Congenital Heart Disease Flashcards
What is the most common congential heart defect?
VSD
(also believed 1/2 of these repair themselves and never come to medical attention)
What is an atrial septal defect?
persistent opening in the interatrial septum after birth that allows for direct communication between the l. and r. atria
What is the prevalence of ASD?
1:1500 live births
10% of all congenital heart disease
What is the consequence of an ASD?
blood ordinary shunted from left–>right
volume overload in right atrium
What is Einsemenger Syndrome?
When a shunt that was formerly left-to-right becomes right-to-left
What is the common presentation for ASD?
- May be asymptomatic
- DOE
- Fatigue
- Recurrent lower respiratory tract infections
What are the exam/test findings with ASD?
RV heave along LSB
S2 wide, fixed splitting
murmur is mid-systolic left USB
cardiac cath - measures higher O2 in right atria
echo - shows shunt on doppler
Rx for ASD?
Surgical-
- direct suture closure
- pericardial or synth patch
- percutaneous apporahc with septal occluder device
How prevalent is VSD?
1.5-3.5:1000 live births
How do you diagnose VSD?
echocardiography
What are the symptoms and signs of VSD?
- depends upon the size of the defect ranges from no symp. to heart failure
- harsh, holosystolic murmur along LSB
- systolic thrill
- if reversed shunt, cyanosis and dyspnea
What is the Rx for VSD?
- 1/2 close spontaneously by age 2
- closure indicated with s/sx of CHF or pulmonary vascular disease
- same as ASD methods for closure
What is the tetraology of Fallot?
4 Defects:
- VSD
- pulmonic stenosis
- overridng aorta (communicates with right ventricle through VSD)
- RVH
How common is tetralogy of Fallot?
Pretty rare: 5:10k live births
but most common cyanotic heart disease in childhood
What are the s/sx of tetralogy of Fallot?
- “Tet” Spells - hyperventilation, cyanosis, syncope and squatting after exertion, feeding, crying
- dyspnea on exertion
- mild cyanosis and clubbing
- RV heave
- systolic ejection murmur at left USB
What is the Rx for tetralogy of Fallot?
surgery-close VSD and increase pulmonary artery width
*if necessary, temporarily create connection between aorta and pulmonary artery to reduce hypertension
What is transposition of the great vessels?
aorta arises from the RV
pulmonary artery arises from the LV
so, aorta pumps deoxygenated blood and the pulmonary artery carries oxygenated blood to the heart
What is the prevelence of transposition of the great vessels?
40:100k births (7% congenital)
most common neonatal cyanosis
What are the s/sx of transposition of the great vessels?
blue baby - extremely hypoxic and cyanotic
How do you diagnose transposition of the great vessels?
echocardiogram
What is the Rx for transposition of the great vessels?
arterial switch surgery is definitive
until that can occur, use prostaglandins to keep ductus arteriosis open (only way to get some oxygenated blood circulating)
Who still gets pre-dental/surgical antibiotic prophylaxis with congenital heart disease?
unrepaired cyanotic heart disease
post-repair for six months
post-repair with residual defects
What is coarctation of the aorta?
narrowing of the aortic lumen
(can be postductal -98% or preductal - 2% for the DA)
how common is coarctation of the aorta?
1:6k live births
What are the s/sx of coarctation of the aorta?
- most asymptomatic, but severe will be evident in a newborn
- preductal has cyanosis in LE
- femoral pulses weak and delayed
- midsystolic murmur
- elevated UE BP
How do you diagnose coarctation of the aorta?
- x-ray may show indented aorta and notches on inferior part of the ribs
- echo confirms
What is the treatment for coarctation?
severe - prostaglandin to keep DA open
definitive - excision and reanastamosis (balloon +/- stenting)
What is patent ductus arteriosus (PDA)
when embryological anastamosis between aorta and pulmonary artery fails to close after birth
What is the prevalence of PDA?
1:2500-5000 live births
What are the s/sx of PDA?
small are asymptomatic
moderate - fatigue, dyspnea, palpitation
large - left-to-right shunt HF
Afib can develop if left atria dilates
“machine” like murmur
How do you diagnose PDA?
echo
What’s the treatment for PDA?
- prostaglandin synthesis inhibitor - indomethacin
- NSAIDs
- surgical ligation or division
- transcather coiling
What is congenital aortic stenosis?
Abnormal development of the aortic valve with resulting obstruction of flow. Usually bicuspid leaflet or unicuspid (more severe)
What are the s/sx of aortic stenosis?
most are symptomatic, but when symptoms occur, looks like AS in grown-ups.
What is pulmonic stenosis and what is its prevalence?
obstruction across the pulmonic valve due to abnormal development of the valve, outflow tract, or artery.
It’s very rare
What are the signs and symptoms of pulmonic stenosis?
usually asymp.
when there are symptoms, DOE, exercise intolerance, right-sided HF
- On exam:
- prominent jugular venous a wave
- RV heave and thrill
- widely split S2 and soft P2
- loud, late-peaking, crescendo-decrescendo systolic murmur at upper LSB
How do you dx and grade pulmonic stenosis?
echo to confirm and assess pressure gradient
Mild <50 mmHg
Moderate 50-80 mmHg
Severe >80 mmHg
What’s the Rx for pulmonic stenosis?
moderate or severe cases treated - transcatheter balloon valvuloplasty
RV hypertrophy usually regresses!
What is rheumatic heart disease?
valvular disease that follows as a sequelae of rheumatic fever
mitral - 75-80%
aortic - 30%
tricuspid and pulmonic - 5%
what is heard on auscultation of an ASD?
- fixed, split S2
- ejection murmur may be heard at left upper sternal border
- diastolic rumble of relative tricuspid stenosis may also be present
what are the common signs on PE of VSD?
holosytolic murmur
diastolic rumble or thrill
large can cause CHF symptoms
What are the common signs on PE of Tetralogy of Fallot
Tet spells
systolic ejection murmur at the upper left sternal border (pulmonic stenosis)
What is Kawasaki disease?
multisystem vasculitis
occurs primarily in young children (>80% are 5 years or less)
what is the treatment for Kawasaki disease?
IVIG
and high-dose aspirin during acute phase (fever)
low dose ASA for 6-8 weeks
echocardiograms every 5 years for life (more often with congenital ht dz)
what are the cardiac signs of Kawasaki disease?
pericardial effusion/pericarditis
myocarditis/endocarditis (usually resolves when fever resolves)
prolongation of the PR and QTc and reduced volatage on EKG
cardiac aneurysms may form and can regress
What are the criteria for diagnosing rheumatic heart fever?
JONES PEACE
Jones are major - joints, oh my heart, nodules, erythema marginatum, syndenham’s chorea
PR interval prolongatio, ESR elevation, arthralgias, CRP elevated, elevated temp.
What is the treatment for rheumatic fever?
Penecillin G IM
anti-inflammatory - ASA or corticosteroids
treat any CHF
treat chorea with phenobarbital, haloperidol, valproic acid, diazepam
What are the criteria for diagnosing Kawaski disease?
FEAR ME
Fever for equal to or greater than 5 days
Eye injection - conjunctivitis
Adenopathy - greater than 1.5 cm
Rash - bullous and vesicular lesions common
Mucous membrane changes - red/cracked lips strawberry tongue
Extremity Changes -palm and sole edema and desquamination
what is the biggest concern in Kawasaki disease?
children less than 1 year are more likely to have atypical symptoms and still have coronary artery aneurysms
What is the inheritance pattern for hypertrophic cardiomyopathy?
autosomal dominant
(sometimes sporadic)
what are the symptoms of hypertrophic cardiomyopathy?
dyspnea on exertion
chest pain
syncope
ventricular gallop (S3)
mumur of mitral regurgitation - holosystolic apical murmur
When should cardiac causes of syncope be suspected?
- when it occurs while lying down
- provoked by exercise
- chest pain with it
- family hx of sudden cardiac death
how do you diagnose hypertrophic cardiomyopathy?
echocardiogram
what is the treatment for hypertrophic cardiomyopathy?
activity restriction
- Beta blockers
- calcium channel blockers
- in severe cases, anti-arrythmics or an ICD
