What are the 5 T’s of R to L shunts?
Tetralogy Transposition Truncus Tricuspid TAPVR
Right to left shunts are _____ cyanosis.
Early
What is the most common cause of early cyanosis?
Tetralogy of Fallot
Persistent Truncus Arteriosus is what?
Failure of truncus arteriosus to divide into pulmonary trunk and aorta
Those with persistent truncus arteriosus are most often accompanied with….
VSD
What is tricuspid atresia?
- No tricuspid valve
- hypoplastic RV
Tricuspid atresia requires what other defects?
ASD and VSD
What is TAPVR?
Pulmonary veins drain into right heart circulation
What other defect is TAPVR associated with?
ASD and sometimes PDA to allow for R to L shunting to maintain CO
What are the L to R shunts in the frequency that they occur?
VSD>ASD>PDA
Left to right shunts are associated with ______ cyanosis.
Late
What drug closes a PDA?
Indomethacin
What heart sounds are characteristic of ASD?
- S1: Loud
- S2: Wide, fixed split
What are the steps in Eisenmenger’s syndrome?
- Uncorrected VSD, ASD, PDA
- Compensatory pulmonary vascular hypertrophy
- Progressive pulmonary hyptertension
- Pulmonary resistance increases
- Shunt reverses to R to L
What are the ultimate effects of Eisenmenger’s syndrome?
Late Cyanosis
Clubbing
Polycythemia
Tetralogy of Fallot is caused by a _________ displacement of the _______ septum
Aterosuperior, infundibular
What are the characteristics of Tetralogy of Fallot?
PROVe:
- Pulmonary infundibular stenosis
- RV hypertrophy
- Overriding Aorta (over VSD)
- VSD
Why does squatting help with TOF?
Reduces blood flow to the legs and thus increases peripheral vascular resistance and decreases the cyanotic R to L shunt across the VSD.
Preferred treatment for TOF is what?
Early surgical correction
What is the cause of the early cyanosis tet spells in TOF?
The pulmonary stenosis forces R to L flow (instead of usual L to R) and RVH
In transposition of great vessels the ______ leaves the RV and the _______ leaves the LV
Aorta, Pulmonary trunk
Because in TGV, there is a _______ of systemic and pulmonary circulations, it is not compatible unless a ________ is present to ________
separation, shunt, allow adequate mixing of blood (VSD, PDA, or Patent foramen ovale)
TGV is due to what?
Failure of the aorticopulmonary septum to spiral
Without surgical correction, infants_______.
die within the first few months of life
Coarctation of the aorta can cause what?
Aortic Regurgitation
What is the infantile type of coarctation of the aorta?
INfantile is IN close to the heart
Aortic stenosis proximal to insertion of ductus arteriosus (preductal)
What is the adult type of coarctation of the aorta?
Stenosis is distal to ligamentum arteriosum (postductal)
For infantile coarctation of the aorta where do you check the pulses of Phyiscal Exam?
Femoral
Infantile Coarctation of the aorta is associated with what syndrome?
Turners syndrome
Adult type coarctation of the aorta is associated with what other things?
- Notching of the ribs (due to collateral circulation)
- Hypertension in the UE
- Weak pulses in LE
Adult type coarctation is associated with what other abnormality?
Bicuspid aortic valve
In the fetal period, shunts are _____ to ______.
Right, Left
In the neonatal period, lung resistance ________ and shunt becomes ______ to ______
Decreases, Left, Right
PDA is associated with what kind of murmur?
Continuous, Machine-like
PDA patency is maintained by what?
PGE and low oxygen tension
Uncorrected PDA can eventually result in what?
Late cyanosis in the lower extremities
What defect is associated with 22q11 syndrome?
- Truncus Arteriosus
- TOF
What defect is associated with Down Syndrome?
- ASD
- VSD
- AVSD (endocardial cushion defect)
What defect is associted with congenital rubella?
- Septal Defects
- PDA
- Pulmonary artery stenosis
What defect is associated with Turner Syndrome?
Coarctation of the aorta (preductal)
What defect is associated with Marfan’s Syndrome?
Aortic Insufficiency and dissection (later)
What defect is associated with infant of a diabetic mother?
TGV
Hypertension is defined as a blood pressure of over ________
140/90 mmHg
What are the risk factors for hypertension?
- Older age
- Diabetes
- Smoking
- Genetics
- Black>White>Asian
90% of hypertension is ________ and 10% is ______
Primary, secondary
Primary hypertension is related to increase _____ and ________
Cardiac output, TPR
Secondary hypertension is related to ________
Renal disease
Malignant hypertension is severe and is a blood pressure of over _________
180/120
Hypertension predisposes to what?
- Atherosclerosis
- LVHypertrophy
- Stroke
- CHF
- Renal failure
- Retinopathy
- Aortic DIssection
What are atheromas?
Plaques in blood vessel walls
What are xanthomas?
Plaques or nodules composed of lipid-laden histiocytes in the skin (especially eyelids aka xanthelasma)
What are Tendinous Xanthoma?
Lipid deposit in tendon, especially achilles
What are corneal arcus?
Lipid deposit in cornea, nonspecific (arcus senilis)
What is Monckeberg Arteriosclerosis?
Calcification in the media of the arteries, especially radial or ulnar
Moncheberg artiosclerosis is usually _______ and occurs in ______ arteries. It _______ obstruct flow and the _______ is not involved.
benign, pipestem, does not, intima
What are the two types of arteriolosclerosis?
Hyaline, hyperplastic
What is hyalanie arteriolosclerosis?
Thickening of small arteries
- essential hypertension
- diabetes mellitus
What is hyperplastic arteriolosclerosis?
Onion skinning
-Malignant hypertension
What is atherosclerosis?
Fibrous plaques and atheromas form in intima of arteries
Atherosclerosis is a disease of ______ arteries and ________ sized muscular arteries
elastic, large and medium
What are the modifiable risk factors for atherosclerosis?
- Smoking
- Hypertension
- Hyperlipidemia
- Diabetes
What are the non-modifiable risk factors for atherosclerosis?
- Age
- Gender (more in men and postmenopause women)
- Family History
__________ is important in the pathogenesis of atherosclerosis
Inflammation
What are the first two steps of atherosclerosis progression?
- Endothelial cell dysfunction
2. Macrophage and LDL accumulation
What are the third and fourth steps of atherosclerosis progression?
- Foam cell formation
4. Fatty streaks
What is the fifth step of atherosclerosis progression?
- Smooth muscle cell migration (involves PDGF and FGF), proliferation, and extracellular matrix deposition.
What are the sixth and seventh steps of atherosclerosis progression?
- Fibrous plaque
7. Complex atheromas
What are the complications of atherosclerosis?
- Aneurysms
- Ischemia
- Infarcts
- Peripheral vascular disease
- Thrombus
- Emboli
What are the locations of atherosclerosis in order of commonality?
Abdominal Aorta>Coronary artery>popliteal artery>carotid artery
What are the symptoms of atherosclerosis?
- Angina
- Claudication
- Can be asymptomatic