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63 Cards in this Set

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In development, the heart folds into an S shape. Where is the arterial chamber and ventricular chamber located?

The arterial chamber lays posterior in S while ventricular chamber lays anterior.

Arterial chamber grows and incorporates what two structures?

Superior vena cava and pulmonary vein

How is blood shunted from the right atrium to the left atrium in the fetus?

Through foramen ovale (of the septum secundum) and ostium secundum (of septum primum)

The truncus arteriosus gives rise to...

Ascending aorta and pulmonary trunk

The bulbus cordis gives rise to...

Smooth parts (outflow tract) of left and right ventricles.

The primitive atria gives rise to:

Trabeculated part of the left and right atria

The primitive ventricle gives rise to:

Trabeculated part of the left and right ventricles

The primitive pulmonary vein gives rise to:

Smooth part of left atrium

Coronary sinus comes from which embryonic structure?

Left horn of sinus venosus

Smooth part of the right atrium comes from which embryonic structure?

Right horn of sinus venosus

Right common cardinal vein and right anterior cardinal vein give rise to:

Superior vena cava

What structure closes the opening between the atrial chamber and the ventricular chamber?

Superior endocardial cushion and inferior endocardial cushion

What disease is associated to endocardial cushion defects?

Trisomy 21

What are the causes of arterial septal defects (ASD)?

Ostium secundum overlapps foramen ovale, Absence of septum secundum, Neither septum secundum nor septum primum develop.

What structure divides truncus arteriosus into aorticpulmonary trunk? What is the origin?

Spiral septum, neural crest derivative

What are the complications of a truncus arteriosus rotation gone wrong?

- Transposition of great vessels


- Tetralogy of Fallot


- Persistent truncus arteriosus


- Dextrocardia, fenestrae, VSD


Explain first 2 steps in ventricle morphogenesis:

muscular ventricular septum forms , Aorticopulmonary septum rotates and fuses with muscle to form membranous intraventricular septum with closes the interventricular foramen.

Endocardial cushion functions:

seperates atria from ventricles and contributes to both atrial seperation and membranous portion of the interventricular septum

How are the interventricular valves formed?

The ventricle remodels.


- The myocardial erodes causing ventricles to become enlarged-residual tissue becomes fibrous and forms chordae tendoneae. This leads to the formation of papilary muscles and AV valves.

What induces the aorticopulmonary septum?

Truncus arteriosus rotates and neural crest and endocardial cells migrate, truncal ridges spiral 180* and form the aorticopulmonary septum.

What are the three fetal shunts?

Ductus venosus, foramen ovale and ductus arteriosus

Organize blood vessles from most oxygenated to less oxygenated in fetal circulation from the moment it leaves the placents until it reaches the RA:

Umbilical vein, ductus venosus, Inferior vena cava

Explain how the foramen ovale closes:

At birth, infants takes a breath; causing decreased resistance in pulmonary vasculature and increased left atrial pressure which closes foramen ovale.

Explain how the ductus arteriosus closes:

Increased O2 from respiration and decreased PG from placental seperation induce closure of ductus arteriosus.

What drug helps close PDA (patent ductus arteroisus)? How?

Indothecin by inhibiting PG

What drug helps keep PDA (patent ductus arteriosus) open? How?

PG-E1 and E2 by dilating vessels

Who maintains right to left shunt as septum secundum is forming?

foramen secundum


Name the most common left to right shunts in congenital heart disease (in order of frequency)

- Ventricular septal defect (VSD)


- Atrial septal defect (ASD)


- Patent ductus arteriosus (PDA)


What is the most common congenital cardiac defect?

VSD

Which CHD is generally asymptomatic, may manifest weeks after birth or remain so throughout life, and many times resolves on its own after 6 months?

VSD, if it does not resolve may lead to LV overload and heart failure.

Cardiac left to right shunt that usually occurs in septum secundum and has a loud S1 and a wide, fixed split S2

Atrial septal defect

Congenital heart defect that is associated with a continuous, machine like murmur.

Patent ductus arteriosus

What mantains patency in PDA?

PGE synthesis and low O2 tension

Uncorrected PDA can eventually result:

Late cyanosis in lower extremities, RVH, LVH and heart failure

Eisenmenger syndrome is due to:

Uncorrected left to right shunt (VSD, ASD, PDA), pathologic remodeling of vasculature, pulmonary arteriolar hypertension

Complications of einsenmenger syndrome:

Cyanosis, clubbing and polycythemia, RVH occurs to compensate

Coarctation of the aorta is associated to:

Bicuspid aortic valve, aortic regurg


Infantile type: turner sx


Adult type: Notching of the ribs, hypertension in upper extremities and weak, delayed pulses in lower extremities.


Preductal coarctation of the aorta is seen in:

Children

Postductal coarctation of the aorta is seen in:

Adults

45 year old man that during a routine check has 160/90 in the right arm and 170/90 in the left arm. Weak, barely audible pulse in the feet. What do you suspect?

Coarctation of aorta

Name the most common right to left shunts in congenital heart diseases?

5 T's


1. Truncus arteriosus (1 vessel)


2. Transposition (2 switched vessels)


3. Tricuspid atresia (3=tri)


4. Tetralogy of fallot (4=tetra)


5. TAPVR (5 letters)

What is a persistant truncus arteriosus?

Failure of the truncus arteriosus to divide into pulmonary trunk and aorta due to failure of neural crests to migrate.

Difference between right to left and left to right shunts?

Left to right shunts are considered "late cyanosis" or blue kids, some are asymptomatic or show up later in life. Whereas right to left shunts are considered "blue babies", Often diagnosed prenatally or become evident directly after birth. Usually require surgical correction and or maintenance of a PDA.

What is D-transposition of great vessels?

Aorta and pulmonary trunk are switched due to failure of aorticopulmonary septum to spiral. There is a separation of systemic and pulmonary circulations. Not compatible with life unless a shunt is present to allow mixing of blood. (maintain open with PGE until surgery)

Without surgical internvention, what is the prognosis for D-transposition of great vessels?

Most infants would die within the first few months of life.

It is an absence of tricuspid valve and a hypoplastic RV

Tricuspid atresia

Requires both ASD and VSD for viability.

Tricuspid atresia

What does PROVe stand for?

The 4 symptoms of tetralogy of fallot.


1. Pulmonary infunibular stenosis


2. RVH - bootshaped heart


3. Overriding aorta


4. VSD

Is tetralogy of fallot a right to left OR left to right congenital heart disease?

Normally left to right but during "TET SPELLS" (crying, feeding, increased activity), pulmonary stenosis forces circulation from right to left, making them cyanotic.

Why do children with tetralogy of fallot squat?

Because squatting increases systemic vascular resistence (SVR), decreases right to left shunt and improves cyanosis.

What is the treatment of tetralogy of fallot?

Early surgical correction

Total anomalous pulmonary venous return (TAPVR)?

Pulmonary veins drain into right heart circulation, associated with ASD and sometimes PDA to allow right to left shunting to maintain

Associated to lithium use

Ebstein anomaly (tricuspid atresia)

Widely split S2, tricuspid regurg

Ebstein anomaly (tricuspid atresia)

What is ebstein anomaly?

Associated to maternal lithium use. Tricuspid leaflets are displaced into right ventricle, hypoplastic right ventricle, tricuspid regurg or stenosis.

80% of patients with an ebstein anomaly have a....

patent foramen ovale with right to left shunt.

Why may ebstein anomaly increase risk of SVT and WPW?

Because of the dilated right atrium

What is the most common case of early childhood cyanosis?

tetralogy of fallot

What is the most important determinant for prognosis in tetralogy of fallot?

Pulmonary infundibular stenosis

Boot shaped heart in children makes you think of ____________, and in adults the same finding makes you think of______________

Tetraology of fallot in children and COPD in adults

What is derived from the 3rd aortic arch?

Common carotid artery and proximal part of internal carotid artery

C is the 3rd letter of the alphabet

What is derived from the 4th aortic arch?

On left, aortic arch; on right, proximal part of right subclavian artery.

What is derived from the 6th aortic arch?

proximal part of pulmonary arteries and (on left only) ductus arteriosus