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M3 Path Block 2 > 9 - The Heart > Flashcards

9 - The Heart Flashcards

(51 cards)

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1
Q

Development of Heart (2)

A

Cardiac precursors originate in lateral mesoderm on 15th day

NOTCH PATHWAY!

1st heart field is left ventricle

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2
Q

Di-George Syndrome Gene

A

TBX1

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3
Q

Holt-Orm Syndrome Gene

A

TBX5

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4
Q

Noonan Syndrome

A

PTPN11 (Signaling Protein)

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5
Q

All ACYANOTIC diseases have what letter in them?

A

D - ASD, VSD, PDA

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6
Q

Congenital Heart Disease: ASD Gene

A

GATA4

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7
Q

Congenital Heart Disease: DiGeorge Syndrome Gene and Defined

A

TBX1 Gene Mutation

Abnormality in the development of third and fourth branchial arches

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8
Q

Congenital Heart Disease: DiGeorge Syndrome Mnemonic

A

CATCH-22

Cardiac Abnormality (VSD/ASD)

Abnormal facies

Thymic aplasia (recurrent infections)

Cleft Palate

Hypocalcemica (parathyroid defet)

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9
Q

Congenital Heart Disease: Teralogy of Fallot Gene

A

NOTCH-2

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10
Q

Congenital Heart Disease: Environmental Factors

A

Congenital rubella in 1st trimester –> PDA

Maternal diabetes –> TOF, VSD

Isotretinion (Accutane) –> Transposition of great vessels

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11
Q

Congenital Heart Disease: General Clinical Classifications - (5)

A
  1. Left-Right Shunt
  2. Right-Left Shunt
  3. Obstruction
  4. Shunt is an abnormal communication between the chambers or blood vessels
  5. Atresia: A complete obstruction of a chamber or vessel
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12
Q

Congenital Heart Disease: Left-Right Shunts

A

ACYANOTIC and have ‘D’

ASD, VSD, and PDA

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13
Q

Congenital Heart Disease: Right to Left Shunts

A

CYANOTIC and have ‘T’ (5Ts)

Tetralogy of Fallot

Transposition of great vessels (2 switched vessels)

Tricuspid Atresia

Truncus arteriosus (1 vessel)

Total Anaomalous Pulmonary Venous Return (TAPVR)

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14
Q

Congenital Heart Disease: Obstructive Lesions (3)

A
  1. Coarctation of Aorta
  2. Aortic Stenosis
  3. Pulmonary Stenosis
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15
Q

Congenital Heart Disease: Left-Right Shunts - ASD Defined

A

Communication between left and right atrium

Not synonymous with patent foramen ovale (which closes 80% at age 2)

Classified based on its location

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16
Q

Congenital Heart Disease: Left-Right Shunts - ASD Types (3)

A

10% - Primum Type (associated with VSD)

90% Secundum (most common) and NOT associated with any conditions

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17
Q

Congenital Heart Disease: - Left-Right Shunts ASD C/F

A

Asymptomatic till the age of 30

Soft systolic murmur can be heard due to defect or pulmonary valve

Complications are rare

Low mortality

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18
Q

Congenital Heart Disease: Left-Right Shunts - ASD Treatment

A

Closure either by surgery or catheter

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19
Q

Congenital Heart Disease: Eisenmenger’s Syndrome

A

Left-Right Shunt (VSD, ASD, PDA) —–> Right-Left Shunt

Due to increased pulmonary blood flow –> pathologic remodeling of vasculature –> pulmonary HTN –> RVH to compensate

LATE CYANOSIS

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20
Q

Patent Foramen Ovale: 4)

A

Persistent defect even after 2 years of age

Unsealed flap opens when there is increased right ventricular pressure –> e.g. in coughing, snezzing, Valsalva

High risk of paradoxical embolization (if defect is large, any thrombus from right side can go into systemic circulation)

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21
Q

Congenital Heart Disease: Left-Right Shunts - VSD

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A

Most common congenital heart disease

Free communication between right and left ventricle

22
Q

Congenital Heart Disease: Left-Right Shunts - VSD Types

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A

10% infundibular - near opening of pulmonary valve

**90% membranous type - defect within septum

23
Q

Congenital Heart Disease: Left-Right Shunts - VSD C/F

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A

MANIFEST EARLY IN PEDIATRIC AGE

50% of small muscular VSD close spontaneously

Large VSD complicates reversal of shunt and reversal of shunt and pulmonary HTN

***Risk for INFECTIVE ENDOCARDITIS (pansystolic murmur)

24
Q

Congenital Heart Disease: Left-Right Shunts - PDA

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A

PG-E2 maintains its patency

25
Congenital Heart Disease: Left-Right Shunts - PDA C/F
Continuous harsh machinery murmur Pulmonary HTN and cyanosis
26
Congenital Heart Disease: Left-Right Shunts - PDA in Premature
Communication is between pulmonary trunk and aortic arch,
27
Congenital Heart Disease: Right to Left Shunts - Tetralogy of Fallot (TOF) Components
Mnemonic - PROV 1. Pulmonary infundibulary stenosis 2. RVH 3. Overriding of the aorta 4. VSD * Commonest congenital cyanotic disease because RV is stiff * Boot-shaped heart (from RVH) and tets spell
28
Congenital Heart Disease: Right to Left Shunts - TOF Clues
Child cries during feeding/while playing OR development of cyanosis during feeding or crying
29
Congenital Heart Disease: Right to Left Shunts - TOF Clinical Features
**PULMONARY STENOSIS*** If stenosis is less than its left to right right --> 'pink tetralogy' Severe stenosis --> cyanotic spell
30
Congenital Heart Disease: Right to Left Shunts - Transposition of Great Arteries Defined (4)
RARE - survival is nil without surgical interventions Abnormal formation of truncal aorto-pulmonary setpa Aorta arises from the right ventricle and pulmonary artery arises from left ventricle --> so venous blood goes to systemic circulation directly because aorta arises from RV. Blood doesn’t go to lungs at all for oxygenation (no gas exchange) Atria are normal
31
Congenital Heart Disease: Right to Left Shunts - Transposition - C/F (4)
Depends on the magnitude of tissue hypoxia Complicated with marked RVH *Increased PHT Without surgical interventions, difficult to survive
32
Obstructive Lesions: Coarctation of Aorta - Defined, Forms, and Associations
Constriction of aorta Infantile form - constriction before the origin of the ductus arteriosus Adult form - construction after the ligamentum teres (after PDA closes) Associated with MR, Berry Aneurysms, VSD, AND TURNER SYNDROME
33
Obstructive Lesions: Coarctation of Aorta - C/F
Depends on the PDA opening or closing. If severe, would occur right after birth * Continuous murmur * Notching of ribs
34
Obstructive Lesions: Coarctation of Aorta - Possible Complications Proximal to Coarctation (3)
1. High BP in upper limbs 2. Risk of rupture of Berry's aneurysm 3. Dilatation of aortic arch --> dissection of aorta
35
Obstructive Lesions: Coarctation of Aorta - Possible complications Distal to Coarctation (3)
1. Decreased renal blood flow 2. Poor distal pulses 3. Leg claudication (gangrene)
36
Heart Failure/CHF: Compensatory Mechanisms (6)
1. Frank-Starlings Law 2. Cardiac hypertrophy/dilatation 3. Activation of neurohormonal substances 4. Norepinephrine 5. Renin-Angiotensin 6. ANP
37
Heart Failure/CHF: Systolic Dysfunction
Deterioration of contractile function Reduced ejection fraction Examples: MI Valvular heart disease Hypertension
38
Heart Failure/CHF: Diastolic Dysfunction
Insufficient expansion of chambers during diastole Examples: Constrictive pericarditis Myocardial fibrosis Amyloid deposition
39
Pathophysiology of Hypertrophy
Persistent stimuli --> increase in protein synthesis --> increased mitochondria number --> increased myocyte leading to increased sarcomeres... (3) ***1. Assembled in parallel -----> Concentric hypertrophy (condition which heart following HTN is forcing AGAINST RESISTANCE: Aortic Stenosis, systemic vascular HTN) 2. In series with sarcomere --> Dilatation
40
Pathophysiology of Hypertrophy 2 - Steps to Heart Failure
Pressure overload --> Volume overload --> increased stress (and regional dysfunction) --> cell stretch --> increase in size and mass of heart (also from increased protein synthesis and deposition of abnormal proteins) ----> Heart failure ****Microscopy: Enlarged, box shape nuclei in heart --> concentric hypertrophy *****
41
Left Sided Heart Failure Steps
Left sided heart failure --> passive congestion in lungs --> pooling of blood in left ventricle --> organ dysfunction
42
Morphological Changes In Heart Failure: Heart (2)
1. Hypertrophy | 2. Myocyte hypertrophy with varying degree of fibrosis
43
Morphological Changes In Heart Failure: Lungs
1. Pulmonary congestion and wet lungs | 2. Perivascular and interstitial edema
44
Morphological Changes In Heart Failure: General (4)
1. Edema in interlobular space gives rise to Kerly A and B lines radiologically (pulmonary edema in septum) 2. Widening of interalveolar septum 3. Accumulation of edema fluid in the alveolar spaces * 4. Heart-failure cells (brown) --> tell-tale sign
45
Heart Failure/CHF: Clinical Features
Limits patient's ability to perform the routine activities Tired, Orthopnea, and Dyspnea
46
Right Heart Failure: Cause and Feature
Commonest cause s left heart failure If cause is chronic pulmonary diseases, it is known as 'corpulmonale' Pulmonary HTN is a feature
47
Right Heart Failure is secondary to (5)
1. COPDs 2. Pulmonary HTN 3. Repeated pulmonary thromboembolism 4. Chronic sleep apnea 5. Altitude sickness (Pulm HTN)
48
Right Heart Failure: Morphology (5)
1. Pedal (ankle) and pretibial edema (dependent parts) 2. Heart shows right atrial hypertrophy and dilation * 3. Nutmeg liver: cetrilobular necrosis and areas of cardiac sclerosis 4. Splenomegaly 5. Fluid (effusion) collects in the pleura, pericardium, and peritoneum
49
Infantile form of coarctation leads to? (2)
Turner syndrome with differential cyanosis (lower half of body)
50
Adult form of coarctation of aorta leads to? (2)
Inferior notching of the ribs and disconcoardance (no cyanosis)
51
Most common association with maternal diabetes?
Transposition of Great Arteries

M3 Path Block 2 (10 decks)

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