Pathology Flashcards
Congenital heart diseases
2 groups
Special names
Reasons
- R to L shunts-early cyanosis-blue babies
2. L to R shunts-late cyanosis with Eisenmengers phenomenon-blue kids
R to L shunts
Cyanosis when
Diagnosed when
Compatibility with life unless
Early cyanosis—“blue babies.”
Often diagnosed prenatally or become evident immediately after birth.
Usually require urgent surgical correction and/or maintenance of a PDA.
What are the R to L shunt disorders
The 5 Ts:
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 in the name)
Persistsnt Truncus arteriosus
Causes
Pathology
Also have what
Truncus arteriosus fails to divide into pulmonary trunk and aorta due to lack of aorticopulmonary septum formation
most patients have accompanying VSD.
TGV Means what Due to Shunts present Prognosis
Aorta leaves RV (anterior) and pulmonary trunk leaves LV (posterior)
Due to failure of the aorticopulmonary septum to spiral.
VSD
ASD
PDA
If no sx die within weeks
Tricuspid atresia
Pathology
Shunts
Absence of tricuspid valve and hypoplastic RV
requires both ASD and VSD for viability.
TOF Commonest what Cause Components 4 Factor determining the prognosis Shape of the heart,due to Special symptom Relieved how RX
Most common cause of early childhood cyanosis.
Caused by anterosuperior displacement of the infundibular septum
Pulmonary infundibular stenosis (most important determinant for prognosis)
Right ventricular hypertrophy (RVH)— boot-shaped heart on CXR
A Overriding aorta
VSD
early cyanotic “tet spells”
Squatting: SVR, right-to-left shunt, improves
cyanosis.
Treatment: early surgical correction.
TAPVD
Means
Via
Shunts 2
Pulmonary veins drain into right heart circulation
SVC, coronary sinus
associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO.
L to R shunt diseases
Cyanosis when
Why
VSD>ASD>PDA
Late cyanosis
Eisenmengers
VSD Common Symptoms at birth Symptoms appear when Resolve how Larger ones can causes
Most common congenital cardiac defect.
Asymptomatic at birth.
May manifest weeks later or remain asymptomatic throughout life.
Most self resolve
larger lesions may lead to LV overload and HF.
ASD Pathology Clinical signs Two types/common/associated abnormalities Symptoms Difference from patent f.ovale
Defect in interatrial septum
loud S1/wide, fixed split S2.
Ostium secundum defects most common and usually occur as isolated findings
ostium primum defects rarer yet usually occur with other cardiac anomalies.
Symptoms range from none to HF.
Distinct from patent foramen ovale in that septa are missing tissue rather than unfused.
PDA Function in fetal period Neonatal period function Later on what happens Diagnostic sign
In fetal period, shunt is right to left (normal).
In neonatal period, lung resistance shunt becomes left to right
progressive RVH and/or LVH and HF-Eisenmengers
Lower extremity cyanosis
Eisenmengers syndrome Caused by How Clinical signs 3 Age of onset
Uncorrected left-to-right shunt (VSD, ASD, PDA)
pulmonary blood flow pathologic remodeling of vasculature pulmonary arterial hypertension. RVH occurs to compensate -shunt becomes right to
left
Causes late cyanosis, clubbing and polycythemia.
Age of onset varies.
Congenital COA Pathology Site Associated conditions 2 Symtopms and signs
Later on CXR changes,due to
Aortic narrowing near insertion of ductus arteriosus (“juxtaductal”).
Associated with
bicuspid aortic valve
Turner syndrome.
Hypertension in upper extremities and weak, delayed pulse in lower extremities (brachial-femoral delay).
With age, collateral arteries erode ribs (notched appearance on CXR).
Congenital cardiac defect s associations
Alcohol exposure in utero (fetal alcohol syndrome)
Congenital rubella
Down syndrome
Infant of diabetic mother
Marfan syndrome
Prenatal lithium exposure Turner syndrome Williams syndrome
22q11 syndromes
Alcohol exposure in utero (fetal alcohol syndrome)-ASD/VSD/PDA/TOF
Congenital rubella-ASD/VSD/PDA/PS
Down syndrome-AV canal defects/ASD/VSD
Infant of diabetic mother-TPGV
Marfan syndrome-MVP/AR/thoracic aorta aneurysms and dissections
Prenatal lithium exposure -Ebstein anomaly(TV defect)
Turner syndrome -COA/bicuspid aortic v
Williams syndrome-supravalvular AS
22q11 syndromes-TA,TOF
HTN
Definition Risk factors Ethnic frequency Types and % Causes for both contrast X-ray in fibromyscular dysplasia What's HT urgency What's HT emergency/eg Complications Explain high renin HT
Defined as persistent systolic BP ≥ 140 mmHg and/or diastolic BP ≥ 90 mmHg
age, obesity, diabetes, physical inactivity, excess salt intake, excess alcohol intake, family history
black > white > Asian
90% -1° (essential) and related to CO or TPR
10% -2°
-renal/renovascular disease-renal artery stenosis (e.g., fibromuscular dysplasia -usually found in younger women-string of beads appearance)
-1° hyperaldosteronism.
Hypertensive urgency—severe (≥ 180/≥ 120 mmHg) hypertension without acute end-organ damage.
Hypertensive emergency—severe hypertension with evidence of acute end-organ damage
(e.g., encephalopathy, stroke, retinal hemorrhages and exudates, papilledema, MI, HF, aortic dissection, kidney injury, microangiopathic hemolytic anemia, eclampsia)
Complications
1.CVS-CAD, LVH, HF, atrial fibrillation
aortic dissection, aortic aneurysm
- CNS-stroke
- Renal-chronic kidney disease (hypertensive nephropathy)
- retinopathy.
B. Renal artery stenosis is a common cause (renovascular hypertension).
Stenosis decreases blood flow to glomerulus.
Juxtaglomerular apparatus (JGA) responds by secreting renin, which converts angiotensinogen to angiotensin 1.
Angiotensin I is converted to angiotensin 11 (ATII) by angiotensin converting enzyme (ACE).
ATII raises blood pressure by (I) contracting arteriolar smooth muscle, increasing total peripheral resistance and (2) promoting adrenal release of aldosterone, which increases resorption of sodium in the distal convoluted tubule (expanding plasma volume).
Leads to HTN with increased plasma renin and unilateral atrophy (due to low blood flow) of the affected kidney; neither feature is seen in primary hypertension
Important causes of stenosis include atherosclerosis (elderly males) and fibromuscular dysplasia (young females).
HLD signs 3
Xanthelesma-
lipid deposited in histiocytes
Seen as plaque sin eye lids
Tendinous xanthomata-
Lipid deposited on tendons
Common Archillies
Corneal arcus-
Deposit in cornea
Old age-senile arcus
Premature if high cholesterol
Arteriosclerosis What types 3 How common Affects which vessels
Thickening of vessel walls-hardening and loss of elasticity
Ateriolosclerosis-
common
Aterioles
Medial calcific sclerosis-
uncommon
Medium size arteries
Atherosclerosis-
very common
Elastic and medium and large muscular arteries
Ateriolosclerosis Common Affect which vessels Types 2 Eg Hyper plastic Appearance on histo,due to Classically seen when
Common.
Affects small arteries and arterioles.
Two types:
1.hyaline (thickening of vessel walls in essential hypertension or diabetes mellitus
classically produces glomerular scarring (arteriolonephrosclerosis)that slowly progresses to
chronic renal failure
2.hyperplastic (“onion skinning” in severe hypertension with proliferation of smooth muscle cells).
fibrinoid necrosis of the vessel wall with hemorrhage
classically causes acute renal failure with a characteristic ‘flea-bitten’ appearance
Medial calcific sclerosis Common Affects Pathology Effect Involves what Not involves Blood flow affected X-ray appearance
Uncommon.
Affects medium-sized arteries.
Calcification of elastic lamina of arteries vascular stiffening without obstruction.
“Pipestem” appearance on x-ray
Does not obstruct blood flow
intima not involved
Atherosclerosis Common Affects what What's it Risk factors Pathophysio Complications Vessels affecting frequency Symptoms
Very common.
elastic arteries and large- and medium-sized muscular arteries
a form of arteriosclerosis caused by buildup of cholesterol plaques.
Risk factors-
Modifiable: smoking, hypertension, hyperlipidemia, diabetes.
Nonmodifiable: age, sex ( in men and postmenopausal women), family history.
Pathophysio Inflammation important in pathogenesis: endothelial cell dysfunction macrophage and LDL accumulation foam cell formation fatty streaks smooth muscle cell migration (involves PDGF and FGF), proliferation extracellular matrix deposition fibrous plaque complex atheromas
Complications Aneurysms ischemia infarcts peripheral vascular disease thrombus emboli.
Frequency-
Abdominal aorta > coronary artery > popliteal artery > carotid artery B .
Symptoms-
Angina, claudication, but can be asymptomatic.
Aortic aneurysms What Symptom of complications Complication Two types. Causes Risk factors Tertiary syphillis causes what ,special name Abdominal aortic aneurysm site High risk of rupture when Abdominal aneurysms occure in who
Localized pathologic dilatation of the aorta.
May cause abdominal and/or back pain
which is a sign of leaking, dissection, or imminent rupture.
Abdominal
Associated with atherosclerosis
Risk factors include history of tobacco use, age, male sex, family history.
May present as palpable pulsatile abdominal mass .
Thoracic
Associated with cystic medial degeneration.
Risk factors include hypertension, bicuspid aortic valve, connective tissue disease (e.g., Marfan syndrome)
Also historically associated with 3° syphilis (obliterative endarteritis of the vasa vasorum).
Below renal vessels above bifurcation
Diameter >5cm
Males >60y,HT
Flank pain/hypotension/pulsatile mass
Aortic dissection What Pathology Risk factors Symtopms Special sign CXR finding Complications 3 Types 2 Involve which parts Treatment If ascending aorta and descending both involves which type
Longitudinal intimal tear forming a false lumen
hypertension
bicuspid aortic valve
inherited connective tissue disorders (e.g., Marfan syndrome).
Can present with tearing chest pain, of sudden onset, radiating to the back +/− markedly unequal BP in arms.
CXR shows mediastinal widening.
Can result in
rupture
pericardial tamponade
death.
Two types:
Stanford type A (proximal):
involves Ascending aorta.
May extend to aortic arch
or descending aorta.
Treatment is surgery.
Stanford type B (distal):
involves descending aorta and/or aortic arch.
No ascending aorta involvement.
Treat medically with β-blockers, then vasodilators.
Stanford A
Ischemic artery disease types
Angina Infarction Coronary steal syndrome SCD Chronic IHD
Angina Means Due to Myocytes damage? Types
Chest pain due to ischemic myocardium
2° to coronary artery narrowing or spasm
no myocyte necrosis
Stable angina
Prinzemetal(variant) angina
Unstable angina
What's stable angina Symptoms Occures when Acute ECG changes Resolves with
Stable—usually 2° to atherosclerosis
exertional chest pain in classic distribution
ST depression on ECG
resolving with rest or nitroglycerin.
Prinze metal angina What's Occurs when Due to Triggers ECG changes RX 3
Chest painoccurs 2° to coronary artery spasm
Occurs at rest
transient ST elevation on ECG
Known triggers include tobacco, cocaine, and triptans, but trigger is often unknown.
Treat with Ca2+ channel blockers, nitrates, and smoking cessation (if applicable).
Unstable angina What's? Due to ECG changes Difference to NSTEMI
Increase in frequency or intensity of chest pain or any chest pain at rest.
thrombosis with incomplete coronary artery occlusion
+/− ST depression and/or
T-wave inversion on ECG
no cardiac biomarker elevation (unlike NSTEMI)
Coronary steal syndrome
Explain
Basis of what
Distal to coronary stenosis, vessels are maximally dilated at baseline.
Administration of vasodilators (e.g., dipyridamole, regadenoson) dilates normal vessels and shunts blood toward well-perfused areas flow and ischemia in poststenotic region.
Principle behind pharmacologic stress tests.
MI Due to what Symtopms Types 2 ECG in both Diagnostic ix
Most often acute thrombosis due to rupture of coronary artery atherosclerotic plaque.
Acute onset typical chest pain
Autonomic symptoms
Lasting more than1/2hr
Not responding to rest or GTN
transmural, ECG may show ST elevations (STEMI)
subendocardial, ECG may show ST depressions (NSTEMI)
Cardiac biomarkers are diagnostic.
