CVS 2 Flashcards

(94 cards)

1
Q

A 3-year-old child is seen by his mother to suddenly turn blue around the lips after an episode of crying. This has happened many times previously and the child quickly adopts the squatting position to relieve his symptoms. On previous examinations, the child has been seen to have multiple cardiac problems, including a boot-shaped heart on chest x-ray.

Based on the most likely diagnosis, what is the most likely diagnosis and what occurs in this?

A

Tetralogy of Fallot

failed migration of neural crest cells contributes to the anterior and superior displacement of the infundibular septum - septum is malpositioned

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2
Q

Give 4 examples of cyanotic heart defects.

A

cyanotic:
- TOF
- tricuspid atresia
- transposition of great arteries
- hypoplastic left heart

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3
Q

Give 4 examples of acyanotic heart defect.

A

acyanotic:
- atrial septal defect
- patent foramen ovale
- ventricular septal defect
- coarctation of aorta

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4
Q

A baby is premature, born at 30 weeks, putting her at higher risk for delayed ductus venosus closure.

Which structures does the ductus venosus act as a communication between in the fetus?

A

umbilical vein to IVC

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5
Q

A 2-week-old female is referred to the paediatrician due to poor feeding and poor weight gain. On cardiac auscultation, a continuous ‘machine-like’ murmur is heard. An echocardiogram reveals a patent ductus arteriosus (PDA).

If this structure had closed at birth, what would the remnant be called?

A

ligamentum arteriosum

(small fibrous band connecting pulmonary trunk to the aortic arch)

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6
Q

A 20-year-old man has been found to have an abnormal electrical connection in his heart causing him to have frequent palpitations, dizzy spells, and shortness of breath. Delta waves are also noted on the ECG. Ablation of the coronary sinus is considered as a treatment for this condition.

What is the condition?

The target for this surgery is derived from which embryological structure?

A

condition: Wolff-Parkinson-White syndrome (delta waves on ECG are giveaway)

in WPW there’s an accessory conduction pathway that bypasses the AV node, causing pre-excitation of the ventricles

they’re talking about ablation of the coronary sinus which develops from the LEFT HORN of the SINUS VENOSUS

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7
Q

What cardiac structures close in a newborn within a few hours of birth? Explain.

A

foramen ovale
- septum primum and septum secundum are forced together which closes foramen ovale

ductus arteriosus
- vessel connecting the pulmonary artery to the aorta, letting blood bypass the non-functional fetal lungs
- closes and becomes ligamentum arteriosum
- if stays open = PDA - blood flows from aorta to pulmonary artery

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8
Q
  1. What is the function of the foramen ovale in the fetus?
  2. What is the adult remnant of the foramen ovale?
  3. What triggers the closure of the foramen ovale after birth?
A
  1. allows blood to flow from right atrium to left atrium, bypassing the lungs
  2. the fossa ovalis
  3. drop in pulmonary pressure after lung expansion increases left atrial pressure, pushing septa together
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9
Q
  1. What is the function of the ductus arteriosus in the fetus?
  2. What is the adult remnant of the ductus arteriosus called?
  3. What causes the ductus arteriosus to close after birth?
  4. What happens if the ductus arteriosus remains open after birth?
A
  1. connects pulmonary artery to the aorta, diverting blood away from the non-functional fetal lungs
  2. ligamentum arteriosus
  3. increased oxygen tension and reduced prostaglandins
  4. PDA causes left to right blood shunting - pulmonary hypertension, HF, arrhythmias
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10
Q

In foetal circulation, what is the role of the following:

  1. Placenta
  2. Ductus Venosus
  3. Foramen Ovale
  4. Ductus Arteriosus
A
  1. acts as a site of gas exchanged, nutrient delivery, waste removal
  2. shunt blood from the umbilical arteries and one umbilical vein
  3. allows blood to flow from right to left atrium, bypassing the lungs
  4. connects the pulmonary artery to the aorta, bypassing the lungs
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11
Q

Fill in the gaps:

Developing __1__ receives __2__ blood from the mother via __3__ and __4__ vein.

A
  1. embryo
  2. oxygenated
  3. placenta
  4. umbilical
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12
Q

Label the image.

A
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13
Q

What are the 3 foetal circulatory shunts and what do they do?

A
  1. ductus venosus
    - foetal blood vessel connecting umbilical vein to IVC
    - carries mostly highly oxygenated blood away from the liver
  2. foramen ovale
    - shunts highly oxygenated blood from right atrium to left atrium
  3. ductus arteriosus
    - connects aorta and pulmonary artery shunting blood away from lungs
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14
Q

Which foetal shunt allows blood to move from the right atrium to the left atrium?

A

foramen ovale

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15
Q

What foetal shunt connects the umbilical vein to the IVC, carrying mostly highly oxygenated blood away from the liver?

A

ductus venosus

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16
Q

What foetal shunt connects the aorta and the pulmonary artery shunting blood away from the lungs?

A

ductus arteriosus

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17
Q

What does the truncus arteriosus give rise to?

A

ascending aorta and pulmonary trunk

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18
Q

A 65-year-old male presents to the emergency department with sudden onset of severe chest pain. He describes the pain as ‘ripping through his body’. He is quickly diagnosed with a Stanford Type A aortic dissection and taken off for surgical repair.

Which of the following embryonic structures gives rise to the area in which the dissection occurred?

A

truncus arteriosus

(stanford type A aortic dissection takes place in the ascending aorta)

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19
Q

A mother brings her baby to the Emergency Department after she notices a pale yellow fluid leaking from its umbilicus. Her doctor explains that this is because of the failed closure of what used to be part of the embryological umbilical ligament.

What is the name of this remnant?

A

urachus

(remnant of the allantois, which is the remnant of the embryological umbilical ligament)

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20
Q

A 35-year-old man presents to his general practitioner (GP) complaining of a fever and malaise. On questioning the GP finds out that he is has been using IV drugs for the past few years and after careful examination suspects infective endocarditis.

The structure most likely affected in this patient is derived from what embryological structures?

A

endocardial cushion

infective endocarditis in patients positive for IVDU most commonly affects tricuspid valve - all valves in the heart are derived from endocardial cushion

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21
Q

A 4-week old boy is brought to the general practitioner (GP) after his mother has seen daily yellowish drainage coming from his umbilicus. The infant had an uncomplicated delivery and is otherwise well.

This problem is most likely caused by abnormal development of which embryological structures?

A

allantois

(persistent allantois can lead to a patent urachus - presents are urinary drainage from the umbilicus)

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22
Q

What does the right atrium develop from?

A

primitive atrium and sinus venosus

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23
Q

What is an unfused fossa ovalis called?

A

patent foramen ovale

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24
Q
  1. What is an Atrial Septal Defect?
  2. What shunting does this lead to?
  3. This can lead to what enlargement?
  4. What complications can this cause?
A
  1. hole/opening in the septum between the atria which persists postnatally and allows blood to flow between the two atria - left atrial pressure is higher than the right
  2. left-to-right shunting because left atrial pressure is normally higher = pushes oxygenated blood back into right atrium
  3. right atrial and ventricular enlargement
  4. pulmonary hypertension, arrhythmias, eisenmenger syndrome
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25
What are the symptoms of Atrial Septal Defect?
early life: - asymptomatic adult life: - dyspnoea - fatigue - recurrent RTIs - palpitations
26
What is the most common congenital heart defect? What is it associated with?
ventricular septal defect - fetal alcohol syndrome - chromosomal abnormalities e.g down syndrome
27
What do the images show?
anatomical variations of VSDs most common is perimembranous VSD
28
What are the main differences between VSD and ASD?
VSD - opening between ventricles - more severe than ASD, can cause HF early - failure of interventricular septum (muscular or membranous) formation ASD - opening between atria - may be asymptomatic early, later on risk of stroke, hypertension, arrhythmias - failure of atrial septa formation or endocardial cushion defects
29
Why do both VSDs and ASDs present with left-to-right shunt?
because pressure in left ventricle (VSDs) and left atria (ASDs) is greater than in the right ventricle and right atria - therefore oxygenated blood flows from right side to left
30
What is a Ventricular Septal Defect?
opening in the septum between the ventricles which persists postnatally
31
What kind of murmur occurs in Ventricular Septal Defect?
S1-S2 pansystolic murmur (caused by continuous leak of blood during the entire ventricular contraction phase)
32
What kind of murmur occurs in Atrial Septal Defect?
splitting of S2 sound and systolic murmur
33
What is Patent Ductus Arteriosus?
it is when the ductus arteriosus - blood vessel connecting the pulmonary artery to the aorta during foetal life - fails to close after birth causes blood to flow abnormally for the aorta back into the lungs - increases workload on the heart
34
1. What is the ductus arteriosus and what is its role in fetal circulation? 2. What happens to the ductus arteriosus after birth? 3. What is patent ductus arteriosus (PDA)? 4. What type of shunt occurs in PDA and what are the consequences?
1. fetal blood vessel connecting the pulmonary artery to the aorta, allowing blood to bypass the non-functioning foetal lung 2. normally closes within first few days after birth and becomes ligamentum arteriosum 3. failure of the ductus arteriosus to close after birth - results in persistent blood flow between aorta and pulmonary artery 4. left-to-right shunt, increased pulmonary blood flow, pulmonary hypertension, HF, arrhythmias
35
What type of murmur would you hear for the following: 1. VSDs 2. ASDs 3. PDA
1. S1-S2 pansystolic murmur 2. splitting of S2 sound and systolic murmur 3. continuous 'machinery' murmur
36
What is Tetralogy of Fallot? List 3 risk factors.
congenital heart defect comprising of four abnormalities: - VSD - pulmonary stenosis - overriding aorta - right ventricular hypertrophy - PKU in mother - alcoholism in mother - diabetes
37
What is a characteristic finding of TOF on x-ray?
boot-shaped heart
38
A 24-year-old woman presents with progressive exertional breathlessness over 6 months and occasional palpitations after heavy exercise. She has a soft ejection systolic murmur at the left upper sternal edge, wide fixed splitting of S2, mild postural dizziness, and a small café-au-lait macule. CXR shows mild cardiomegaly and prominent pulmonary arteries. ECG reveals incomplete RBBB and right axis deviation. Echocardiogram demonstrates mild RA and RV dilation, left-to-right flow at the atrial level, and mild tricuspid regurgitation. What is the most likely diagnosis and the underlying haemodynamic abnormality?
Atrial Septal Defect (ASD), secundum type. Underlying haemodynamic abnormality → Left-to-right shunt at the atrial level → ↑ pulmonary blood flow → RA & RV dilation → wide fixed splitting of S2.
39
An 11-month-old boy presents with slow growth, feeding fatigue, and 3 episodes of bronchiolitis/pneumonia this year. Exam reveals: - Pansystolic murmur at the left lower sternal edge with palpable thrill - Mild hepatomegaly and fine basal crackles - Mild tachypnoea but no cyanosis Investigations show: - ECG → sinus tachycardia, biventricular hypertrophy - Echocardiogram → moderate perimembranous VSD, left-to-right shunt, mild LV & LA dilation, trace TR What is the most likely diagnosis and underlying haemodynamic abnormality?
Diagnosis → Ventricular Septal Defect (VSD), perimembranous type Haemodynamics → Left-to-right shunt at ventricular level → ↑ pulmonary blood flow → volume overload in LA/LV → pulmonary congestion & recurrent chest infections.
40
A 2-month-old preterm infant (born at 34 weeks) presents with feeding fatigue, tachypnoea, and poor weight gain. Exam shows: - Continuous “machinery-like” murmur at the left infraclavicular area - Bounding pulses & wide pulse pressure (80/40 mmHg) - Bibasal crackles but no cyanosis - Echocardiogram would likely reveal a vascular connection between the pulmonary artery and the aorta. What is the most likely diagnosis and underlying fetal structure involved?
patent ductus arteriosus (PDA) Underlying fetal structure → Ductus arteriosus (normally closes after birth, becomes ligamentum arteriosum) Haemodynamics → Left-to-right shunt → ↑ pulmonary blood flow → pulmonary congestion, feeding difficulty, failure to thrive
41
A 2-week-old boy has recurrent episodes of cyanosis during crying and feeding. Parents note that he sometimes squats after crawling. Examination shows SpO₂ 88%, harsh ejection systolic murmur at the left upper sternal border, single S₂, and boot-shaped heart on CXR with decreased pulmonary vascular markings. ECG shows right axis deviation and RV hypertrophy. What is the most likely diagnosis and the embryological defect responsible?
tetralogy of fallot (TOF) - cyanotic congenital heart defect - harsh ejection systolic murmur (pulmonary stenosis) - squatting relieves symptoms (high systemic vascular resistance) - boot-shaped heart on CXR (RV hypertrophy) - cyanosis (overriding aorta) due to anterocephalad deviation of the infundibular (conal) septum → malalignment VSD and RV outflow obstruction
42
Which foetal structure becomes the coronary sinus in the adult heart?
left sinus horn
43
Fill in the gaps: The right anterior cardinal vein becomes the ___1___, while the left anterior cardinal vein contributes to the formation of the __2____.
1. SVC 2. left brachiocephalic vein
44
The spiral nature of the aorticopulmonary septum ensures what?
correct alignment of the aorta and pulmonary trunk
45
Which congenital heart defect is most commonly associated with Turner syndrome?
coarctation of the aorta
46
List three possible complications of a large PDA that remains open into adulthood.
pulmonary hypertension right ventricular hypertrophy respiratory distress syndrome
47
Fill in the gaps: In a secundum ASD, blood flows from the ___1___ atrium to the ___2___ atrium due to the pressure gradient.
1. left 2. right
48
Which congenital defect is classically associated with differential cyanosis (cyanosis in the lower limbs but not the upper limbs)? a. Coarctation of the aorta b. Ventricular septal defect c. Tetralogy of Fallot d. Transposition of the great arteries
coarctation of the aorta
49
A newborn is diagnosed with transposition of the great arteries (TGA). Explain why survival depends on another concurrent shunt.
in TGA the aorta (normally carries oxygen-rich blood from the left ventricle to the body) comes off the right ventricle and the pulmonary artery (carries oxygen-poor blood from right ventricle to lungs) comes off the left ventricle basically the aorta and pulmonary artery are switched - means oxygen poor blood to circulate around the body and oxygen-rich to circulate in lungs only not compatible with life so shunt is needed after birth
50
Fill in the gaps: Eisenmenger syndrome can develop in large unrepaired ventricular septal defects. Chronic left-to-right shunting leads to increased pulmonary blood flow and pulmonary vascular ______1_____. Eventually, reversing of the shunt leads to the clinical sign of _____2______.
1. hypertension 2. cyanosis
51
What is the blood flow order in the heart?
1. IVC/SVC (deoxygenated blood, towards heart) RA -> tricuspid valve -> RV RV -> pulmonic vavle -> pulmonary artery -> lungs _____________ 2. Lungs (oxygenated, away from heart) pulmonary vein -> LA LA -> mitral valve -> LV LV -> aortic valve -> aorta -> rest of body
52
Which veins bring deoxygenated blood back to the heart?
IVC and SVC
53
After entering the right atrium, through which valve does blood flow into the right ventricle?
tricuspid valve
54
From the right ventricle, where does the blood go next?
through pulmonary valve into pulmonary artery - carries blood to the lungs
55
1. Into which heart chamber does the oxygenated blood from the lungs enter? 2. From this structure, which valve does the blood pass through to reach the next structure?
1. left atrium 2. mitral (bicuspid) valve
56
Summarise the path of blood flow starting from the body and returning back to it.
Body → IVC/SVC → RA → tricuspid valve → RV → pulmonary valve → pulmonary artery → lungs → pulmonary veins → LA → mitral valve → LV → aortic valve → aorta → body.
57
A 52-year-old man presents to the general practice complaining of shortness of breath on exertion. He has no chest pain. There is no cough. He has no history of smoking. On cardiac auscultation, you find an ejection systolic murmur. Suspecting a valvular defect to be the cause of the gentleman's symptoms, you refer him for echocardiography. The imaging shows no evidence of a valvular defect but does show an atrial septal defect (ASD). An ASD allows the shunting of blood between the left and right atria. In foetal life, the left and right atria are connected by which structure?
foramen ovale
58
A 65-year-old man with heart failure presents to his GP. He has a stroke volume of 33% and asks what affects stroke volume. 1. What would increase stroke volume in a normal individual? 2. What are the 4 factors affecting stroke volume?
1. increased central venous pressure (stroke volume is the amount of blood pumped out of the left ventricle) - cardiac size - contractility - preload - afterload
59
A fourth-year medical student is asked to examine the abdomen of a 59-year-old man who presented to hospital with generalised abdominal pain. He has a past medical history of chronic obstructive pulmonary disease. The student reported generalised abdominal tenderness but with no peritonism, masses or organomegaly. The student described the liver as 'bouncing up and down periodically' on the tips of her fingers. What is the likely cause for this? Why?
tricuspid regurgitation - presents with pulsatile hepatomegaly due to backflow of blood into the liver during cardiac cycle
60
What does the 1st aortic arch become? What does the 2nd aortic arch? What does the 3rd aortic arch become?
mostly regresses but contributes to the MAXILLARY ARTERY mostly regresses, but forms the STAPEDIAL and HYOID arteries COMMON CAROTID ARTERIES and PROXIMAL INTERNAL CAROTID ARTERIES
61
What does the right and left 4th aortic arch form?
left: part of aortic arch right: part of subclavian artery
62
What does the left and right 6th aortic arch form?
left: pulmonary artery and ductus arteriosus right: right pulmonary artery
63
What do the left and right 7th intersegmental artery become?
left and right subclavian
64
What does the left dorsal aorta become? What happens to the right dorsal aorta?
left: descending thoracic aorta right: mostly regresses but contributes to part of the right subclavian artery
65
What do the left and right aortic sac contribute to?
left: part of the aortic arch right: brachiocephalic trunk
66
What is the purpose of the Vitelline system?
drains the GI tract and gut derivatives - forms the portal venous system - contributes to part of the hepatic sinusoids (blood channels in the liver)
67
What is the purpose of the Umbilical System?
carries oxygenated blood from the placenta to the embryo fate: - right umbilical vein disappears - left umbilical veins persists, forms connection with the ductus venosus = shunts oxygenated blood directly into IVC bypassing liver - AFTER BIRTH = left umbilical vein closes -> ligamentum teres hepatis ductus venosus closes -> ligamentum venosus
68
What is the purpose of the Cardinal System?
drains the head and neck and body wall, major venous drainage of the embryo itself fate: - forms the systemic venous system SPECIFICALLY: - anterior cardinal veins = internal jugular veins, SVC - posterior cardinal veins mostly regress - subcardinal and supracardinal veins -> IVC segments, renal veins
69
Which veins form a portal system that drains blood from the abdominal foregut, the midgut, and the upper part of the anorectal canal?
right and left vitelline veins
70
What do the right and left vitelline veins do?
form a portal that drains blood from the abdominal foregut, the midgut and the upper part of the anorectal canal
71
Which umbilical vein disappears and which one persists? The vein which persists forms what after birth?
right umbilical vein disappears left umbilical vein persists -> forms ligamentum teres hepatis after birth
72
Which vein anastomoses with a structure in the liver? What is the purpose of this?
left umbilical vein anastomoses with the ductus venosus in the liver shunting oxygenated placental blood into the IVC and to the right side of the heart
73
What do the panels show?
panel A: EARLY STAGE - embryo has paired vitelline veins (drain yolk sac) and paired umbilical veins (bring oxygenated blood from placenta) - veins drain into the SINUS VENOSUS (primitive heart inflow) - liver buds starting to grow and vitelline veins pass through the panel B: HEPATIC SINUSOIDS FORM - liver grows and vitelline veins fragment into a network of channels called hepatic sinusoids - umbilical and vitelline blood now flows through liver sinusoid before reaching heart panel C: DUCTUS VENOSUS appears panel D: FINAL REMODELING - near birth - right vitelline vein = hepatic portion of the IVC and hepatic veins - left vitellin vein = contributes to portal vein - left umbilical vein = carries oxygenated blood from placenta -> through ductus venosus to IVC
74
What are the primary venous structures in the early embryo responsible for draining blood from the body back to the heart?
cardinal veins
75
How many types of cardinal veins are there? What is their function?
1. ANTERIOR CARDINAL VEINS - drain blood from the head and upper body - right anterior cardinal vein = SVC (returns deoxygenated blood from upper half of the body to the right atrium of the heart) - left anterior cardinal vein = mostly regresses but contributes to formation of left brachiocephalic vein 2. POSTERIOR CARDINAL VEINS - drain blood from lower body - largely replaced by the SUBCARDINAL and SUPRACARDINAL veins as embryo develops (remaining portions contribute to the azygos and hemiazygos) 3. SUBCARDINAL VEINS - initially drain the mesonephroi and later contribute to development of of permanent venous system - form portions of the IVC (which returns deoxygenated blood from lower half of the body to right atrium) - develops into renal veins (drains kidneys) - form gonadal veins (ovarian in females and testicular in males) 4. SUPRACARDINAL VEINS - develop later to support venous return from the body wall - contribute to the formation of the azygos and hemiazygos veins = drain the thoracic wall - form part of the IVC
76
1. What do the anterior cardinal veins drain in the embryo? 2. What does the right anterior cardinal vein form in adults? 3. What happens to the left anterior cardinal vein?
1. head and upper body 2. SVC 3. mostly regresses but contributes to the left brachiocephalic
77
1. What do the posterior cardinal veins drain? 2. What replaces the posterior cardinal veins during development? 3. What do the remaining portions of the posterior cardinal veins form?
1. lower body of embryo 2. subcardinal and supracardinal veins 3. parts of the azygos and hemiazygos veins
78
1. What do the subcardinal veins initially drain? 2. What adult structures are formed from the subcardinal veins? 3. What adult structures are formed from the supracardinal veins?
1. the mesonephroi (embryonic kidney) 2. portions of the IVC, renal veins, gonadal veins 3. azygos and hemiazygos and part of the IVC
79
Which embryonic veins drain blood from the head and upper body?
anterior cardinal veins
80
Which embryonic vein forms the superior vena cava (SVC)?
right anterior cardinal vein
81
Which embryonic vein mostly regresses but contributes to the left brachiocephalic vein?
left anterior cardinal vein
82
Which embryonic veins drain blood from the lower body?
posterior cardinal veins
83
Which venous systems replace the posterior cardinal veins during development?
subcardinal and supracardinal
84
Label the image.
85
In the 5th week, the pulmonary vein starts to undergo incorporation into what?
left dorsal wall of the primitive (primordial) atrium
86
In the 5th week, which vein starts to undergo incorporation into the left dorsal wall of the primitive atrium?
pulmonary vein
87
The muscular part of the interventricular septum is formed by the end of which week? This occurs as a result of what?
by end of week 7 as a result of dilation of the two primitive ventricles (right and left conus swellings) which causes the medial walls to become apposed and fuse together the proliferation of the right and left conus swellings, combined with the proliferation of the inferior atrioventricular cushion, eventually closes the interventricular foramen and forms the membranous portion of the IV septum
88
What forms the membranous portion of the IV septum?
proliferation of the right and left conus swellings, combined with the proliferation of the inferior atrioventricular cushion, eventually closes the IV foramen and forms the membranous portion of the IV septum
89
Septation occurs between which two weeks? This changes the heart from a what to a what?
week 4 and week 7 from a single-channel peristaltic pump to a dual-channel, synchronously contracting device with one-way valves - FORMS 3 MAJOR SEPTA
90
Which 3 major septa are formed during septation of the heart?
1. INTERATRIAL SEPTUM FORMATION - starts with primary septum growing down from the roof of the atrium toward the endocardial cushions - gaps remain: FORAMEN PRIMUM and FORAMEN SECUNDUM - secondary septum grows, leaving an oblique flap = FORAMEN OVALE - ALLOWS RIGHT TO LEFT SHUNT OF BLOOD IN THE FETUS 2. INTERVENTRICULAR SEPTUM FORMATION - separates left and right ventricle - if membranous part fails to form - VSD 3. SEPTATION OF VENTRICULAR OUTFLOW TRACT - a spiral aorticopulmonary septum forms (from neural crest cells) -> it twists to divide the TRUNCUS ARTERIOSUS INTO PULMONARY TRUNK AND AORTA
91
What is the role of the primary septum in atrial development? What is the role of the secondary septum?
it grows down toward the endocardial cushions - leaving the foramen primum (which later closes) it forms next to the primary septum, leaving the foramen ovale (a flap valve)
92
What is the role of the foramen secundum?
it forms in the upper primary septum to maintain right to left blood flow
93
Which septum forms between the ventricles? How does this form?
the interventricular septum a muscular septum grows upward from the apex, then a membranous part closes the gap via the endocardial cushions and neural crest
94
The IVC is a large vein that carries the deoxygenated blood from the lower and middle body into the right atrium of the heart. The IVC is formed during a left-to-right shift in the embryonic veins. What are its 4 parts derived from?