asx, murmur
- mod-large: sweating, poor feeding/ failure to thrive, rsp infections. Murmur + thrill, and diastolic rumble in mitral area
- CHF - dyspnea, persistent cough, pulmonary vascular resistance
- Eisenmenger’s syndrome
tx VSD
- MC close on their own
- Rx for sx pts:
- Anticongestives
- Diuretics (reduce system overload)
- Higher calorie feeds (calories for Heart and to Grow) - surgery
- Repair larger shunts by age 2 (prevent pulm HTN)
- Patch closure over VSD (preferred tx)
- Transcatheter closure: Mesh to close VSD (higher risk)
indications for surgical closure VSD
- Large VSD w/ medically uncontrolled symptomatology & continued FTT.
- Pulmonary HTN
- Aortic insufficiency
- LA/LV dilation
Persistence of the normal fetal vessel that joins the PA to the Aorta.
patent ductus arteriosus (PDA)
PDa nornally closes when?
1st wk of life
PDA is MC in which sex?
female > male (2:1)
s/s small PDA?
What would you hear on ausculation in infants and adults?
Usually axs
Neonates: holosystolic “machine-line” murmur on auscultation Infants, children, adults: continuous murmur
s/s moderate PDA
- Exercise intolerance
- Continuous murmur
- Wide systemic pulse pressure
- Displaced ventricular apex
s/s larger PDA
Infants: leads to heart failure
Children: SOB, fatigability, Eisenmenger syndrome
imaging/diagnostics for PDA
Echo - 2D suprasternal echo
CXR - Normal/cardiomegaly
Other - ECG : LV hypertrophy, LAE
tx for small ax PDA
monitor
tx for neonates with PDA
Close PDA using prostaglandin inhibitor
tx for Symptomatic moderate/large PDA
- HF: Digoxin, furosemide
- Surgery
indications for surgery in symptomatic moderate/large PDA
symptoms of L-to-R shunting, L-sided volume overload, reversible pulmonary arterial HTN
3 scenarios for pulmonary stenosis
- Stenosis of the valve itself (MC)
- Thickened muscle below valve
- Stenosis of the pulmonary artery below valve
pathophys of pulmonary stenosis
Obstruction of blood flow across the pulmonary valve = increased work by R ventricle = thicken over time = pressure overload into R ventricle = RVH
pulm stenosis - When obstruction is severe, increased pressure can cause a ____ shunt
What condition to occur at the atrial level through a PFO?
R-to-L
Eisenmenger Syndrome
In neonates with critical pulmonary stenosis, the only way to get blood into the lungs is through a PDA. So, ____ is given at the time of birth to keep the DA open
Prostaglandin
s/s of pulmonary stenosis
- Usually asymptomatic with normal health if mild to moderate PS. Pulses are normal. May show sx later in adolescence or adulthood
- Systolic ejection murmur at the LUSB which increases with inspiration
- S₂ followed by opening click that becomes louder with expiration
- RV lift on palpation of the precordium
diagnostics for PS
CXR - nml heart size; post stenotic dilation of main pulm artery and L pulm artery
EKG - nml with mild obstruction, RVH with mod-severe PS
ECHO - confirms
cardiac cath - reserved for therapeutic balloon valvuloplasty
tx for pulmonary stenosis
- Mild - Moderate - no intervention
- Moderate-Severe - Percutaneous balloon valvuloplasty; Surgery needed when balloon valvuloplasty is unsuccessful
what is coarctation of the aorta
Part of the aortic arch (usually the proximal descending aorta) is narrower than usual causing blockage of normal blood flow to the body, backing blood flow into the left ventricle.
This causes the muscles to work harder to get the blood out of the heart
coarctation of the aorta is MC in which sex?
males > females
females with coarctation of the aorta are associated with what other condition?
Turner syndrome
leading cause of HF in the first month of life
coaractation of aorta - alone or in combo
pathophys of coarctation of the aorta
Narrowed segment of aorta
- Upstream issues
- Blood flow increases into aortic branches before coarctation—> blood flow, pressure increases in UE, head - Downstream issues
- Decreased blood flow, decreased pressure in LE
- Kidneys receive less blood —> activate RAAS —> secondary HTN
types of coarctation of the aorta
- Preductal coarctation
- Associated with Turner syndrome, PDA
- May go unnoticed unless severe. Presents as postductal coarctation - Postductal coarctation
- Distal to ligamentum arteriosum
- Presents in adulthood
- BP higher upstream, lower downstream
s/s coarctation
Depends on presence/severity of PDA
- Murmur
- Systole: diamond-shaped murmur
- Diastole: high-pitched decrescendo murmur - Infants
- Lower extremity cyanosis
- Absent/delayed femoral pulse
- Failure to thrive/poor feeding
- BP higher in UE compared to LE - Secondary HTN
- Severe HF, shock if/when PDA closes
- Other sx more apparent with age: CP, cold extremities, claudication on exertion; LV impulse palpable, sustained; Pulsations felt in intercostal spaces
s/s adult coarctation
HTN (most common)
Hypotension in lower extremities
Bilateral lower extremity claudication
Dyspnea on exertion
Delayed/weak femoral pulses
diagnostics for coarctation
- Angiogram - Visualize narrowing in aorta, anatomy & severity
- CXR - Rib notching: 3-sign (narrowed aorta resembles notch of number 3 d/t prestenotic of aortic arch & postenotic of descending aorta dilatation)
- Echo - Visualize location, size, blood turbulence
- Others: ECG - LVH, LAE
tx coarctation
- prostaglandin E - increases flow to LE
- Surgery: Resection with end-to-end anastomosis
- If unfeasible, bypass graft across area of coarctation
- Long-segment coarctation: subclavian aortoplasty
- Prosthetic patch aortoplasty (rarely)
- Balloon angioplasty with possible stent
prognosis for coarctation?
What is mandatory for kids before participating in sports?
- Survival through neonatal period w/o developing HF - good
- Infective endocarditis - rare before adolescence, but can occur in both repaired and unrepaired
- correction after age 5 yrs - increased risk for HTN and myocardial dysfunction
- Exercise testing is mandatory for these children prior to their participation in athletic activities
3 types of AS
- valvular - 75%
- subvalvular - 23%
- supravelvular - 2%
s/s of AS
- Harsh, systolic ejection murmur at the upper right sternal border with radiation to the neck
- Systolic ejection click at the apex
- Dilation of the ascending aorta on CXR
dx AS?
What if resting gradient is 60-80mmhg?
- ECHO is the standard for diagnosis and for following the severity
- Cath for resting gradient has reached 60-80 mmHg and in whom intervention is planned
tx for AS
- Percutaneous balloon valvuloplasty: Standard initial tx with valvular AS
- subvalvular or supravalvular AS - interventional cath is not effective; surgery is required
- Consider surgery in sx pts with a high resting gradient
an alternative to mechanical valve placement in infants and children for AS
Ross procedure
prognosis AS (activity levels for kids)
- Mild - moderate obstruction - normal O₂ consumption and maximum exercise capacity. Children with nml resting and exercise (stress) EKGs: can participate in vigorous physical activity, including non-isometric competitive sports
- Severe obstruction - predisposed to ventricular dysrhythmias and refrain from vigorous activity; avoid all isometric exercise
criteria for cyanosis lab-wise/VS
Presence > 3 g/dl deoxy HgB correlates with 80-85% SpO2.
Cyanosis can be misdiagnosed or mistaken as
CCHD if there is acrocyanosis, pulmonary causes, CNS causes
- Great tool in absence of ECHO for suspected CHD
- Differentiates cardiac and non cardiac causes of cyanosis
hyperoxia test
what med can be started based on results of hyperoxia test?
prostin
hyperoxia test findings and what the values mean
- < 100 - intracardiac shunting; most likely ductal dependant lesion so initiate prostin
- 100-250 – intracardiac mixing lesions
- > 250 - no structural cyanotic heart hisease
Infant must be how old for CCH screening
> 24 hours of age and more accurate if older
5 T’S of cyanotic heart disease
- Truncus Arteriosus
- TGA (d-Transposition of the great arteries)
- Tricupsid Atresia
- Tetralogy of Fallot
- Total anomalous pulmonary venous return
- Pulmonary Atresia
- Ebstein’s anomaly
what is the tetralogy of fallot
- Pulmonary stenosis
- Large VSD
- Overriding Aorta
- Right Ventricular Hypertrophy
MCC of cyanosis in infancy/childhood
tetralogy of fallot
tetralogy of fallot - Severity of cyanosis proprortional to ?
severity of RVOT
on CXR the heart shows RVH, almost shoe/boot -shaped, what is the dx
tetralogy of fallot
what is tet spell?
children with tetralogy of fallot exhibiting bluish skin during episodes of crying or feeding
- Hyper cyanotic episode
- Decreased SVR causing increased R->L shunt, increasing cyanosis
- Decreased SVR (hot bath, fever, exercise)
- Agitation – worsens dynamic sub PS obstruction
- Life threatening if untreated
management for serious “tet spells”
- keep baby calm
- give O2
-
fluids, med to improve pulm blood flow
- Morphine IV - decrease agitation, decrease dynamic RVOT obstruction
- Bicarbobate - corrects met acidosis; decrease PVR
- Phenylephrine - increases SVR
- BB - decreases dynamic RVOT obstruction - surgery during neonatal/infant period regardless of pt size; before age 2
- palliative tx when complete correction is risky - Balloon dilation, Stent placement
The two main arteries carrying blood out of the heart - pulmonary artery and aorta, are switched in position
transposition of the great arteries
- Aorta comes off RV
- PA comes off LV
- Considered a critical heart defect
different types of transposition of the great arteries
- D-TGA: dextro-TGA/complete TGA (dextro = aorta on right)
- L-TGA: levo-TGA/congenitally corrected TGA (Levo = aorta on left)
what other congenital heart defects can help infants survive TGA
- VSD
- PDA
Second most common cyanotic congenital heart defect
TGA
TGA is MC in which sex
Male > female (3:1)
pathophys of TGA
- Survival is impossible unless there is a way to mix the pulmonary and systemic circuits (now running in parallel)
- The majority of mixing occurs at the atrial level so an interatrial communication (PFO or ASD) is critically important. Without this, patient will be severely cyanotic at birth
- Left unrepaired, transposition is associated with a high incidence of early pulmonary vascular obstructive disease
RF for TGA
- Diabetes
- Rubella
- Poor nutrition
- Vonsumption of alcohol
- > 40 years old
s/s TGA
- In utero: asymptomatic
- d-TGA: Cyanosis, unchanged with supplemental oxygen (less severe if VSD present), Tachypnea, Acidosis
- L-TGA: asx
diagnostics for TGA
- Echo - Evaluate heart function, structure
- CXR - Classic triad
- Heart appears as egg on its side/”egg on a string” appearance
- Lung congestion
- Cardiomegaly - Angiogram - Pre-surgery
tx TGA
- Prostaglandin E: short-term solution. Keeps ductus arteriosus open
- Surgery
- Balloon atrial septostomy: short-term solution. Hole created in atrial septum
- Surgery - switch great vessels
Underdeveloped left ventricle, ascending aorta
hypoplastic Left heart syndrome
hypoplastic left heart syndrome
Without ___ or ___: heart not capable of sustaining life outside womb
ASD, PDA
With ASD, PDA: right heart function present but impaired; sometimes asymptomatic at birth
pathophys of hypoplastic left heart syndrome
- Right heart functions normally
- oxygenated blood enters left atrium
- flow backs up due to small mitral valve, small left ventricle
- high pressure in LA, blood circulated ineffectively by left ventricle
s/s hypoplastic left heart syndrome
- cyanosis
- rsp distress
- poor feeding/failure to thrive
- LHF
- cardiogenic shock
- death if not tx
diagnostics for hypoplastic left heart syndrome
- prenatal US
- cardiomegaly
- other: ECG - RVH after birth
tx for hypoplastic left heart syndrome
- Prostaglandin E: short-term solution. Keeps ductus arteriosus open
-
3-step surgery
- 1st - Norwood - 1-2 wk old
(“new aorta” to RV & then “tube” to PA)
- 2nd - Bidirectional Glenn - 4-6 mo (PA & SVC connection; upper body blood to lungs)
- 3rd - Fontan - ~2 y (PA & IVC; lower body blood to lungs)
Children who undergo surgical repair for hypoplastic left heart syndrome can participate in recreational activities but are restricted from ?
competitive and vigorous athletics
6 common innocent murmurs of childhood
- newborn murmur
- Peripheral pulmonary artery stenosis (PPS)
- still’s murmur
- pulmonary ejection murmur
- venous hum
- neck/supraclavicular “carotid bruit”
First few days of life
LLSB without radiation
Soft SEM
Resolves by 1 mo
what type of murmur
newborn murmur
- Caused by nml branching of the PA
- ULSB, back, axillae
- Soft SEM
- Disappears by age 2
- ECHO needed to differentiate from harmful murmurs
what type of murmur
Peripheral pulmonary artery stenosis (PPS)
- MC innocent murmur of childhood
- 2 - 7 y/o
- Apex and LLSB
- Vibratory, soft systolic
- Loud when supine; disappears with inspiration or sitting
- Loud with anemia and fever
what type of murmur
still’s murmur
- After age 2
- Right infraclavicular area
- Continuous, musical, Grade I-III
what type of murmur
venous hum
- supraclavicular area 30-40% of children
- Aortic stenosis below clavicle
- Brief; physiologic
- SEM, harsh, Grade II-III
what type of murmur
Neck / supraclavicular “carotid bruit”
