Pediatric Cardiology I - Nazeri

Question 1

only 2 veins that carry arterialized (oxygenated) blood?**

Answer 1

pulmonary veins and umbilical veins

Question 2

know the fetal circulation (slide 3)**

Answer 2

• umbilical veins –> liver –> ductus venosus –> IVC –> RA
• venous blood in RA –> RV –> pulmonary artery –> PDA bc lungs are blocked –> descending aorta
• arterialized blood in RA –> LA by foramen ovale –> LV –> aorta to enter systemic –> supply brain and upper extremity

Question 3

what 2 organs get the highest % of oxygenated blood?***

Answer 3

liver and brain

Question 4

what to look for when evaluating congenital heart disease?

Answer 4

Location of the PMI
The character of heart sounds
The location, radiation and character of any heart murmur

look for:**

• cyanosis on a pulse Ox
• pulmonary vascular markings on chest Xray
• left or biventricular hypertrophy on EKG

Question 5

pulse Ox

Answer 5

-used to detect subclinical ductal dependent cyanotic heart disease the 1st 24-48hr.

Question 6

when do you do ECHO in newborn?***

Answer 6

• pulse Ox in right hand or either foot <90% –> urgent ECHO**
• pulse Ox b/w 90-95% –> check every hr. 3x –> ECHO if still unchanged
• pulse Ox b/w right hand and either foot >3% –> urgent ECHO

-O2 level can be lower in left arm bc left subclavian comes off aorta near PDA

Question 7

acyanotic congenital heart disease

Answer 7

• left to right shunts
• blood is oxygenated
• 1. volume overload –> ASD, VSD, AV valve regurge, cardiomyopathies
• 2. pressure overload –> pulmonary/aortic stenosis, aortic coarctation

Question 8

right ventricle to left ventricle murmur**

Answer 8

• RV builds up pressure in left to right shunts –> switch to right to left shunt (Eisenmenger)
• loud murmur with NARROW VSD** (holosystolic)
• might not have murmur with wide VSD

Question 9

ostium primum defect**

Answer 9

aka AV canal defect

• leads to VSD and endocardial cushion defect (aka AV canal defect)
• may have no inter ventricular septum

Question 10

ostium secundum** defect**

Answer 10

leads to ASD (left to right shunt)

Question 11

patent ductus arteriosus (PDA)**

Answer 11

• acyanotic congenital heart bc higher pressure in aorta pushes oxygenated blood into PDA
• closure at 2-3 weeks

Question 12

manifestations of left to right shunts**

Answer 12

• large VSD may be asymptomatic at first –> progress to decreased pulmonary compliance –> pulmonary edema (tachypnea, wheezing, crackles) and heart failure (not really failure bc LV output is not reduced)
• over time if not treated –> have reversal of left to right shunts (Eisenmenger)** due to a higher pressure building up on right side and in pulmonary artery –> cyanosis

Question 13

increased pressure loads

Answer 13

• severe pulmonic stenosis –> right side heart failure –> hepatomegaly and peripheral stenosis by shunting into foramen ovale
• severe aortic stenosis/coarctation –> left side heart failure –> pulmonary edema, low peripheral perfusion, and right side heart failure

Question 14

double aortic arch**

Answer 14

• compress trachea –> airway obstruction
• in lower 1/3 –> wheezing**
• in middle –> inspiratory dyspnea**
• enter pleural tissue at lower 1/3**

Question 15

cyanotic congenital heart disease causes

Answer 15

1. obstruct RV outflow –> higher pressure leads to right to left shunt
2. defects causing intracardiac admixtures of venous oxygenated blood and arterial blood return
3. persistent pulmonary HTN of newborn (retained foramen ovale, PDA)

Question 16

tetrology of fallot**

Answer 16

-cyanotic due to right to left shunt and displacement of aorta

1. Pulmonic valve stenosis
2. VSD
3. Deviation of the aortic root to the right
4. Right ventricular hypertrophy

Question 17

Blalock-Taussig shunt

Answer 17

• shunt b/w the right subclavian artery and the right pulmonary artery (forms due to tetralogy of fallot)
• deoxygenated blood from right subclavian enters right pulmonary to go back to lungs again –> fixes O2 problem

Question 18

TET spell

Answer 18

• complete obstruction of RV outflow –> no blood enters pulmonary circulation –> cyanosis
• due to pulmonary artery or muscle spasm
• fatal in newborns
• improved by squatting, IV fluids, morphine, propranolol

Question 19

simple transposition of great vessels

Answer 19

• aorta on RV
• pumonary artery on LV
• 2 separate circulations
• need foramen ovale or PDA to mix blood
• D transposition –> open forame ovale
• L transposition –> open VSD

Question 20

scimitar vein

Answer 20

-anomalous venous return from the right lung into IVC

Question 21

Norwood procedure

Answer 21

-treatment for hypoplastic left heart syndrome

Question 22

persistent pulmonary HTN of newborn (PPHN)**

Answer 22

• severe cyanosis with tachypnea, often labile
• may have myocardial ischemia –> papillary muscle dysfunction –> mitral/tricuspid regurge

predisposing factors*

• oligohyramnios**
• pulmonary hypoplasia from diaphragmatic hernia
• maternal NSAID use constricting ductus arteriosus
• cyanotic CHD
• alveolar capillary dysplasia

diagnosis
-PaO2 difference b/w right radial and umbilical arteries

treatment

• PGE1,2 to keep PDA open
• Pre iNO, Post iNO** –> endothelial derived and relax vascular smooth muscle

Question 23

ECMO*

Answer 23

-cardiopulmonary bypass to increase systemic perfusion/gas exchange

1. veno-arterial –> ligate carotid artery and attach to right internal jugular
2. veno-venous –> right internal jugular to right atrium