Pediatric Cardiology

Front 1

What causes reverse differential cyanosis?

Back 1

Transposition of the great arteries with right to left shunting of oxygenated blood via the ductus arteriosus.

Front 2

What causes a paradoxical split S2?

Back 2

Conditions causing delayed LV outflow via aorta e.g. Aortic stenosis, LBBB or Hypertrophic Cardiomyopathy.

Front 3

List the 5 benign pediatric murmurs?

Back 3

PPS (peripheral pulmonic stenosis), pulmonary flow murmur, Stills murmur, Carotid bruit and Venous hum.

Front 4

What conditions cause left axis deviation in kids?

Back 4

Tricuspid atresia, osmium primum ASD, AV canal defect or left venticular hypertrophy.

Front 5

Formula for calculation of corrected QT interval (QTc)

Back 5

QT/sqrt(R-R int)

R-R int is in seconds

Front 6

Conditions that prolong QTc

Back 6

Tricyclic overdose, low Ca/Mg or K, class Ia and IIa anti-arrhythmics (e.g. Procainamide, quinidine, amiodaeone, sotalol), CNS insult

Front 7

ECG findings in right atrial enlargement (RAA) and left atrial enlargement (LAA)

Back 7

RAA (peaked p waves in II and V1)
LAA (wide/notched p wave in II, increased neg deflection in V1)

Front 8

What are the 2 mechanisms of abnormal arhhythmias?

Back 8

Reentry or automaticity

Front 9

Mechanisms of SVT in kids?

Back 9

Accessory tract (e.g. WPW) or AV node reentry

Front 10

Treatment of acute rheumatic fever? In a PCN allergic patient?

Back 10

1.2 mill U Benzathine PCN G IM or 600,000 U of procaine PCN qday IM x10 days. Erythromycin 1 gm PO x 10 days in PCN allergy.

Front 11

What are the major and minor manifestations of acute rheumatic fever?

Back 11

Major (carditis, chorea, migratory polyarthritis, subcutaneous nodules, erythema marginatum) Minor (arthralgia, fever, high CRP/ESR, prolonged PR interval)

Front 12

Criteria for dx of acute rheumatic fever?

Back 12

a) 2 major or 1 major + 2 minor criteria
b) evidence of recent/concurrent S pyogenes infection (+ throat cx for GAS or rapid strep antigen or elevated/rising strep antibody titer)

Front 13

Mechanism of action of Digoxin?

Back 13

a) Inhibit Na+-K+ ATPase --> increased intracellular Na--> increased intracellular Ca (by activation of Na+-Ca2+ exchange) --> increased inotropy
b) Inhibit sympathetic response, increase parasympathetic tone --> decreased cardiac metabolic demand

Front 14

Treatment of post-pericardiotomy syndrome? (immune med rxn usually 1-4 wks after surgery, unto 6 mos)

Back 14

Bed rest, Aspirin 80-100 mg/ kg/day and when acute attack is under control, wean off aspirin over 6 weeks; 10-15% recurrence risk!

Front 15

What is Becks triad?

Back 15

Cardiac Tamponade physiology (elevated JVP, hypotension, muffled heart sounds)
Other Tamponade signs = Kussmaul sign (JVP rise with inspiration), pulsus paradoxus (exaggerated fall in aortic BP with inspiration), narrow pulse pressure

Front 16

Signs of Digoxin toxicity? Treatment?

Back 16

Nausea, vomiting, diarrhea, color vision changes, confusion, vertigo, palpitations or arrhythmias (AV block, SVT or VT).
Digoxin antibodies (Fab fragments or Digibind) for severe toxicity.

Front 17

Physical exam, CXR changes in pulmonary hypertension?

Back 17

Exam - narrow split or single loud S2; diastolic decrescendo murmur from Pulmonary regurgitation if present; syncope, chest pain or sudden death if severe.
CXR - large proximal PA or RA/RV hypertrophy

Front 18

Usual presentation of anomalous origin of the left coronary artery?

Back 18

Usually present in infants 2 weeks - 6 mos with heart failure from MI or ischemia. Poor feeding, tachypnea, respiratory symptoms, restlessness, cardiomegaly, anteroom-lateral infarction changes on ECG (abnl Q waves in I/aVL and anterior chest leads).

Front 19

Most common aortic arch abnormality? Most common symptomatic anomaly?

Back 19

Most common aortic arch anomaly = aberrant R subclavian artery from descending aorta (posterior to esophagus, usually no symptoms).
Most common symptomatic anomaly = double aortic arch (encircling of esophagus and trachea).

Front 20

Other congenital abnormalities associated with truncus arteriosus? Manifestations?

Back 20

Deletion of chromosome 22 (Di George) or interrupted aortic arch. Manifestations = increased pulmonary blood flow and L-R shunt (dyspnea, wheezing, FTT, no significant cyanosis, cardiomegaly + increased pulmonary markings, right aortic arch in 30-50%).

Front 21

Surgical stages of management of hypo plastic left heart syndrome?

Back 21

a) Norwood - cut the MPA, ligate PDA, reconstruct ascending aorta and aortic arch to connect RV to aorta; systemic to pulmonary shunt e.g. BT shunt); atrial septectomy if small ASD;
b) Bi-directional Glenn: SVC to PA shunt
c) Fontan: IVC to PA connection

Front 22

Types of total anomalous pulmonary venous return? CXR findings?

Back 22

a) 1/3 have pulmonary venous return via left vertical trunk into L innominate vein and then SVC.
b) 1/4 below diaphragm into ductus venous and then IVC (more likely to be obstructed)
c) Rest 30-40% directly connect into RA or coronary sinus.
CXR (unobstructed): snowman or figure 8 silhouette (dilated L vertical and innominate veins and R SVC)

Front 23

What causes an Ebstein anomaly?

Back 23

Posterior and septal leaflets of the Tricuspid valve are displaced down and attach to the RV wall dividing the RV into 2 sections - proximal atrial like enlarged segment and a distal ventricular like segment. Usually see significant tricuspid regurgurgitation. Usually patients are cyanotic after birth from atrial right to left shunt but this resolves later on in childhood/ adolescence. Paroxysmal SVT common (ECG shows RBBB or WPW)

Front 24

Most common cause of cyanosis in a newborn during the first few days of life?

Back 24

Complete transposition of the great arteries or d-TGA (Tetralogy of Fallot is most common at all ages!!)

Front 25

ECG and CXR findings in d-TGA?

Back 25

ECG: persistently positive T wave in R precordium around 5 days of age.
CXR: egg shaped heart with narrow mediastinum/ small thymus (seen in 1/3 of infants).

Front 26

Components of Tetralogy of Fallot? CXR findings?

Back 26

RV outflow obstruction (sub pulmonary valve stenosis), VSD, overriding aorta, RVH.
CXR: boot shaped heart (RVH)

Front 27

Treatment of a tet spell?

Back 27

Prostaglandin in a neonate (alleviate pulmonary flow obstruction), knee chest position (increased systemic resistance and decreased R-L shunt), oxygen, morphine/avoid agitation, vasopressors (e.g. Phenylephrine) to increase systemic resistance, keep Hematocriova round 50-55%; may give Propranolol to prevent attacks!

Front 28

Most common congenital heart lesion?

Back 28

1) VSD
Followed by Pulmonic stenosis, ASD and PDA.

Front 29

Factors that determine impact of a left to right shunt?

Back 29

1) Size of the shunt
2) Pressure difference between the two vessels/areas shunted
3) Total outflow resistances

Front 30

Most common location for VSD based on age?

Back 30

a) < 1 year: muscular septum (usually close spontaneously by age 1)
b) > 1 year: membranous septum

Front 31

Physical exam findings with ASD? CXR and ECG findings?

Back 31

Fixed split S2 (delayed closure of PV due to increased volume from L-R shunt), pulmonary flow murmur, sometimes early or mid diastolic murmur across TV from increased shunting.
CXR: cardiomegaly, increased pulmonary blood flow
ECG: RAD/RVH, rsR' or rSR' in the right precordium, notched S wave in inferior leads.

Front 32

Most common heart defect in Down syndrome? Presentation?

Back 32

AV canal defect.
Exam: VSD murmur +/- mid diastolic rumble (increased pulmonary venous return and flow across AV valve), mitral regurg murmur if mitral cleft significant, heart failure in the first few months.
ECG- left axis deviation!!

Front 33

Explain the connections in l-TGA? (corrected transposition)

Back 33

RA- mitral valve- LV- PA
LA- tricuspid valve- RV- left sided aorta
ECG: Q waves in the right septal leads and not on the left (septal activation right to left!)
- usually associated with VSD or pulmonic stenosis

Front 34

What is an Austin Flint murmur?

Back 34

Low pitched mid-diastolic murmur at the apex due to regurgitant aortic ject striking the anterior leaflet of the mitral valve.

Front 35

Causes of aortic regurgitation in kids?

Back 35

Congenital aortic stenosis ( e.g. secondary to a bicuspid AV), Marfan syndrome, Rheumatic fever, Infective endocarditis

Front 36

Worldwide most common cause of MItral regurgurgitation?

Back 36

Rheumatic fever (#1 cause in the US is MVP)

Front 37

Associated conditions with mitral valve prolapse?

Back 37

Marfan, Ehlers-Danlos or the muopolysaccharidoses.
Exam = late systolic crescendo murmur preceded by a click; louder with lesser volume (e.g. sitting or stander versus lying supine)

Front 38

Most common causes of pulmonary regurgitation?

Back 38

Post op complication of pulmonary stenosis or tetralogy of fallot; congenital PR is rare.

Front 39

How does one estimate RV pressure?

Back 39

Using RA pressure + gradient across TV (I.e. 4* v squared) where v = velocity of tricuspid regurgitant jet.

Front 40

Presentation of severe congenital aortic stenosis?

Back 40

Early, systolic murmur at right or left sternal border with early ejection click, diminished perfusion and pulses --> shock; marked cardiomegaly on CXR and severe pulmonary edema.

Front 41

What is the Ross procedure?

Back 41

Moving the pulmonary valve ring with valve intact into the aortic annulus and placing a homografts aortic valve into the RV outflow tract; no anticoagulation needed, low risk of restenosis.

Front 42

Genetic inheritance of HOCM?

Back 42

Autosomal dominant with variable expression; 1/2 the mutations are found on chromosome 14.

Front 43

Characteristics of HOCM murmur?

Back 43

Systolic murmur that is crescendo decrescendo which becomes louder with Valsalva or standing (reduced venous return --> decreased LV volume and increased obstruction)

Front 44

Most common cause of sudden deaths in athletes at sporting events?

Back 44

Arrhythmias from HOCM.

Front 45

Supravalvar AS is associated with what genetic syndrome?

Back 45

Williams syndrome (idiopathic infantile hypercalcemia, mental retardation, cocktail personality, elf life facies, narrowing of peripheral systemic and pulmonary arteries)

Front 46

Interrupted aortic arch is commonly associated with what syndrome?

Back 46

22q11 micro deletion
(most common physical location is the part of the aorta between the origin of the left subclavian and the ductus attachment)

Front 47

Manifestations of coarctation of the aorta in older kids? CXR findings?

Back 47

Hypertension, epistaxis, claudication like sxs in lower extremities with exercise, strokes (rupture of berry aneurysms), weak or delayed femoral pulses), posterior murmur at left scapular angle or murmur from aortic stenosis/ bicuspid aortic valve.
CXR: figure of 3 sign, rib notching