Fa: Cardiovascular - Pathology - 1

Front 1

What are R→L congenital shunts?

Back 1

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 (Total Anomalous Pulmonary Venous Return - 5 letters in name)

Front 2

What are the similarities of the R→L congenital shunts?

Back 2

- Cause of early cyanosis: "blue babies"
- Often diagnosed prenatally or becomes evident immediately after birth
- Usually requires urgent surgical correction and/or maintenance of a PDA

Front 3

What kind of congenital shunts are Trunctus Arteriosus, Transposition, Tricuspid Atresia, Tetralogy of Fallot, TAPVR? Treatment?

Back 3

- They are all R→L shunts causing early cyanosis (blue babies)
- Requires urgent surgical correction and/or maintenance of PDA

Front 4

What happens in a persistent Truncus Arteriosus?

Back 4

- Failure of truncus arteriosus to divide into a pulmonary trunk and aorta
- Most patients have an accompanying VSD

- R→L shunt causes early cyanosis "blue babies"

Front 5

What happens in a D-transposition of the great vessels?

Back 5

- Aorta leaves RV (anterior)
- Pulmonary trunk leaves LV (posterior)
- Leads to separation of systemic and pulmonary circuits
- Not compatible with life unless a shunt is present to allow mixing of blood (eg, VSD, PDA, or patent foramen ovale)

- R→L shunt causes early cyanosis "blue babies"

Front 6

What is the cause of a D-transposition of the great vessels?

Back 6

Failure of the aorticopulmonary septum to SPIRAL

Front 7

What is the prognosis for patients with D-transposition of the great vessels?

Back 7

- Without surgical intervention, most infants die within the first few months of life
- Need a shunt to allow mixing of blood (eg, VSD, PDA, or patent foramen ovale)

Front 8

What happens in Tricuspid Atresia?

Back 8

- Absence of tricuspid valve and hypoplastic RV
- Requires both ASD and VSD for viability

- R→L shunt causes early cyanosis "blue babies"

Front 9

What is the prognosis for patients with Tricuspid Atresia?

Back 9

Non-compatible with life unless there is both an ASD and VSD

Front 10

What is the cause of Tetralogy of Fallot?

Back 10

Anterosuperior displacement of the infundibular septum

Front 11

What is the most common cause of early childhood cyanosis?

Back 11

Tetralogy of Fallot

Front 12

What happens in Tetralogy of Fallot?

Back 12

PROVe
1. Pulmonary infundibular stenosis (most important determinant for prognosis)
2. RV hypertrophy (boot shaped heart on CXR)
3. Overriding aorta
4. VSD

Pulmonary stenosis forces R→L flow across VSD → early cyanotic "tet spells" and RVH

- R→L shunt causes early cyanosis "blue babies"

Front 13

How can you improve symptoms in Tetralogy of Fallot?

Back 13

- Squatting: ↑ SVR (systemic vascular resistance), ↓ R→L shunt, improves cyanosis

- Treatment: early surgical correction

Front 14

What happens in Total Anomalous Pulmonary Venous Return (TAPVR)?

Back 14

- Pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc)
- Associated with ASD and sometimes PDA to allow for R→L shunting to maintain CO

- R→L shunt causes early cyanosis "blue babies"

Front 15

What are L→R congenital shunts? Which are more common?

Back 15

VSD > ASD > PDA
- Ventricular Septal Defect
- Atrial Septal Defect
- Patent Ductus Arteriosus
- Eisenmenger Syndrome

Front 16

What are the similarities of the L→R congenital shunts?

Back 16

- Causes late cyanosis
- "Blue kids"
- VSD > ASD > PDA

Front 17

Which is the most common congenital cardiac defect?

Back 17

Ventricular Septal Defect

Front 18

What happens in a Ventricular Septal Defect?

Back 18

- Asymptomatic at birth
- May manifest weeks later or remain asymptomatic throughout life
- Most self resolve, larger lesions may lead to LV overload and heart failure

- L→R shunt causes late cyanosis ("blue kids")

Front 19

What are the possible complications of a Ventricular Septal Defect?

Back 19

Larger lesions may lead to LV overload and heart failure

Front 20

What happens with an Atrial Septal Defect?

Back 20

- Defect in interatrial septum, usually occurs in septum secundum; septum primum defects usually occur with another anomalies
- Loud S1; wide fixed split S2
- Symptoms: none to heart failure

- L→R shunt causes late cyanosis ("blue kids")

Front 21

How is an Atrial Septal Defect distinct from a Patent Foramen Ovale?

Back 21

Septa is missing tissue rather than unfused

Front 22

What heart sound are associated with an Atrial Septal Defect?

Back 22

- Loud S1
- Wide, fixed split S2

Front 23

What happens in Patent Ductus Arteriosus?

Back 23

- In fetal period, shunt is R→L (normal)
- In neonatal period, ↓ lung resistance → shunt becomes L→R → progressive RVH and/or LVH and heart failure
- Associated with a continuous "machine-like" murmur

Front 24

How can you maintain the patency of the Ductus Arteriosus?

Back 24

PGE synthesis and low O2 tension

PGE kEEps it open

Front 25

How can you close a patent Ductus Arteriosus?

Back 25

Indomethacin (ends patency of PDA)

Front 26

What are the potential complications of an uncorrected Patent Ductus Arteriosus?

Back 26

Can eventually result in late cyanosis in the lower extremities (differential cyanosis)

Front 27

When would you administer PGE to a newborn?

Back 27

To maintain patency of the Ductus Arteriosus (may be necessary to sustain life in conditions such as transposition of the great vessels)

Front 28

When is PDA normal? When should it close?

Back 28

- Normally open in utero
- Normally closes only after birth

Front 29

Which syndrome consists of an uncorrected L→R cardiac shunt (VSD, ASD, or PDA) that eventually switches to R→L, ultimately leads to pulmonary arteriolar hypertension, compensatory RVH, late cyanosis, clubbing, and polycythemia?

Back 29

Eisenmenger syndrome

Front 30

What are the characteristics of Eisenmenger syndrome?

Back 30

- Uncorrected L→R shunt (eg, VSD, ASD, PDA) → ↑ pulmonary blood flow → pathologic remodeling of vasculature → pulmonary arteriolar HTN

- RVH occurs to compensate → shunt becomes R→L

- Causes late cyanosis, clubbing, and polycythemia
- Age of onset varies

Front 31

What are the other heart anomalies besides the R→L and L→R shunts?

Back 31

Coarctation of the Aorta
- Infantile type
- Adult type

Front 32

What is coarctation of the aorta associated with?

Back 32

Bicuspid aortic valve, other heart defects

Front 33

What happens in the infantile type of Coarctation of the Aorta?

Back 33

INfantile: IN close to the heart
- Aorta narrows proximal to the insertion of the ductus arteriosus (PREDUCTAL)
- Associated with Turner Syndrome
- Can present with closure of the ductus arteriosus (reverse w/ PGE2)

Front 34

What happens in the adult type of Coarctation of the Aorta?

Back 34

aDult: Distal to the Ductus
- Aorta narrows distal to ligamentum arteriosum (POSTDUCTAL)
- Associated with notching of the ribs (collateral circulation), HTN in upper extremities, and weak, delayed pulses in lower extremities (radiofemoral delay)

Front 35

What finding is associated with notching of the ribs (collateral circulation), hypertension in upper extremities, and weak, delayed pulses in lower extremities (radiofemoral delay)?

Back 35

Adult type of Coarctation of the Aorta (aorta narrows distal to the ligamentum arteriosum)

Front 36

Which disorder is associated with Truncus Arteriosus and Tetralogy of Fallot?

Back 36

22q11 syndromes

Front 37

Which disorder is associated with ASD, VSD, and AV septal defect (endocardial cushion defect)?

Back 37

Down Syndrome

Front 38

Which disorder is associated with septal defects, PDA, and pulmonary artery stenosis?

Back 38

Congenital Rubella

Front 39

Which disorder is associated with a bicuspid aortic valve and coarctation of the aorta (preductal)?

Back 39

Turner Syndrome

Front 40

Which disorder is associated with MVP (mitral valve prolapse, thoracic artery aneurysm and dissection, and aortic regurgitation?

Back 40

Marfan Syndrome

Front 41

Which disorder is associated with transposition of the great vessels?

Back 41

Infant of diabetic mother

Front 42

What congenital cardiac defects are associated with 22q11 syndromes?

Back 42

- Truncus arteriosus
- Tetralogy of Fallot

Front 43

What congenital cardiac defects are associated with Down Syndrome?

Back 43

- ASD
- VSD
- AV septal defect (endocardial cushion defect)

Front 44

What congenital cardiac defects are associated with Congenital Rubella?

Back 44

- Septal defects
- PDA
- Pulmonary artery stenosis

Front 45

What congenital cardiac defects are associated with Turner Syndrome?

Back 45

- Bicuspid aortic valve
- Coarctation of the Aorta (preductal)

Front 46

What congenital cardiac defects are associated with Marfan Syndrome?

Back 46

- MVP (mitral valve prolapse)
- Thoracic aortic aneurysm and dissection
- Aortic regurgitation

Front 47

What congenital cardiac defects are associated with an infant of a diabetic mother?

Back 47

Transposition of the great vessels

Front 48

What is the definition of Hypertension?

Back 48

- Systolic BP ≥ 140 mmHg And/Or
- Diastolic BP ≥ 90 mmHg

Front 49

What are the risk factors for Hypertension?

Back 49

- ↑ Age
- Obesity
- Diabetes
- Smoking
- Genetics
- Black > White > Asian

Front 50

What are the causes of hypertension?

Back 50

- 90% Primary (Essential), related to ↑ CO or ↑ TPR
- 10% Secondary to renal disease, including fibromuscular dysplasia in young patients

Front 51

What is the definition of a hypertensive emergency?

Back 51

Severe hypertension (≥ 180/120 mmHg) with evidence of acute, ongoing target organ damage (eg, papilledema, mental status change)

Front 52

What does hypertension predispose to / risk factor for?

Back 52

- Atherosclerosis
- LVH
- Stroke
- CHF
- Renal failure (picture - hypertensive nephropathy)
- Retinopathy
- Aortic dissection

Front 53

What does this slide show?

Back 53

Hypertensive Nephropathy - renal arterial hyalinosis on PAS stain

Front 54

What are the signs of hyperlipidemia?

Back 54

- Xanthomas
- Tendinous Xanthomas
- Corneal Arcus

Front 55

What are xanthomas? Cause?

Back 55

- Plaques or nodules composed of lipid laden histiocytes in the skin = A
- Especially common on the eyelids (xanthelasma = B)
- Sign of hyperlipidemia

Front 56

What are Tendinous Xanthomas? Cause?

Back 56

- Lipid deposits in tendons (C)
- Especially common in Achilles
- Sign of hyperlipidemia

Front 57

What are corneal arcus?

Back 57

- Lipid deposits in cornea
- Appears early in life with hypercholesterolemia
- Common in elderly (arcus senilis = D)

Front 58

What is this? Cause?

Back 58

Xanthoma
- Plaques or nodules composed of lipid laden histiocytes in the skin
- Sign of hyperlipidemia

Front 59

What is this? Cause?

Back 59

Xanthelasma
- Plaques or nodules composed of lipid laden histiocytes in the skin
- Especially common on the eyelids
- Sign of hyperlipidemia

Front 60

What is this? Cause?

Back 60

Tendinous Xanthoma
- Lipid deposits in tendons
- Especially common in Achilles
- Sign of hyperlipidemia

Front 61

What is this? Cause?

Back 61

Corneal Arcus
- Lipid deposits in cornea
- Appears early in life with hypercholesterolemia
- Common in elderly (arcus senilis = D)

Front 62

What are the types of arteriosclerosis?

Back 62

- Arteriolosclerosis (common)
- Mönckeberg (medial calcific sclerosis)

Front 63

What are the types of arteriolosclerosis?

Back 63

- Hyaline - thickening of small arteries in essential HTN or DM) (left)
- Hyperplastic - "onion skinning" as seen in severe HTN (right)

Front 64

What is the uncommon form of Arteriosclerosis? Characteristics?

Back 64

Mönckeberg (Medial Calcific Sclerosis) Arteriosclerosis
- Uncommon
- Calcification in the media of arteries, especially radial or ulnar
- Usually benign; "pipestem" arteries on x-ray
- Does not obstruct blood flow
- Intima not involved

Front 65

What is the term for the thickening of small arteries in essential hypertension or diabetes?

Back 65

Hyaline Arteriolosclerosis

Front 66

What is the term for the "onion skinning" appearance of small arteries seen in severe hypertension?

Back 66

Hyperplastic Arteriolosclerosis

Front 67

What is the term for the usually benign process that causes a "pipestem" appearance of arteries on x-ray? Which arteries are typically affected? Implications?

Back 67

Mönckeberg (Medial Calcific Sclerosis) Arteriosclerosis
- Uncommon
- Calcification in the media of arteries, especially radial or ulnar
- Does not obstruct blood flow
- Intima not involved

Front 68

What is affected by atherosclerosis?

Back 68

Disease of elastic arteries and large- and medium-sized muscular arteries

Front 69

What are the modifiable risk factors for atherosclerosis?

Back 69

- Smoking
- Hypertension
- Hyperlipidemia
- Diabetes

Front 70

What are the non-modifiable risk factors for atherosclerosis?

Back 70

- Age
- Sex (more in men and postmenopausal women)
- Family history

Front 71

How does atherosclerosis progress?

Back 71

- 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, and ECM deposition → fibrous plaque → complex atheromas (picture)

Front 72

What is the appearance of atheromas?

Back 72

Cholesterol crystals
- Fatty streaks

Front 73

What are the complications of atherosclerosis?

Back 73

- Aneurysms
- Ischemia
- Infarcts
- Peripheral vascular disease
- Thrombus
- Emboli

Front 74

What are the more common locations of atherosclerosis?

Back 74

Abdominal aorta > Coronary artery > Popliteal artery > Carotid Artery (picture)

Front 75

What are the symptoms of Atherosclerosis?

Back 75

- Angina
- Claudication
- Can be asymptomatic

Front 76

What is an aortic aneurysm? Location?

Back 76

Localized pathologic dilation of the aorta
- Abdominal AA
- Thoracic AA

Front 77

What does it mean if the aortic aneurysm is painful?

Back 77

Sign of leaking, dissection, or imminent rupture!!

Front 78

What is an abdominal aortic aneurysm associated with?

Back 78

- Associated with atherosclerosis
- Occurs more frequently in hypertensive male smokers >50 years old

Front 79

What is a thoracic aortic aneurysm associated with?

Back 79

- Associated with cystic medial degeneration due to hypertension (older patients) or Marfan syndrome (younger patients)
- Also historically associated with 3° syphilis (obliterative endarteritis of the vasa vasorum)

Front 80

What is this a CT of?

Back 80

Abdominal aortic aneurysm
- Suprarenal
- Eccentric mural thrombus

Front 81

What is this a CT of?

Back 81

Thoracic aortic aneurysm
- Ascending aorta
- Dissection (arrow)

Front 82

What happens with an aortic dissection?

Back 82

Longitudinal intraluminal tear forms a false lumen
- The false lumen can be limited to the ascending aorta, propagate from the ascending aorta, or propagate from the descending aorta

Front 83

What is aortic dissection associated with?

Back 83

- HTN
- Bicuspid aortic valve
- Inherited CT disorders (eg, Marfan syndrome)

Front 84

How does a patient with an aortic dissection present?

Back 84

- Tearing chest pain of sudden onset
- Radiates to back
- +/- markedly unequal BP in arms
- CXR shows mediastinal widening

Front 85

What are the possible outcomes of a patient with aortic dissection?

Back 85

- Pericardial tamponade (fluid accumulates in the pericardium)
- Aortic rupture
- Death

Front 86

What are the manifestations of ischemic heart disease?

Back 86

- Angina (stable, unstable/crescendo, variant/Prinzmetal)
- Coronary steal syndrome
- Myocardial infarction
- Sudden cardiac death
- Chronic ischemic heart disease

Front 87

What is the term for chest pain due to ischemic myocardium 2° to coronary artery narrowing or spasm, without myocyte necrosis?

Back 87

Angina

Front 88

What are the characteristics and types of angina?

Back 88

- Chest pain due to ischemic myocardium
- 2° to coronary artery narrowing or spasm
- No myocyte necrosis
- Types: stable, variant / Prinzmetal, and unstable / crescendo

Front 89

What is the cause of Stable Angina? Characteristics?

Back 89

- Usually 2° to atherosclerosis
- Exertional chest pain in classic distribution (usually with ST depression on ECG)
- Resolves with rest

Front 90

What is the cause of Variant / Prinzmetal Angina? Characteristics?

Back 90

- Occurs at rest 2° to coronary artery spasm
- Transient ST elevation on ECG
- Known triggers include tobacco, cocaine, and triptans (but often unknown)
- Treat with CCB, nitrates, and smoking cessation (if applicable)

Front 91

What is the cause of Unstable/Crescendo Angina? Characteristics?

Back 91

- Thrombosis with incomplete coronary artery occlusion
- ST depression on ECG (↑ in frequency or intensity of chest pain; any chest pain at rest)

Front 92

If a patient's angina is triggered by tobacco, cocaine, or triptans, what is the cause?

Back 92

Variant angina (Prinzmetal) = coronary artery vasospasm

Front 93

What ECG signs are there to distinguish the types of angina?

Back 93

- Stable: ST depression
- Variant / Prinzmetal: ST elevation
- Unstable / Crescendo: ST depression

Front 94

What is the principle behind pharmacologic stress tests?

Back 94

Coronary Steal Syndrome
- Distal to coronary stenosis, vessels are maximally dilated at baseline
- Administration of vasodilators (eg, dipyridamole, regadenoson) dilates normal vessels and shunts blood toward well-perfused areas
- Leads to decreased flow and ischemia in post-stenotic region

Front 95

In the coronary steal syndrome, where is there decreased flow and ischemia?

Back 95

The area distal to the coronary stenosis gets decreased flow and ischemia because after administration of vasodilators the normal vessels dilate and shunt blood towards the well-perfused areas

Front 96

What is the most common cause of Myocardial Infarction?

Back 96

Acute thrombosis due to coronary artery atherosclerosis with complete obstruction of coronary artery

Front 97

What happens in tissue that has had a Myocardial Infarction?

Back 97

- Myocyte necrosis
- If transmural, ECG will show ST elevations
- If subendocardial, ECG may show ST depressions
- Cardiac biomarkers are diagnostic

Front 98

What are the signs on EKG of a Myocardial Infarction?

Back 98

- Transmural: ST elevations
- Subendocardial: ST depressions