Pedi Cardio

Question 1

What can congenital heart disease lead to?

Answer 1

Heart failure, arrhythmias, conduction abnormalities, and death

Question 2

What are some of the generalized symptoms we look for in a baby that might have a cardiovascular problem?

Answer 2

Difficulty feeding, increased respiratory rate (or labored breathing), sweating, cyanosis, and syncope

Question 3

In a little person, how do we make the diagnosis of a cardiovascular problem?

Answer 3

Clinical, PE, IMAGING IS EVERYTHING (echo), ECG, chest x-ray, and cardiac cath

Question 4

There are 6 major complications of cardiovascular disease in a child, what are they?

Answer 4

CHF, Cyanosis, polycythemia, stroke, retardation of growth, pulmonary arterial hypertension, pulmonary vascular obstructive disease (PVOD)

Question 5

What is PVOD?

Answer 5

destruction of the pulmonary vascular bed in the presence of continuous pressure overload – leading to an increase in pulmonary vascular resistance and elevation of pulmonary artery pressure

Question 6

What is pulmonary arterial hypertension?

Answer 6

When systemic arterial pressure is very high leading to increased pressures in the right side of the heart; with lower pressures in the pulmonary arterioles it causes constriction to protect the capillaries (that can lead to narrowing and fibrosing)

Question 7

If we see cyanosis is a baby, what does that commonly indicate?

Answer 7

Common with defects that result in right to left shunting of blood or heart failure

Question 8

If a child is hypoxic and it is due to heart failure, will they respond to oxygen? What about right to left shunting?

Answer 8

Heart failure = RESPONDS to O2

R to L Shunting does NOT respond to O2

Question 9

What would we look for on PE with cyanosis?

Answer 9

Clubbing of the finger with long-standing cyanosis

Question 10

What else would a child have with right to left shunting or chronic hypoxemia?

Answer 10

Polycythemia = Hct >60

Question 11

What can polycythemia from right to left shunting directly lead to?

Answer 11

Intracranial thrombus (stroke)

Question 12

What else can cause a stroke in a child?

Answer 12

Paradoxical embolus

Question 13

A baby presents for a check-up and while listening to their heart, you hear a holosystolic murmur along the left sternal border, what might it be?

Answer 13

VSD

Question 14

What other murmur in adults has a holosystolic murmur?

Answer 14

Mitral regurg

Question 15

What part of the ventricle would a child most likely have a ventricular septal defect?

Answer 15

The thin membranous septum (80%)

Muscular septum (20%)

Question 16

What way does the blood move with a ventricular septal defect?

Answer 16

From left to right

Question 17

If a child has a large VSD, what can happen?

Answer 17

Pulmonary artery hypertension is common and PVOD develops over time

Large defect results in LV dilation & failure

Question 18

What would a small “restrictive” VDS cause?

Answer 18

Small L to R shunt that is typically well tolerated

Question 19

What does a large VSD lead to?

Answer 19

Heart failure early in life

Question 20

How would you diagnose VSD in a baby?

Answer 20

Echo

Question 21

How would you manage a small VSD?

Answer 21

Regular follow-up with periodic echo-doppler to confirm closure

Some close by 18 months, 50% by 4 years

Question 22

How would you treat a moderate to large VSD?

Answer 22

Treat like heart failure in adults

Surgical repair once heart failure is improved

Question 23

What are the longer term complications of VSD?

Answer 23

If PAH continues over time → irreversible PVOD and then surgery carries high risks

Question 24

If a 29 year old man presents after a long flight with left arm numbness and weakness with a small visual deficit, what diagnosis?

Answer 24

Atrial septal defect

Question 25

What is the most common congenital heart defect that is often not detected until adulthood?

Answer 25

Atrial septal defect

Question 26

How are the two atria formed?

Answer 26

Formed by fusion of 2 overlapping planes of tissue making the atrial septum

Question 27

What is the problem with an atrial septal defect?

Answer 27

Large enough defect to allow free communication between the atria Lack of fusion occurs in 25% of adults, leaving a patent foramen ovale

Question 28

Where do most atrial septal defects occur?

Answer 28

In the mid septum (due to lack of tissue for overlap) AKA Ostium Secundum

Question 29

What is it known as if the defect is in the lower atrium?

Answer 29

Ostium primum

Question 30

What is it known as if the defect is high in atrium?

Answer 30

sinus venous defect

Question 31

What are some of the complications of an atrial septal defect?

Answer 31

Right atrial, ventricle, and pulmonary artery enlargement – due to volume overload Pulmonary hypertension Left to right shunting

Question 32

Why is their left to right shunting is ASD?

Answer 32

Because the right ventricle is more compliant than the left, pulmonary vascular resistance is less than systemic vascular resistance, and the right atrium is lower pressure than the left atrium

Question 33

How does a child present with ASD?

Answer 33

Asymptomatic

Question 34

How does a 20-year-old present with ASD?

Answer 34

fatigue, dyspnea, and decreased stamina

Question 35

How does ASD present in the 3rd & 4th decades of life?

Answer 35

Increasing fatigue, dyspnea, and atrial arrhythmias (Afib)

Question 36

What is one of the most unfortunate side effects of ASD?

Answer 36

Paradoxical emboli → stroke

Question 37

How does a paradoxical emboli travel?

Answer 37

DVT → Venous emboli → RA → through the PFO → LA → LV → Right carotid → brain

Question 38

What do you look for on PE with ASD?

Answer 38

S2 widely split through inspiration/expiration Grade 2-3 midsystolic crescendo-decrescendo murmr (present during childhood)

Question 39

How do you confirm diagnosis of ASD?

Answer 39

ECG & Echo-Doppler Catheter to close ASD

Question 40

How would you treat an ASD if no symptoms are present?

Answer 40

If the Qp:Qs (pulmonary to systemic blood flow) is >1.5:1 or if PAH present

Question 41

If a young boy who is otherwise healthy and is presenting with fatigue and SOB with strenuous activity. On PE you note a harsh crescendo/decrescendo murmur at the upper left sternal border, what diagnosis?

Answer 41

Pulmonic stenosis

Question 42

What is the pathology for pulmonic stenosis?

Answer 42

Domed shaped stenosis of the pulmonary valve along with right ventricular hypertrophy

Question 43

What does right ventricular hypertrophy occur?

Answer 43

Because it has to push higher pressures to get past the stenotic valve

Question 44

What is considered severe pulmonic stenosis vs. moderate?

Answer 44

Severe is a pressure gradiant between the RV & the PA >75 Moderate >40mmHg

Question 45

What would you look for on PE with pulmonic stenosis in a child?

Answer 45

Systolic thrill, early systolic click on the left sternal border, and a LOUD/HARD CRESCENDO-DECRESCENDO at upper LSB that radiates towards the clavicle and is louder with inspiration.

Question 46

What diagnostics would you do to confirm pulmonic stenosis?

Answer 46

ECG has RVH, Echo shows the obstruction and severity

Question 47

What can happen to an adult if severe pulmonic stenosis is not identified and treated?

Answer 47

Poor prognosis with RV failure

Question 48

How do you treat pulmonic stenosis is a child?

Answer 48

Balloon valvuloplasty (has replaced surgery)if gradient is >75 No intervention is gradient is

Question 49

In utero, how does the heart function?

Answer 49

It develops with a right to left shunt through the patent ductus arteriosus

Question 50

How long does it take for the PDA to close?

Answer 50

within the first 2-3 days after birth

Question 51

When does the dutcus most often remain open?

Answer 51

In pre-term deliveries

Question 52

What happens if there is a moderate ductus opening?

Answer 52

Elevated PAP and significant shunting

Question 53

What happens if there is a large ductus opening?

Answer 53

Major left to right shunting, pulmonary congestion, LV dysfunction and failure

Question 54

How does PDA present?

Answer 54

HF develops in the first weeks of life

Question 55

What do you look for on PE with PDA?

Answer 55

A continuous systolic thrill that is continuous through systole and diastole “Machienery murmur”

Question 56

How would you confirm diagnosis of a PDA?

Answer 56

Chest X-ray to see increased LA & LV ECG – LVH Echo Doppler – LVH MRI & CT – identify PDA

Question 57

How do you treat a premature infant with PAD?

Answer 57

Indomethacin = first line treatment Surgical or catheter closure

Question 58

If a young boy presents with hypertension and leg fatigue when running, what diagnosis?

Answer 58

Coractation of the Aorta

Question 59

What does coarctation of the aorta cause?

Answer 59

Cause obstruction to outflow to the lower half of the body, LVH due to pressure overload, and arterial hypertension

Question 60

How does coractation present in an infant?

Answer 60

Heart Failure

Question 61

How does coractation present in older children?

Answer 61

Fatigue, dyspnea, and fatigue of the lower extremities + Hypertension in childhood!!

Question 62

What would be another cause of hypertension in childhood?

Answer 62

Renal artery stenosis

Question 63

What would you look for on PE in this child with hypertension and leg fatigue?

Answer 63

Differential blood pressure between arms & legs (difference of >10mmHg is highly suspicious) Remember! BP is NORMALLY HIGHER in the LE when compared to UE Well-developed upper body & thin legs

Question 64

How would you confirm coractation?

Answer 64

Chest x-ray – Figure 3 sign & indentations into the ribs ECG – LVH Cardiac MRI//MRA & CT Cardiac cath

Question 65

How do you treat coractation?

Answer 65

Surgical direct resection/repair or stent (hypertension may persist if correction is after age 6)

Question 66

What would cause RVH in a child?

Answer 66

Tetralogy of Fallot

Question 67

What occurs in a tetralogy of fallot?

Answer 67

Biventricular origin of the aorta

Question 68

What is one of the most common cause of sudden cardiac death in the young?

Answer 68

Hypertrophic cardiomyopathy (along with Brugada syndrome & Marfan’s)

Question 69

When a young athlete is in for a sports physical, what must we always ask about?

Answer 69

Family history of sudden death, history of chest pain, dizziness, syncope, and dyspnea

Question 70

What should our cardiac exam include for our young athletes’ sports physical?

Answer 70

Thorough evaluation for murmurs!! ECG & Echo Doppler should be obtained when structural cardiac pathology is suspected