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075. Cardiopulmonary Exam 1 > 14. Heart/Congenital Heart Defects > Flashcards

14. Heart/Congenital Heart Defects Flashcards

1
Q

What day does the first heart beat?

A

22days (4th week) post fertilization

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2
Q

What is the heart derived from?

A

Splanchnic Mesoderm
Mesenchyme (NEURAL CREST)
Angioblastic tissue (blood vessels – later)

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3
Q

Heart fields are at the most cranial ends of the embryo, formed from splanchnic mesoderm. The primary heart fields are derived from the primitive streak during gastrulation and give rise to which parts of the heart?

A

Left and right atria and left ventricle

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4
Q

Secondary heart fields from pharyngeal arches (mesoderm) and give right to what parts of the heart?

A

Right ventricle and the outflow tract (aorta n pulmonary)

conus cordis/ truncus arteriosus

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5
Q

what does the intraembryonic ceolom give rise to?

A

the pericardial cavity

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6
Q

Cardiogenic cords are another name for the heart (primary heart fields). When first forming the heart, there are 2, one from each side. When and how do they come together?

A

During lateral/horizontal folding, the endocardial heart tubes approach eachother and fuse, forming the heart

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7
Q

A mesentary, also known as the dorsal mesocardium, does what?

A

a double layer of splanchnic mesoderm which provides a route for blood vessels, nerves, and lympathic to get to and from organs

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8
Q

All of the heart is from splanchnic mesoderm, including endo cardium, myocardium, and epicardium? Where are all of these layers?

A

Endocardium: internal endothelial lining
Myocardium: muscular wall
Epicardium: outer covering of heart

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9
Q

The dorsal mesocardium which connects the heart to the posterior body wall and will degenerate, to form the?

A

transverse pericardial sinus which can be used to block blood flow to aorta/pulmonary trunk for surgery

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10
Q

Neural crest cells also contribute and come from myelencephalon, which migrate from 3,4,6 and do what?

A

participate in the formation of the truncus arteriosus and aorticopulmonary septa

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11
Q

What are the genes that regulate the migration and differentiation of neural crest cells?

A

Hox genes, NF1, Pax3, and RETINOIC ACID

If too much retinoic acid from MOM, can lead to cardiac defects because disrupts migration of neural crest cells

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12
Q

During heart formation after fusion of the endocardial heart tubes from either side during lateral folding, forming the sinus venosus, atrium, ventricle, bulbus cordis, and truncus arteriosus. What do the venosus, bulbus and truncus do?

A

Sinus venosus embryological veins bring into heart

Bulbus cordis/truncus arteriosus, outflow track which both form into pulmonary tunk and aorta (DAY23)

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13
Q

By day 35, the truncus arteriosus forms what?

A

The aortic sac, coming from here, aortic arch arteris and then vessels form

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14
Q

Bulbus cordis and ventricles grow very quickly making the heart bend on itself forming what?

A

Bulboventricular loop which bends to the right, making the atrium and sinus venosus go dorsal or to the back and the ventricle moves downwards

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15
Q

A right handed folding loop is normal, what occurs during a left handed folding loop?

A

Dextrocardia: mirror image of what it should be, commonly with sinus inversus, heart sounnds will not be on the normal side

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16
Q

Septation events (all at the same time) start mid 4th week and complete by the 8th week. Why does septation need to occur?

A

To seperate the ventricles into left and right, the atriums into left and right, outflow track into pulmonary and aortic

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17
Q

Atrioventricular canal septation involves Endocardial (AV) cushions from mesoderm on the dorsal and ventral walls, cusions grow together. How do they fuse?

A

Ventral and dorsal endocardial cusions migrate and fuse together. forming ventricular canals going from atria to ventricles

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18
Q

Pectinate muscles are the primitive heart tube, anything that is smooth in the atrium is from sinus venosus (venous structure into heart). Which contains a left and right horn. What forms from these?

A

Left horn is remodeled into the coronary sinus

Right horn is remodeled is incorporated into the wall of the atrium.

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19
Q

Orficies (opening) of the superior and inferior vena cava veins and the orfice of the coronary sinus comes from what?

A

Right horn of sinus venosus

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20
Q

The right horn of the sinus will fold on itself making the right sinal atrial orfice, which has what on either side?

A

Left and right sinuatrial (venous) valves, which fuse cranially and caudally and fuse with septum spurium

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21
Q

The cranial right sinuatrial / venous valve gives rise to the crista terminalis which does what?

A

seperates the pectinate musclle from the sinus venosum

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22
Q

The caudal right sinuatrial / venous valve will give rise to what valves?

A

valve of the coronary sinus and the valve of the inferior vena cava

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23
Q

Septation of the atrium is done by forming two different septums, septum primum and septum secundum. What are the foramen for the septum primum and what do they do?

A

Foramen primum: shunt between right and left atrium, fuses with endocardial cushions
Formane secundum: forms before foramen primum disappears, strengthens formane primum shunting

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24
Q

What does the septum secundum, which overlaps septum primum form?

A

foramen ovale, which fuses with endocardial cusion and has a hole in the septum secundum for embryological blood flow

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25
Q

What does the hole in foramen secundum from the septum primum and the hole in the foramen ovale from the septum secundum permit?

A

allows blood flow from the right atrium to the left atrium, shunting by early 7th week

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26
Q

What is the first septum in the embryological heart?

A

Fossa ovale

27
Q

Ventricle septation is done by interventricular septum, the muscular portion. What does the interventricular septum do?

A

Migrates up to the endocardial cusion, but DOES NOT make it all the way, leaving an interventricular foramen

28
Q

Bulbus cordis and truncus arteriosus septation is initiated by neural crest cells from pharyngeal arches 4&6, do what?

A

migrate into the truncus arteriosus and bulbus cordis (conus cordis), forming ridges

29
Q

Neural crest cells form ridges called what on the bulbus cordis and truncus arteriosus?

A

bulbar ridges/ conotrunkal ridges and truncal ridges

30
Q

The neural crest cells form the aorticopulmonary septum (separtaing) as it forms, what does it do?

A

It will rotate 180 degrees, dividing bulbis cordis and truncus arteriosus into ascending aorta and pulmonary trunk… FUSES WITH ENDOCARDIAL CUSION

31
Q

The bubus cordis (inferior part) on the right and left side will form what?

A

Right side forms conus arteriosus (infundibulum)

left side forms aortic vestibule

32
Q

The bulbar and truncal ridges migrate to the middle and fuse, forming what?

A

the aorticopulmonary septum, which spirals

33
Q

Once the musclar portion of the interventricular septum is formed, there is still a hole near the endocardium cusion where it did not fuse, what covers the hole?

A

membranous interventricular septum made from endocardial cushion, and NC R/L bulbar ridges

34
Q

Once the right and left bulbar ridges fuse to the endocardium cushions, the interventricular foramen disappears and forms what?

A

Membranous part *** MOST COMMON defect in membrane d/t Neural crest

35
Q

What does the membranous interventricular divide?

A

separate left and right ventricle, also separates left ventricle from right atrium

36
Q

To form semilunar and atrioventricular valves, right and left bulbar ridges from NC fuse, and dorsal/ventral valve swelling is derived from?

A

Mesoderm and NC (mesenchyme)

37
Q

Heart has been beating since day 22. How do the valves form?

A

Blood if forced through the developing valves and when at rest, blood drops down, hitting the ventral and dorsal swelling, eroding, forming strong CT and the cusps!

38
Q

What occurs to the baby when there is no shunt or a left to right shunt?

A

Acyanotic bb

39
Q

What occurs to the baby when there is a right to left shunt?

A

cyanotic bb

40
Q

In fetal circulation, we are not oxygenating our own blood so what occurs?

A

We shunt blood from right atrium to left atrium, which from there it will pump to the baby’s body

41
Q

if the ductus arteriosus if still prevelant after birth (connects pulmonary trunk to aorta) what will happen?

A

Will be acyanotic, because the high pressure from the aorta will cause blood to go into pulmonary trunk, into lungs, so rich O2 blood is shunted to O2- blood, Left to right shunt

42
Q

What are the main causes of patent ductus arteriosus (PDA) (2)? Treatments?

A

1Low oxygen content, 2 too must prostaglandin E2 (PGE2-vasodilator), produced by placenta mediated by COX2
Treatment is to give COX2 inhibitors, ibuprofen, indomethacin, will close 72 hours after birth

43
Q

What are characteristics of PDA?

A

continuous murmur, poor eating, sweating crying, if persists can destroy capillary beds in lungs

44
Q

On xray, PDA can be seen by pulmonary edema in lungs, and what?

A

heart is enlarged

45
Q

Atrial septal defects are more common in females than males. Can occur in secundum ASD (high) or primum ASD (LOW). What happens?

A

Baby is acyanotic, because blood is shunted from the left atrium to the right side.

46
Q

Ostium/foramen secundum defect, includes patent foramen ovale. How is this caused?

A

excesive cell death during foramen secundum, and reabsorption of septum primum or by inadequate development of septum secundum

47
Q

What causes probe patent foramen ovale in 25% of people?

A

No direct access from left to right, but can put probe through one atrium to the other. -incomplete adhesion between foramen ovale and septum secundum

48
Q

foramen/ Ostium primum, low Atrioseptal defects occur how?

A

septum premum does not fuse with the endocardial cushion, associated with mitral valve cleft

49
Q

Ventricular septal defects occur in more male than females… does it cause cyanosis?

A

Ventricular septum defects cause a hole in the left ventricle to right ventricle, cause acyanosis because the pressure from the left pumps it O2 blood into deoxygenated, but still gets O2 blood to body

50
Q

Ventricular septal defects are caused by what embryological defect?

A

Neural crest issues: membranous part not formed by bulbar ridges and endocardium cushion

51
Q

What can be heard during VSDs?

A

murmur in left/lower sternal border or left 3-4th intercostal space

52
Q

AV septal defects have complete defects which are characterized by a primum ASD that is contiguous with a VSD and a common AV valve. What is characteristic of a partial AV septal defect?

A

characterized by a primum ASD, a single AV valve annulus with 2 separate valve orfices…. anterior leaflet of mital valve is cleft

53
Q

What is the embryological basis for the AV septal defect?

A

Endocardial cushions do not fuse leading to a left to right shunt = acyanotic, common in 20% of people with Downs

54
Q

What features do you commonly see with atrioventricular septal defects? (3)

A
  1. Atrial septal defect
  2. Ventricular septal defect
    3, Abnormal valve leaflet

All 3 for complete, 1&3 for partial

55
Q

What defect involves the right atrium going to left ventricle, left atrium going to right ventricle, right ventricle going to aorta, and left ventricle going to pulmonary trunk AND interventricular septal defect?

A

Corrected transposition of the Great Vessels

56
Q

Is corrected transposition of the great vessels defect acyanotic or cyanotic, and what embryological event does this?

A

Acyanotic because left to right shunt, caused by neural crest and improper separation of outflow tract.
Reversed rotation of the heart

57
Q

What occurs in the defect: transposition of great vessels?

A

great vessels are from wrong ventricles, septal defects VSD or ductus arteriosus needed! otherwise will never get oxygenated blood to body

58
Q

How does transposition of great vessels occur embryologically?

A

CYANOTIC because oxygenated blood goes to pulmonary trunk

improper spiraling of the neural crest cells, no 180 degress spiral.

59
Q

Double outlet right ventricle causes a right to left shunt, cyanosis. How does this occur?

A

Abnormal migration of bulbar ridges during septation, and misalignement in ventricular of interventricular septtum because right ventricle is so big, it sends it blood to both aorta and pulmonary trunk

60
Q

What defect is truncus arteriosus and what causes it?

A

Is a single great vessel, cause by a interventricular septal defect, right to left shunt-cyanotic caused by absence of bulbar/truncal ridges to form/migrate to the midline and form separate valves

61
Q

What defect is very serious and common, and presents with pulmonary stenosis-small, interventricular septal defect, over-riding aorta-large and right ventricular hypertrophy?
Cyanotic, right to left shunt, and caused by abnormal septation of the outflow tract (NC)

A

Tetralogy of Fallot

62
Q

What is commonly seen with critical pulmonary stenosis?

A

cusps of pulmonary valves are fused or thickened, leading to cyanosis d/t decreased blood flow to lungs

63
Q

What is commonly seen with critical aortic stenosis?

A

tachypnea, poor feeding, poor perfusion, can lead to hypoplastic left heart syndrome

cyanotic d/t decreased systemic blood flow

64
Q

What defect includes features of mitral valve stenosis, left ventricle hypoplastic, aortic valve stenosis, and aortic arch hypoplastic
Cyanotic due to NO LEFT VENTRICLE, PDA and ASD will help with defect so patient does not die

A

Hypoplastic Left Heart Syndrome

075. Cardiopulmonary Exam 1 (23 decks)

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