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Congenital, EKG Flashcards

1
Q

What is a PFO?

A
  • Patent foramen ovale
    • one of two fetal cardiac shunts, allowing blood to bypass the fetal lungs, which cannot work until they are exposed to air
    • occurs when the foramen ovale fails to close after birth
    • later forms the “fossa ovalis”
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2
Q

What are the notable 22q11.2 deletion syndromes?

A

Multiple phenotypes

  • Tetralogy of Fallot
  • Pulmonary Stenosis
  • Interrupted arch
  • VSD
  • Double outlet right ventricle
  • D-transposition of the great arteries
  • DiGeorge syndrome: CATCH-22
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3
Q

Explain the features of Digeorge Syndrome

A

CATCH-22

  • Cardiac abnormality
    • commonly - interrupted aortic arch, truncus arteriosus, tetralogy of Fallot
  • Abnormal facies
  • Thymic aplasia and immune deficiencies
  • Cleft palate
  • Hypocalcemia/hypoparathyroidism
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4
Q

Chromosomal abnormality leading to:

  • Down syndrome
A

Trisomy 21

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5
Q

Chromosomal abnormality leading to:

  • Turner Syndrome
A

absence or abnormality in one of X chromosomes

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6
Q

Chromosomal abnormality leading to:

  • Williams Syndrome
A

Microdeletion on 7q and others

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7
Q

Mendelian gene/chromosomal mutation associated with:

  • Marfan Syndrome
A

Fibrillin-1 mutation on chromosome 15q21

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8
Q

Mendelian gene/chromosomal mutation associated with:

  • Loey-Dietz syndrome
A

TGF beta receptor disorder (TGFBR1 or TGFBR2)

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9
Q

Mendelian gene/chromosomal mutation associated with:

  • Holt-Oram Syndrome
A

TBX5 gene mutation

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10
Q

What congenital defect has the highest risk of transmission to progeny?

A
  • Bicuspid aortic valve and/or aorthopathy
    • up to 30% transmission rate
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11
Q

What is the general rate of transmission to offspring, for most congenital heart defects?

A

2-4%

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12
Q

What is/are the most common congenital heart pathology:

  • Down syndrome
A
  • 60% have some congenital heart lesion
  • AV septal defects (complete or partial)
    • ASDs
    • VSD’s
    • Both ASD and VSD’s
    • Cleft AV leaflets
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13
Q

What is/are the most common congenital heart pathology and features:

  • Holt-Oram Syndrome
A
  • Secundum ASD’s (occassionally others)
  • Abnormal digits, usually thumbs; can be both upper limbs
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14
Q

What is/are the most common congenital heart pathology and features:

  • Noonan Syndrome
A
  • Dysplastic pulmonary valve
  • Web neck, hypertelorism, low set ears, micrognathia
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15
Q

What is/are the most common congenital heart pathology:

  • Marfan Syndrome
A
  • Aortic aneurysm
  • MVP
  • Aortic valve prolapse
  • Pulmonary artery dilatation
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16
Q

Which echocardiographic scan plane is most optimal to define a secundum ASD?

A
  • Subcostal 4-chamber view
    • view which is optimally perpendicular to the atrial septum
    • eliminates the greatest degree of potential drop out
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17
Q

What is the most common associated anatomic lesion found with a sinus venosus ASD?

A

Anomalous right pulmonary venous connection

  • either a single RUPV or the RU and middle pulmonary veins insert anomalously to the SVC or the SVC-right atrial junction
  • these can also be located inferiorly near the entrance of the IVC into the RA
  • sinus venosus ASD’s are most commonly found in the superior portion of the atrial septum creating a “biatrial” insertion of the SVC
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18
Q

What is the most common associated anatomic lesion found with a inlet VSD’s?

A

AV septal defects

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19
Q

What is the most common associated anatomic lesion found with bicuspid aortic valve?

A

coarctation of the aorta

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20
Q

What is the most common associated congenital defect in a patient with Down Syndrome and an AV septal defect (AVSD)?

A

Tetralogy of Fallot

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21
Q

What is the most common anatomic finding in a complete AVSD?

A

LVOT is “sprung” anteriorly

  • LV inflow is shortened and LVOT is elongated (“goose-neck deformity”) –> LV inlet / LV outlet ratio < 1
  • Presence of a common AV valve –> AV no longer wedged between AV valves and is pushed anteriorly (“sprung”)
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22
Q

What are the anatomic hallmarks of AVSD’s?

A
  • Clef in the anterior leaflet of the left AV valve
  • Lateral rotation of the LV papillary muscles
  • Attachments of the left and right AV valves at the same level at the cardiac crux
  • LV inlet / LV outlet ratio < 1 (“goose-neck deformity)
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23
Q

What is the best echo view to delineate a subpulmonary (supracristal, doubly committed) VSD?

A

parasternl short axis view

  • can also be demonstrated from subcostal and apical windows with appropriate angulation
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24
Q

What is the most characteristic acquired lesion resulting from a subpulmonary (supracristal, doubly committed) VSD?

A

Aortic Insufficiency

  • occurs as a result of prolapse of the aortic cusp into the subpulmonary VSD
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25
Q

What is the most characteristic physiologic effect of a large VSD?

A

Equalization of the RV and LV pressures

as well as

elevated pulmonary arterial pressure

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26
Q

What is the most common anatomic type of subaortic stenosis?

A

Discrete membrane

  • located proximal to the aortic valve within the LVOT
  • most often circumferential and can be adherent to both the aortic valve as well as the anterior leaflet of the mitral valve
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27
Q

When are the Glenn and Fontan procedures employed?

A

whenever a congenital anatomy requires routing blood from the systemic venous system to the pulmonary arterial system

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28
Q

Describe the Glenn procedure:

A

creation of a cavopulmonary connection between the SVC and the disconnected right pulmonary artery (RPA)

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29
Q

What is the major complication of the Glenn procedure?

What is done to correct this complication?

A
  • patients only perfused the right lung via the SVC –>
    • pulmonary AV malformations (sometimes quite large)
    • cyanosis
  • Correction –> bidirectional Glenn procedure which allowed blood to go to both lungs
    • attaching SVC to RPA + oversewing of pulmonary valve
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30
Q

Describe the Fontan procedure

A
  • multiple ways to perform the procedure, all involve routing
    • IVC –> lungs
  • Classic Fontan = IVC –> right atrial appendage –> main PA
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31
Q

Why is it important are the Glenn and Fontan procedures performed at different times?

A
  • prevent competitive flow conflict
  • prevent increased pulmonary resistance
    • lung will not be accustomed to the flow
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32
Q

What is a solution to the initial high pressure in the Fontan conduit at placement?

A
  • “fenestrated” release into the right atrium to allow “pop-off” flow to allow decreased pressure in the conduit
  • once lungs have adapted to the new increase in flow –> fenestration is generally closed with a closure device
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33
Q

What pathophysiology causes the Fontan conduit to fail?

What is required to allow the Fontan conduit to function correctlly?

A
  • Pulmonary hypertension (from any cause)
    • pulmonary vascular disease, elevated pulmonary venous pressure from ventricular dysfunction, AV valve regurgitation
  • Systemic venous pressure must exceed the PA pressure and active early diastolic relaxation of the systemic ventricle be normal
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34
Q

What are options to reduce atrial arrhythmias that occur after a Fontan procedure?

A
  • Ablation and/or antiarrhythmics are typically trialed prior to these procedures
  • MAZE procedure
    • reduces the amount of atrial tissue available to sustain the arrhythmia
  • Conversion to extracardiac Fontan + removal of redundant atrial tissue
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35
Q

What are long-term complications in patients with the Fontan Procedure?

A
  • No tolerance to Pulmonary Hypertension from ANY cause
  • Atrial arrhythmias (poorly tolerated)
  • Coagulopathy
    • clots within conduit and atria
  • Liver dsyfunction and Cirrhosis
  • Liver cancer (hepatocellular carcinoma)
  • Protein-losing enteropathy
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36
Q

What lab tests should be checked yearly in patients with prior Fontan procedure?

A

LFT’s and AFP

  • rule out hepatocellular carcinoma
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37
Q

What is a poor prognostic sign in patient’s with prior Fontan procedure?

How is this diagnosed/monitored?

A

Protein-losing enteropathy

Diagnosis/monitoring

  • Monitoring: serial albumin measurements demonstrating a decline or drop over time
  • Diagnosis: fecal protein alpha-1 antitrypsin
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38
Q

What are the two most important measurable parameters in cardiopulmonary exercise stress testing in patients with congenital heart disease?

A
  • VO2 max
    • maximal aerobic capacity
  • Slope of the VE/CO2 max
    • minute ventilation-carbon dioxide output relationship
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39
Q

What parameters/trends in cardiopulmonary exercise stress testing will indicate severe impairment and poor prognosis?

A

Low VO2 max

+

Very rapid VE/CO2 slope

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40
Q

What implies an adequate cardiopulmonary exercise stress test in patients with CHD?

A

peak respiratory exchange rate of ≥ 1.10

  • ratio between VCO2 and VO2
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41
Q

Define VO2 max

A
  • amount of O2 a person takes up and delivers to the tissue at peak exercise at the point where the oxygen consumption plateus despite increasing work rate

***Level < 14 L/kg/min is considered a threshold value to move forward with transplantation

42
Q

Define VE/CO2 slope

A
  • index of ventilator efficiency and expresses the number of liters of ventilation per liter of CO2 exhaled
  • Normal is around < 30
    • high values = insufficient ventilation due to hyperventilation, increased dead space, or poor gas exchange
    • high values are expected in heart failure
      • cardiomyopathy (poor prognosis) = > 34
43
Q

Which congential heart disease demonstrate the worst overall exercise tolerance?

A

Eisenmenger syndrome

44
Q

What are the 3 most common causes of sudden cardiac death in the young?

A
  • Hypertrophic cardiomyopathy - 26%
  • Anomalous coronay arteries - 20%
  • Commotio cordis (direct trauma) - 14%

**** Most common cause –> unknown

45
Q

What is an EKG predictor of SCD in patients with TOF?

A

QRS > 180 msec

  • generally indicates more dilation of the RV secondary to repair or PR
47
Q

What is the general rule for chamber measurements in regards to heart rate and beats?

A
  • Perform on more than one cycle (inter-beat variability)
    • Sinus rhythm - average 3 beats
    • Atrial fibrillation - average 5 beats
48
Q

What Echo modes can be utilized in linear measurements?

49
Q

What Echo mode can be utilized to obtain volumetric measurements?

50
Q

Where should the LV end-diastolic dimension be measured?

A
  • perpendicular to the LV long axis
  • at or immediately below the mitral valve leaflet tips
  • 2D >> M-mode (will OVERESTIMATE)
    • will avoid oblique measurements
    • use guided M-mode if needed
51
Q

What are the disadvantages of linear LV measurements in 2D-mode?

A
  • lower frame rates than M-mode
  • assume normal shape ventricle
52
Q

What are the advantages of linear measurements in M-mode?

A
  • reproducible
  • high temporal resolution
  • published data
53
Q

What is the preferred method of LV volume measurement?

A
  • 2D Biplane Disc Summation (modified Simpsons)
    • sum of πr2h
54
Q

How do you perform the 2D Biplane Disc Summation (modified Simpsons) method of LV volume measurement?

A
  • trace the blood-compacted tissue interface in 2 and 4 chamber apical views
  • straight line at mitral valve level (connect opposite sections of mitral ring)
55
Q

What are methods for calculation of LV systolic function?

A
  • Volumetric LVEF
    • (LVEDV-LVESV)/LVEDV x 100
  • Biplane method of discs (modified Simpson’s) ***currently recommended
  • 3D can be used when available/feasible
56
Q

What is a severely abnormal LVEDD (cm)?

  • males
  • females
A
  • > 6.8 cm
  • > 6.1 cm
57
Q

Define End-Diastole in the Echo Cardiac Cycle

A

-frame after MV closure

or

-frame with largest LV dimension/volume

58
Q

Define End-systole in the Echo Cardiac Cycle

A

-frame after AV closure

or

-frame with smallest LV dimension/volume

59
Q

Where should the LV end-diastolic dimension be measured?

A
  • perpendicular to the LV long axis
  • at or immediately below the mitral valve leaflet tips
  • Prefer 2D instead of M-mode (will overestimate and obtain oblique measurements)
60
Q

What is the standard IVSd (cm) for men and women?

A

men: < 1.0cm
women: < 0.9cm

61
Q

What is the standard PWT (cm) for men/women?

A
  • men: < 1.0 cm
  • women: < 0.9cm
62
Q

What is IVSd?

A

interventricular septal end-diastolic dimension

63
Q

what is PWT?

A

posterior wall thickness

64
Q

What is LVIDd?

A

left ventricular internal diameter end-diastolic dimension

65
Q

what is LVIDs?

A

left ventricular internal diameter end-systolic dimension

66
Q

What is the standard measurement for LVIDd?

A
  • men: < 5.8cm
  • women: < 5.2cm
67
Q

What is the standard measurement for LVIDs?

A
  • men: < 4.0cm
  • women: < 3.5cm
68
Q

What is normal LVEF?

A
  • men: > 52%
  • women: > 54%
69
Q

What is normal LVEDd in males?

Severely abnormal?

A
  • 4.2-5.8 cm
  • > 6.8 cm
70
Q

What is the (males) normal LV diastolic volume index (mL/m2)?

Normal LV systolic volume index (mL/m2)?

Severely abnormal LV diastolic volume index (mL/m2)?

A
  • < 74 mL/m2
  • < 31 mL/m2
  • > 100 mL/m2
71
Q

What is normal LVEDd in females?

Severely abnormal?

A
  • 3.8-5.2 cm
  • > 6.1 cm
72
Q

What is the (females) normal LV diastolic volume index (mL/m2)?

Normal LV systolic volume index (mL/m2)?

Severely abnormal LV diastolic volume index (mL/m2)?

A
  • < 61 mL/m2
  • < 24 mL/m2
  • > 80 mL/m2
73
Q

How to calculate indexed measurements?

A

Indexed measurements = measurement / BSA

74
Q

56 year old female has LV mass of 98 g/m2 and relative wall thickness of 0.38. What is her LV chamber geometry?

A

Eccentric hypertrophy

75
Q

What is the formula for RWT?

A

RWT = 2 x PWT / LVEDD

76
Q

What is the linear method (cube formula) for determining LV mass?

A

LV mass = 0.8 x 1.04 [(IVS + LVIDD + PWT)3 - LVIDD3] + 0.6g

  • LV mass = (LV volume epicardium - LV volume endocardium) * 1.04

**1.04 = specific gravity of myocardium

77
Q

Describe method for determining LV mass/LV mass index/Relative wall thickness

79
Q

What adverse cardiovascular outcomes are associated with increased left atrial size?

A

Increased incidence of:

  • Atrial fibrillation
  • Stroke
  • post-MI mortality
  • death and hospitalization in patients with dilated cardiomyopathy
80
Q

What is alternative method of calculating the LV mass?

A
  • Truncated ellipsoid
  • Area-length
  • 3D (direct measurement)
81
Q

What is the relationship between pulmonary arterial hypertension and left atrial size?

A

PAH is not associated with increased left atrial size

82
Q

What are teh three major physiologic roles that affect LV filling and function?

A
  • contractile pump that delivers 15%-30% of the entire LV filling
  • reservoir that collects pulmonary venous return during ventricular systole
  • conduit for the passage of stored blood from the left atrium to the LV during early ventricular diastole
83
Q

What is the recommended approach to assess left atrial size?

A
  • TTE
  • measured at LV end-systole (largest at this time)
  • utilization of dedicated 4- and 2-chamber left atrial views
    • helps to avoid foreshortening as LA and LV frequently lie in different planes
84
Q

What must be included/excluded in tracing of the left atrial border?

A
  • Excluded:
    • LAA
    • confluences of the pulnonary veins
  • Included:
    • tip of the mitral leaflets should represent the atrioventricular interface
85
Q

What is the Law of LaPlace?

A
  • describes the factors that determine left ventricular wall stress, which is a major determinant in myocardial oxygen demand
  • LV wall stress is the force acting against the myocardial cells
  • LV wall stress is directly proportional to LV pressure and radius
86
Q

What is LaPlace’s law (equation)?

A

LV wall stress = (LV pressure x LV radius) / 2 x LV wall thickness

87
Q

Describe the changes to LaPlaces law with pressure overload states?

A
  • Concentric hypertrophy (HTN, AS)
  • Sarcomeres added in parallel
  • Normal or small LV cavity size with thick walls
  • Increased:
    • LV pressure
    • LV wall thickness
    • LV mass
    • RWT

LV wall stress = ( Î LV pressure x LV radius) / 2 x Î LV wall thickness

88
Q

Describe the changes to LaPlaces law with volume overload states?

A
  • Eccentric hypertrophy
  • Increased diastolic chamber size
  • No change in RWT
  • Sarcomeres added in series
  • Increased:
    • LV radius (volume)
    • LV wall thickness
    • LV mass

LV wall stress = ( LV pressure x Î LV radius) / 2 x Î LV wall thickness

89
Q

What is a normal LV global longitudinal strain?

90
Q

Explain how to estimate RA pressure using IVC measurements?

A
  • IVC diameter < 2.1 cm + collapse > 50% (with sniff) –> RA pressure = 3 mmHg (normal
  • IVC diameter > 2.1 cm + collapse < 50% ( with sniff) –> RA pressure = 15 mmHg
  • RA pressure = 8 mmHg –> IVC parameters don’t fit this schema
91
Q

What are two situations in which estimation of RA pressures are unable to be assessed?

A
  • Ventilators
  • Young healthy athletes

**

92
Q

What are the six quantitative parameters to evaluate RV systolic function (and abnormal values)?

A
  • TAPSE ( < 17 mm)
  • Tricuspid annular velocity (< 9.5 cm/s)
  • RV 2D fractional area change ( < 35%)
  • RV index of myocardial performance ( > 0.43 for pulsed Doppler and > 0.54 for tissue doppler)
  • 3D RV EF ( < 45%)
  • RV free wall strain ( > - 20%)
93
Q

What two RV quantitative parameters are associated with poor prognosis in patients with CHF and PH?

A
  • TAPSE
  • Abnormal RV free wall strain
95
Q

What is the ductus venosus?

A
  • fetal shunt which shunts a portion of the left umbilical vein blood flow directly to the IVC
96
Q

What is the ductus arteriosus?

A
  • fetal shunt allowing blood flow to bypass the lungs
  • connects the main pulmonary artery to the proximal descending aorta
  • at birth becomes “ligamentum arteriosum”
98
Q

Define RBBB (complete) on EKG

A
  • rsR’, rsr’, or rSR’ complexes in V1 or V2 with the secondary R wave (r’ or R’) usually wider than the initial R wave (r)
    • minorityy of patients may have a wide and often notched R wave pattern in V1 and/or V2
  • Prolonged QRS duration, > 120 ms in adults
    • > 100ms in children ages 4-16 years
    • > 90ms in children < 4 years
  • S wave of greater duration than R wave or > 40 ms in leads I and V6
  • Normal R wave peak time in leads V5 and V6 but > 50 ms in lead V1

***Of the above criteria, the first 3 should be present to make the diagnosis. When a pure dominant R wave with or without a notch is present in V1, the 4th criteria should be satisfied

*****RBBB results in secondary ST-T segment changes (ST depression or T wave inversion), unrelated to ischemia, in V1-V2.

RBBB can be seen in normal adults without structural heart disease; however, in the setting of known CAD, a RBBB carries a 2-fold increase in mortality

99
Q

Define the main criteria used for LVH

A
  • Cornell Criteria: R avL + S V3 =
    • > 28mm in males
    • > 20 mm in females
  • Sokolow-Lyon criteria: SV1 + R V5 or V6 > 35 mm
  • Sokolow-Lyon “stand-alone” criteria: R aVL > 11mm

***Sokolow-Lyon “stand-alone” criteria most specific (92%)

****Cornell criteria is most specific (31%)

100
Q

Define EKG criteria for RVH:

A
  • Right axis deviation
    • Mean QRS axis greater than or equal to 100 (degrees)
  • *R/S ratio in V1 > 1
  • *R/S ration in V5 or V6 < 1
  • *qR complex in V1
  • *R wave > 7 mm
  • Secondary ST-T segment changes (ST depression or T wave inversion) in right precordial leads
101
Q

What EKG changes can mimick RVH?

How do you differentiate between the two?

A
  • posterior or inferposterolateral MI
  • Factors that favor a diagnosis of RVH:
    • concomitant RAD
    • TWI in V1-V2
  • Factors that favor posterior MI
    • inferior Q waves
102
Q

Define ST and/or T wave abnormalities suggesting myocardial injury:

A
  • > 1 mm of ST-T segment elevation in at least 2 contiguous

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