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1. Heart Article Clinicles
By: Demetrice Conday

2. Sudden Infants Death
Syndrome (SIDS)
• AKA: Crib Death or SIDS
• What is it? Unexpected death of healthy infant within
its 1st year of life.
• How is it diagnosed? Not clarified as SIDS until all
other explanations have been effectively ruled out.
• Suggested Causes: Abnormalities in brainstem
development, neuroregulation of cardio-respiratory
control, and Hearts Electrical Conduction System.

3. Dextrocardia
• What is it? Normally heart positioned more on left side
with apex pointing to left. But, with this position
abnormality it’s reversed & more positioned towards
right, with apex pointed at right, and all vessels also
reversed. Mirror Image Effect.
• Cause? When heart tube bends to left instead of right,
displacing the heart to the right.
• May or May not occur with? Situs Inversus (All organs
are reversed)
Most common positional abnormality of the heart
Liver develops on the left
side instead of the right,
the stomach develops on
the right side instead of the
left

4. Ectopia Cordis
• What is it? Heart ends up outside thoracic
cavity (Congenital).
• Cause? Occurs when lateral folds fail to fuse
form thoracic wall during the 4th week. Will
result in abnormal development of sternum
and pericardium.
• Treatment: Most infants die right after birth
due to cardiac failure. But if there are no
severe cardiac defects the surgery is an
option. The heart will be covered with skin
(surgical procedure).

5. Atrial Septal Defects
• What is it? Defect allows left to right shunting of blood:
• Oxygenated blood flows from LA to RA & mixes w/ deoxygenated
blood. (then to RV)
• Occurs Most Frequently in: Females
• Most Common Type: Patent Foramen Ovale
• Probe Patent Ovale: A prob can be passed from one
atrium to the other through the fossa ovalis. If
opening is small, the defect is not clinically
significant.
• Cause: Failure of flap of valve of formen ovale to
fuse with septum secundum after birth  Leaving
communication b/w atria

6. Atrial Septal Defects (Cont)
• 4 Types of ASD’s
• Ostium Secundum Defect (most common)
• Ostium Primum Defect (endocardial cushion defect with a foramen primum defect)
• Sinus Venosus Defect
• Common Atrium

7. Atrial Septal Defects (Cont)
• Ostium Secundum ASDs
• Involves defect in septum primum and septum secundum
• Abnormal resorption of septum primum during development of foramen secundum
leads to patent foramen ovale.
• Aberrant Resorption
• Causes: The septum primum adopts a fenestrated appearance
• Excessive Resorption
• Causes: The septum primum will be too short to effectively close the foramen ovale.
• Septum Secundum fails to develop normally = Atypical Large Foramen Ovale
• Causes: May be too large to be closed by a normal sized septum primum

8. Atrial Septal Defects (Cont)
• Ostium Primum ASDs
• Causes: When septum primum fails to fuse with endocardial cushions.
• Results in: Patent Foramen Primum.
• Cleft in anterior cusp of mitral valve tends to co-occur.
• Commonly Associated With: Down Syndrome (Trisomy 21)

9. Atrial Septal Defects (Cont)
• Sinus Venosus ASDs
• Where does this form? In interatrial septum near entry of SVC in RA
• Cause? Incomplete resorption of sinus venosus within RA, or maldevelopment of
septum secundum.

10. Atrial Septal Defects (Cont)
• Common Atrium ASDs
• What is it? Complete absence of interatrial septum
• Causes? Combination of ostium secundum, ostium primum, and sinus venosus
defects.

11. Atrial Septal Defects (Cont)
• Signs and Symptoms
• Shortness of Breath
• Easy Fatigability
• Edema of lower limb or abdomen
• Frequent respiratory infections
• Palpitations
• Main Association
• Murmur
• 2nd Heart Sound
• Amount of blood is increased as it moves from L to R atrium & enters pulmonary circulation.
, which results in a delay in closure of pulmonic valve.

12. Ventricular Septal Defects
(VSD)
• Location: Membranous part of IV Septum. Can also occur in muscular part of IV Septum,
but less common.
• Small VSDs
• Usually close on there own during the first year of life.
• Large VSDS
• Remain and allow oxygenated blood from the LV to the RV.
• Infants with severe VSDs often have other cardiac anomalies as well, including
transposition of the great arteries or an underdeveloped outlet chamber.
• On Cardiac Auscultation
• VSD presents with a harsh holosystolic murmur that is loudest in tricuspid
area, the 4th intercostal space at the left sternal boarder.
ASD & VSD + Associated
w/ Down Syndrome

13. Patent Ductus Arteriosus
• What is it? When ductus arteriosus, which usually closes shortly after birth
and forms the ligamentum arteriosum, fails to do so and instead remains
open.
• Causes? L to R shunt, allows oxygenated blood from the high-pressure aorta
to the flow into the low-pressure pulmonary artery and mix poorly oxygenated
blood heading to lungs.
• Most frequent in: Females
• Associated with: Machine-Like Murmur (heard in infraclavicular region)

14. Eisenmenger
Syndrome
• Cause? When a left to right shunt (ASD, VSD,
PDA) is left uncorrected, Eisenmenger Syndrome
can eventually result.
• What is it? When RV and pulmonary vasculature is
remodeled due to the shunting of blood.
• Subsequently, increased pressure in RV results in R
ventricular hypertrophy.
• What can right ventricular hypertrophy cause? For
the left to right shunting of blood to be reverse.
Therefore, poorly oxygenated blood from the R
ventricle will mix with oxygenated blood in the L
ventricle.

15. Persistent Truncus Arteriosus
• Cause? When truncal ridges & aorticopulmonary
septum fail to separate truncus arteriosus into aorta &
pulmonary trunk, creating a R to L shunt.
• Most Common Type: Single Arterial Trunk (Gives off
ascending aorta and pulmonary trunk, supplying the
systemic, pulmonary, and coronary circulations).
• Defect ALWAYS occurs with a VSD & is 1 of the main
cardiac defects associated with DiGeorge (22q11)
syndrome.

16. Transposition of the Great
Arteries
• What is it? Normally:
• Aorta arises from LV
• Pulmonary trunk arises from RV.
But w/ defect relationship = Reversed:
Aorta arise from RV
Pulmonary trunk arise from LV
• Cause? Defective neural crest migration, and or abnormal
development of the conus arteriosus during the time that the
bulbus cordis is being incorporated into the ventricles.
• Results in? AV connections are normal b/w both ventricles,
therefore there is a complete separation between systemic
and pulmonary circulation.
• Most Common cause of Cyanotic Disease in neonates.

17. Transposition of the Great
Arteries
• Incompatible with life UNLESS:
• ASD, VSD, or PDA also present in order to create R to L
shunt that allows blood to mix & oxygenated blood to be
delivered to aorta.
• Will result in:
• Right Ventricular Hypertrophy since RV in now
part of a high resistance systemic circuit
delivering blood to the body.
• Left Ventricular Atrophy since it is now apart of
low-resistance pulmonary circulation.

18. Unequal Division of the
Truncus Arteriosus
• What is it? Unequal division of truncus
arteriosus results when division of
truncus arteriosus above the level of the
valves creates 1 large artery and 1 small
artery.
• This will lead to uneven
aorticopulmonary septum.
• This anomaly is associated with 2 types
of pulmonary stenosis:
• 1. Pulmonary Valve Stenosis
• 2. Infundibular Stenosis

19. Unequal Division of the Truncus
Arteriosus (Cont)
• Pulmonary Valve Stenosis
• Cusp of pulmonary valve fused together 
This forms a dome, narrowing valvular
opening
• Infundibular Stenosis
• When conus arteriosus of RV can’t fully
develop. If outflow of blood is severely
obstructed hypertrophy of RV will be
observed as well.

20. Tricuspid Atresia
• What is it? Complete closure of the AV canal.
• Cause? Fusion of the tricuspid valve leaflets or
absence of the tricuspid valve altogether, such that the
AV Canal is blocked and blood cannot flow from the
RA to the RV and into the pulmonary circulation.
• Results in: Hypoplastic RV and hypertrophic LV & is
not compatible with life W/O ASD or VSD

21. Tetralogy of Fallot
• The most common cause of
cyanosis in early childhood.
• A Classical Constellation of
Four Cardiac Abnormalities
• Pulmonary Infundibular
Stenosis
• Overriding Aorta
• VSD
• R Ventricular Hypertrophy
PROV

22. Tetralogy of Fallot (Cont)
• Pulmonary Stenosis
• A “Tet Spell” occurs when right ventricular outflow tract
obstruction is aggravated, such as from crying, exercise, or
fever. This increases shunting of blood from the R to L
ventricle, worsening cyanosis.
• In response, a child will squat  This increases systemic
vascular resistance, which increases L ventricular pressure
& decreases the R to L shunt,  Reducing cyanosis.
• Cyanosis
• Important sign of tetralogy of Fallot. Babies may tire easily
while feeding. They are not able to gain weight or grow as
quickly as children who have healthy hearts.
• Diagnostic Test
• Chest X-Ray
• Echocardiography
• Electrocardiogram
• Pulse Oximetry
• Treatment
• Open heart surgery either soon or later in the infancy
Along with persistent truncus arteriosus, Tetralogy of Fallot is associated with
diGeorge syndrome (in which case one will also see characteristic facial
features, such as wide set eyes).

23. Total Anomalous Pulmonary
Venous Return
• What is it? Pulmonary Veins containing blood
oxygenated by lungs-drain into RA instead of
left.
• Symptoms
• May appear soon after birth, or they may be
delayed depending in part on the blockage
of the veins draining towards heart.
• Occurs with ASD or PDA
• Necessary to allow blood from the R heart
to be shunted to the L in order to maintain
cardiac output.

24. Aortic Stenosis &
Atresia
• What is it?
• Aortic valve completely fused together,
reducing flow from LV into aorta.
• Leads to L ventricular hypertrophy
• Cardiac Auscultation = systolic crescendo-
decrescendo heart murmur loudest at right
sternal boarder in the 2nd intercostal space.
• Cause
• Aortic Stenosis occurs when the edges of
the aortic valve leaflets fuse.
• Can be congenital (or acquired) through
damage to the valve and dystrophic
calcification.

25. Subaortic Stenosis
• What is it? A ring of fibrous tissue
narrows the aorta just below the valve.
• Cause? when tissue that forms valves
fails to degenerate.

26. Aortic Atresia
• What is it? Occurs when the aortic valve or part of the
aorta itself is completely obstructed.

27. Coarctation of the Aorta
• What is it? Constriction of the Aorta.
• Two Main Types
• Infantile (preductal) Coarctation
• Adult (postductal) Coarctation

28. Coarctation of the Aorta (Cont)
• Infantile (preductal) Coarctation
• Cause? The aortic arch proximal to ductus arteriosus is hypoplastic.
• Results in? Narrowing of the aorta b/w the left subclavian artery and the ductus
arteriosus, which is often patent.
In response to
the increased
blood flow, the
pulmonary trunk
dilates and the
right heart
becomes
hypertrophied.

29. Coarctation of the Aorta (Cont)
• Adult (ductal) Coarctation
• Cause? A ridge tissue forms and constricts the aorta adjacent to the ligamentum
arteriosum.
• Tissue is made up of smooth and elastic fibers that are continuous with the aortic
media. These tissues increase pressure in the vessels and will result in dilation of
the aorta and its branches (brachiocephalic trunk, left common carotid, and
left subclavian artery) & hypertrophy of LV

30. Congenital Long QT
Syndrome
• What is it? Inherited disorder names for its
characteristic electrocardiogram (ECG or EKG)
finding of a prolonged QT-interval.
• Causes? Reflects increase in amount of time it
takes for cardiac muscle to repolarize b/w
heartbeats & is due to defects in ion channels on
cardiac myocytes.
• Disorder increases risk of a pt developing a
polymorphic ventricular tachycardia (ventricles
pumping to quickly) called Torsades de Pointes.

31. Congenital Long QT Syndrome (Cont)
• Types
• Jervell and Lange Nielsen Syndrome
• An autosomal recessive condition in which patients experience conduction
abnormalities of the heart as well as sensorineural deafness
• Romano-Ward Syndrome
• An autosomal dominant inheritance, but not associated with deafness.

32. Brugada Syndrome
• What is it? When hearts normal rhythm is
disrupted
• Results in? Irregular heartbeats and a
subsequently heightened risk of developing
ventricular tachyarrhythmias, which can lead to
syncope (fainting), seizures, and potentially sudden
cardiac death.
• Patients usually are asymptomatic
• Common Among Asian Males (Autosomal
Dominant)
• Classic ECG pattern reflects pseudo-right bundle
branch block, with elevations of the ST segment in
leads V1-V3 (reflecting abnormalities in the septal
and anterior regions of the heart).

33. Ebstein’s Anomaly
• What is it? Specific abnormality of the R heart. Main pathology
lies within tricuspid valve, which is displaced downward towards
RV.
• Anterior leaflet enlarged.
• Causes? Fetal exposure to lithium during pregnancy due to
treatment of the mother (for bipolar disorder, for example)
• Results in? Tricuspid regurgitation. This can eventually lead to
right heart failure.
• Symptoms? Exertional dyspnea, palpitations, and cyanosis. Other
children may develop a murmur and arrhythmias.
• Primarily diagnosed by echocardiography (ECG).

34. Clinical Case