Cardiology Flashcards
Acceptable upper limit for a normal QTc for boys and girls
<0.45 boys
<0.47 females
Treatment for long QT Syndrome
Beta blockers
Avoid prolonging medications
Inheritance pattern and hearing for two most common congenital long QT syndromes
Romano-Ward = autosomal dominant, normal hearing
Jarvell and Lange Nielsen = autosomal recessive, SNHL
What is Torsades de Pointes and what situations can it occur in?
Polymorphic VT
Long QT, hypoMg, hypoK, anti-arrhythmic drugs
What arrhythmia do we worry about with WPW?
SVT
What to look for on an ECG for hypertrophy?
RVH = tall R in V1 and deep S in V6 LVH = tall R in V6 and deep S in V1
What to look for on an ECG in a patient where tachycardia (?SVT) has resolved?
Findings of WPW: short PR (<120), wide QRS (>110), and delta wave
Steps included in a Norwood procedure
Ligate PDA
Make ASD
Make neo aorta out of PA
Connect RV with L PA with Sano shunt (or subclavian to PA with BT shunt)
Risk factors for coronary artery involvement in KD
- Late dx, delayed tx
- Age <1 y/o or >9 y/o
- Male
- Fever >14d
- Na <135, WBC >12
Timeline when children are at highest risk for coronary aneurysm development after fever
- 4-6 weeks after onset of fever
Indications for SBE prophyalxis
- Prosthetic heart valve or who have had a heart valve repaired with prosthetic material
- History of endocarditis
- Heart transplant with abnormal heart valve function
- Congenital heart defects including: Cyanotic congenital heart disease, congenital heart defect that has been completely repaired with prosthetic material or a device for the first 6 months after the repair procedure, repaired congenital heart disease with residual defects
x8 cyanotic CHD lesions
6 T’s:
- Transposition of the great arteries
- TOF
- Tricuspid atresia
- Total anomalous pulmonary venous connections
- Truncus arteriosus
- “Tingle” ventricle (single ventricle)
2 A’s:
- Pulmonary atresia
- Ebstein’s anomaly
4 stages of ECG changes for pericarditis
- ST elevation with PR depression
- T wave flattening
- T wave inversion
- Resolution
CHF - ethology in the first week of life (6)
Think of obstructive!!
- HLHS
- Severe AS
- Coarctation
- Asphyxia
- Severe MR and TR
- Uncontrolled tachycardias
CHF - causes for week 2-6 of life (3)
Think left to right shunts!!
- VSD
- PDA
- AVSD
CHF - causes in older children (3)
Think pump failure
- Dilated cardiomyopathy
- Myocarditis
- Tachycardias
CHF - how to improve contractility?
- epinephrine, norepinephrine, dopamine, dobutamine, milrinone
CHF - how to decrease preload?
Diuretics
Fluid restriction
CHF - how to decrease after load?
ACEi, ARBs
Path finding for rheumatic fever
Aschoff bodies
Criteria for Rheumatic Fever
For new diagnosis = x2 major OR x1 major plus 2 minor
Major = carditis, polyarthritis, chorea, subcutaneous nodules, erythema marginatum
Minor = fever, polyarthralgia, prolonged PR, elevated acute phase reactants
Definition of cor pulmonale
Right heart dysfunction secondary to pulmonary disease
Findings of PHTN on ECG
RVH and RV strain
Characteristic cardiac feature of Tuberous Sclerosis
Cardiac rhabdomyoma
WAGR Syndrome
Wilms tumor, aniridia, GU abnormalities, intellectual disability (retardation)
ASD heart sounds - and why?
- mid systolic pulmonary flow or ejection murmur accompanied by the fixed split second heart sound
- murmur = from more blood flowing across pulmonic valve (not from ASD itself)
- split S2 = because more blood passing across PV so delayed closure
What position is best to listen to S2?
Both supine and sitting - as normal children can have a relatively wide S2 split when supine
Types of AV heard block?
1st degree: Prolonged PR
2nd degree: Gradually longer PR until QRS drop (type 1), normal PR but dropped QRS (type 2)
3rd degree: No P wave conducted to ventricles
HCM - genetic syndromes
- noonan syndrome
- BW
- Costello
- cardiofaciocutaneous
HCM - metabolic disorders
- lysosomal disorders - Pompeii disease
- mitochondrial disorder - freidrich’s ataxia
Prostaglandin SE (top 3)
- apnea
- hypotension
- hypoglycaemia
First line for long QT syndrome
Beta blockers
What cyanotic heart lesion has decreased pulmonary blood flow?
TOF
TOF - genetic syndrome
DiGeorge
Snowman XR
TAPVR
Egg on string
Transposition
Ebsteins -maternal medication
Lithium
What kind of murmur does TGA have?
No murmur
What does TGA require to live?
ASD
PDA - give prostaglandin
Why are tricuspid atresia kids blue?
Right to left shunting at atrial level
Are we more worried about supracardiac TAPVR or infracardiac?
Infra because vein can go behind the heart to the IVC through the ductus venousus - more likely to be obstructed
3 obstructive critical lesions
Critical AS
Critical coarctation
HLHS
What genetic syndrome is associated with coarctation?
Turners
Dose of prostaglandin in emergency situation
0.1 mcg/kg/min
Does perimbranous vs membranous VSD close spontaneously usually?
Membranous
What ECG finding is seen in AVSD?
Superior axis deviation
What cardiac lesion presents with a wide pulse pressure?
PDA
When does ALCAPA present and why?
Not for weeks until PVR falls
Then coronary artery coming off PA has blue blood and lower pressures
How long do you treat pericarditis with NSAIDS for?
Ibuprofen for 2-3 weeks
Where will you here a HCM murmur?
LLSB
What murmur is located at the apex?
Mitral regurgitation
What murmur gets worse with standing?
HCM
Venous hum
Classically what is HR for infants and children in SVT?
> 220
>180
What is the concern for WPW apart from SVT?
At risk for sudden cardiac death - if have atrial fibrillation or flutter they have an accessory pathway to send fast signals to ventricles —> VT or Vfib
How do you give adenosine for SVT?
Via right arm with flush very quickly
With what QTc length are you at greater risk for torsades?
> 500 ms
What to give In an emergency situation for torsades?
Magnesium sulphate
Major differential diagnosis for TOF
TGA
Causes of myocarditis
Infection: viral, bacterial, fungal
Autoimmune/inflammatory: SLE, KD, RA
Chemicals/drugs: Radiation, doxorubicin, lead, scorpion
Indications for IE dental procedure prophylaxis
- Prosthetic valve
- Previous IE
- CHD: unrepaired lesion (including palliative shunts), <6 mo post-repair with prosthetic material/device, residual defect post-repair
- Cardiac transplant with valvulopathy
- Rheumatic disease if prosthetic valve/material
4 classic and “fun” CXR findings for specific CHD
- Rib notching = coarct
- Boot = TOF
- Egg on string = TGA
- Snowman = TAPVR
4 bony abnormalities on CXR that increase likelihood of CHD
- Hemivertebrae + rib anomaly = VACTERL
- 11 rib pairs = Down’s syndrome
- Chest deformities (pectus excavatum, scoliosis) = Marfan’s
- Rib notching = coarct
x2 cardiac complications of maternal rubella
PDA + pulmonary stenosis
Cardiac complication of lithium
Epstein
Difference between vascular ring vs sling. Clinical presentation.
Ring = completely encircled. Sling = compression. Presentation = stridor, respiratory distress, feeding difficulty, swallowing dysfunction
Investigations to consider for ?vascular ring/sling
Echo, CXR (R aortic arch), upper GI swallowing study, MRI
SVT - most common cause, ECG findings, and associated lethal arrhythmia
- WPW
- ECG = short PR (bypassing AV node), slurred QRS (delta wave), wide QRS, ST + T wave non-specific changes
- Lethal = a.fib leads to rapid ventricular responses given accessory pathway –> Vfib
Causes of prolonged QT
- Congenital = hereditary (e.g., ion channelopathies), sporadic
- Acquired = drug induced, metabolic/lyte abN (hypoCa, hypoK, nutrition), CNS/autonomic d/o (head trauma, stroke), CVS (myocarditis, coronary artery disease)
ECG findings for hyper/hypoK
Hyper = tall peaked T waves, prolong PR, wide QRS Hypo = depressed ST, flat T waves, long QT
ECG findings for hyper/hypoCa
Hyper = short QT Hypo = long QT
Cardiac lesion for 22q11?
TOF
Cardiac lesions (x2) for Marfan?
Aortic dilation > mitral valve prolapse
Cardiac lesions (x2) for Turner syndrome?
- BAV
- CoA
Cardiac lesion for Noonan syndrome?
Pulmonary stenosis
Cardiac lesion for William’s syndrome?
Supra-valvular AS
Loud single S2
- Pulmonary hypertension
- TGA
Regurg at LLSB vs apex
LLSB: VSD»_space; TR
Apex: MR»_space; VSD
Top x3 most common cardiac causes of sudden death in athletes?
- Hypertrophic cardiomyopathy
- Coronary anomaly
- Myocarditis
Top x3 cardiac questions on family history for syncope?
- Pacemaker/ICD implant
- Death <50 years old from heart problem, seizure, drowning/car accident, SIDS
- Any HCM, Marfan’s, Brugada, LQT
Definition of pulmonary hypertension?
Elevated PA pressures above normal - mean PA pressure >25mmHg
What might you hear on cardiac exam for a patient with pulmonary HTN?
- Single S2
- TR or PR murmurs
Most common type Long QT Syndrome
LQT1
Triggers for the types of long QT syndrome
- LQT1: exercise
- LQT2: arousal
- LQT3: rest
Acquired causes of heart block (x4)
Athletes, high vagal tone, hypothyroidism, anorexia
Congenital causes of heart block (x7)
Neuromuscular, myocarditis, cardiomyopathy, neonatal SLE, lyme disease, rheumatic fever, idiopathic
What % of children with KD will have coronary artery involvement if not treated vs treated?
20% vs 5%
Length of ASA treatment (+ additional treatments) for KD?
-No coronary involvement: 6-8 weeks
-Small aneurysm (z = 2.5-5): ASA until resolves
-Medium aneurysm (z = 5-10, <8mm): ASA for life
-Large aneurysm (Z = >10, >8mm): ASA for life + LMWH
+clopidrogrel if high risk patient
High risk lesions for SBE prophylaxis
- Prosthetic heart valve or who have had a heart valve repaired with prosthetic material
- History of endocarditis
- Heart transplant with abnormal heart valve function
- Congenital heart defects including: Cyanotic congenital heart disease, Congenital heart defect that has been completely repaired with prosthetic material or a device for the first 6 months after the repair procedure, Repaired congenital heart disease with residual defects, such as persisting leaks or abnormal flow at or adjacent to a prosthetic patch/device
First line for SBE prophylaxis?
Amoxicillin
How can you differentiate a re-entry circuit vs ectopic focus for a narrow complex rhythm?
Look at R-P interval compared to PR interval
- Short R-P interval = re-entry circuit
- Long R-P interval = ectopic focus
What narrow complex rhythm will adenosine work and what one will it not work?
- Good for re-entry circuit
- Not responsive for ectopic focus
Dose of adenosine
0.1 mg/kg –> then double the dose
Investigations for palpitations
- Labs: Lytes, Ca, Mg, TSH
- ECG (goal to correlate with symptoms)
- Holter
- Home ECG device
- Exercise testing
Criteria for hypertrophy on ECG
- RVH: Tall R wave in V1, deep S wave in V6
- LVH: Tall R wave in V6, deep S wave in V1
What would strain look like on an ECG with hypertrophy?
T wave inversion or flattening
What to look for on ECG for RBBB and LBBB?
- RBBB = bunny ears V1
- LBBB = bunny ears V6
Difference between complete BBB vs incomplete BBB?
- Complete = wide QRS
- Incomplete = only bunny ears, normal QRS
Duke Criteria
- Need clinical OR pathologic criteria
- Clinical criteria = 2 major OR 1 major + 3 minor OR 5 minor
- Major (2) = positive culture (with typical bug), echo evidence (veg, abscess, new regurg)
- Minor (5) = fever, vascular (emboli, conjunctival hemorrhage, Janeway lesions), immune (GN, Osler nodes, Roth spots, RF), positive culture (non-typical bug), high risk patient (IVDU, known cardiac RF)
- Pathologic criteria = histological evidence, +gram stain/Cx from specimen
What endocarditis derm lesion is painful?
Osler nodes
RF Jones criteria
- Need 2 major OR 1 major+2 minor
- Major: Carditis, polyarthritis, chorea, subcutaneous nodules, erythema marginatum
- Minor: Fever, polyarthralgia, prolonged PR, elevated acute phase reactants
How long do patients need to be on antibiotic prophylaxis for in RF?
10 years - until 21 yo
What part of the heart is affected in RF carditis?
All parts - peri/myo/endocardium
List of cyanotic congenital heart lesions
- 6 T’s: Tingle ventricle, truncus arteriosus, transposition, tricuspid atresia, TOF, total anomalous pulmonary venous return
- 2 A’s: Epstein’s anomaly, pulmonary atresia
For truncus arteriosus - what would the CXR look like and what would pre-post saturations be?
- CXR = increased pulmonary vascularity
- Pre/post saturations = the same
What does HLHS need to stay “stable”?
-ASD + PDA
CXR finding in HLHS
Increased pulmonary vascularity
CXR for TOF (in terms of vascularity)
Decreased pulmonary vascularity
How to address a TOF tet spell
- Quiet/calm
- Knee-chest or squatting (to increase afterload to decrease R to L shunting)
- O2, fluid
- Morphine, phenylephrine, propranolol
CXR for tricuspid atresia
Decreased pulmonary vascularity
What is Ebstein anomaly?
Apical displacement of the tricuspid valve –> atrialized right ventricle
CXR for transposition
Increased pulmonary vascularity
Complications of a Fontan procedure
- Aplastic bronchitis
- Protein losing enteropathy
- Thromboembolism
- Arrhythmias
- Cyanosis (collaterals, pulmonary AVMs)
Aspects of Norwood procedure
- Create ASD
- Close PDA
- BT shunt (subclavian to PA) or sano shunt (RV to PA)
- Neo aorta
Aspects of Glenn procedure
-Connect SVC to PA
Aspects of Fontan
Connect IVC to PA
What is the typical cardiac lesion for (a) double inlet and (b) double outlet?
(a) DILV
(b) DORV
CHF etiology in the first week
- think obstruction*
- HLHS
- Severe aortic stenosis
- Coarctation
- Asphyxia or hypoxic ischemic injury
- Severe mitral or tricuspid regurg
- Uncontrolled tachy
CHF etiology in 2-6 weeks of life
- think L to R shunt*
- VSD, AVSD
- PDA
x3 cardinal features of CHF in infants
- Tachycardia
- Tachypnea
- Hepatomegaly
Management plan for CHF
- Supportive: HOB elevated, fluid + salt restriction, O2, tube feeds, high cal formula, immunization
- Pharma: improve contractility (dopamine, ER, NER, milrinone), decreased preload (diuretics), decrease afterload (ARB/ACEi), minimize ongoing damage (B-blockers)
- Surgery
What x2 cardiac lesions could present with cyanosis on day 2 of life? And what would be the most common presentation:
(a) bounding pulses/active precordium
(b) poor perfusion + pulses
(c) tachypnea + nasal flaring
- TOF + TGA
- Resp distress = c
Dx of POTS
- Orthostatic changes
- Normal supine HR
- HR change of at least 40 bpm during 10 minute stand test (or tilt table) with no hypotension
Characteristics of a venous hum (+how to differentiate from PDA)
- Best heard superior to R clavicle
- Radiates to back
- Continuous murmur
- Goes away when supine
In the setting of an ASD, what is an ECG most likely to show?
RV overload - R axis deviation, hypertrophy, and large P waves
What cardiac defect are you most likely to get in fetal alcohol syndrome?
Septal defects
Best treatment for hypertrophic cardiomyopathy? And why?
Beta blockers - slows the heart down to allow for a full heart + slow enough rate to allow for filling
What to think about for an infant with left-sided ischemia on ECG (with Q waves)
ALCAPA
What is the most common cardiac lesion in IDM?
Transient hypertrophic cardiomyopathy
x3 steps of pharmacological treatment for SVT
- Adenosine 0.1 mg/kg
- Adenosine 0.2 mg/kg
- Amiodarone
Features on auscultation of ASD murmur
- Wide fixed S2
- SEM to LUSB (pulmonary flow)
- Diastolic rumble at LSB (tricuspid overflow)
Long term complications of a large, unrepaired ASD
- pHTN (Eismenger)
- RV dilation + dysfunction
- Atrial arrhythmias
- MR + TR
- Paradoxical emboli
What dx do you think of with right aortic arch (+ then what other structure to look for)?
DiGeorge - vascular ring
Dx of Marfan - criteria
-Top 4 most important: aortic root dilation, ectopia lentis, family history, systemic features (=/> 7)
Systemic features: MVP, scoliosis, myopia, facial features, reduced US/LS with increased arm/height, wrist + thumb sign, PTX, hind foot abnormality, pectus carnitatum, dural ectasia, protrusion acetabuli, striae, reduced elbow extension