Congenital Heart Disease Flashcards by Aboudi el masri

What are the 4 most common congenital heart defects?

Ventricular septal defect
ductus arteriosis
Atrial septal defect
pulmonary/aortic stenosis

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Is coarctation of the aorta a congenital heart defect?

Yes

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What are the 5 Ts for cyanotic lesions?

TGA - Transposition of the great arteries
Tetralogy of Fallot
Tricuspid atresia (or pulmonary atresia)
Truncus arteriosis
Total anomalous pulmonary venous return

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list 3 acyanotic shunted lesions

Shunted means that it bypasses something => there is a shift from left to right (note than very early in life the shunt is in the opposite direction)
Ventricular septal defect
arterial septal defect
ductus arteriosis

Note: acyanotic here is only if isolated. usually these occur with other cardiac issues and hence may still present cyanotic.

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list 2 acyanotic non-shunted lesions

aortic stenosis
coarctation of the aorta

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When is the antenatal anomaly scan conducted to detect the presence of CHD in utero?

20 weeks gestation

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Pulse oximetry may be used to screen for these diseases.
What is the cutoff in room temperature that warrants further investigation?
What is meant by pre and post ductal oxygen sat?

<95% sat under room air conditions

the “duct” refers to ductus arteriosis. Pre-ductal is taken from the right arm as the right subclavian artery occurs before the ductus arteriosis. Post-ductal is taken on either foot as the abdominal aorta is after the duct.

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What investigations would you carry out to investigate a suspected case of CHD?

History and physical exam (CVS and resp)
MUST INCLUDE: Vitals and growth parameters including pre and post-ductal sats and 4 limb blood pressure
Blood gas and hyperoxia test
ECG
CXR
Cardiology consult (get used to saying that) for ECHO
Genetic evaluation
For 5/5 must include plot weight and height centiles

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If the suspected CHD is duct-dependent (ductus arteriosis), what must you ensure is given to the infant while they are awaiting transfer to a tertiary hospital?

Prostin which is a prostaglandin used to either induce labour in pregnant women or to keep the duct open temporarily.

Note: Prostaglanding synthetase inhibitors are used to seal off patent ductus arteriosis. makes sense!

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What are the 4 components of the tetralogy of Fallot?

Severe pulmonary stenosis
Right ventricular hypertrophy
large ventricular septal defect
Overriding aorta that lays astride the ventral septal defect rather than the left ventricle

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What is hypoplastic left heart syndrome (2 things)

Left side of heart poorly developed
Left sided valves (mitral and aortic) small or absent

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What is the transposition of the great arteries. How do these babies survive?

Complete separation of pulmonary and systemic circulation with the right ventricle pumping into the aorta and left ventricle pumping to the pulmonary artery. => desaturated blood circulates the body while saturated blood continuously circulates the lungs

=> these babies need a septal defect or patent ductus arteriosis to survive and mix the 2 systems.

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When does TGA (transposition of great arteries) normally present?

first few days of life

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Tricuspid atresia usually presents with severe cyanosis.
What is tricuspid atresia?
What other CHD is it associated with?
How do these babies survive?

Absent tricuspid valve with hypoplastic right ventricle and and pulmonary artery (=> right ventricle and pulmonary artery are poorly developed)

A/w TGS (transposition of great arteries)

Requires associated defects for survival (septal defect of PDA)

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Patent ductus arteriosis (also applies for AVSD):
What would you prescribe to close a patent ductus arteriosis
What would you prescribe to try and keep the defect open? Why would you want to do that?

Prostaglandin synthetase inhibitor (e.g. Ibuprofen)
PGE1 (prostaglandin E1) is used to keep it open. We would want to do that in cases where the survival of a child with a congenital heart defect (such as tetralogy of fallot and transposition of the great arteries) depends on mixing via PDA or AVSD. Also maybe for transferring to a tertiary center.

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What % of all newborns have congenital heart disease
What % of down syndrome patients have a congenital heart defect?

1%
50%

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How is neonatal CHD screening done in Ireland for discharge?

Involves taking O2 sats where it is >95% on room air via pulse oximetry

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What is the characteristic presentation of CHD in infants?
What findings on a cardiac exam would support CHD diagnosis?

Murmur + Resp distress + poor feeding/failure to thrive/weight stagnation

Prominent cardiac impulse
Weak pulse
Liver edge
murmurs (Red flag = S3 gallop)

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What is the gold standard method of diagnosing CHD? Give other methods

ECHO gold standard. Also X-ray and ECG.
4 limb BP and pre+post ductal O2 sats (right arm and lower limb)

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What information are you trying to get, related to CHD, from an X-ray?

Pulmonary oedema (esp ASD, VSD,AVSD)
Cardiomegaly (esp in TOF, aortic stenosis)

How does fetal circulation become normal circulation?

In fetal circulation, blood from placenta goes through ductus venosis into the inferior vena cava and uses the foramen ovale to get to the left atrium before being pumped to the rest of the body. Once the chord is clamped 2 things happen
1) cord clamping deprives infant of endogenous placental prostaglandins and
2) Exposure to high oxygen content (compared to fetal SpO2 of 65%)

A patient with ASD presents with labored work of breathing. What do you expect to find on examination?
When do you expect this patient to present?
What do you expect to find on ECHO?
How would this be treated?

Right ventricular heave
Splitting of 2nd HS

Presents usually in first few years of life

ECHO: atrial septal defect and dilation of right heart
tx: easily repaired via device closure or surgery if needed

An infant at 6 weeks of life comes into the 6 week check with labored work of breathing and refusing to feed. Both pulmonary arterial and venous pressures are elevated. They do not have any facial dysmorphic features. What is the most likely diagnosis?

What murmur do you expect? Where would you listen for it?

How would you manage this patient?

VSD

Ejection systolic (same as aortic stenosis)
Left Lower Sternal edge or over the tricuspid valve

Diuretic therapy + high calorie feed + surgery at 4-6 months

An infant with brushfield, cynodactyly, and flatted nasal bridge presents. What is the most likely CHD?

How would you manage this patient?

AVSD

Diuretic therapy + high calorie feed + surgery at 4-6 months (slightly later than VSD but same)

What defects can mimic congenital heart failure?

VSD, AVSD

On examination, you note a weak femoral pulse. What will you proceed to do to confirm? Where else would you auscultate? What is your primary diagnosis? How would you manage this patient?

I will compare radial and femoral pulses Also auscultate the back as it might radiate there. Coarctation of the aorta Urgent surgery

You suspect coarctation of the aorta and proceed to compare the radial and femoral pulse strengths. What is the difference in systolic BP required for diagnosing coarctation of the aorta?

>20mmHg Note: remember to do 4 limb BP, pre and post ductal O2 sats, and ECHO

Midwife comes and tells you that there is a murmur in the delivery suite. You note an ejection systolic murmur. What is the most likely diagnosis? Why does this occur? The infant remains in respiratory distress despite resus efforts. what is your management plan?

Aortic stenosis, radiates to the carotids. Aortic valve is congenitally bicuspid instead of tricuspid Management: Urgent balloon dilatation followed by valve repair/replacement

A patient with micrognathia, low-set ears, hypertelorism, and cleft palate presents to the emergency department with labored breathing and the mother reports refusal to feed. What is the most likely diagnosis? On examination what color do you expect? You conduct an X-ray, what do you expect to see? The infant suddenly becomes cyanosed and work of breathing increases significantly. What is happening and what should you do? What is your management plan?

Di george's => Tetralogy of Fallot (can also be Truncus arteriosis but that is very rare) Pink-mild cyanosis X-ray - Boot-shaped heart due to RV hypertrophy This is called a TET spell or episode of cyanosis. Immediately perform the knee-chest maneuver, administer IV Bolus, and IV morphine Surgery at 4-6 months and prostaglandin therapy if getting worse

An infant experiences rapid cardiogenic shock in first few days of life. What is the most likely diagnosis? What is it? Management plan? What syndrome is this related to?

Truncus Arteriosus (can also be hypoplastic left heart syndrome but that should've been caught antenatally). This is when there is one trunk coming off both ventricles which then splits into the pulmonary and aorta Urgent surgery Di George's syndrome

What CHD require Prostaglandin therapy

Duct-dependent CHD Transposition of the great arteries Hypoplastic left ventricle TOF

Infant remains cyanosed in the delivery suite despite resuscitation efforts. They do improve on prostaglandin therapy. What is the likely diagnosis? What is your immediate management of this case? What is your management plan?

Transposition of the great arteries. It is duct dependent due to it being 2 different circulations => the prostaglandin is needed especially for transfer to a tertiary hospital. Assuming prostaglandin is administered, a balloon atrial septostomy which is used to enlarge the foramen ovale is needed to improve mixing Management plan: Continue on prostaglandin therapy until Atrial switch operation at 1 week

Infant remains cyanosed in the delivery suite despite resuscitation efforts. Prostaglandin therapy is administered but it has made the situation worse. What are you suspecting? What are the 2 types of this CHD?

Total Anymous Pulmonary Venous Drainage. This is due to an extra vein connecting to the RA instead of the LA => keeping it open would be worse cuz the heart is otherwise normal. Can be supracardiac => into SVC or infracardiac => into liver/IVC.

You meet a mother who has told you on the 20 week anomaly scan (gestation), that her fetus has a left heart than has not grown properly. What is the diagnosis? How would you manage?

Hypoplastic left heart syndrome (usually diagnosed antenatally otherwise cardiogenic shock) The left ventricle is basically useless => this is also duct dependent => prostaglandin therapy will help. This is the most complex => 3 surgeries needed at 1st week 3-4 months 2-3 years + lifelong medical care and supervision.

Give Maternal RFs for congenital heart disease. State the strongest RF

Insulin-dependent diabetes Smoking/alcohol Multiple births congenital rubella infection (strongest RF) Antidepressants/antiepileptic drugs

What congenital heart defects are associated with noonan's

Pulmonary stenosis Hypertrophic cardiomyopathy

What congenital heart disease is associated with Alagille syndrome?

Peripheral pulmonary artery stenosis

What congenital heart disease is associated with Hand-heart syndrome. Give the medical name for this syndrome too

Holt Oram syndrome Secundum ASDs

What are the signs and symptoms to look out for when considering a congenital heart disease?

Tachypnea Failure to thrive/gain weight Active precordial impulse Added heart sounds (especially gallop) Palpable liver edge 2-3 cm below costal margin

What area of the precordium is most associated with innocent/still's murmur? Describe the murmur. What other defect may have a murmur at the same place?

Left lower sternal edge (tricuspid) Sounds vibratory and musical. and is typically a soft ejection systolic murmur. One of the best ways to distinguish an innocent murmur is via the grade. this is usually grade 2/6 VSD is at the same location. high pitched whoosh sound

Describe a mitral regurg murmur

whoosh. Heard loudest at the apex and is holo-systolic

Describe a murmur for ductus arteriosus

Continuous murmur, difficult to discern heart sounds 1 and 2 (that is just my version of it) Heard loudest at the left infraclavicular region (left of pulmonary valve)

You are checking an ECG and note a right BBB. Where did you notice it? (what lead) What congenital heart defect is associated with it?

You will notice as rsR complex or M in V1 Associated with atrial septal defect

What syndrome is associated with coarctation of the aorta?

Turner's also aortic stenosis (bivalve)

ECHO shows right ventricular hypertrophy. What do you expect to see on an ECG? Ho about left ventricular hypertrophy?

Upright T wave in V1 Very sharp Q waves in aVL

You conduct an ECHO on a down syndrome patient and note AVSD. What do you expect to find on ECG?

Superior or northwest WRS axis

You note deep Q waves in leads 1, AVL, V5, and V6. What is the most likely diagnosis?

ALCAPA which causes angina in an infant => immediate cardiology referral (just like the other ECG findings discussed)

What is each small square in an ECG Large square How do you calculate heart rate from an ECG?

Small box = 0.04s large = 0.2s Total number of R waves x10