Cardiology Flashcards
From what embryologic structure does the heart form?
Mesoderm
First system to function in utero
What embryologic structures form the tube of the heart structure and when?
Two sheets of mesodermal angiogenic cells (Day 15) –>
Upper sheet enlarges to encircle other sheet (Day 17)–>
Beating initiated in upper tube (Day 20)
How and when does the heart tube structure form ventricles?
Tube bends to the right (D-loop) (Day 21)–>
Chambers form (Day 22)–>
Ventricles migrate to side-by-side (Day 28)
How and when does cardiac septation occur?
Atrial septum: grows within atria and forms 2 septum (Day 34)
Ventricular septum: Cells near inferior single ventricle grow upward to form septum (Day 38-46)
When is cardiac structure complete?
7-8 weeks
Describe the position of the atria relative to the viscera
1st letter:
S- solitus
I - inversus
A- ambiguous
Describe the position of the ventricle:
2nd letter:
D-loop: right ventricle on right side
L-loop: mirror image
Describe the position of the great arteries:
3rd letter:
S- solitus (aorta to right and posterior of pulmonary artery)
I- inversus/mirror image
What are the cardiac designations for:
- Normal
- TGA (Right and left)
- Situs inversus totalis
Normal: SDS
D-TGA: SDD (right)
L-TGA: SLL (left)
Situs inversus: ILI
When do cardiac defects occur during embryogenesis?
Most by 8 weeks
Cardiac morphogenesis- when does it occur and why?
Can occur progressively throughout pregnancy
Occurs due to acquired conditions or decreased blood flow
Where is most blood volume in the fetal heart?
Right ventricle
Where is the smallest blood volume in the fetal heart?
Right atrium
What percentage of fetal blood volume shunts through the PDA?
60%
What percentage of fetal blood flow goes to the lungs?
10%
What percentage of fetal blood volume is each of the 4 heart chambers?
Right atrium (20-25%)–>
Left atrium (27%)–>
Left ventricle (34%)–>
Right ventricle (65-70%)
Blood from the upper body drains to the ____ ventricle which then supplies the _____ body
Right
Lower
Blood from the lower body and placenta drain to the_____ then 1/3 cross the PFO to ___________ and 2/3 supplies the upper body
IVC
Cerebral and coronary arteries
The ______ ventricle supplies the majority of cardiac output in the fetus
Right
The highest oxygen content in the fetus is in the __________
Umbilical veins (70%)
Cardiac output equals
Systemic blood pressure/
Total peripheral vascular resistance
OR
HR X Stroke volume
Heart rate or stroke volume have a bigger impact on cardiac output?
Heart rate
What 3 things keep the ductus arteriosus open in utero?
Prostaglandin 2
Prostacyclin
Thromboxane A2
What medication maintains an open doctor’s arteriosus?
Prostaglandin 1
The lowest oxygenation content in the fetus is in the
Umbilical artery
Fetal hemoglobin contributes to lower pO2 tolerance by
Higher oxygen affinity
Low p50
Left shift in oxyhemoglobin curve (easier to release O2)
Increased hemoglobin levels for increased O2 carrying capacity
Anaerobic metabolism
Calculation of cardiac output:
HR X SV
OR
SBP/TPVR
Right ventricle stroke work is roughly equal to _____ left ventricle stroke work
1/6
An increased cardiac contraction resulting from increased preload/stretch is described by the
Frank Starling principle
Cardiac contractility is increased by
Catecholamines
Thyroid hormone
Pulmonary: systemic shunt calculation:
sO2 (ao) - sO2 (SVC)/
sO2 (LA or Pulm v) - sO2 (PA)
Pulmonary vascular resistance calculation:
P PA- P LA/
P blood flow
Systemic vascular resistance calculation
P Ao - P RA/
Sys blood flow
Cyanosis is visible if hemoglobin decreases by
3 to 5 g reduced Hgb
What causes differential cyanosis and what is it?
Critical coarctation with PDA and increased PVR
Right to shunting causing cyanosis in the lower body more than the upper body
What is reverse differential cyanosis?
Upper body more cyanotic than lower body Due to TGA with intact septum, associated with pulmonary hypertension, interrupted aortic arch, or coarctation of the aorta, and PDA
PaO2s associated with critical preductal coarctation of the aorta with PDA and increased PVR are
Right radial paO2 = 250. ^^^^^
Umbilical artery paO2 = 45. vvvvv
PaO2s associated with TGA with intact ventricular septum, PDA and (PHTN, interrupted aortic arch, or preductal CoA) are
Right radial paO2 = 50
Umbilical artery paO2 = 250
PaO2s associated with infradiaphragmatic TAPVR are
Umbilical artery paO2 = 90
Umbilical venous paO2 = 250
S1 heart sound represent
Closure of mitral and tricuspid valves
A widely split s1 can represent
Right bundle branch block
Ebstein’s anomaly
S2 represents
Closure of the aortic valve in pulmonary valve
A widely split S2 can represent
ASD
PAPVR
A single S2 can represent
Pulmonary hypertension
VSD murmur is characterized by
Holostystolic murmur beginning with S1 and continuing to S2
A crescendo ejection murmur can represent
Stenotic aortic or pulmonic valves
Valvular regurgitation sounds like a
Blowing murmur
Diastolic murmurs are _____ and can represent ________
Always pathologic
Aortic regurgitation
Pulmonic regurgitation
Tricuspid or mitral stenosis
Continuous murmurs occur with
PDA AV fistula Venous hum Collateral vessels Truncus arteriosus Aortopulmonary window
Narrow pulse pressure occurs with
Pericardial tamponade
Aortic stenosis
Intravascular depletion
Wide pulse pressure occurs with
PDA Thyrotoxicosis AV fistula Aortic regurgitation Truncus arteriosus
Mean blood pressure is calculated as
Diastolic blood pressure + 1/3 ( systolic blood pressure - diastolic blood pressure)
Tachycardia in early compensated shock is due to
Catecholamine release
In neonates the most common type of shock is
Hypovolemic shock
Kerley B lines indicate
Congestive heart failure, linear densities in lungs due to interstitial edema
Recurrence of congenital heart disease in a subsequent sibling is
2-5%
Risk of congenital heart disease in a child to a mother with Congenital heart disease is
If the father had CHD
~7%
~2%
Most inheritable type of congenital heart defects are
Left-sided obstructive lesions
The most common CHD is ______ at ____ percentage
VSD
16%
Most common cyanotic heart disease presenting in the first week of life is _____ at the _____ percentages
TGA
5-10%
Cyanotic heart disease most likely to present in the first week of life and cause mortality is
HLHS
2%
Most common cyanotic heart disease beyond infancy is
TOF
8-10%
Name the cyanotic heart disease listed in the 5T/DO/ESP
Truncus arteriosus Transposition of the great arteries Tricuspid atresia Tetralogy of Fallot TAPVR DORV Ebstein's anomaly Single ventricle Pulmonary atresia
CHF related to congenital heart defects tend to be ______ type of defects
Obstructive
HLHS with restrictive atrial defect Severe TR or PR Large systemic aortovenous fistula Obstructed TAPVR TGA Ebstein's anomaly Critical AS or PS Preductal CoA
TGA is more common in
Males
About 50% of TGA will also be associated with a
VSD
The more common form of TGA has the aortic valve positioned
Interior, inferior, and to the right of the pulmonary valve
LTGA is also known as
Congenitally corrected TGA
Aortic valve is anterior and left of the pulmonary valve
Which type of TGA is most likely to have severe cyanosis at birth?
D-TGA
Which congenital heart defect has the appearance of egg on a string on x-ray and no murmur?
TGA
EKG in TGA tends to show
Right QRS axis
Right ventricular hypertrophy
Right atrial hypertrophy
Immediate management of TGA includes
PGE1
Rashkind (balloon septostomy)
Treat CHF
Arterial switch (Jatene) and VSD/PS repairs at older age
25% of tetralogy of fallot have
Right aortic arch
Four abnormalities associated with tetralogy of fallot are
VSD
RVOT
RVH
OAo
What distinguishes a pink tet from a blue tet?
Severity of right ventricular outflow tract obstruction
Decreased pulmonary blood flow, decrease pulmonary veins return to left atrium
How does increased pulmonary vascular resistance and decreased systemic vascular resistance lead to a tet spell?
Changes in pulmonary and systemic resistance lead to increase right to left shunting which causes decreased pulmonary blood flow–>
Decreased blood flow through the pulmonary arteries decreases the PO2 causing acidosis and increased carbon dioxide
What methods counteract a tet spell?
Knees to chest Morphine Bicarb Vasoconstrictors Propranolol or esmolol Fluid bolus
Surgical management of TOF involves
Blalock Taussig shunt
VSD closure and RVOT obstruction repair
Survival with pulmonary atresia is dependent on the presence of
ASD or PFO with PDA
RVH occurs in pulmonary Atresia due to
RVOT
EKG for pulmonary Atresia will show
Normal QRS
LVH» RVH
RAH 70%
In addition to PGE, PA is treated with
Angiography to determine anatomy
BT shunt +/-RVOT reconstruction (if not RV dependent)
Truncus arteriosus is associated with
DiGeorge syndrome
TA’GD
Truncus arteriosus increases the risk of
Right aortic arch
Interrupted aortic arch
What other defect is always associated with the truncus archeriosis?
VSD
Which type of TA is most common and has the main pulmonary artery branch from the truncus then split?
Type 1, 50-70%
What is the difference between type 2 and 3 truncus arteriosus?
Type 2 PA branches posteriorly
Type 3 PA branches laterally, least common type
Truncus arteriosus is marked clinically by
Cyanosis CHF Wide pulse pressure Bounding pulses Pansystolic murmur Single S2
Truncus arteriosus is associated with right aortic arch ____%
50%
Surgical repair plan for truncus arteriosus is
Early, complete repair
Tricuspid Atresia without a _____ has a worse severity.
VSD- poor RV development, ductal dependent
30% of tricuspid Atresia also have
Great arteries transposed
Clinical findings in tricuspid Atresia are
Severe cyanosis CHF Systolic murmur and single S2 \+/- Pulmonary vascular markings if PBF Left superior QRS LV>>RV Arterial hypertrophy
Following PGE1, surgical plan for tricuspid Atresia is
Rashkind (balloon septostomy)
PA banding if ++ PBF
The Congenital heart defect associated with maternal lithium use
Ebstein’s anomaly
A heart defect with an enlarged right atrium, displaced tricuspid valve, small right ventricle, and 80% with an ASD is
Ebstein’s anomaly
20% of ebstein’s anomaly have this arrhythmia
WPW
The high mortality of ebstein’s anomaly is attempted treatment with:
PGE1
Treat CHF
Airway stabilization
If severe symptoms, surgical intervention
A non-cardiac complication of ebstein’s anomaly is
Airway compromise due to dramatic cardiomegaly
Asplenia or polysplenia are increased in which congenital heart defect?
Single ventricle
Single ventricle usually looks like
Single left ventricle with left transposition (Aorta comes off small leftward RV)
Absent ventricular septum (complete mixing)
Symptoms of single ventricle dependent on:
PBF-
- Increased: CHF, mild cyanosis, enlarged heart/PVM
- Decreased: mod-severe cyanosis, mild CHF, normal heart size/PVM
Surgical treatments for single ventricle are
CHF-> PA banding
Palliative -> Glenn procedure
Definitive -> Fontan procedure
TAPVR is defined as
Pulmonary veins drain to RA
PV can be supracardiac, cardiac, or infracardiac
PV draining to coronary sinus in TAPVR is
Cardiac
PV draining to portal vein or IVC in TAPVR is
Infracardiac
*Most likely to be obstructive
The TAPVR type with the worst severity is
Infracardiac/obstructive
Surgical repair urgently
Treating TAPVR with PGE1 can cause
worsened pulmonary congestion/edema
If the aorta and pulmonary artery both come from the RV, this is ________.
The other anomalies associated with DORV are
Double outlet right ventricle
VSD
Great arteries can be side by side or transposed, +/- PS
Clinical presentation in DORV is dependent on
Size/type of VSD
+/- PS
EKG: RVH
Arrhythmia associated with DORV is
First degree heart block
What kind of surgical repair is indicated for DORV?
Depends on severity of VSD and PS
What is the most common cause of CHF after the 2nd week of life?
VSD
Most common type of VSD is
Perimembranous (70%)
Inlet and outlet VSD’s each make up ____% of all VSD’s.
~7%
Describe the presentation of a mod -large VSD
Clinically silent for 2-3 days, then CHF, poor feeding, respiratory distress, then harsh holostystolic murmur
Definitive surgery for VSD is indicated when
Significant left to right shunt (2:1)
Severe CHF
Poor growth
Increased pulmonary artery pressure
Second most common cardiac defect is
ASD (6-11%)
ASD is more common in
Females
RV overload occurs in ASD because
Left to right shunting due to increased RV»_space; LV compliance
In contrast to VSD, EKG in ASD shows
RVH, RAD
Surgery in ASD is
At 2-5 years, definitive closure if RV overload
Molecules that prompt PDA closure
PGFa, acetylcholine, bradykinin, oxygen
Physiology of a PDA mimics a
VSD
A continuous or systolic machinery murmur suggests a
PDA
Complete AV canal defect is associated with
Trisomy 21
Associated defects with AV canal defects are
PDA and TOF (10%)
Primum ASD, inlet VSD, and common AV valve is a
Complete AV canal defect
Complete and partial AV canal defects are differentiated by
Partial:
+/- cleft in MV
No VSD
Normal TV
Symptoms in AV canal defects are
Dependent on ASD/VSD contributions
AV canal murmur is
Systolic due to VSD
+/- apical diastolic murmur, +/- gallop
Surgical correction of AV canal defect involves
ASD/VSD closure
AV valve separation
Partial anomalous pulmonary venous return is
One or more PV drain into RA
RIGHT»> LEFT (2:1)
In PAPVR, left pulmonary veins most often drain
To the innominate vein
Clinically PAPVR mimics
ASD
Pulmonary congestion in PAPVR is dependent on
Number of anomalous veins
ASD qualities
PVR
Similar to ASD, PAPVR clinically has
ASD murmur
RVH, RAE
increased PVM
EKG: RVH, RV conduction delay
Does PAPVR need surgical correction?
Only for clinically significant left to right shunt
Obstructive cardiac lesions are
Coarctation of the aorta
Pulmonic stenosis
Aortic stenosis
Infracardiac TAPVR
Coarctation of the Aorta of associated with
Turners syndrome (30% of Turner’s patients)
Cardiac defects associated with CoA
Bicuspid aortic valve
VSD
The form of CoA with the worst severity is
Preductal
Presents after birth
Differential cyanosis
Shock following PDA closure
Associated with other defects
The CoA most likely to have collateral vessels:
Juxtaductal and postductal
Rib notching in CoA is a sign of
Collateral vessels
Juxta- and post- ductal CoA commonly presents with
Hypertension or BP gradient
CoA murmur is
Systolic rejection at left interscapular area
+/- AR bicuspid valve murmur
CoA surgery involves
End to end anastomosis
+/- balloon angioplasty
Surgery often followed by hypertension
Pulmonic stenosis and aortic stenosis are each approximately ___% of CHD
5%
_________ stenosis is more common in males.
Aortic (4:1)
Williams syndrome is associated with ________ stenosis
Both pulmonic and aortic stenosis, supravalvular
Subvalvular pulmonic stenosis is associated with ______
TOF
Bicuspid aortic valve is associated with ______ ________ stenosis
Valvular aortic stenosis
Valvular positioning of pulmonic and aortic stenosis can result in
Post-stenotic dilation
If mild, both pulmonic and aortic stenosis present
Asymptomatic No cyanosis Election click Split S2 Normal heart size
Severe pulmonic and aortic stenosis both require
PGE1
balloon valvuloplasty
Pulmonic stenosis murmur
ULSB Systolic ejection with radiation to back
Grade proportional to stenosis
Aortic stenosis murmur
URSB, left midsternal systolic ejection murmur, radiation to LLSB
Grade is INDEPENDENT of degree of stenosis
Increased stenosis–> 2nd R intercostal thrill
Aortic valve regurgitation: Phys Presentation CXR/EKG Management
Incr preload/SV-> aorta dilation
Wide pulse pressure, bounding pulses. Early diastolic murmur.
LVE, dilated Aorta. ST depression, T wave inversion
Treat CHF, valve repair
Mitral valve regurgitation Phys Presentation CXR/EKG Management
LA/LV overload–> hypertrophy
Systolic blowing & diastolic murmurs at apex
Increased LV
Treat CHF, valvuloplasty vs MV replacement
Tricuspid valve regurgitation Phys Presentation CXR/EKG Management
RA/RV overload RV dysfunction+/- cyanosis if R-L Systolic blowing murmur, diastolic murmur at LLSB Hepatic congestion RAH Conservative, surgery only if necessary
HLHS is most common in
Males
The second most common CHD with cyanosis in the guest week of life is
HLHS
Aortic or mitral valve atresia/stenosis, hypoplastic LV and aortic arch hypoplasia is
HLHS
Patients with HLHS shows symptoms when
The PDA closes–>
CHF, metabolic acidosis, shock
HLHS EKG/CXR show
+PVM, cardiomegaly, RVH, RAD
Management of HLHS
PGE1, inotropes->
balloon septostomy –>
Norwood—>
heart transplant if needed
Hypertrophic cardiomyopathy is associated with
Pompe
Hurler
Noonan
IDM
Dilated cardiomyopathy is associated with
Myocarditis
Carnitine deficiency
Abnormal perfusion
Post-arrhythmia
Cardiac dysfunction in dilated cardiomyopathy is due to
Decreased ventricular function due to overstretch/dilation of LA/LV
Cardiomyopathy with CHF and MR murmur is
Dilated cardiomyopathy
The least common form of cardiomyopathy is
Restrictive
Marked arterial»ventricular dilation with abnormal ventricular filling is
Restrictive cardiomyopathy
EKG in HCM shows
LVH, ST and T wave changes
Possible arrhythmia
If obstructive, HCM should be treated with
Preload
Myomectomy
Avoid digoxin, inotropes
An S4 gallop is seen in
Dilated cardiomyopathy
EKG In dilated cardiomyopathy shows
Increased LV, ST and T wave changes
Q waves
possible arrhythmia
Dilated cardiomyopathy should be treated with
Vasodilators
Treat CHF
Possible anticoagulation
Possible heart transplant
Restrictive cardiomyopathy can mimic
Pulmonary hypertension
Persistent pulmonary overcirculation, pulmonary vasculature remodeling, increased PVR and RA/RV enlargement are due to
Eisenmengers complex- progression of untreated CHD
Treatment of eisenmengers complex includes
Nifedipine
Prostacyclin
Nitric oxide
Lung transplant
Acquired pulmonary vasoconstriction leading to RVH and RV dysfunction that results from severe lung disease is
Cor pulmonale
Although irreversible, Cor pulmonale is treated with
Addressing underlying disease, diuretics, pulmonary vasodilators, oxygen
Pulsus paradoxus from rapid fluid accumulation is
Pericardial effusion–> cardiac tamponade
Pericardial effusion can be caused by
Pericarditis
Severe anemia/CHF
Post cardiac surgery
CVL leakage
Treatment of pericardial effusion
Pericardiocentesis
Treat underlying disease
Left coronary artery originating from pulmonary artery is
Anomalous origin of the LCA from the PA (ALCAPA)
4 stages of ALCAPA are marked by
1- elevated PVR, adequate perfusion
2- decreased PVR and ALCA flow, increased dependence on collaterals
3- asymptomatic phase, collaterals maintaining perfusion
4- decrease PVR-> decrease collateral flow to ALCA (pulmonary-coronary steal)–> LV ischemia, LAD infarction, pulmonary congestion
Clinical presentation of ALCAPA is
Onset 2-3 months Respiratory distress Feeding intolerance FTT Transient ischemia: pallor, paroxysmal crying, diaphoresis
When ALCAPA reaches stage 4 (CHF), EKG shows
Deep Q waves (I, aVL, v4, v5, v6)
ST elevations v4-6
Diagnosis and treatment of ALCAPA include
EKG (showing infarction)
Echo with Doppler
Cardiac Cath if echo non-confirmatory (incr Pulm:syst blood flow)
ALCA anastamosis to aorta
Prognosis of ALCAPA is
Dependent on early intervention before significant myocardial injury
May require transplant
High mortality of undiagnosed
In general, in utero cardiac tumors can cause
Hydrops
Fetal arrhythmia
Can be asymptomatic
The most common cardiac tumor is
Rhabdomyoma
Qualities and treatment of rhabdomyomas
Usually multiple
Commonly involve ventricle and septum
Associated with tuberous sclerosis
Surgery if outflow obstruction
A well circumscribed, fibrous single cardiac tumor is
Fibroma
Most in LV
A cardiac tumor found in adulthood in the LA that can cause inflow obstruction
Myxoma
A rare cardiac tumor of cardiac myocytes is
Sarcoma
An intrapericardial tumor that can cause pericardial effusion is
Teratoma
The cardiac malformations associated with asplenia are
Always severe
Bilateral ______ sidedness is associated with asplenia.
Right
Bilateral left sidedness and less severe cardiac malformations are associated with
Polysplenia
TAPVR, bilateral SVC, AVC, TGA, PS/PA, single ventricle, dextrocardia are associated with both asplenia and polysplenia, but ________ are associated specifically with asplenia.
Aorta/IVC juxtaposition (100%)
Non cardiac sequelae of asplenia are
2 right lungs, gallbladders Malrotation Howell-Jolly & Heinz bodies Strep pneumo Cyanosis
Poor prognosis
Non cardiac sequelae of polysplenia are
2 left lungs
Biliary atresia
Malrotation
Cyanosis
Poor prognosis
Fibromuscular septum divining the LA to two compartments is
Cor triatriatum
Cor triatriatum presents
Decreased peripheral pulses Tachypnea FTT pulmonary edema Loud P2 Increased PVM EKG: RAD, RVH
Treatment of Cor triatriatum
Treat pulmonary edema
Surgery if necessary
Cyanide heart disease more prone to pulmonary over circulation are
TAPVR
HLHS
TGA/VSD
Truncus arteriosus
Chest x-ray finding of a boot shaped heart is concerning for
Tetralogy of Fallot
Chest x-ray finding of an egg on a string is concerning for
D-TGA
Chest x-ray finding of a snowman shaped heart is concerning for
TAPVR, supracardiac
Chest x-ray finding of an extremely large heart with decreased pulmonary markings is
Ebstein’s anomaly
Chest x-ray findings of a small heart with increased pulmonary markings is
Obstructive TAPVR
Chest x-ray findings concerning for right aortic arch with increased pulmonary markings is
Truncus arteriosus
Chest x-ray findings concerning for right aortic arch with decreased pulmonary markings is
Tetralogy of Fallot
QTc calculation
QT/ √RR interval
In infants over 6 months, normal QT is
<0.45s
RV hypertrophy on EKG is
V1: increased R, persistent upright T, increased R/S ratio, possible Q wave
LV hypertrophy on EKG is
V6: increased R, Q wave (+v5), peaked T waves, increased R/S ratio
Neonates <1 months and those with TOF have an EKG axis
+90- +/- 180°
Infants with tricuspid Atresia or AV canal have an EKG axis
0- -90°
Infants older than 1 month or with PA with intact septum have any EKG axis
0- +90°
Upright T wave in V1 after 72h suggests
RVH
Normally, p wave is ______° and QRS/T are ________°
0-90°
100-150°
Arrhythmia affect ___% of fetuses
1-2%
Fetal magnetocardiography (FMCG) increases into about arrhythmias with
Observation of QRS and QT interval, beat variability, and presence of T waves
Most tachyarrhythmias are
SVT (70-80%)
SVT typically presents at _____ weeks
28-32
Factors that determine perversion to groups in SVT
Prematurity and duration of SVT
Top 3 treatments for fetal SVT:
Digoxin
Amiodarone
Procainamide/flecainide/sotalol
Side effect of procainamide for fetal SVT
Can induce contractions
Flecainide and sotalol are limited to 2nd line treatment for SVT due to increased
Mortality
7-15% flecainide
30% sotalol
For tachyarrhythmias, a treatment that works in SVT but not Atrial flutter is
Amiodarone
Both tachyarrhythmias are associated with an increased risk of
Necrotizing enterocolitis
Cardiac change associated with maternal thyrotoxicosis
Sinus tachycardia
AV block is associated with ______ and presents at ___ weeks
Maternal SSA/SSB antibodies
Any gestational age
AV block with maternal antibodies is at risk for
Needing cardiac pacing at birth Cardiomyopathy b(30%)
AV block with a poorer prognosis has
No maternal antibodies
Additional cardiac defects
Higher risk for hydrops/CHF/demise
+/- a-/poly- splenia
There is an increased risk of SVT in the first month of life if the fetus has
Fetal ectopy
PACs are associated with
Hyperthyroidism
CHD
Cardiomyopathy
Central line misplacement
PVC’s can be caused by
Digoxin toxicity Infection Electrolyte abnormalities Hypoxemia Acidosis CHD Aminophylline/caffeine Myocarditis
Premature junctional contractions
Lack p waves with normal QRS
WPW, atrial flutter, atrial fibrillation, SVT, and ventricular tachycardia are examples of ______, the most common type of tachyarrhythmia.
Reentrant tachycardia
Reentrant tachycardia can be treated with
DC cardioversion
Junctional ectopic tachycardia, ectopic atrial tachycardia and ventricular tachycardia are _______ tachyarrhythmia.
Abnormal automatic focus
Typically refractory to DC conversion
Abnormal p waves with atrial ectopic focus and rapid rate is
Ectopic atrial tachycardia
Treated with beta blocker/antiarrhythmic
Junctional ectopic tachycardia happens most often _______ with EKG __________
Post operatively
Ventricular rate faster than atrial rate
Junctional ectopic tachycardia happens most often _______ with EKG __________
Post operatively
Ventricular rate faster than atrial rate
Junctional ectopic tachycardia is treated with
Normalize electrolytes
Limit inotropes
Arterial pacing+/- amiodarone, procainamide
Adenosine or ice given to an infant with atrial flutter causes
Allowed HR that can reveal sawtooth pattern
Atrial flutter is treated with
Digoxin
DC synchronized cardioversion/esophageal pacing if unstable
Atrial rate of 360+ bpm is __________ and treated with _____________
Atrial fibrillation
DC defibrillation, digoxin
Tachycardia with a wide QRS should be assumed to be
Ventricular tachycardia
Ventricular tachycardia is usually treated with
Lidocaine
If unstable, DC cardioversion
Causes of ventricular tachycardia
CHD Electrolyte abnormalities Hypoxemia Myocarditis Cardiac tumors Digoxin toxicity Prolonged QT Cardiomyopathy
Rapid irregular rate with abnormal QRS
Ventricular fibrillation
Causes of ventricular fibrillation
CHD Prolonged hypoxia Hyperkalemia Myocarditis Medications Cardiomyopathy Tumors
Prolonged PR is a
First degree AV block
Increasing PR interval leading to a dropped atrial impulse is
second degree AV block
Mobitz I
Wenckebach
Abrupt missing atrial beat without change in PR interval is
Second degree AV block
Mobitz II
Dissociated AV construction with independent ventricular and atrial rates
Complete AV block
Third degree AV block
Associated with LTGA, AVC, lupus
Outcome of compete heart block
Poor prognosis of HR <55, prolonged QT, wife QRS, ventricular dysfunction
pacing required if symptomatic
RBBB is associated with
Ebstein’s anomaly
Prolonged QT interval is ______ and can lead to _______
> 0.45s
Ventricular tachycardia, SIDS
Treatment for prolonged QT is
Propranolol
Pacing if necessary
SA node injury after cardiac surgery is
Sick sinus syndrome
Can be associated with atrial flutter/fibrillation
Cardioversion dosing
0.25-0.5j/kg
Max dosing 2j/kg
Defibrillation dosing
1-2 J/Kg
Max dosing 4j/kg
A prolonged QRS with slurring is
WPW
WPW is associated with
form of SVT
Ebstein’s anomaly
L-TGA
WPW is treated similarly to
SVT
If stable, vagal maneuvers, adenosine, digoxin, propranolol
If unstable, synchronized DC cardioversion
Verapamil is contraindicated in infants less than 12 months due to
Increased risk of sudden death
EKG changes in hypercalcemia are
Shortened QT interval
EKG changes in hypocalcemia are
Prolonged QT interval
EKG changes in hyperkalemia
Peaked T wave, short QT, depressed ST–>
Prolonged PR, wide QRS, flat P wave–>
Absent P, sinusoidal QRS, systole/ventricular fibrillation
EKG changes in hypokalemia
Wide QRS, depressed ST, biphasic T-> u wave
–> Flat T, prominent U, prolonged PR, sinoatrial block
Shortening fraction calculation
LV diastolic dimension- LV systolic dimension/
LV diastolic dimension
X 100
On echo, AVC is best viewed
By apical view
On echo, ASD is best viewed
by subcostal view
Aortic arch is best seen on echo by
Suprasternal notch view
Left heart and valves are best seen on echo by
Parasternal view
Optimal timing of fetal echo is
18-32w
Transvaginal can be fine as early as 10 weeks
CHD difficult to visualize on fetal echo are
CoA,
Minor valve anomalies
VSD
ASD
Cardiac A1 receptors are located ______ and cause _______
Vascular smooth muscle
Cardiac myocytes
Increase contractility
Vasoconstriction
Cardiac a2 receptors are located ________ and cause _________
CNS
Sympathetic nerves
Block NE release
Inhibits sympathetic response
Vascular smooth muscle relaxation
Cardiac B1 receptors are located _____ and cause ________
Sinoatrial node
Cardiac muscle
Conduction cells
Increased HR (SA node) Increased contractility
Mechanism of action of no milrinone/amrinone is
Phosphodiesterase inhibitor
Leads to accumulation of cAMP
Effects of phosphodiesterase inhibitors are
Chronic inotropy
Decreases SVR
Some pulmonary vasodilation
cAMP accumulation by phosphodiesterease inhibitors causes
Inhibited cAMP breakdown leading to increased calcium cellular entry, increased contractility, relaxation of vascular smooth muscle
Mechanism of action of digoxin is
Inhibits sodium potassium ATPase pump
Increases calcium influx
What are the clinical effects of digoxin
Negative chronotropic
Inotropy
Decreased SVR
N/K ATPase pump inhibition by digoxin causes
Decreased afterload
Anti-arrhythmic via decreased AV conduction
Digoxin toxicity can cause
GI symptoms Bradycardia Prolonged PR AV block Hyperkalemia, hypercalcemia
Mechanism of action of dobutamine
Acts on beta 1»_space;> beta 2 receptors
Clinical effects of dobutamine are
Chronotropy
Inotropy
Decreased SVR
Dobutamine acts as a
Synthetic catecholamine
Limited effects on afterload make dobutamine useful in settings of
Cardiogenic shock and myocardial dysfunction
Mechanism of action of dopamine
Endogenous catecholamine
Inhibits N/K ATPase and Na/H pump
Epinephrine and norepinephrine precursor
Promotes endogenous release of norepinephrine
Clinical effects of dopamine
++ Chronotrope
Inotropy
Dose dependent SVR increase
Clinical benefits of dopamine are
LOW DOSE: Renal vasculature dilation
MODERATE DOSE: B1 and dopaminergic receptors -> inotropy, ^HR
HIGH DOSE: B1 and A1
Mechanism of epinephrine
Potent vasopressor
B1, B2»_space; a
Clinically effects of epinephrine
Strong Chronotropy
Inotropy
Dose dependent SVR increase
Can improve coronary artery perfusion
Epinephrine adverse effects include
Hypokalemia
Local tissue ischemia
Renal vascular ischemia
Severe hypertension
Mechanism of isoproterenol
Synthetic catecholamine
B only
Clinical effects of isoproterenol
+++++ Chronotropy
–> useful for complete heart block
Inotropy
Decreased SVR
Not helpful in shock
Effects of nitroprusside
Decreased SVR
Vasodilator
Increases intracranial pressure
Can cause cyanide toxicity, tissue necrosis
Mechanism of norepinephrine
A> B1 > B2
Chronotropy, inotropy
Increases SVR
Adverse effects of norepinephrine
Second line to epinephrine Profound vasoconstriction Renal vasoconstriction Hypocalcemia Hypoglycemia
Mechanism of indomethacin
Cyclooxygenase inhibitor
Blocks prostaglandin synthesis
60-80% successful PDA closure, 30% relapse
Mechanism of prostaglandin
Vasodilator, maintains PDA
6-15% of patients receiving prostaglandin will have
Apnea
PGE1 relatively contraindicated in
Obstructive TAPVR
HLHS with intact septum
TGA, restrictive septum
Mitral valve Atresia
Endocarditis prophylaxis is indicated for
Unrepaired CCHD Repaired CCHD with prosthetic material 6 months post-op Incompletely repaired CCHD Cardiac transplant Prosthetic valve Prior bacterial endocarditis Rheumatic heart disease HCM MV prolapse+ regurg
Fetal valvuloplasty is offered to prevent HLHS for patients with:
Critical AS <26 weeks without progression to LV hypoplasia
Fetal valvuloplasty is offered to prevent hypoplastic right ventricle to patients with
PA with intact ventricular septum
Fetal ASD creation is offered to palliate HLHS to patients with
HLHS with intact septum
Or
TGA with intact septum
The rashkind procedure is a ________ and is used in
Septostomy
D-TGA
Severe MS
HLHS with inadequate mixing
The Blalock-taussig procedure creates _________ and is used for
Shunt between subclavian artery and ipsilateral pulmonary artery
Increases PBF
TA, PA, TOF with severe PS
The Glenn procedure creates _________ and is used for
Connection between SVC and PA to increase PBF without RV
TA
Single ventricle with severe PS
Mustard, Senning, Rastelli and Jatene are all procedures used for treating
D-TGA
The mustard procedure creates
An intraatrial baffle after atrial septum removed
SVC/IVC -> LA -> LV -> PA
Senning procedure differs from the Mustard
Because the baffle is made using atrial septum and RA wall instead of pericardium
The Rastelli procedure
Patches the VSD to decrease deoxygenated blood mixing into aorta.
Used in d-TGA with VSD and PS
The procedure to switch the arteries position in d-TGA is the
Jatene procedure
The procedure to reroute the SVC and IVC to the PA is
Fontan
Used in tricuspid Atresia and single ventricle
The 3 stages of the Norwood to repair HLHS are
1- septostomy, MPA division, hypoplastic aorta repair, shunt to increase PBF
2- Bidirectional Glenn, shunt removed, SVC connected to PA
3- Modified Fontan (SVC/IVC to PA)
Syndromes with a >50% likelihood of CHD
C- Carpenter and CHARGE
E- Ellis van Creveld
H- Holt-Oram
N- Noonan
T- Tri 13, 18, 21
V- VACTERL
W- Williams
Most common CHD in carpenter syndrome
VSD, ASD
Most common CHD in cat eye syndrome
TAPVR
Most common CHD in CHARGE
TOF
Most common CHD in Cornelia-de-Lange
VSD
Most common CHD in cri du chat
Varies
Most common CHD in DiGeorge
Aortic arch (Truncus arteriosus)
Most common CHD in Ehler-Danlos
Aortic root dilation, MV prolapse
Most common CHD in Ellis van Creveld
Common atrium
Most common CHD in glycogen storage diseases 2a (Pompe)
HCM
Most common CHD in Goldenhar
VSD, PDA, TOF
Most common CHD in Holt-Oram
ASD
Most common CHD in homocysteinuria
Arterial/venous thromboses
Most common CHD in Hurler
Thickened valves
CAD
HCM
Most common CHD in Klinefelter
TOF
MV prolapse
Most common CHD in Klippel-Feil
VSD
Most common CHD in Marfan
Aortic dilation
Aneurysm
MV prolapse
Most common CHD in Meckel-Gruber
ASD, VSD
Most common CHD in Noonan
Dysplastic pulmonary valve
LVH, HCM
Most common CHD in Rubenstein -Taybi
VSD, ASD
Most common CHD in TAR (thrombocytopenia, absent radius)
TOF
ASD
Most common CHD in trisomy 13
VSD/PDA (80-90%)
50% with other anomalies
Most common CHD in trisomy 18
VSD, PDA, PS, CoA
Most common CHD in trisomy 21
Complete AVC
VSD
PDA
Most common CHD in Turner
Bicuspid aortic valve
CoA
AS
Most common CHD in VACTERL
VSD
TOF/CoA
Most common CHD in Williams
Supravalvular subaortic stenosis
Infectious causes of myocarditis are
Coxsackie B
Parvovirus B19
Rubella
CHD due to rubella are
AS
PS
TOF
Maternal medications that can cause VSD are
Ethanol Hydantoin Phenytoin Trimethadione Valproic acid
Maternal medications that can cause ASD are
Ethanol Hydantoin Phenytoin Trimethadione Valproic acid
Maternal medications that can increase risk of HLHS
Retinoic acid
Trimethadione
Maternal medications that increase risk of pulmonary hypertension
Aspirin
SSRI
Thalidomide can cause
Conotruncal malformations
The medication that can cause TGA is
Retinoic acid
_____ % of mother’s of an infant with complete heart block have lupus
30-60%