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M3 Path Block 2 > 9 - The Heart > Flashcards

9 - The Heart Flashcards

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

A

Most common congenital heart disease

Free communication between right and left ventricle

22
Q

Congenital Heart Disease: Left-Right Shunts - VSD Types

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

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

A

PG-E2 maintains its patency

25
Q

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

A

Continuous harsh machinery murmur

Pulmonary HTN and cyanosis

26
Q

Congenital Heart Disease: Left-Right Shunts - PDA in Premature

A

Communication is between pulmonary trunk and aortic arch,

27
Q

Congenital Heart Disease: Right to Left Shunts - Tetralogy of Fallot (TOF) Components

A

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
Q

Congenital Heart Disease: Right to Left Shunts - TOF Clues

A

Child cries during feeding/while playing OR development of cyanosis during feeding or crying

29
Q

Congenital Heart Disease: Right to Left Shunts - TOF Clinical Features

A

PULMONARY STENOSIS*

If stenosis is less than its left to right right –> ‘pink tetralogy’

Severe stenosis –> cyanotic spell

30
Q

Congenital Heart Disease: Right to Left Shunts - Transposition of Great Arteries Defined (4)

A

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
Q

Congenital Heart Disease: Right to Left Shunts - Transposition - C/F (4)

A

Depends on the magnitude of tissue hypoxia

Complicated with marked RVH

*Increased PHT

Without surgical interventions, difficult to survive

32
Q

Obstructive Lesions: Coarctation of Aorta - Defined, Forms, and Associations

A

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
Q

Obstructive Lesions: Coarctation of Aorta - C/F

A

Depends on the PDA opening or closing. If severe, would occur right after birth

  • Continuous murmur
  • Notching of ribs
34
Q

Obstructive Lesions: Coarctation of Aorta - Possible Complications Proximal to Coarctation (3)

A
  1. High BP in upper limbs
  2. Risk of rupture of Berry’s aneurysm
  3. Dilatation of aortic arch –> dissection of aorta
35
Q

Obstructive Lesions: Coarctation of Aorta - Possible complications Distal to Coarctation (3)

A
  1. Decreased renal blood flow
  2. Poor distal pulses
  3. Leg claudication (gangrene)
36
Q

Heart Failure/CHF: Compensatory Mechanisms (6)

A
  1. Frank-Starlings Law
  2. Cardiac hypertrophy/dilatation
  3. Activation of neurohormonal substances
  4. Norepinephrine
  5. Renin-Angiotensin
  6. ANP
37
Q

Heart Failure/CHF: Systolic Dysfunction

A

Deterioration of contractile function

Reduced ejection fraction

Examples:
MI
Valvular heart disease
Hypertension

38
Q

Heart Failure/CHF: Diastolic Dysfunction

A

Insufficient expansion of chambers during diastole

Examples:
Constrictive pericarditis
Myocardial fibrosis
Amyloid deposition

39
Q

Pathophysiology of Hypertrophy

A

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)

  1. In series with sarcomere –> Dilatation
40
Q

Pathophysiology of Hypertrophy 2 - Steps to Heart Failure

A

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
Q

Left Sided Heart Failure Steps

A

Left sided heart failure –> passive congestion in lungs –> pooling of blood in left ventricle –> organ dysfunction

42
Q

Morphological Changes In Heart Failure: Heart (2)

A
  1. Hypertrophy

2. Myocyte hypertrophy with varying degree of fibrosis

43
Q

Morphological Changes In Heart Failure: Lungs

A
  1. Pulmonary congestion and wet lungs

2. Perivascular and interstitial edema

44
Q

Morphological Changes In Heart Failure: General (4)

A
  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
Q

Heart Failure/CHF: Clinical Features

A

Limits patient’s ability to perform the routine activities

Tired, Orthopnea, and Dyspnea

46
Q

Right Heart Failure: Cause and Feature

A

Commonest cause s left heart failure

If cause is chronic pulmonary diseases, it is known as ‘corpulmonale’

Pulmonary HTN is a feature

47
Q

Right Heart Failure is secondary to (5)

A
  1. COPDs
  2. Pulmonary HTN
  3. Repeated pulmonary thromboembolism
  4. Chronic sleep apnea
  5. Altitude sickness (Pulm HTN)
48
Q

Right Heart Failure: Morphology (5)

A
  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
  3. Splenomegaly
  4. Fluid (effusion) collects in the pleura, pericardium, and peritoneum
49
Q

Infantile form of coarctation leads to? (2)

A

Turner syndrome with differential cyanosis (lower half of body)

50
Q

Adult form of coarctation of aorta leads to? (2)

A

Inferior notching of the ribs and disconcoardance (no cyanosis)

51
Q

Most common association with maternal diabetes?

A

Transposition of Great Arteries

M3 Path Block 2 (10 decks)

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