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Peds Cardiology > 1. Cardiac Anatomy & Physiology > Flashcards

1. Cardiac Anatomy & Physiology Flashcards

1
Q

LV isovolumic contraction continues until…

A

Aortic valve opens

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2
Q

What causes aortic valve to open?

A

Lower pressure in the aorta compared to LV after a period of isovolumic contraction

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3
Q

What happens in the heart at end diastole?

A

MV closes (LV filling has completed)

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4
Q

What valve are Libman-Sacks lesions typically on?

A

Mitral (left sided valves)

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5
Q

Verrucous, non-bacterial valve lesion in a patient with SLE?

A

Libman-Sacks endocarditis

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6
Q

What % of patients newly diagnosed with SLE have Libman-Sacks lesion?

A

10%

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7
Q

Describe echo findings of a Libman-Sacks lesion?

A

Irregular vegetation <0.5cm in diameter on valve/chordal apparatus

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8
Q

Metabolic factor derived from breakdown of high energy phosphate?

A

Adenosine

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9
Q

True or False: ATP can’t be regenerated at times of low O2 tension

A

True

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10
Q

What happens to ATP in states of low O2 tension?

A

AMP made, breaks down to adenosine which causes coronary artery vasodilation

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11
Q

What does NO induce?

A

cGMP

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12
Q

What does NO induction of cGMP cause?

A

Muscle relaxation

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13
Q

What does Endothelin 1 cause?

A

Tonic vasoconstriction

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14
Q

What does prostaglandin cause?

A

Smooth muscle relaxation

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15
Q

Describe abdominal anatomy in polysplenia

A
  1. Multiple spleens on same side of vertebral column as stomach
  2. Gallbladder single (may have biliary atresia)
  3. Abdominal situs variable
  4. IVC commonly interrupted with azygous continuation to SVC
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16
Q

In a mature cardiac myocyte, where is the majority of calcium involved in the binding of troponin C/initation of myocyte contraction is stored where?

A

SR

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17
Q

What stores the most important source of Ca involved in initiatino of myocyte contraction?

A

SR

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18
Q

How does Ca enter the myocyte during an action potential?

A

L-type voltage gated Ca channel

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19
Q

What happens after Ca enters a myocyte during an action potential?

A

Ca activated Ca release via ryanodine receptor - Causes release of Ca from SR

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20
Q

What is different about Ca in a immature cardiac myocyte v. adult?

A

Function/organization of SR isn’t mature… activation more dependent on flow via L-type Ca channels v. Ca induced Ca release from SR

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21
Q

Most common form of bicuspid aortic valve?

A

R/L fusion (75%)

-Next:
R/N
L/N

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22
Q

What can fusion of 1+ cusp on an aortic valve result in?

A

Unicuspid valve

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23
Q

What hormone is released in response to decreased renal arterial pressure and induces cleaving of angiotensinogen to angiotensin 1?

A

Renin

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24
Q

What does the juxtaglomerular apparatus of the kidney secrete in response to low renal perfusion pressure?

A

Renin

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25
Q

What does renin do?

A

Cleaves angiotensinogen to angiotensin 1

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26
Q

What converts angiotensin 1 to angiotensin 2?

A

ACE

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27
Q

Where does ACE convert angiotensin 1 to angiotensin 2?

A

Lungs/vasculature

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28
Q

What does Angiotensin 2 do?

A

Induces vasoconstriction and stimulates ADH (vasopressin) secretion

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29
Q

What factor has the greatest impact on pressure change across 2 points in a vessel?

A

Radius

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30
Q

What equation describes the resistance between 2 points?

A

Poiseuille-Hagen relationship

-Resistance between 2 points is a function of pressure/flow

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31
Q

What is the Poiseuille-Hagen equation?

A

R = (8 x Length of vessel x viscosity) / (pi x radius2)

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32
Q

Normal left aortic arch is derived from what embryologic aortic arch?

A

Left 4th

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33
Q

Majority of the aortic arch arises from what embryologic structure?

A

Left 4th arch

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34
Q

What does the right 4th arch give rise to?

A

Proximal portion of right subclavian

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35
Q

Where do the pulmonary arteries and ductus arise from?

A

Left 6th arch

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36
Q

What does the 5th aortic arch give rise to?

A

Nothing… typically involutes

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37
Q

What other defect is most commonly seen in patients with ToF?

A

ASD/PFO

-80% of patients, “Pentalogy of Fallot”

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38
Q

True or False: Left heart abnormalities are common in patients with ToF?

A

Flase

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39
Q

A right aortic arch occurs in what % of patients with ToF?

A

25%

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40
Q

Most common coronary artery abnormality in TGA?

A

Left circumflex from the RCA (16%)

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41
Q

Most common coronary artery pattern in D-TGA?

A

Normal (67%)

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42
Q

After left circumflex from the RCA, what are the next most common coronary abnormalities in D-TGA?

A
  1. Single right

2. Inverted right and left circumflex with normal origin of LAD from anterior facing sinus

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43
Q

Echo-dark space around aortic sinus in PSSA view that extends anterior to SVC and posterior to ascending aorta and pulmonary trunk?

A

Transverse sinus

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44
Q

What is the transverse sinus?

A

Intrapericardial space between the great arteries anterosuperiorly and atrial walls posterioinferiorly

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45
Q

What can be seen in post-operative patients as an echo-dark space around the aortic sinus in PSSA if fluid is present?

A

Transverse sinus

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46
Q

What can the transverse sinus be mistaken for in a neonate if there is a small amount of fluid in the sinus?

A

Take-off of LCA

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47
Q

What is the space between the posterior LA and reflections of great veins (SVC and pulmonary veins)?

A

Oblique sinus

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48
Q

How does ANP work on the kidney?

A

Decreases distal tubular resorption of sodium

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49
Q

What causes release of ANP?

A

Stretch from either atrium

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50
Q

What does ANP do to the kidney?

A

Dilates afferent arteriole, constricts efferent arteriole

Increases GFT

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51
Q

2 effects of ANP?

A
  1. Vasodilatory

2. Cardioinhibitory

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52
Q

Average ratio of ventricular septal thickness to LV free wall thickness in adolescent heart?

A

1.1

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53
Q

Average ratio of ventricular septal thickness to LV free wall thickness in heart in first 2 decards?

A
  1. 1 (range 0.8-1.4)

* Radio increases slowly into adulthood averaging >1.2 by 70

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54
Q

What diseases can affect the average ratio of ventricular septal thickness to LV free wall thickness?

A

HCM

*Diseases with asymmetric hypertrophy

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55
Q

What is the average ratio between LV/RV thickness?

A

3 (range 2-5)

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56
Q

Is the average ratio between LV/RV thickness higher or lower in fetuses and neonates as compared to adults?

A

Lower

*High right-sided pressure in utero

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57
Q

What causes a diastolic murmur in a VSD?

A

AI from herniation of aortic valve leaflet though defect

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58
Q

Which aortic cusp is most likely to herniate through a VSD?

A

Right

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59
Q

What types of VSDs can you see herniation of the right aortic cusp?

A
  1. Subarterial (supracristal/infundibular)

2. Perimembranous

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60
Q

What % of VSDs are subarterial (supracritsal/infundibular)?

A

5%

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61
Q

What ethnicity are subarterial VSDs more common in?

A

Asians

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62
Q

What is the definition of an overriding AV valve?

A

Empties into 2 ventricles

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63
Q

What is an overriding AV valve always assocaited with?

A

Malalignment VSD

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64
Q

What is it called if you have an AV valve that empties into 2 ventricles and has anomalous insertions of the chordae tendineae into the contralateral ventricle?

A

Overriding and straddling

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65
Q

Which chamber is most likely to rupture following chest wall trauma?

A

RV

*Thin walled and more anterior

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66
Q

True or False: Ventricular wall rupture is more common than atrial rupture

A

True

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67
Q

What is the most common site of an atrial rupture?

A

Appendage

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68
Q
  1. Descending aorta and IVC on same side of vertebral column
  2. High incidence of malrotation
  3. Variable position of stomach
  4. Typicall only 1 gallbladder
  5. Biliary atresia uncommon
  6. Liver most commonly midline with 2 mirror-image right lobes
A

Asplenia

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69
Q

4 changes with endurance training

A
  1. Increased blood volume/stroke volume
  2. Decreased HR
  3. Decreased resting arterial BP
  4. Decreased myocardial oxygen demand
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70
Q

Most reliable feature of normal AV valve that distinguishes mitral from tricuspid?

A

Level of attachment at cardiac crux

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71
Q

Which AV valve inserts higher into the cardiac crux?

A

Mitral (between 0-8mm/m2 higher than tricuspid)

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72
Q

In Ebstein’s the tricuspid valve is how much lower than the mitral?

A

> 8mm/m2

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73
Q

What 2 lesions have no difference between where the mitral and tricuspid valve inset?

A
  1. Partial AVSD

2. DILV

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74
Q

What shifts O2 dissociation curve left?

A
  1. Alkalosis
  2. Decreasing temp
  3. Decreasing 2,3-DPG
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75
Q

What shifts O2 dissociation curve right?

A
  1. Acidosis
  2. Increased temp
  3. Increased 2,3-DPG
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76
Q

Where is the sinus node in right juxtaposition of the atrial appendages?

A

Normal position

*Usually single SA node

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77
Q

Describe sinus node in right atrial isomerism

A

Bilateral

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78
Q

Describe sinus node in left atrial isomerism

A

Absent or malpositioned

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79
Q

Describe sinus node in left-sided juxtaposition of the atrial appendages

A

Displaced anteriorly or inferiorly

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80
Q

What type of lesions is left juxtaposition of the atrial appendages associated with?

A

Abnormal ventriculoarterial connections

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81
Q

What type of lesions is right juxtaposition of the atrial appendages associated with?

A

Simple (ASD, VSD)

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82
Q

A decrease in pO2 causes the systemic vasculature to do what?

A

Dilate- Local tissues trying to get more O2 through increased volume of flow

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83
Q

Name 3 things that cause local vasodilation

A
  1. Increased pCO2
  2. Acidosis
  3. Increased K
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84
Q

What does some tissue release as a vasodilator in response to increased oxygen demand?

A

Adenosine

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85
Q

What is the valvular remnant at the IVC/RA junction?

A

Eustachian valve

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86
Q

What is the crescent-shaped valvular remnant at the os of the coronary sinus?

A

Thebesian valve

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87
Q

What is the fine, filamentous structure that represents the persistence of the valves of the sinus venosus?

A

Chiari network

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88
Q

Where does the Chiari network typically extend from?

A

Crista terminalis to Eustachian or Thebesian valves

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89
Q

What is the role of the Eustachian valve?

A

Directs blood flow from SVC/IVC through PFO to LA

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90
Q

What does the crista terminalis form from?

A

Resorption of the right valve of the sinus venosus

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91
Q

What is the crista terminalis?

A

Ridge in the RA that separates the sinus venosus portion of the RA from the muscular RA

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92
Q

Describe an intermediate AVSD

A
  • Large atrial septal and ventricular septal defect
  • Common atrioventricular valve
  • Separate left and right orifices

*Rare subtype of a complete AVSD

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93
Q

What is a partial AVSD?

A
  • Septum primum ASD
  • Cleft left AV valve in anterior leaflet
  • L/R AV valves are separate
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94
Q

What is a transitional AVSD?

A

Subtype of partial defect… primum ASD, cleft left AV valve, separate L/R AV valves + inlet VSD

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95
Q

What fetal venous structure has the lowest O2 saturation?

A

CS and SVC

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96
Q

Most reliable anatomic feature that distinguishes the normal RV from LV?

A

Level of insertion of AV valve at cardiac crux

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97
Q

Which valves are in fibrous continuity in the normal heart?

A

Aortic and mitral

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98
Q

The direction of blood flow through an ASD is primarily related to what?

A

Relative compliances of LV/RV

*Otherwise normal patient, RV is more compliant so get L-R shunting

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99
Q

Most common anatomic form of HLHS?

A

AA/MA

*AS/MS and AA/patent MV are similar in occurence

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100
Q

In the cardiac sarcomere, what includes the entirety of the myosin contractile elements?

A

A-band

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101
Q

What bisects the A-band in the cardiac sarcomere?

A

M-line

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102
Q

What contains purely actin elements of the cardiac sarcomere?

A

I-band

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103
Q

What bisects the I-band in the cardiac sarcomere?

A

Z-disk

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104
Q

What is the central subsection of the A-band that doesn’t include the areas of myosin-actin overlap?

A

H-zone

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105
Q

Most common truncal valve morphology in truncus arteriosus?

A

Tricuspid (70%)

*Next:
Quadricuspid (20%)
Bicuspid (10%)
Pentacuspid (<1%)
Unicommisural (<1%)
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106
Q

What valves is a truncal valve in fibrous continuity with?

A

Mitral in all patients

-Can rarely be in fibrous continuity with tricuspid

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107
Q

The resting potential of which ion is primarily responsible for the baseline (phase 4) resting conductance of cardiac myocytes?

A

K

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108
Q

What current is the dominant resting conductance of the myocyte keeping it negatively polarized around -85mV until an AP arrives to activate the cell into phase 0?

A

Ik1 K current

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109
Q

The rapid depolarization of cardiac myocytes (phase 0) is driven by the rapid influx of which ion into the myocytes?

A

Na

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110
Q

When an AP arrives to the cardiac myocyte, what channels open resulting in rapid depolarization?

A

Na

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111
Q

What provides the prolonged phase 1/2 depolarization required to achieve muscle contraction?

A

K and Ca conductance

Phase 2: Ca
Phase 3: K

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112
Q

SCN5A mutations involve which channel gene?

A

Na

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113
Q

SCN5A mutations can cause which 2 conditions?

A
  1. Type 3 Long QT

2. Brugada

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114
Q

Neonate with hypoxia + decreased vascular markings?

A

PS

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115
Q

Where is a sinus venosus ASD relative to the fossa ovalis?

A

Posterior/superior

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116
Q

What defect is typically secondary to absence of usual rim of tissue between the right pulmonary veins and the RA?

A

Sinus venosus

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117
Q

What commonly occurs with sinus venosus ASD?

A

Anomalous right upper pulmonary venous return

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118
Q

What is the most abundant non-myocyte cardiac cell in the mature heart?

A

Fibroblast

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119
Q

What type of cells are vital for structural integrity of the heart and play a large role in remodeling and development?

A

Fibroblasts

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120
Q

List 2 roles of cardiac fibroblasts

A
  • Responsible for deposition of extracellular matrix and contribute to remodeling through secretion of metalloproteinases
  • Also involved in secretion of cytokines and growth factors which influence neighboring cells
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121
Q

What are contractile cells that help regulate blood flow in capillaries?

A

Pericytes

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122
Q

What type of cells are mostly found in medium caliber blood vessels in the heart?

A

Vascular smooth muscle cells

123
Q

Where are macrophages in the heart?

124
Q

What type of cells line the inner wall of the ventricles?

A

Endothelial cells

125
Q

Most common additional abnormality seen in patients with Ebstein?

A

ASD

  • > 80% of patients
  • Next most common is PS (even functional or true PA), then VSD
126
Q

What has to be determined prior to designing treatment plans for a neonate with severe Ebstein?

A

Degree of RVOT obstruction

127
Q

True or False: Left-sided lesions are common in Ebstein?

A

False

*LVNC reported in some genetic association studies, but otherwise uncommon

128
Q

What 2 structures have the lowest O2 saturation in fetus?

129
Q

What structure passes under the transverse aortic arch in normal heart?

A

RPA and left bronchus

130
Q

The PA at its bifurcation is to what side of the ascending aorta?

131
Q

Where does the LPA travel in relation to the left bronchus?

A

Over the left bronchus

132
Q

What arterial vessel is partially formed by the remnants of the 3rd aortic arch?

A

Common carotid

133
Q

What does 1st aortic arch form?

A

Part of maxillary artery

134
Q

What does 2nd aortic arch form?

A

Part of stapedial artery

135
Q

What does 3rd aortic arch form?

A

Common carotid artery

Proximal portion of the internal carotid artery

136
Q

Which structures connect cardiac myocytes end to end and are responsible for structural integrity and synchronized contraction of cardiac tissue (transmission of electrical impulses)?

A

Intercalated discs

137
Q

What are intercalated discs made of?

A

Adherens junctions
Desmosomes
Gap junction

138
Q

Name 2 proteins that connect the myocytes to the extracellular matrix

A

Dystrophin

Costameres

139
Q

What are invaginations of the sarcolemma which allows transmission of the action potential to the inner part of the cell?

140
Q

What nerve is a branch of the vagus nerve that travels inferiorly around the aortic arch before traveling superiorly to innervate muscles in the larynx?

A

Left recurrent laryngeal nerve

141
Q

What is a potential cause of post-op hoarseness and stridor?

A

Injury to the left recurrent laryngeal nerve

142
Q

What types of surgery have risk to the left recurrent laryngeal nerve?

A

Anything with manipulation of the aortic arch

143
Q

What structure does the right recurrent laryngeal nerve loop around?

A

Right subclavian artery

144
Q

Most common aorto-pulmonary positions in patients with DORV?

A

Side-by-side with aorta to the right of the PA (2/3 of DORV)

145
Q

Most common ASD?

A

Secundum (75%)

146
Q

Where are secundum ASDs found?

A

Region of fossa ovalis

147
Q

Second most common type of ASD?

A

Septum primum (20%)

148
Q

2 least common types of ASD?

A
  1. Sinus venosus (5%)

2. Coronary sinus (<1%) - often associated with heterotaxy

149
Q

Name 3 possible factors that can contribute to the formation of a secundum ASD

A
  1. Deficient growth of septum secundum
  2. Excessive resportion of septum primum
  3. Deficient valve tissue
150
Q

Normal right SVC is derived from what embryologic structure?

A

Right anterior cardinal vein and right common cardinal vein

151
Q

If the left anterior cardinal vein persists, what does it become?

A

Left SVC

*Left anterior cardinal vein typically regresses

152
Q

What is the ductus venosus remnant termed?

A

Ligamentum venosus

153
Q

The round ligament of the liver is part of what?

A

Umbilical vein remnants

154
Q

What is the most common hemodynamically significant extracardiac AV malformation in neonates?

A

Vein of Galen

155
Q

Which gender is more affected by Vein of Galen malformations?

156
Q

How does a Vein of Galen malformation present?

A

High-output cardiac failure soon after birth

157
Q

What exam finding suggests a Vein of Galen malformation?

A

Bruit in the fontanelles

158
Q

List morphologic features of the tricuspid valve

A
  1. Septal chordal attachments
  2. Triangular-shaped orifice at the mid-leaflet level
  3. Presence of 3 leaflets and commissures
  4. Emptying into a morphologic RV
159
Q

List morphologic features of the mitral valve

A
  1. 2 leaflets
  2. Elliptical-shaped orifice
  3. 2 commissures
  4. Empties into morphologic LV
160
Q

Which fetal remnant delivers blood from right/left hepatic veins to RA?

A

Ductus venosus

161
Q

What does the ductus venosus do?

A

Receives blood flow from the hepatic veins and umbilical vein and delivers it to the RA

162
Q

What happens to the ductus venosus after birth?

A

Becomes the ligamentum venosum

163
Q

Where is the aortic isthmus located?

A

Between the origin of the left subclavian artery and the ligamentum arteriosum

164
Q

What do the umbilical arteries do?

A

Bring deoxygenated blood from internal iliac arteries to placenta

165
Q

What coronary abnormality needs to be identified in a ToF patient who will be undergoing a transannular patch?

A

LAD from RCA (5%)

*Coronary then takes a course anterior to the RVOT

166
Q

Besides LAD from RCA, what is another potential coronary abnormality in ToF patients?

A

Accessory LAD (large conal branch), 10-15%

167
Q

What is the association between the atrial and fossa ovalis?

A

LA: Valve of fossa ovalis
RA: Limbus of fossa ovalis

168
Q

Name an anatomic hallmark of the morphologic RA

A

Ostium of the IVC

*Suprahepatic IVC nearly always connects to the RA

169
Q

A finger-like appendage and smooth walled surfaces are characteristic of which atria?

170
Q

True or False: Pulmonary vein ostia are good predictors of atrial morphology?

A

False- High frequency of anomalous pulmonary venous return

171
Q

Most common relationship of great arteries in tricuspid atresia?

A

Normal (70-80%)

172
Q

Relationship between VSD size and PS in patients with tricuspid atresia?

A
  • Bigger VSD with widely patent pulmonary outflow

- Smaller VSD with PS

173
Q

During relaxation phase of cardiac muscle contraction, majority of Ca is removed from sarcoplasm by what?

A

Reuptake in the sarcoplasmic reticulum via SERCA pumps

  • 80% of the Ca that is sequestered during the relaxation phase of the cardiac muscle contraction is via Ca-ATPase SERCA pumps located on the SR
  • 20% is removed from the cell through Na-Ca and Ca-ATPase pumps located on the sarcolemma
174
Q

Where is the AV node in tricuspid atresia?

A

Floor of RA

175
Q

Where is the bundle of His in tricuspid atresia?

A

Courses from AV node on floor of RA to the crest of the intraventricular septum (muscular) and runs posterior to the VSD rim

*Any surgery involving VSD, worry about heart block

176
Q

List features of the RV in the normal heart

A
  1. Tripartite (inlet, trabecular, outlet regions)
  2. Coarse septal surface
  3. Prominent apical trabeculations
  4. Tricuspid/pulmonary valves not in continuity
177
Q

When can you see a tricuspid/pulmonary valve in continuity?

A

ToF with membranous VSD extension

178
Q

Most common additional cardiac finding in TGA?

A

VSD (40-45%)

*Majority perimembranous, muscular, or malalignement

179
Q

What are 3 less common additional cardiac findings in TGA?

A
  1. Isolated LVOTO (5%)
  2. CoA, arch hypoplasia or IAA (5%)
  3. Left juxtaposition of atrial appendages (2-5%)
180
Q

What structure is often abnormal in TGA, but is rarely functionally significant?

A

Mitral valve (20%)

-Cleft MV most common

181
Q

What causes cyanosis in a patient with severe Ebstein with severe TR?

A

R-L atrial shunting

*Often worsens with exercise in older patients

182
Q

What is the most common associated finding in Ebstein?

A

ASD or PFO (80%)

183
Q

Anatomic hallmark of morphologic LA?

A

Valve of fossa ovalis (septum primum)

184
Q

Anatomic hallmark of morphologic PA?

A

Limbus of fossa ovalis and entrance of IVC

185
Q

Which atria is more posterior?

186
Q

Describe the atrial appendages

A

LA: Fingerlike, trabeculated
RA: Broad-based

187
Q

What is fetal Hgb composed of?

A

Alpha/Gamma subunits

188
Q

Does fetal or adult Hgb have a higher affinity for O2?

189
Q

What is adult Hgb composed of?

A

Alpha/Beta subunits

190
Q

Why does fetal Hgb have a higher affinity for O2 compared to adult?

A

Needed for transport of o2 across placenta

191
Q

In a normal newborn, fetal Hgb is replaced by adult Hgb by what age?

192
Q

Through what pathway does norepinephrine activate B1 adrenergic receptors?

A

Gs dependent activation of adenylate cyclase… increases cAMP

193
Q

Norepinephrine activates B1 adrenergic receptors by doing what?

A

Activating the Gs subunit of the G-protein complex which activates adenylate cyclase

194
Q

What does adenylate cyclase do?

A

Converts ATP to cAMP

195
Q

What does cAMP do?

A

Activates protein kinase A

196
Q

What does protein kinase A do?

A

Phosphorylates multiple proteins involved in muscle contraction and AP of heart

*Increases chronotropy and inotropy

197
Q

What happens to pleural pressure and intra-abdominal pressure with inspiration?

A
  • Fall in pleural pressure

- Rise in intra-abdominal pressure

198
Q

What happens to right sided venous return and RV stroke volume with inspiration?

A
  • Increased right sided venous return

- Increased RV stroke volume

199
Q

What happens to the E’ velocity in the tricuspid and mitral valve with inspiration?

A
  • E’ increases across tricuspid (5-10%)

- E’ decreases across mitral (5-10%)

200
Q

What is characterized by absent or abnormal chordal insertions for the mitral valve?

A

Mitral arcade or hammock mitral valve

  • Leaflet edges are often thickened and rolled
  • Leaflets are relatively tethered with poor coaptation due to direct insertion of leaflets
  • MR common, but functional MS can occur
201
Q

What causes an ostium primum ASD?

A

Abnormal endocardial cushion development

202
Q

Where is a primum ASD located?

A

Anterior to the fossa ovalis in the inlet portion of the ventricular septum

203
Q

What 2 things result from failure of the endocardial cushions to develop?

A
  1. Primum ASD

2. AVCD

204
Q

What happens when there is failure of the common pulmonary vein to connect to the LA?

A

Anomalous pulmonary venous return

205
Q

What are 2 common etiologies behind secundum atrial septal defects?

A
  1. Excessive cell death/resorption of the septum primum

2. Insufficient growth of the septum secundum

206
Q

A persistent L-SVC is a remnant of what?

A

Left horn of the sinus venosus

207
Q

Where is a L-SVC seen on echo?

A

-TTE: Runs lateral and leftward to PA on high parasternal short-axis views with flow heading inferiorly. –TEE: Runs between left pulmonary veins and left atrial appendage (just posterior and superior to mitral valve)

208
Q

What might an L-SVC be mistaken for?

A

Descending aorta

209
Q

Where does a L-SVC run before joining the CS in the left AV groove?

A

Posteriorly along LA

210
Q

How much deoxygenated blood is needed to visualize true cyanosis?

A

At least 5g/dL

211
Q

In what patient would a relatively low O2 not result in expected clinical cyanosis?

A

Anemia- Not enough deoxygenated blood

212
Q

What area is the best indicator of cyanosis and why?

A

Tongue- right vascular supply and minimal pigmented cells

213
Q

Mutations in which protein may cause disassociation of the intracellular cytoskeleton and the extracellular matrix in cardiac myocytes in addition to causing muscular dystrophy?

A

Dystrophin

214
Q

What does dystrophin do?

A

Protein that links extracellular matrix and cytoplasmic actin providing link between extracellular and intracellular structures of the myocyte

215
Q

Mutations in the gene that encode for what protein can cause muscular dystrophy and DCM?

A

Dystrophin

216
Q

Name 2 proteins that are involved in the linking of intracellular and extracellular structures and can cause long QT or SIDS if mutated

A

Caveolin

Syntrophin

217
Q

Mutations in cytoplasmic actin or dystroglycan complex can cause what?

218
Q

In the sarcomere, which protein binds to Ca and allows cross-bridges to form permitting contraction?

A

Troponin C

  • Ca binds to troponin C, changing the tertiary structure of troponin C and other troponin subunits
  • Allows tropomyosin to shift positions and allows myosin and actin binding leading to muscle cell contraction
219
Q

Children living at high elevations have higher _ pressures as compared to those living at sea level?

A

Mean PA pressure

*More exacerbated with exercise

220
Q

What might explain the relatively increased risk of persistence of the PDA in children living at high altitude?

A

Higher mean PA pressures

221
Q

Where are baroreceptors located?

A

Carotid sinus

Aortic arch

222
Q

Increased arterial pressure on the baroreceptors in the carotid sinus or aortic arch cause what?

A

Decreased BP via decreased HR and vasodilation

223
Q

What is the primary mechanism that the myocardium uses to compensate for increased O2 demand?

A

Increased coronary blood flow

v. any change in O2 extraction

224
Q

Why is coronary O2 tension low (20-25mmHg)?

A

High baseline O2 extraction levels by the myocardium

225
Q

Relative to fossa ovalis, where is a coronary sinus ASD?

A

Anterior

Inferior

226
Q

What is a coronary sinus ASD typically seen in association with?

A

Unroofing of CS and persistent L-SVC

227
Q

Why is the 2nd heart sound often single and loud in d-TGA?

A

Anterior position of aorta

228
Q

What causes a narrow mediastinum and “egg on a string” sign on CXR in d-TGA?

A

Aorta/PA are AP in position to each other

229
Q

What is the most common associated cardiac lesion in DORV?

A

PS

*50% of patients with DORV

230
Q

After PS what is the next most common associated cardiac lesion in DORV?

A

ASD: 25% secundum, 8% primum

*Can see subaortic stenosis and right aortic arch, but less common

231
Q

A mitral valve in which all of the chordal attachments occur to a single papillary muscle is called what?

A

Parachute mitral valve

  • There is a variant with attachments to 2 distinct papillary muscles, but most to just 1
  • Chordae can be short and thick
232
Q

What is it called if the mitral valve leaflets insert directly onto the papillary muscle?

A

Mitral arcade

233
Q

True or False: The pulmonary veins contain cardiac myocytes instead of smooth muscle cells in the last 1-3cm before insertion to LA

A

True

*Allows them to minimize retrograde flow during atrial systole

234
Q

What is a potential EP problem due to the pulmonary veins having cardiac myocytes instead of smooth muscle cells in the last 1-3cm before insertion to LA?

A

A-fib

*Why PV isolation procedures sometimes used in adults

235
Q

What is the most common location of the PAs in truncus arteriosus?

A

-MPA from left postero-lateral aspect of truncus right above valve (48-68%, Type I)

  • 29-48% type II, Branch PAs from posterior surface
  • 6-10% type III, Branch PAs from lateral sides of truncus
  • Type IV, Branch PAs from descending aorta
236
Q

In truncus, when there is a single PA, what one is likely to absent?

A

PA on side of the aortic arch

*Same outflow, same side

237
Q

In ToF, when there is a single PA, what one is likely to absent?

A

PA on opposite side of arch

238
Q

What happens to the embryonic 5th arch?

A
  • Typically rudimentary and doesn’t develop into any known vessels in a normal neonate
  • Rare cases of persistence, can be asymptomatic or associated with other cardiac findings
239
Q

What is the clinical scenario of a persistent 5th arch, but interruption of normal 4th arch?

240
Q

What is seen in around 80% of patients with CoA?

A

Bicuspid AoV

*Combo is considered to be indication of a true aortopathy

241
Q

What proportion of CoA patients have “simple” CoA with normal intracardiac anatomy other than a bicuspid AoV?

A

1/2

-Then 1/2 with CoA + Associated anomalies

242
Q

Besides a bicuspid AoV, what is the most common associated anomaly with CoA?

243
Q

LV isovolumic relaxation continues until what event?

A

MV opens

  • End systole, LV ejection is complete and AoV closes
  • This begins isovolumic relaxation with drop in LV pressure
  • Next, MV opens allowing filling of ventricle due to lower pressures in the ventricle
244
Q

Where is AV node located?

A

Triangle of Koch

245
Q

What are the borders of the triangle of Koch?

A

Tendon of Todaro
Attachment of septal leaflet of tricuspid valve
Ostium of CS

246
Q

What is the most common type of TAPVR?

A

Supracardiac (46%)

  • Pulmonary veins form a confluence posterior to LA and blood is directed through a venous channel to left cardinal system
  • Most common anatomical site is left innominate vein
247
Q

What are frequencies of the different types of TAPVR?

A
  1. Supracardiac (46%)
  2. Infracardiac (23%)
  3. Cardiac (20%)
  4. Mixed (11%)
248
Q

Elevated pH
Normal pCO2
High bicarbonate

A

Acute metabolic alkalosis

249
Q

What are causes of acute metabolic alkalosis?

A

Vomiting
Hypokalemia
Use of alkalotic meds (bicarbonate, hyperaldosteronism, Bartter syndrome)

250
Q

What coronary artery typically supplies the inferior LV wall and posteromedial papillary muscle of the mitral valve?

251
Q

What supplies the anterolateral papillary muscle of the mitral valve?

A

Dual: LAD and cicumflex

252
Q

What supplies the posteromedial papillary muscle of the mitral valve?

253
Q

What supplies the inferior wall of the LV?

254
Q

In fetal circulation, which structure has the highest O2 content?

A

Umbilical vein

*Brings blood from placenta to the fetus (UV - DV/L Hepatic Vein- Atria)

255
Q

What 2 structures typically have the lowest O2 concentration in the fetus??

256
Q

What drains deoxygenated blood into the CS?

A

Great cardiac vein

257
Q

What is the name of the valve at the ostium of the cardiac vein?

A

Veiussens valve

258
Q

What is Vieussens valve?

A

Bicuspid valve at the site of the cardiac vein that merges with the CS

259
Q

Where is the Eustachian valve?

A

Entrance to IVC

260
Q

Where is the Thebesian valve?

A

Where CS drains to RA

261
Q

The AV nodal artery arises from which coronary artery?

A

Right

  • 90%: AV nodal artery from RCA
  • 10%: AV nodal artery from left circumflex
  • Comes from the dominant coronary artery
262
Q

What is the most common coronary artery abnormality seen in patients with otherwise normal heart?

A

Anomalous origin of the left circumflex from right main coronary artery

*1/3 of major coronary artery anomalies

263
Q

After anomalous origin of the left circumflex from the right main, what is the next most common coronary artery anomly?

A

Anomalous origin of the RCA from the left sinus of Valsalva

*Just <30%

264
Q

What % of coronary artery anomalies are single coronary arteries?

265
Q

True or False: Anomalous origin of the left or right coronary from the posterior sinus of Valsalva is a rare finding

266
Q

What is the most common abnormality of the aortic arch?

A

Anomalous right subclavian from the left

  • Occurs in 0.5% of the general population, usually asymptomatic
  • Often found at autopsy or imaging for something else
267
Q

What population is it common to see an anomalous right subclavian artery from the left?

A

Trisomy 21 with CHD (>30%)

268
Q

What is Uhl anomaly?

A

-Partial or complete absence of the RV myocardium (epicardium and pericardium opposed to each other)

  • Rare
  • Valvular morphology often normal
269
Q

What is characterized by fatty infiltration and replacement of the RV myocardium and arrhythmias or SCD?

A

Arrhythmogenic RV dysplasia

270
Q

What is the definition of a straddling AV valve?

A

Involves anomalous insertion of chordae tendineae

  • Have to have VSD
  • May limit repair options
271
Q

What is most responsible for high PVR in a fetus?

A

Low blood and alveolar O2 tension

*Fetus also produces vasoconstrictive substances such as thromboxane and leukotrienes (but doesn’t have as significant an effect)

272
Q

What is the initial connection between the pulmonary venous plexus and the heart?

A

Common pulmonary vein

*Typically has 4+ feeding veins which become the left and right upper/lower pulmonary veins

273
Q

What eventually happens to the common pulmonary vein?

A

Incorporated into the back wall of the LA and individual pulmonary veins connect independent to the atrium

274
Q

What phase of the cardiac AP is characterized by influx of Ca2+ into cell through L-type voltage gated Ca2+ channels?

A

Phase 2 (plateau)

275
Q

Which phase of the cardiac AP is characterized by rapid depolarization due to Na+ entry?

276
Q

Which phase of the cardiac AP is characterized by early repolarization with K+ efflux from the cell?

277
Q

Which phase of the cardiac AP is characterized by K+ efflux from the cell?

A

Phase 3 (repolarization phase)

278
Q

Which phase of the cardiac AP is characterized by return of resting membrane potential and is maintained by Na/K ATPase channels?

279
Q

Describe a tet spell

A
  • Abrupt onset of cyanosis, hypoxemia, dyspnea, agitation
  • Usually starts after 2 months
  • Crying/agitation increase PVR which increases R-L shunting and reduces PBF
  • Infundibular spasm may also be involved
280
Q

What is an indication to proceed with surgical repir of ToF?

A

Presence of hypercyanotic spells

281
Q

What structure is a continuation of the sarcolemma?

282
Q

What is an enveloping myofibril at the level of the Z-disks?

283
Q

What are T-tubules important for?

A

Rapid activation of the entire myocyte after the arrival of AP (excitation-contraction coupling)

284
Q

What type of channels are present in T-tubules?

A

L-type Ca channels: Provide the initial Ca flow into the cell during excitation

285
Q

What are intercalated disks?

A

Junctions between adjacent myocytes

286
Q

What structure links myofibrils to costameres (intracellular protein complexes) and transmit force from the sarcomere to the extracellular matrix and adjacent myocytes and functions to link the cytoplasmic actin filament to the cytoskeleton and connect intermediate filaments between adjacent cells?

A

Intercelated disks

287
Q

What does decreasing end diastolic volume do to myocardial O2 consumption?

A

Decreases it

288
Q

Myocardial O2 consumption is determined by what?

A

Wall tension- Related to intraventricular pressure and radius

*Decreasing EDV decreases pressure and radius of ventricle

289
Q

What effect does cardiac contractility have on wall tension and thus myocardial O2 consumption?

A

-Increased work so increased wall tension and myocardial O2 consumption

290
Q

What does increased HR do to O2 consumption over time?

A

Increases it- Increased frequency of generating pressure during LV contraction

291
Q

What are the normal branching patterns of the RPA and LPA?

A

RPA: Anterior to the RUL bronchus
LPA: Posterior to the LUL bronchus

292
Q

In situs inversus, what is the branching pattern of the RPA and LPA?

A

RPA: Posterior to RUL bronchus
LPA: Anterior to LUL bronchus

293
Q

With bilateral morphologic right lungs, what is the branching pattern of the PAs?

A

Both anterior to the upper lobe bronchus

294
Q

With bilateral morphologic left lungs, what is the branching pattern of the PAs?

A

Both posterior to the upper lobe bronchus

295
Q

What is the power stroke in cardiac muscle contraction?

A

Interaction of myosin head and actin that allows myosin to pull actin filament inward

296
Q

What powers the power stroke in cardiac muscle contraction?

A

ATP hydrolysis and conversion to ADP

297
Q

What is troponin made up of?

A

Troponin T, C, I

298
Q

In diastole, what prevents cross-bridging between actin and myosin?

A

Tropomyosin and troponin I

299
Q

In systole, what binds troponin C, which then binds troponin I and moves it from the ATP-reactive site on actin?

300
Q

What happens when troponin T binds to tropomyosin?

A

Causes a conformation change what allows cross-bridging of actin and myosin

301
Q

What structure has intrinsic ATPase activity?

A

Myosin head

302
Q

If the myosin head is uninhibited, what happens?

A

ATP becomes hydrolyzed which allows the myosin head to interact with actin and pull the filament inward … Power stroke

303
Q

What happens after the power stroke?

A

ADP released which allows myosin and actin to detach

Peds Cardiology (20 decks)

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