General Question Review Flashcards
1
Q
The following constellation of symptoms is consistent with what type of shock: warm extremities, flash capillary refill, wide pulse pressure, high mixed venous saturation?
A
Septic shock
- you expect the peripheral systemic vascular resistance is low and the cardiac output is high with impaired oxygen extraction, leading to lactic acidosis in the presence of a high mixed venous saturation.
2
Q
name factors that can impact an accurate NIRs reading?
A
hyperbilrubinemia can artifically lower NIRs
3
Q
a normal SvO2 is
normal A-V O2 sat difference is
A
~65-80%
~20-35%
4
Q
when it comes to NIRs what trends should lead to investigation?
-what % change from baseline
-what percent lower than SaO2
-what overall %
A
->20% change from baseline
-> 40% lower than saO2
-< 50% Iin the absence of an intracardiac shunt
5
Q
explain how PPV leads to decreased LV workload?
A
-impact on wall stress
-wall stress= LV pressure- intrathoracic pressure
ex) normal: 100-(0) =100
PPV: 100-(mean airway pressure ~15)=85
NPV w/ atelectasis: 100 - (-25)= 125
*therefore PPV, helps reduce the LV wall stress
6
Q
ELN mutation
A
ELN mutation (William syndrome)
7
Q
PTPN11 mutation
A
PTPN11 mutation (Noonan syndrome)
Increased nuchal translucency, cystic hygroma, and polyhydramnios can be seen in fetuses
Cardiac manifestations after birth: ventricular hypertrophy, supra-valve pulmonary stenosis
8
Q
HRAS mutation
A
HRAS mutation (Costello syndrome)
Arrhythmias with hypertrophic cardiomyopathy
9
Q
TSC1 or TSC2 mutations are seen in
A
TS, INHERITANCE AD OR spontaneous
Think cardiac rhabdomyomas
Regress after 2 years of life
10
Q
Fetal svt first line management, what is goal dosing
Consideration if hydrops is present
A
Digoxin is first line therapy goal dose between 1-2ng/ml
However if hydrops → sotalol better transplacental availability
11
Q
Best next step for fad patient with concern for stroke vs fontan
A
Ct for hemorrhagic stroke 2/2 anticoagulation
MRI for thrombotic stroke
12
Q
Non dysmorphic SVAS in 2 first degree relatives should signal need for testing of what condition with what mode of inheritance
A
Elastin, AD OR de novo
13
Q
microdeletion of 7q11.23 causes what disease? associated abnormalities include
Cardiac manifestations….
A
Williams, elfian faces, DD, hyperca, cocktail personality
peripheral pulmonic stenosis and coronary ostial stenosis
14
Q
Management of anticoagulation in a preg lady 1st trimester with mechanical valve
A
If thearptic on <5mg warfarin continue even in 1st trimester
Best for mom, risks for teratogen highest in 1st trimester but tolerable if less than 5 mg dose
15
Q
What is the definition of overriding valve? How is double inlet defined?
A
By definition, an overriding AV valve empties into both ventricles. An atrium is considered to join the ventricle into which more than 50% of the valve orifice empties
16
Q
Tachycardia that starts off wide and narrows without change in R-R interval is what type of tachycardia?
What is the name of this process
A
SVT
Ashmans
17
Q
short stature, external ophthalmoplegia, and ataxia has an ekg with heart block what is the underlying abnormality
A
Kearns sayre
worsening ptosis and eventual loss of control of eye movements
neurologic abnormalities (cerebellar ataxia, myopathy, and sensorineural hearing loss) and endocrine disorders (diabetes, growth hormone deficiency, and hypoparathyroidism). Patients can develop variable and progressive atrioventricular (AV) conduction deficits that may present years after other symptoms
18
Q
Dax for bidirectional VT—name 3
A
CPVT
Digoxin toxicity
Anderson Tawail syndrome (long qt type 7)
19
Q
Barth syndrome is associated with what cardiac defects and what gene mutations and what non cardiac findings
A
CM most commonly LVNC and second most common manifestation is HCM
X linked recessive inheritance pattern
TAZ gene
Neutropenia
Skeletal myopathy
20
Q
glucosidase alpha acid (GAA) gene mutations cause what cardiac defects
A
Pompe glycogen storage disease
HCM
21
Q
lysosomal associated membrane protein 2 (LAMP2) cause what cardiac defect
A
Danon disease
lysosomal transport disorder classified as a glycogen storage disease
Males are typically more severely affected with skeletal myopathy, mild intellectual disability, and cardiomyopathy. Hypertrophic cardiomyopathy is the most common cardiomyopathy, although dilated cardiomyopathy is also seen
Affected females can develop more mild form of CM later in life
22
Q
Mutations in myosin heavy chain 7 (MYH7) cause what defects
A
MYH7 is a common cause of autosomal dominant hypertrophic (35% of cases), left ventricular noncompaction, dilated and restrictive cardiomyopathies
23
Q
TR jet velocity is used to estimate
A
Added to RA/CVP presssure=RV systolic pressure and systolic PA pressure
24
Q
End diastolic PI jet can be used to estimate
A
+CVP=Diastolic PA pressure
25
Early diastolic PI jet can be used to calculate
+CVP=Mean PA pressure
26
Natural hex of unrepaieed asd…
Progress to systolic dysfunction or arrhythmias
The natural history of unrepaired atrial septal defect suggests progressive risk of arrhythmias as patients age, such that over 50% will have atrial arrhythmias without repair by middle to late adulthood
Unlikely to develop systolic dysfunction
27
What sports are okay to play if you have a bicuspid aortic valve and ascending aorta with a score <3.5?
Golf, baseball, tennis
Avoid static sports, think lifting and avoid high contact sports at a competitive level
28
Patients with tof who have VT typically have ectopic focus or reentry circuit
Remember we place an ICD to shock and break the rhythm therefore it is a reentrant circuit
29
What are typical locations for the reentrant circuit in tof
The mechanism of ventricular tachycardia in patients with repaired tetralogy of Fallot is typically reentrant, using critical isthmuses (Tissue that slowly conducts electrical impulse) bordered by unexcitable tissue from the ventriculotomy/outflow tract patch, ventricular septal defect patch, tricuspid valve annulus, and pulmonary valve annulus
30
Persistence of the azygous vein after Glenn can lead to
Desaturation via venovenous collateral directing blood away from lungs
31
Class I meds for management of chronic HF as outpatient
ACEi/ARB
diuretics
Mineralcortcoid antagonists
Bblockade is a class II indication
32
During spontaneous respiration what is the effect on pleural pressure and r heart filling?
Negative pleural pressure
Increases R H FILLING
33
When considering respiratory mechanics, what is the impact of PPV on your filling pressures and what is the impact on your LV transmural pressure
Increases your filling pressure, therefore decreased RH filling
Increases your LV TRANSMURAL pressure= Ao pressure-pleural pressure
34
VT is distinguished by accelerated ventricular rhythm by a cutoff of X% faster than underlying rate?
What is the prognosis for accelerated ventricular rhythm in a newborn
15%
Accelerated ventricular rhythm in a newborn in the absence of any hemodynamic compromise, electrolyte abnormalities, or systemic or metabolic illness, and with no structural or functional cardiac abnormalities, is a benign rhythm and has excellent prognosis
35
What are the indications for pulmonary valvuloplasty?
Critical PS (ductal dep plum blood flow)
Peak to peak gradient >40
Any gradient with evidence of RV dysfunction
36
When considering pulm valvuloplasty is the presence of mpa dilation a bad sign?
Nope! It’s a good sign. Lack of post stenosis mpa dilation is typically concerning for valsculopathy and is more commonly seen in the setting of a dysplastic valve
37
What is a suicide RV
Relieve PV stenosis with valvuoplasty and uncover significant infundibular stenosis leading to insufficient Qp
38
What is the only class # (contraindication) to pulmonary valvuloplasty
PA /IVS w/RV dep coronaries
39
What is the accepted balloon to annulus ratio when considering pulm value interventions?
1 to 1.2-1.4, >1.4 will increase risk of PI
40
What is the accepted balloon: annulus ratio with aortic valvuloplasty?
0.8 to 1
41
What is the cath measured peak gradient required to move forward with aortic valve intervention?
>50, if asymptomatic
>40 if symptomatic
*note these cutoffs created in the setting of moderate sedation
42
Bicuspid AV, what is the most to least common morphology?
(70-85%) L +R>>R+N (10-15%)> L+N (<1%)
43
What are some factors that will make you more likely to have an unsuccessful aortic valvuloplasty?
<3 months, >40 ( greater risk of calcification)
Undersized ballon
Higher pre-dilation valve gradient
Smaller annulus z score
-resend of us repaired via
Prior procedure
Valve calcification
44
Between PV and AV which is more likely to require reintervention after valvuloplasty as an infant?
AV
45
Which groups falls into a class I indication for MV valvuloplasty ?
Rheumatic MS or congenital MS
Rheumatic MS
Moderate MS
If symptomatic or asymptomatic with signs of PH
46
Name the mean gradient for mild, moderate and severe ms?
Mild < 5, PA systolic pressure <30
Moderate 5-1 PA 30-50
Severe >10, PA > 50
47
What is class indication for coa?
Recoa with gradient >20
48
What balloon size considerations for CoA with valvuloplasty
2-3X diameter of stenosis
Up to > 1mm of proximal or distal vessel
Whichever is smaller to reduce vessel rupture
49
When considering valvuloplasty Vs stent placement for branch PAs vs arch which is gentler approach
Stent because we need exceed vessel diameter
50
What is the gold standard for native coa Vs recurrent CoA
Surgery for native CoA
Stent for recurrent if can be dilated to adult size otherwise angioplasty
51
Are there any class I indications for dilating an RV to PA conduit
NO!! We would consider stent placement tho for the following
-significant stenosis and stenting will buy you significant time before surgery, PI will be better tolerated than, no risk for coronary compression
Also stent OR dilation should never exceed the size of native conduit due to risk for rupture
52
During ASD device closure deficiency of what rim increases risk for device erosion
Retoraortic, not a contraindication to closure
53
What type of allergic rxn can be seen after asd device closure
Nickel, typically within 1 month and responsive to medical therapy
54
Asd closure post cath management
ASA AND SBE PPX X6 months
55
Post pda closure cath management
Yes Abe ppx X6 months no ASA required
56
Using spatial arrangements another name for the retroaortic rim is..
AV valve rim…
SVC rim…
Retroaortic: Superior and anterior
AV: inferior and anterior
SVC: superior and posterior
57
What is the risk of serious arrhythmia following ASD device closure?
<1%
58
What is the risk of erosion with amplatzer device, per X # of people people?
1:1,000
59
When is Yasui procedure undertaken? What does it entail
IAA with severe LVOTO
DKS with arch reconstruction , VSD baffle, RV to PA conduit
60
What is the long term complication most commonly seen with IAA
LVOTO
61
What do you need to consider from an airway perspective after post IAA repair
L bronchial compression
62
When thinking of transposition physiology what is the physiologic benefit of the pda
Increases Qp, increases la pressure to help promote atrial level mixing
63
What are the 4 surgical considerations for dTGA with LVOTO
1) ASO
2)Rastelli—baffle lv to Ao with ra to pa conduit
3) rev- similar except you are coming out more of the conus or infundibulum and then pa reconstruction +- conduit
4) Nikadoh- translocate Ao posteriorly, pa reconstruction, risk of coronary kinking
64
What is the most common complication post arterial switch
Supravalvar PS
65
Long term complication post ASO
Neo- aortic root dilation
66
Surgerical repair of what VSD is associated with a higher incidence of injury to conduction system
-supracristal or doubly committed juxtaarterial
-mid muscular
-high muscular
-inlet
-apical
Inlet!
Conduction system lies inferior and posterior to defect
Remember with muscular VSDs the defect is far away from conduction system
67
Rastelli classification for AVSD is determined by the superior or inferior bridging leaflet? What are the types and how are they classified
Superior bridging leaflet
Type a- division with attachments to the crest
B- straddling attachments classically seen in unbalanced AVSD
C-no division of superior bridging leaflet with no attachments to crest of the ventricular septum
68
When defining DORV by type of VSD what is the GA relationship in sub pulmonary and subaortic VSD? What outflow tract obstruction can be seen with each? Taussig bing anomaly refers to what…
Subaortic, normally related GA, risk for possible ps
Subpulmonary, transposed GA, risk for LVOTO and arch hypoplasia
69
Awhat is the triglyceride and LDL level that prompts a call to a real lipid specialist
TG >500, LDL> 250
70
What is normal LDL and TG thresholds
<130 for LDL
<100 for TG
71
Elevated LDL prompts 6 months of lifestyle and diet modification after this what LDL level will get you a statin
>190
160-190 w/ some risk factors
140-160 w/ LOTS of risk factors
72
Aortic arch repair with a Dacron graft has what LONG term complication
Aortic aneurysms
one-third of those who had aneurysms developed spontaneous rupture of the aneurysm, with most of those ruptures resulting in death
73
In a pulmonary sling where does the lpa arise from
The RPA
74
Can a LPA sling cause respiratory distress
YES either 2/2 bronchial compression OR tracheal rings
75
You have a patient with respiratory distress with an echo concerning for lpa sling? What is your next step
CT to evaluate tracheal anatomy
Barium swallow can show anterior indentation on esophagus but won’t tell us the trachea anatomy
Bronchoscopy does not provide insight to the distal bronchial tree anatomy
76
Intubation causes increased after load on what ventricle
RV!
Intubation reduces the LV transmural pressure and therefore decrease LV afterload
77
When evaluating for aortic stenosis on echocardiogram which measurement will best correlate with cath derived peak to peak pressure?
Max pressure gradient or the mean gradient
We look at the PV which tells us the max or peak pressure gradient
BUT it’s the mean gradient which matches up best with cath measurements
78
Which is non-selective—Dihydropyridine or non-Dihydropyridine?
Verarpamil and diltazem belong to what class
Amlodpine and nicardpidine and nifedipine belong to what
Which is more dangerous in overdose?
Non is nonselective
Pines are Dihydropyridines
Non more dangerous-bradycardia,hypotension, metabolic acidosis, hyperglycemia
79
FBN1 mutations are seen in what d/o
This d/o presents with what cardiac manifestation
Marfan syndrome
severe MR that can be medically refractory
80
Newborn escape rate in structurally nml heart that warrants pacemaker
In CHD
<50 in structurally nml heart
<60-70 in CHD
81
Newborn with heart block, mom has no known autoimmune disease. What do you do?
Send ab for antiSSA and anti SSB or Ro/La
70% of mothers with these antibodies maybe carriers, BUT NOT have autoimmune disease
82
Mom is a carrier for anti SSA or SSB or Ro/La what is the risk of heart block for her 1st baby?
What is the risk for subsequent babies if the first baby has heart block
2% for first baby, up to 20% for subsequent babies if the first had CHB
83
Babies who develop autoimmune mediated congenital heart block have what % risk of developing DCM
5-30%
84
What is the prognosis for Newborns that present with 2:1 block 2/2 to prolonged QT
Poor! Almost 50% die within the first year, they need both a pacemaker and then tx with bblocker
85
How thick can the ventricular septum be in 2% of male athlete hearts
13-15mm
86
What sex and race is less likely to have hypertrophic as an athlete
What are some characteristic features of an athletes heart
Participation in endurance or isometric sports increases odds of hyper trophy
How can ESTs help
White females
Athlete’s heart is associated with
-left ventricular dilation greater than 55 mm, whereas HCM more commonly presents with a left ventricular cavity smaller than 45 mm in adults.
-Both HCM and athlete’s heart are associated with left atrial dilation; however, dilation is more pronounced in HCM.
-Diastolic function is expected to be normal in athlete’s heart and abnormal in HCM.
-Finally, magnetic resonance imaging can also be useful to closely assess hypertrophy, chamber size, and evidence of myocardial fibrosis, which is almost universally pathologic
Athletes would be expected to have a supranormal oxygen consumption, whereas patients with HCM would not fare as well and may have an abnormal or blunted blood pressure response to exercise
Endurance!
87
What is the differential Dx for short RP tachycardia
-typical AVNRT
-JET (VA dissociation with adenosine)
-AVRT
88
Sinus rhythm a long or short RP rhythm
Name another rhythm with the same pattern
Long
PJRT
89
Mutations in BMPR2 are associated with what
PH
90
Mutations in JAG1 are seen in
Allagielle
91
Mutations in PTNP11 are seen in
Noonan syndrome
92
Sildenafil MOA
Phosphodisterase inhibitor blocks the breakdown of cGMP in smooth muscles and promotes vasodilation
93
What is the MOA of Bosentan and Ambrisentan
ERA endothelial receptor antagonist
94
What two clinical signs are poor prognostic inductors with restrictive cardiomyopathy
Cardiomeagly and pulmonary venous congestion on CXR
95
What is the most common genetic pattern of inheritance for DCM
Autosomal dominant
If you have genotype +DCM, 50% chance that your kid will inherit it
96
What is the side effect of taco when levels are high
Irritablity and tremulousness
97
How long after ACEi discontinuation do symptoms subside
1 week
98
Mechanical valve without additional procoagulant RF…what is the advice on periprocedural anticoagulation
Stop warfarin 48 to 72 hours prior to OR
No heparin bridge necessary
Restart within 24 HOURS
99
In restrictive physiology, what Is the mitral e/a ratio? What is the e’ on tissue Doppler?
E/A >2, E’ <10
**when you have equalization of la and of edp, when then mitral valve opens you have a brisk influx of blood (very high e) but by the time your atria contract the pressure is so high that nothing really happens
Normal e/a is 2 and e’ is >10
100
SVT has what type of VA association? Is it classically orthodromic or antidromic? If we were to categorize by a type of arrhythmia is it reentry or ectopic and what is the name for it? In typical SVT do you see preexcitation when you are having SVT?
1:1 VA association, rentry pathway, orthodromic down the native conduction up the accessory pathway (95%) of the time
Antidromic is a wide complex SVT because the signal conducts retrograde up the native conduction and antegrade via the accessory pathway
No you don’t see the delta wave during SVT because your accessory pathway is being used for retrograde conduction not antegrade at that time
101
PJRT is a long or short RP tachycardia
Do you see preexcitaion on baseline ecg
How do the p waves look
Long RP tachycardia (like sinus)
No pre excitation —tells you there is no antegrade conduction only retrograde
They’re upside down in II and III
102
A Mahiam fiber allows for antegrade or retrograde conduction via accessory pathway. Remember it’s rare
Only antegrade —wide complex tachycardia
Rare
103
In preexcited patients what two meds should you avoid
Verapmil and digoxin—slowing the conduction chronically thru the AV node increases the risk for antegrade conduction across the pathway and possible VF
104
What causes sudden death in patients with WPW
Rapidly conducted atrial fibrillation antegrade that leads to VF
105
What RR interval of the accessory pathway increases the risk for SCD
<250 msec
106
Asymptomatic preexcitated patient shows up in clinic
What do you do
1) holter—if persistently pre-excitated then you do a treadmill
If they lose the preexcitation for the boards okay for cards follow up, in real life send them to then lab for any result
ERP <250msec high risk for antegrade conduction and SCD
107
What is the differential diagnosis for long RP tachycardia
PJRT
atypical AVNRT
Sinus tachycardia
AET
108
I’m typical AVNRT you go up the …PATHWAY
Up the fast, R to P giving you a short RP interval
109
Pt presents with flutter, after what time do you need to consider the risk of clots? If they present after that time how do you proceed
>48 hours
1) rate control, anticoagulate X 3 weeks plan for DC cardioversion and then continue anticoagulation for another 4 weeks
2) TEE, bolus anticoagulation, cardiovert and anticoagulate X4 weeks after
110
After what duration of time is VT considered to be sustained
> 30 seconds
111
What is the threshold for PVC burden after which you are at increased risk for developing arrhythmia induced cardiomyopathy
>10%
112
VT originating from the RVOT typically has what appearance and what axis
LBBB and inferior axis so positive in II,III, and aVF
113
Fasciular Or bellhausens VT has what appearance and axis
RBBB, comes from the LV apex so it is superior axis and negative in II,III.avf
114
Maternal hx of rubella increases chances of developing what cardiac defect?
PS
115
Maternal PKU can cause what CHD
L sided defects, septal defects or ToF
116
In patients with Williams what tends to get better over time
Supravalvar PS or AS
PS gets better
AS gets worse
117
In a patient who recently underwent cardiac repair a widely split S2 with no murmur is indicative of
RBBB
118
Flow across a large VSD is primarily restricted by size or relative resistances
Resistances across pulmonary and systemic beds
119
Where does the bundle of his pass relative to a PM VSD
Compared to inlet VSD
Remember the PM prime minister has a PI
Posterior and inferior
If you ask new parents, AS is the IN thing to do
And sleep is the IN the IN thing to do
120
A premie with NEC and a large PDA meets indication for medical surgerical or cath based closure
Immediate surgical closure
Improves mortality
121
class 1c agents are….
They are contraindicated in what population
Flecainde, propfanone
Increased arrhythmias and higher mortality in patients with CAD and LV dysfxn
122
What is the effect of flecinade on dig and propranolol levels
Increases dig and propanolol
123
What is the impact of amidoarone on flecainade
Increases flec levels
124
What are the two B blockers that are non-selective?
Propanolol Nadolol
Impact both B1 cardiac and B2 receptors
125
What class III antiarrythmic has the least negative iontrophy and therefore is the drug of choice for HF
Amidoarone
126
What are the negative SE of amidoarone
Abml thyroid fxn hyper or hypo
LFTs
Corneal deposits
Photosensitivity
And the most feared is irreversible interstitial lung fibrosis which you are at higher risk for the longer you’re on the Med
127
What is the advantage of using ibuprofen vs indomethacin for ductal closure?
Less effect on cerebral blood flow and less renal injury
Same success with closure of the ductus
Equal risk of bleeding
128
Name the anticoagulant that works by binding to fibrin
-ASA
-warfarin
-streptokinase
-tpa
-heparin
Tpa
129
Name the anticoagulant that works by acetylation of cyclooxygenase to inhibit production of thromboxane A2
-ASA
-warfarin
-streptokinase
-tpa
-heparin
ASA
130
Name the anticoagulant that works as a mucopolysaccharide to increase the rate of anti-thrombin III neutralizing thrombin, factor X, and IX, XI, XII
-ASA
-warfarin
-streptokinase
-tpa
-heparin
Heparin
131
Name the anticoagulant that works by interfering with the post translational modification of vitamin K rep coag factors
-ASA
-warfarin
-streptokinase
-tpa
-heparin
Warfarin
132
What are the three risk factors for developing irreversible PH
1) cyanosis
2) pulmonary overflow
3) increased PA pressure
133
Water ruins pott leaves
Waterston shunt RPA
Potts shunt LPA
134
In truncus what type of valve is the most common
Tri—> quadracuspid—> bi
135
What coronary is diminutive and what is prominent in truncus
LAD diminutive
Prominent R consul branch
136
What age and PVR are prohibitive to repAir of truncus
PVR 8, age > 8
137
What causes a split s2 in truncus
delayed closure of abml truncal valve
138
What is the most common papillary muscle arrangement in parachute mitral valve
Presence of two separate papillary muscles with asymmetric chordal attachment
139
What is the characteristic of mitral arcade
Mitral valve leaflets are thickened and chorded are markedly shortened or absent
140
In patients with AS and hypoplastic nonapex forming LV what AV and MV annulus size are the cutoffs when considering SV vs biv repair
It is better to proceed with single v…
1) AV annulus < 5 mm
2) MV annulus < 9 mm
141
specify what would cause you to restrict a patient with MVP from exercise
1)rhythm
2)degree of MR
3)LV EF < x
Repetitive no sustained or sustained tachycardia
Severe MR
EF <50%
142
In an isolated mitral cleft, the regurgitation jet is directed in what direction
Anteriorly toward the LVOT
Compared to the nml cleft in AVSD where the jet is directed posteriorlu toward the inlet septum
143
You are asymptomatic and want to get pregnant OR play competitive sports what is the peak to peak gradient in cath needed to justify aortic valvuloplasty
>50mmHg
In normal people who aren’t trying to do above the cut off is >60
We don’t do aortic valvuloplastys in asymptomatic patients with peak to peak < 40 unless you have dysfxn
144
You are working up a patient for cardiomyopathy, cath shows LA and LVEDP > 5mmHg than RVEDP and RA pressure, a bolus is given and the difference increases to >10mmHg. Is this restrictive or constrictive physiology
Restrictive— L sided filling pressure 5-10mmHg greater than right, finding that can be enhanced with volume loading
145
What EKG changes are seen in DMD
-q waves where
Q waves in I, avL, V5 and v6 as well as prominent R wave in R precordial leads
146
What is the most common rhythm abml in children with restrictive cardiomyopathy
Atrial flutter followed by 2nd and 3rd degree heart block
147
You suspect bath syndrome, describe the patient, clinical tracheotomy and abml urine finding
Infant with myopathy, hypotonia, neutropenia, poor clinical prognosis with many dying at a young age
Elevates 3 methylglutaconic acid
148
A patient with suspected long QT on nadolol presents with CHF, you admit to the CICU with plan to start milrinone. Any concern with doing so if patient is appropriately compliant with nadolol
Nope! Milrinone retained its full hemodynamics effects in the setting of b blockade
149
What are the four risk factors in HCM that increases the risk of sudden cardiac death?
What do we do to decrease the risk—myectomy and or ICD?
1) family hx of sudden cardiac death
2) personal hx of cardiac arrest or sustained VT
3)massive Lv hypertrophy defined as LV wall thickness > 30 mm
4)syncope
Myectomy for symptom control
ICD to reduce risk of SCD
150
What is the physiology impact of squatting on preload and afterload
Increases both!
Helps improve venous return thru muscule contraction and increases afterload
151
What is the physiologic impact of hand grip to preload and afterload
Increased afterload
No change to preload
152
What does valsava do to cardiac preload and afterload
It increases intrathroacic pressure therefore decreasing preload
153
How do you increase murmur or gradient in HCM?
Have a patient stand, give isoprel, or amyl nitrate(vasodilator) or exercise
154
How is HCM inherited?
AD inheritance pattern
155
When do you start and how often do you screen 1st degree family members of a patient with HCM, in lieu of genetics
1-1.5 years starting at age 12 and can space to q5 years after 21 if no evidence of LVH
156
Partial pericardial defects are rare and can be associated with significant symptoms
-typically occurs on R or L side
-symptoms caused by anatomic construction of
-what does a CXR look like
-echo or MRI to dx
Typically L sided
CXR will show leftward displacement of the cardiac border and Geri still if LA appendage which may appear like dilated MPA
MRI to dx May find tongue of pulmonary tissue between the pa and aorta
Symptoms include syncope chest pain arrhythmias
2/2 ro incatcetation of the LA appendage, torsion of GA, or constriction if coronary arteries
157
In patients with cctga where is the AV node located?
Anterior to the AV ring, near the ASD
158
What type of tumor typically presents with signs of obstruction, PE, and or systemic illness?
Most common location in the heart?
Pedunculated or not
Myoxma
LA
Pedunculated
159
Most common causes of IE in neonates
Don’t have teeth—so we don’t worry about oral flora
Staph aureus, coag neg staph, candida
