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Cardiology - Congenital Heart / Cardiac Diseases (Congenital Cardiac…
Cardiology - Congenital Heart / Cardiac Diseases
*Congenital Cardiac Pathology
shunts are either
:white_small_square: left --> right
:white_small_square: right --> left
Left - Right *Shunts
"VAP"
VAP = VSD > ASD > PDA
PDA = *Patent Ductus Arteriosus
ductus is kept open for fetus due to prostaglandin PGE2 production by the placenta
when born, idecreased pulmonary pressure and lowered PGE2 closes the Duct
PDA is most common in premature babies
Clinical Cases
Clinical Case
Notes
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note that
Clinical Case
Clinical Case
Clinical Case
Notes
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*ASD and PFO
ASD = Atrial septal defect
PFO = patent foreman ovale
can be both causes of cryptogenic stroke
--> unkown cause
ASD Sequalae
laminated medial hypertrophy of the pulmonary arteries
--> hardenning of the pulmonary arteries
Eisenmenger Syndrome
--> where the ASD or VSD reverses direction to become a Right --> left shunt
ASD and laminated medial hypertrophy of the pulmonary arteries
--> hardenning of the pulmonary arteries
Clinical Case
Notes
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note that
Clinical Case
Formation of Atrial Septum
septum primum and septum secundum are needed to fuse
Crytptogenic stroke and ASD and PFO
Clinical Case
Notes
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note the key here in development of the septum is that the septum primum and septum secundum both neeed to fuse to cut off the Foreman ovale
for looking for a cause in cryptogenic stroke they will often try to rule out ASD and PFO by putting a cathedar into the right atrium and seeing if bubbles are seen in the left atrium thorugh the sopen septum
VSD = *Ventricular Septal Defect
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Right - Left *Shunts
"5 Ts of Tetralogy vessels"
4 = Tetralogy of Fallot
--> rest go by number
*Tetralogy of Fallot
DROP
Tetra conditions
better on Squatting
"SQUATTING is EAZY for TOUFF MVP HOCM athletes"
*TOF presentation = TET spells of cyanosis
in TOF babies have TET spells = get blue and cyanotic after feeding or crying
Clinical Cases
Severity of Tetralogy of Fallot is the degree of RVOT
ROVT = right ventricular outflow tract
Tetralogy of Fallot case 2
Clinical Case
Notes
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note that .
Tetralogy of Fallot case
Clinical Case
Notes
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note that
Notes
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TOF is the most common cause of congenital cyanosis in young infants
note that squatting improving it is key
makes the TPR higher
--> more pressure on the aorta forces blood through the pulmonary valve
TOF = HOLE in my BOOT shaped Heart
HOLE = VSD --> Very SounD loud
cowboy sitting on the aorta holding the pulmonary artery = pulmonary stenosis
heel of the boot = RVH
Tetralogy of Fallot = TOF = Tired of FALLING = DROP
D = Displaced aorta
R = RVH
O = openning in vetricle septum = VSD
P = pulmonary stenosis --> loud systolic murmur
*Squatting Manouver Improves TOF
"SQUATTING is EAZY for TOUFF MVP HOCM athletes"
--> TOF MVP HOCM athletes are both QUIET on squatting
--> Squatting = increase preload = LV dilation
--> LV dilation helps HOCM by openning the outflow obstruction from the SEPTAL thichenning
--> LV dilation helps MVP by pulling the loose chords tight
--> in TOF mainly AFTERLOAD increase from squatting closes the Aortic valve and opens the stenotic pulmonary valve
Congenital Cardiac Disorders
*Ehler Danlos Syndrome
collagen 3
--> THREE = Ehlors
*Marfan's Syndrome
Case example:
Notes
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Marfan's Syndrome is from a mutation in the fibrillin-1 gene = FBN1, that codes for a connective tissue protein that is used for the production and maintenance of elastic fibers
people with Marfan's syndrome all have the genetic mutation of FBN-1 gene, but have variable expressivity
--> meaning they may have different phenotype, they may have many symptoms or be asymptomatic from the exact same gene mutation
Marfan's syndrome is also autosomal dominant
ASD = Atrial Septal Defect
Notes
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note that ASD has a sudden change in the O2 pressure from the IVC or SVC because as the blood enters the right atrium, it is mixed with oxygenated blood, giving it a medium level of O2 in comparison to the left side of the heart
note that ASD has an increased volum of blood that is shunted from left to right atrium during systole
--> this causes a ASD association with a murmour heard over the pulmonic valve
--> also a fixed splitting of the S2 heart sound due to the excess flow of blood
--> the valve takes longer to close
Case Presentation:
Cardiac Hypertrophy / Dilated Cardiomyopathies
DCM vs HCM
*DCM = Dilated Cardiomyopathy
most common cardiac hypertrophy
--> 80% + of cases vs. LVH?
eccentric hypertrophy
--> cells build adjacent to each other and not concentrated = concentric (LVH)
DCM Notes:
DCM is autosomal DOMINANT
--> but it has incomplete penetrance so even with the mutation, it may not present clinically in some patients
because it is dominant, this makes it more common than others
DCM Case example 1
Notes
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note
Case presentation:
Pathophys:
recall MY TITZ for sarcomeres
--> M line has MYocytes
connects with Titin to Z line
--> where actin are attached
most common mutation that leads to DCM
--> TTN gene mutation for titin protein
--> myosin are loosley held on Z lines by defective titin
--> leads to dilation of the cardiomyocytes
*HOCM = hypertrophic Obstructive Cardiomyopathy
autosomal dominant inheritance --> STRONG family history
stage 1 = compensated VH hypertrophy Diastolic heart failure
stage 2 = decompensated LV hypertrophy diastolic HF
HOCM Inheritance
autosomal Dominant
--> strong family Hx ALWAYS
genes for sarcomere proteins
--> sarcomere have large heavy myosin chains
--> +/- myosin binding complex protein C
HOCM
:
LOFO
--> aortic stenosis of modified type (preload exception)
CHF and viscoius RAAS system cycle of decompensation from kidney
renal perfusion sense low blood pressure --> activates RAAS system
this increased BP even more and increases afterload
--> viscoius cycle of more and more LVH
S4 sound on heart exam
-S4 sound heard when you have the atrial kick right before the S1 closure of the atrial valves
S4 sound comes from blood from atrial kick hitting against the hardenned LVH
= stiff ventricle
diastolic HF
note even if athletes are in high intensity sports you have to rule out HOCM
Physiological Changes of LV and Heart
main thing is increased thickness and mass in the ventricular septum
smaller LV volume
poor CA network to perfuse the thickened LV
HOCM increased thickness and mass in the ventricular septum
HOCM and Viscoius RAAS Renal Decompensation CHF
note that in LVH of HOCM, you get high RAAS activation to compensate for the heart failure
HOCM and diastolic HF --> RAAS viscoius cycle case
Notes
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note that
Clinical Case
HOCM and Maneuvers
EVERYTHING opposite to Aortic stenosis before the valve
--> afterload still quiets the murmur
stenosis here is due to the LOFO and not the valve itself
more preload = better
HCM and LOFO
LOFO = left outlet flow obstruction
Notes
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note that LOFO in HCM causes an aortic stenosis, but it is a special and different kind of Aortic stenosis
it is not due to the narrowing of the valve itself , but to the outflow tract right before it
it can thus be differentiated from a normal aortic stenosis since for normal aortic stenosis the rule is
increasing preload = increase in Frank starling = increase CO and aortic stenosis murmur
opposite in HCM aortic stenosis LOFO
LOFO - increase in preload = inspiration = increase in stetch of the LV and LOFO
--> LOFO opens wider and murmur lower
LOFO - decrease in preload = valsalva pool jump = abrupt standing up = decrease in stetch of the LV and LOFO tightens
--> LOFO tightens and murmur louder
HOCM LOFO and Drugs
AVOID vasodilators and diuretics in HOCM and LOFO
anything that reduces the preload to the LV will make HOCM LOFO worse
note that for vasodilators, CCBs dihydropyridines are used for anti HTN because they vasodilate and also work a bit on the heart
--> dihydro CCBs = vasodilators = CANNOT use with HOCM and LOFO
nondihydro CCBs = verapamil and diltiazem CAN be used in HOCM since they work on the ventricle more and relax it
Clinical Cases
2 more items...
Treatment of HOCM
beta blockers = first line?
Clinical Cases
Clinical Case
Notes
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note that
Clinical Case
*Marfan's Syndrome
fibrilin-1 mutation
