Cardiovascular Development Clinical Relevance

Front 1

Dextrocardia

Back 1

Heart on the R hand side

Due to abnormal heart looping

Apex beat palpated in R 5th intercostal midclavicular line

If isolated, can be severe; usually accompanied by other severe cardiac anomalies, such as single ventricle/ ventricular septal defect

Front 2

Situs invertus

Back 2

All organs are inverted i.e. liver and appendix are on the left side instead of the right side

if heart is on the right side with other organs, you will still survive

Front 3

Where is apex beat usually palpated for?

Back 3

Left 5th intercostal mid-clavicular line

**mitral valve auscultation

Front 4

Atrial septal defects (list them only)

Back 4

10% of congenital heart diseases are ASD's:

1. osteum secundum

2. endocardial cushion defect with osmium primium defect

3. sinus venosus defect

4. common atrium "tricolare biventriculare"

Front 5

Osteum secundum

Back 5

Atrial septal defect (ASD)

Second hole where apoptosis occurs can get larger or can hop in another spot

Front 6

Endocardial cushion defect with osteum primium defect

Back 6

Atrial septal defect (ASD)

Septum doesn't grow all the way to endocardial fusion mesoderm; osteum prim. not closing towards EC mesoderm

Front 7

Sinus venosus defect

Back 7

Atrial septal defect (ASD)

Opening of superior vena cava; pulmonary veins come out from R atrium instead of L atrium. Surgery needed

Front 8

Common atrium

Back 8

"Cor tricolare biventriculare"

Atrial septal defect (ASD)

Rare; three chambered heart

(cortriloculare biventriculare) = 3 chambers with 2 ventricles i.e.biventricle and thus one atrium

Front 9

Probe Patent Foramen Ovale

Back 9

1. 1 in 4 people have this (25% population)

2. Usually asymptomatic

3. If symptomatic, you have another cardiac defect

4. Common association with migraines

5. Probe between R and L atriums

6. doesn't close; analogy to VSD

Front 10

Premature closing of foramen ovale

Back 10

Before birth leads to death shortly after delivered

Results in hypertrophy of the right side of the heart and under-development of the left side

Front 11

Ductus venosis

Back 11

Umbilical vein -->Highly o2 blood bypasses liver into R atrium

Front 12

Foramen ovale

Back 12

post natal "fossa ovales"

R atrium bypasses R ventricle to R pulmonary trunk to lungs and goes straight to L atrium

Front 13

Ductus arteriosus

Back 13

Shunt system used to treat TRANSPOSITION OF GREAT VESSELS; blue baby

Prostaglandins keep this open

NSAID's keep this closed (PDA)

Since some blood does go to pulmonary trunk; bypasses lungs and enters Aorta

Front 14

Persistent Truncus Arteriosus

Back 14

1:1000 live born infants

Failure to separate Truncus Arteriosus from persistent Conus Cordis

(Conus cordis= cranial bc)

1 outflow tract. Failure to divide into ventral aorta, aortal sac, and pulmonary trunk

No neural crest cell migration to membranous intraventicular septum

ALWAYS: ventricular septum defect of membranous type. mixing of blood// no oxygenated blood

cyanosis: blue baby

Front 15

Transposition of Great Vessels

Back 15

Overall annual incidence: 20-30 per 100,000 live births. Males >Females 3:1

No spiral septum; formed "straight down" aorticopulmonary septum (normally separates into 2 tubes spirally)

Aorta -R ventricle/ Pulmonary trunk- L ventricle.

Oxygenated blood stays in lungs and never goes to body

Tx: Prostaglandins- opens Ductus arteriosus shunt system: Bypass of lungs and into Aorta from Pulmonary trunk (picture)

Front 16

Tetralogy of Fallot

Back 16

Anterior displacement of aorticopulmonary septum

Tetralogy:

1. pulmonary stenosis (smaller pulmonary trunk)

2. overriding aorta (larger aorta)

3. Membranous type- ventricular septal defect

4. R ventricular hypertrophy caused by higher pressure on the R side (lots of blood trying to get through vessel)

Cyanosis; blue baby poor oxygenation; decreased of blood to lung depending on stenosis

Tx: surgical

Front 17

Ventricular septal wall defect - muscular type

Back 17

swiss cheese

Front 18

Ventricular septal wall defect-membranous type

Back 18

More common due to dual embryological origins in endocardial cushion mesoderm and aorticopulmonary septum

1. shunting of oxygenated blood from R ventricle to L ventricle

2. mixed blood to lungs = cyanosis blue babies

3. less oxygenated blood going through systemic circulation

4. pulmonary HTN

5. cardiac failure

Front 19

Common ventricle

Back 19

Ventricular septal wall defect

Cor triculare biatrium

2 atriums, 1 ventricle

3 chambered heart

no ventricular septum whatsoever

Front 20

VSD's summary

Back 20

membranous and muscular defects

majority are small

not as many as atrial septal defects

usually membranous more common bc more complex due to dual embryological origin

unlike probe patent foramen ovale, VSD's do close.*can be detected before birth

Front 21

Persistent Stapedial artery

Back 21

2nd Aortic Arch artery becomes spatial arteries which degenerates in adults and is normally are fetal only

congenital vascular anomaly

tinnitus

carotid formation defects

Front 22

1st Aortic Arch Artery

Back 22

Symmetric R and L

Maxillary arteries

Front 23

2nd Aortic Arch Artery

Back 23

Symmetric R and L

Stapedial arteries (fetal only- role in carotid arteries)

Front 24

3rd Aortic Arch Artery

Back 24

Symmetric R and L

1. Carotid arteries

long left, short right

2. Internal carotid arteries (distally)

Front 25

4th Aortic Arch Artery

Back 25

Asymmetric

LEFT: majority of arch of aorta

RIGHT: proximal R subclavian artery

(7th intersegmental arteries--> distal segment)

Subclavian artery has dual embryo origin

Front 26

5th Aortic Arch Artery

Back 26

no structures

Front 27

6th Aortic Arch artery

Back 27

Asymmetric

RIGHT: R Pulmonary artery (majority of it)

LEFT: L Pulmonary artery and Ductus Arteriosus (shunt from pulmonary artery to aorta)

Front 28

Pre-ductal Coarctation of Aorta

Back 28

Constriction before it reaches Ductus Arteriosis

No alternative route; baby dies

Front 29

Post-ductal Coarctation of Aorta**

Back 29

Constriction after it reaches Ductus Arteriosus

Alternative route: COLLATERAL CIRCULATION: Left subclavian-->Internal thoracic artery-->Anterior intercostal artery (1-6) anastomoses with Posterior intercostal artery (3-9)--> Descending Aorta/Thoracic Aorta

Front 30

Double Aortic Arch

Back 30

Cranial to T4

Two arches instead of one

Right Dorsal Aorta (arch) not degenerating as it should normally

constriction of esophagus and trachea**

Stridor &dysphagia

Front 31

5th intersegmental artery becomes?

Back 31

1. Common iliac artery

2. Internal iliac artery**

3. External iliac artery

Proximal segment of internal iliac artery fuses with proximal umbilical arteries pre-natal

Front 32

Vitilline arteries become what 3 gut arteries?

Back 32

Vitilline aa. fuse with abdominal aorta (fusion caudal to T4 from dorsal aorta):

1. Coeiliac artery (T12- foregut)

2. Superior Mesenteric Artery (L1-midgut)

3. Inferior Mesenteric Artery (L3-hindgut)

Front 33

Umbilical arteries become?

Back 33

Retained: proximal umbilical arteries --> 1. distal segments of internal iliac arteries (because it fused with the proximal segments of internal iliac arteries) and 2. superior vesicle arteries

Obliterated: distal umbilical arteries --> 3. medial umbilical ligaments (when we cut the cord)

**umbilical veins obliterate after arteries

Front 34

Which veins drain into sinus venosus into the primitive R atrium? What kind of oxygenation do they provide? Origins?

Back 34

1. vitelline veins (yolk sac- deoxygentated)

2. umbilical veins (placenta - highly oxygenated)

3. cardinal veins (embryo body- deoxygenated)

Front 35

Ductus arteriosus becomes?

Back 35

Ligamentum arteriosus

Front 36

Cranial LEFT vitelline vein becomes?

Back 36

Hepatocardiac channel - Left Hepatic Vein

Front 37

Cranial RIGHT vitelline vein becomes?

Back 37

Hepatocardiac channel:

1. Right Hepatic vein

2. Hepatic portion of Inferior vena cava***

Front 38

Central vitelline veins become?

Back 38

1. Hepatic sinusoids

2. Ductus venosus--> "ligamentum venosum"

Front 39

Caudal vitelline veins become?

Back 39

1. Portal vein (plexus around duodenum)

2. Splenic vein

3. Superior mesenteric vein

4. Inferior mesenteric vein

Front 40

Umbilical vein drains into? R or L?

Back 40

R umbilical vein obliterates by 2nd month

LEFT umbilical vein PERSISTS and drains into Ductus venosus (formed from central vitelline v) highest oxygenated blood

Front 41

Umbilical veins become?

Back 41

Left umb. v's closes shortly after umb. aa's close

L umbilical vein obliterates--> ligamentum teres hepatic "round ligament of liver"

Front 42

Left Brachiocephalic vein formed how? When?

Back 42

How: Anastomoses between Left Anterior Cardinal vein and Right Anterior Cardinal vein

When: Caudal part of Left Anterior Cardinal vein degenerates!!

Front 43

What happens to posterior cardinal veins?

Back 43

Majority degenerate!!!

Remaining portions=

1. Root of Azygos

2. Common iliac veins

5th Intersegmental artery becomes common iliac artery*

Front 44

What happens from the anastomoses of supra cardinal veins and sub cardinal veins?

Back 44

1. azygos and hemizygos veins

2. renal inferior vena cava

Front 45

Inferior vena cava formation

Back 45

1. hepatic- RIGHT vitelline v

2. prerenal- RIGHT subcardinal v

3. renal- anastomoses of sub and supra

4. postrenal- RIGHT supracardinal vein (left goes away)

Front 46

Absence of hepatic segment of IVC

Back 46

Failure of hepatic segment to form

Blood from caudal parts of body drains via collateral circulation of azygos and hemiazygos to R atrium

You won't die

Front 47

Double Inferior Vena Cava

Back 47

Inferior portion of left supra cardinal persists

Left IVC ends at left renal vein, and joins right IVC

Front 48

Superior Vena Cava formation

Back 48

Forms from anastomoses of

RIGHT anterior cardinal vein and RIGHT COMMON cardinal vein

Front 49

Double SVC

Back 49

1. Persistence of Left Anterior Cardinal vein (which is supposed to degenerate for brachiocephalic vein to form!!)

2. Failure of brachiocephalic vein to form

Result: left SVC drains blood from L side and dilates to accommodate the increased blood flow

Front 50

Left SVC in isolation

Back 50

1. Anastomoses of LEFT Anterior Cardinal v & LEFT COMMON cardinal vein

2. Obliteration of RIGHT common cardinal and RIGHT anterior cardinal vein ::: no R SVC is formed (this anastomosis is what normally makes the normal SVC)

Result: blood from R side drained by L SVC into R atrium via coronary sinus which enlargens due to increased blood flow

Front 51

Patent Ductus Arteriosus

Back 51

Occurs commonly in preterm infants. Can close spontaneously (by Day 3 in 60% of normal term neonates). Remainder are usually ligated and with little risk. Medications: NSAID's (ibuprofen) helps close PDA in premature babies only. NSAIDS block prostaglandin which keeps PDA open **rmr tx for transposition of great vessels