Congenital, EKG Flashcards
(102 cards)
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”
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
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
4
Q
Chromosomal abnormality leading to:
- Down syndrome
A
Trisomy 21
5
Q
Chromosomal abnormality leading to:
- Turner Syndrome
A
absence or abnormality in one of X chromosomes
6
Q
Chromosomal abnormality leading to:
- Williams Syndrome
A
Microdeletion on 7q and others
7
Q
Mendelian gene/chromosomal mutation associated with:
- Marfan Syndrome
A
Fibrillin-1 mutation on chromosome 15q21
8
Q
Mendelian gene/chromosomal mutation associated with:
- Loey-Dietz syndrome
A
TGF beta receptor disorder (TGFBR1 or TGFBR2)
9
Q
Mendelian gene/chromosomal mutation associated with:
- Holt-Oram Syndrome
A
TBX5 gene mutation
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
11
Q
What is the general rate of transmission to offspring, for most congenital heart defects?
A
2-4%
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
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
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
15
Q
What is/are the most common congenital heart pathology:
- Marfan Syndrome
A
- Aortic aneurysm
- MVP
- Aortic valve prolapse
- Pulmonary artery dilatation
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
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
18
Q
What is the most common associated anatomic lesion found with a inlet VSD’s?
A
AV septal defects
19
Q
What is the most common associated anatomic lesion found with bicuspid aortic valve?
A
coarctation of the aorta
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
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”)
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)
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
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
25
What is the most characteristic physiologic effect of a large VSD?
Equalization of the RV and LV pressures
as well as
elevated pulmonary arterial pressure
26
What is the most common anatomic type of subaortic stenosis?
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
27
When are the Glenn and Fontan procedures employed?
whenever a congenital anatomy requires routing blood from the systemic venous system to the pulmonary arterial system
28
Describe the Glenn procedure:
creation of a cavopulmonary connection between the SVC and the disconnected right pulmonary artery (RPA)
29
What is the major complication of the Glenn procedure?
What is done to correct this complication?
* 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
30
Describe the Fontan procedure
* multiple ways to perform the procedure, all involve routing
* IVC --\> lungs
* Classic Fontan = IVC --\> right atrial appendage --\> main PA
31
Why is it important are the Glenn and Fontan procedures performed at different times?
* prevent competitive flow conflict
* prevent increased pulmonary resistance
* lung will not be accustomed to the flow
32
What is a solution to the initial high pressure in the Fontan conduit at placement?
* "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
33
What pathophysiology causes the Fontan conduit to fail?
What is required to allow the Fontan conduit to function correctlly?
* 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
34
What are options to reduce atrial arrhythmias that occur after a Fontan procedure?
* 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
35
What are long-term complications in patients with the Fontan Procedure?
* 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
36
What lab tests should be checked yearly in patients with prior Fontan procedure?
LFT's and AFP
* rule out hepatocellular carcinoma
37
What is a poor prognostic sign in patient's with prior Fontan procedure?
How is this diagnosed/monitored?
Protein-losing enteropathy
Diagnosis/monitoring
* Monitoring: serial albumin measurements demonstrating a decline or drop over time
* Diagnosis: fecal protein alpha-1 antitrypsin
38
What are the two most important measurable parameters in cardiopulmonary exercise stress testing in patients with congenital heart disease?
* VO2 max
* maximal aerobic capacity
* Slope of the VE/CO2 max
* minute ventilation-carbon dioxide output relationship
39
What parameters/trends in cardiopulmonary exercise stress testing will indicate severe impairment and poor prognosis?
Low VO2 max
+
Very rapid VE/CO2 slope
40
What implies an adequate cardiopulmonary exercise stress test in patients with CHD?
peak respiratory exchange rate of ≥ 1.10
* ratio between VCO2 and VO2
41
Define VO2 max
* 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
Define VE/CO2 slope
* 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
Which congential heart disease demonstrate the worst overall exercise tolerance?
Eisenmenger syndrome
44
What are the 3 most common causes of sudden cardiac death in the young?
* Hypertrophic cardiomyopathy - 26%
* Anomalous coronay arteries - 20%
* Commotio cordis (direct trauma) - 14%
\*\*\*\* Most common cause --\> **unknown**
45
What is an EKG predictor of SCD in patients with TOF?
QRS \> 180 msec
* generally indicates more dilation of the RV secondary to repair or PR
46
47
What is the general rule for chamber measurements in regards to heart rate and beats?
* Perform on more than one cycle (inter-beat variability)
* Sinus rhythm - average 3 beats
* Atrial fibrillation - average 5 beats
48
What Echo modes can be utilized in linear measurements?
* 2D
* M-mode
49
What Echo mode can be utilized to obtain volumetric measurements?
* 2D
* 3D
50
Where should the LV end-diastolic dimension be measured?
* 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
What are the disadvantages of linear LV measurements in 2D-mode?
* lower frame rates than M-mode
* assume normal shape ventricle
52
What are the advantages of linear measurements in M-mode?
* reproducible
* high temporal resolution
* published data
53
What is the preferred method of LV volume measurement?
* 2D Biplane Disc Summation (modified Simpsons)
* sum of πr2h
54
How do you perform the 2D Biplane Disc Summation (modified Simpsons) method of LV volume measurement?
* 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
What are methods for calculation of LV systolic function?
* Volumetric LVEF
* (LVEDV-LVESV)/LVEDV x 100
* Biplane method of discs (modified Simpson's) \*\*\*currently recommended
* 3D can be used when available/feasible
56
What is a severely abnormal LVEDD (cm)?
* males
* females
* \> 6.8 cm
* \> 6.1 cm
57
Define End-Diastole in the Echo Cardiac Cycle
-frame after MV closure
or
-frame with largest LV dimension/volume
58
Define End-systole in the Echo Cardiac Cycle
-frame after AV closure
or
-frame with smallest LV dimension/volume
59
Where should the LV end-diastolic dimension be measured?
- 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
What is the standard IVSd (cm) for men and women?
men: \< 1.0cm
women: \< 0.9cm
61
What is the standard PWT (cm) for men/women?
* men: \< 1.0 cm
* women: \< 0.9cm
62
What is IVSd?
interventricular septal end-diastolic dimension
63
what is PWT?
posterior wall thickness
64
What is LVIDd?
left ventricular internal diameter end-diastolic dimension
65
what is LVIDs?
left ventricular internal diameter end-systolic dimension
66
What is the standard measurement for LVIDd?
* men: \< 5.8cm
* women: \< 5.2cm
67
What is the standard measurement for LVIDs?
* men: \< 4.0cm
* women: \< 3.5cm
68
What is normal LVEF?
* men: \> 52%
* women: \> 54%
69
What is normal LVEDd in males?
Severely abnormal?
* 4.2-5.8 cm
* \> 6.8 cm
70
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)?
* \< 74 mL/m2
* \< 31 mL/m2
* \> 100 mL/m2
71
What is normal LVEDd in females?
Severely abnormal?
* 3.8-5.2 cm
* \> 6.1 cm
72
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)?
* \< 61 mL/m2
* \< 24 mL/m2
* \> 80 mL/m2
73
How to calculate indexed measurements?
Indexed measurements = measurement / BSA
74
56 year old female has LV mass of 98 g/m2 and relative wall thickness of 0.38. What is her LV chamber geometry?
Eccentric hypertrophy
75
What is the formula for RWT?
RWT = 2 x PWT / LVEDD
76
What is the linear method (cube formula) for determining LV mass?
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
Describe method for determining LV mass/LV mass index/Relative wall thickness
79
What adverse cardiovascular outcomes are associated with increased left atrial size?
Increased incidence of:
* Atrial fibrillation
* Stroke
* post-MI mortality
* death and hospitalization in patients with dilated cardiomyopathy
80
What is alternative method of calculating the LV mass?
* Truncated ellipsoid
* Area-length
* 3D (direct measurement)
81
What is the relationship between pulmonary arterial hypertension and left atrial size?
PAH is not associated with increased left atrial size
82
What are teh three major physiologic roles that affect LV filling and function?
* 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
What is the recommended approach to assess left atrial size?
* 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
What must be included/excluded in tracing of the left atrial border?
* Excluded:
* LAA
* confluences of the pulnonary veins
* Included:
* tip of the mitral leaflets should represent the atrioventricular interface
85
What is the Law of LaPlace?
* 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
What is LaPlace's law (equation)?
LV wall stress = (LV pressure x LV radius) / 2 x LV wall thickness
87
Describe the changes to LaPlaces law with pressure overload states?
* 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
Describe the changes to LaPlaces law with volume overload states?
* 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
What is a normal LV global longitudinal strain?
~ - 20%
90
Explain how to estimate RA pressure using IVC measurements?
* 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
What are two situations in which estimation of RA pressures are unable to be assessed?
* Ventilators
* Young healthy athletes
\*\*
92
What are the six quantitative parameters to evaluate RV systolic function (and abnormal values)?
* 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
What two RV quantitative parameters are associated with poor prognosis in patients with CHF and PH?
* TAPSE
* Abnormal RV free wall strain
94
95
What is the ductus venosus?
* fetal shunt which shunts a portion of the left umbilical vein blood flow directly to the IVC
96
What is the ductus arteriosus?
* fetal shunt allowing blood flow to bypass the lungs
* connects the main pulmonary artery to the proximal descending aorta
* at birth becomes "ligamentum arteriosum"
97
98
Define RBBB (complete) on EKG
* 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
Define the main criteria used for LVH
* 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
Define EKG criteria for RVH:
* 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
What EKG changes can mimick RVH?
How do you differentiate between the two?
* 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
Define ST and/or T wave abnormalities suggesting myocardial injury:
* \> 1 mm of ST-T segment elevation in at least 2 contiguous
103
