Cardiology Flashcards

Question

Causes of cardiomyopathy in a structurally normal heart

Answer 1

Myocarditis Familial dilated cardiomyopathy Tachycardia induced Muscular dystrophies Mitochondrial Metabolic

Answer 2

Diuretics ACEi Spironolactone Beta blockage (carvedilol) IV Inotropes particularly dobutamine, milrinone Levosimendan (Ca sensitising agent)

Answer 3

De George syndrome (22q11) - p/w w interrupted aortic arch, tet fallot, RAA, truncus Turners syndrome - coarctation, bicuspid aortic valve, aortopathy T21 - ASD, VSD, AVSD, ToF, PDA Williams Syndrome (supravalvular AS, peripheral PS) VACTERL - VSD CHARGE syndrome Marfans (aortopathy)

Answer 4

Change in pressure = 4 x (distal velocity)^2

Answer 5

1D assessment of function on echo Should be around about 30%

Answer 6

VEntricular volumes at end of diastole and systole EF = (EDV-ESV)/EDV Should be around 60%

Answer 7

Hemodynamics - shunts - pulmonary vasc resistance/pressure Angiography - Great for vascular information Intervention - may need diagnostic cath before cardiac surgery - stents - valves - creat/dilate holes - close holes/block vessels - balloon angioplasty or valvuloplasty

Answer 8

high BP in pulmonary arteries pulmonary arterial pressure: * mild \> 25mm Hg * mod \>35 * severe \>45

Answer 9

SVC/IVC, RA, RV, pa - 70% pulm veins, la, lv, aorta - 95%

Answer 10

Pink patient with EXTRA pulmonary flow ('heart failure') - breathless, soggy/plethoric CXR

Answer 11

Blue/cyanosed patient with REDUCED pulmonary flow (normal CO around body but LESS around lungs) Blood from lungs mixes with that from body and results in blood that has reduced oxygenation

Answer 12

Day - 19: vasculogenesis in cardiac region - 21: primitive heart tube formed - 22: heart beats - 28: folding of heart completed - 56: outflow tracts and ventricles separated - 63: semilunar valves complete

Answer 13

RA pressure 2-8 (70ms after onset of P wave) RV peak systolic 17-32 RV end diastolic 2-8 (RV contraction is 65ms after q wave) Pulm artery - mean pressure 9-19 - peak systolic 17-32 - end diastolic 4-13 (RV ejection into PA is 80ms after Q wave) LA mean pressure 2-12 (LA contract is 85ms after P wave onset) LV peak systolic 90-140 LV end diastolic 5-12 (LV ejection is 115ms after LV ejection) Aorta mean pressure 70-105

Answer 14

Pressure in ventricles increasing but aortic valve still closed (VOLUME CONSTANT BUT PRESSURE INCR) When pressure ventricle \> aortic pressure, valve opens -\> EJECTION PHASE (LV pressure increases a bit longer whilst volume decr in chamber until aortic pressure \> LV pressure, then aortic valve closes = at End systolic pressure)

Answer 15

Average systolic BP that is \>95th centime for age, gender and height on \>= 3 occasions

Answer 16

HR x SV However impossible to truly measure SV so use this formula for children: ~ 4-5 L/min/m^2 (BSA)

Answer 17

Measures CO (direct method) Catheter fed through SVC into RA → RV → and tip in PA - \> Solution injected - \> Time/temp curve produced **-\> CO = area under curve**

Answer 18

Indirect measurement of CO - Measures O2 consumption = O2 consumption/(arterial - venous content difference)

Answer 19

Estimates extent to which pulmonary blood flow is increased or reduced Flow (Q) - surrogate for CO Q pulmonary = pulmonary vein saturation - pulmary artery sat Q systemic = systemic arterial sat - mixed venous sat Qp:Qs = Qsystemic / Qpulm = (SatAorta-SatSVC)/(SatPulmonary Venous - SatPulmonary Artery) Normally = 1:1 Left to right shunts \> 1.0 Right to left shunts \<1.0

Answer 20

1. PDA - Aortic blood mixes into ain pulmonary artery 2. VSD - LV blood (high pressure ) pushed across into RV both resulting in increasing saturation in main pulmonary artery

Answer 21

ACYANOTIC ASD - Childhood presentation - Murmur, exercise intolerance - *R heart enlargement* as shunting occurs at atrial level , *volume load occurs in RV* PDA nad VSD Shunting L-\> R: volume load occurs straight into pulmonary artery -\> lungs -\> then straight into L side of heart so get volume loading of L side, not right side of heart Presentation: - These may not be evident in the neonatal period due to high pulmonary vasc pressures (R heart pressures = systemic pressure) and thus reduced shunting. Appear at 12 weeks/3mo of age as the PVP back to low baseline and shunting is at its maximum - Murmur, heart failure, FTT - L heart enlargement VSD (30% of all CHD) PDA ASD AVSD (Downs)

Answer 22

Sx - Onset depends on severity of pulmonary stenosis; - cyanosis may appear in infancy (2 to 6 months of age) or in childhood - other symptoms include hypercyanotic spells or decreased exercise tolerance Clinical - Central cyanosis - Clubbing of nail beds - Grade 3 or 4 long systolic ejection murmur heard at ULSB - May have holosystolic murmur at LLSB - Systolic thrill at ULSB - Normal to slightly increased S1 - Single S2

Answer 23

Sx - Small defects: usually asymptomatic - Medium or large defects: CHF, symptoms of bronchial obstruction, frequent respiratory infections Murmur - Small defects: loud holosystolic/ES murmur at LLSE (may not last throughout systole if defect is very small) - Medium and large defects: increased right-to-left ventricular impulses; thrill at LLSE; split or loud single S2; holosystolic murmur at LLSE ECG - SMall VSD: normal ECG - Large VSD will produce right ventricular hypertrophy with right axis deviation. At this point there is either an rsR' pattern in the right precordial leads, or more commonly, a tall monophasic R wave in the right precordial leads reflecting RVH. Also deep S waves in the lateral precordial leads and tall peaked P waves

Answer 24

Sx - May be asymptomatic; can cause easy fatigue, CHF, and respiratory symptoms Clinical - Continuous machinery murmur (grade 1 to 5) in ULSE (crescendo in systole and decrescendo into diastole) - normal S1 - S2may be “buried” in the murmur - thrill or hyperdynamic left ventricular impulse may be present

Answer 25

CENTRAL CYANOSIS **Increased pulmonary blood flow** - Truncus arteriosus - TGA - Total anomalous pulmonary venous return PRESENTATION --\> heart failure with low sats --\> pulmonary congestion as PVR drops --\> subtle desalts (93-94%) with high pulmonary blood flow **Reduced pulmonary blood flow** - Tetralogy of fallot (obstruction of blood flow to lungs) - Tricuspid atresia (no tricuspid valve so no blood flow across PA) - Ebstein's anomaly (abnormality of tricuspid valve that may obstruct blood flow to lungs and affect ability of RV to pump bloods to lungs) PRESENTATION --\> Cyanosis --\> Oligaemic lung dields --\> Significant deterioration clinically with closure of PDA (results in significantly reduced pulmonary blood flow)

Answer 26

Pressure drop across the pulmonary/systemic circulation per unit flow in a specific period of time = pressure drop between mean pulmonary artery pressure and LA pressure Rp = mean PA - mean Lap (mmHg)/Qp (L/min/m^2) Normal PVR \<3.5 If high this could be normal if in a neonate due to increased pulmonary vascular resistance -\> need to then test patients in 100% oxygen to look at vasodilation of pulmonary arteries (should be reversible) --\> if doesn't increase, indicated idiopathic pulmonary HTN HIGH PVR: - hypoxic - elevate Co2 - incr symptoms tone (inotropes) - polycytheamic - PE - pulm oedema - pulm effusion (causes compression) LOW PVR - Oxygen - adenosine - inhaled NO - prostacycin - Ca channel blockers

Answer 27

1/6 systemic vascular resistance

Answer 28

Ventricle is relaxing whilst volume stays constant (after ejection when aortic valve has closed again) when ventricular pressure \< atrial pressure, mitral valve opens and blood flows from atrium into ventricle Relaxation phase occurs until mitral phase shuts

Answer 29

Increased end diastolic volume in LV Larger stroke volume Increased stroke work (area under pressure volume curve)

Answer 30

Higher pressure needs to be reached before \>aorta but stroke volume is slightly reduced Stroke work (area under pressure volume curve) stays about the same

Answer 31

blood under more pressure in ejection phase so have longer ejection phase Ejection phase ends when LV p = aortic pressure. Results in larger stroke volume Overall higher stroke work (area under pressure vol curve)

Answer 32

1. SVC and IVC -\> deoxy blood to **RA -\>tricuspid** valve (3 cuspsl) -\> RV 2. RV -\> pulmonary valve (3 cusps) -\> deoxy blood to lungs via pulmonary artery for oxygenation 3. pulmonary veins bring oxygenated blood to **LA -\> mitral valve** (2 cusps) -\> LV 4. LV -\> aortic valve (3 cusps) -\> oxygenated blood to systemic circulation via aorta 5. Systole atrial contraction - firing of SA node induces depolarisation of heart = p wave = atrial contraction -\> atrial pressure increases -\> ventricular pressure increases as blood flows into ventricles through AV valves. 6. **AV valves (mitral and tricuspid) close = S1 heart sound** 7. _Isovolumetric contraction_ (pressure in ventricles increases as volume stays same, valves closed) 8. _Rapid ventricular ejection_ (blood ejected from ventricles into aorta/PA 9. _Reduced V ejection_ (as pressure in ventricles decrease and aortic pressure also falls, atrial pressure increases as they passively fill) 10. _Diastole_ - starts at *end of T wave* on ECG 11. _Isovolumetric ventricular relaxation_ - when P aorta \> P ventricles, **aortic valve closes to prevent backflow of blood (s2) then pulmonary valve closes shortly after**. Pressure in ventricles decr whilst volume stays same 12. _Rapid ventricular filling_ - when atrial pressure \> ventricular pressure, AV valves open and leads to rapid filling of ventricles 13. _Diastasis (passive ventricular filling)_ - 90% of ventricular filling occurs before atrial contraction whilst aortic pressure continues to fall

Answer 33

* Fibrous Parietal - lines fibrous layer * Visceral - lines outside of heart muscle * Serous fluid (pericardial fluid) between partietal and visceral layers) * heart muscle - myocardial cells and connective tissue between them

Answer 34

S1 - tricuspid and mitral valves snapping shut when L and R ventricles contract Then systole (contraction and ejection of blood) S2 - aortic and pulm valves snap shut, ending systole Then diastole (relaxation)

Answer 35

1. Umbilical cord is cut/clamped -\> removal of placenta from foetal circulation -\> umbilical arteries and vein constrict/blood clots 2. Baby takes first breath, air enters blood and lungs expand -\> air pushes fluid out -\> lung pressure/resistance drops -\> promotes blood flow into lungs -\> Blood returns to L side of heart after going through lungs -\> pressure L atria rises -\> foramen ovale and PDA closes (detects incr oxygenation of blood in LA and decr prostaglandins as was secreted by placenta; closes by 48 hrs)

Answer 36

Foramen ovale - hole between R and L atrium (bypasses foetal lungs which are filled with fluid) Patent ductus arteriosus - connection between PA and aortia These both allow a -\> Right to left shunt -\> Blood bypasses lungs and goes straight to aorta Foetus isn't breathing anyway so relies on Oxygen to be delivered from placenta via umbilical VEIN (x1; note is oxygenated blood) in cord -\> ductus venous in liver -\> IVC -\> RA -\> 1. RV 2. via patent foramen to LA -\> LV -\> around foetal circulation via aorta RV blood can also go to pulm artery to lungs or via PDA to aorta Blood delivered to foetal organs/tissue -\> deoxy blood returned via umbilical arteries (x2) back to placenta for reoxygenation Note foetal and maternal blood do NOT mix in placenta RBC stay in their own circulation but they transfer only O2 and co2 across placenta -\> foetal Hb has more affinity for O2 than maternal Hb

Answer 37

The conduit from umbilical vein (carries oxygenated blood from mums placenta to foetus) to the fetal IVC -\> IVC then carries oxygenated blood to foetal RA

Answer 38

High R side pressures due to high pulmonary resistance (fluid, no oxygen -\> pulm vessels constrict to redirect blood flow) This means that blood flow is directed through foramen ovale into LA (as pressures are lower) rather than through pulm A to lungs

Answer 39

Connection between pulmonary trunk and aorta Pressure aorta \< pressure pulmonary trunk Means that blood that DOES enter the pulmonary trunk/artery ends up being redirected to the aorta -\> systemic circulation (bypasses lungs)

Answer 40

Carotid and aortic arch baroreceptors (Stretch detection; more pressure = more stretch) Incr stretch detected -\> signals to brain -\> PS activation -\> decr HR, decr SV and vasodilation -\> decr BP Decr BP -\> Decr stretch detected -\> symathetic NS activation -\> incr HR and SV and vasoconstriction -\> incr BP OCCURS in sec-min (very rapid)

Answer 41

Flow (Q) x Resistance (R) = (SV x HR) x R

Answer 42

Pulmonary artery wedge pressure = Left Atrial Pressure Most commonly used to quantify degree of mitral stenosis (also will result in high LA pressure) Also gives indication of LV function (LV failure -\> high LA pressure \> 20mmgHg) Method: PCWP is measured by inserting balloon-tipped, multi-lumen catheter (Swan-Ganz catheter) into a peripheral vein (e.g., jugular or femoral vein), then advancing the catheter into the right atrium, right ventricle, pulmonary artery, and then into a branch of the pulmonary artery

Answer 43

Red flags that increase the likelihood of a pathologic murmur include - a holosystolic or diastolic murmur - grade 3 or higher murmur - harsh quality - an abnormal S2 - maximal murmur intensity at the upper left sternal border - systolic click - increased intensity when the patient stands.

Answer 44

Semilunar valve (aortic or pulm valve) stenosis

Answer 45

Upper right sternal border (URSB) - aortic valve Upper left sternal border (ULSB) - pulmonary valve clicks Lower left sternal border (LLSB) - Tricuspid valve and ventricular septal defects Apex - aortic or mitral valve

Answer 46

Sx - Usually asymptomatic and incidentally found on physical examination or echocardiography; large defects can be present in infants with CHF Clinical - Grade 2 or 3 systolic ejection murmur best heard at ULSB; wide split fixed S2; absent thrill; may have a grade 1 or 2 diastolic flow rumble at LLSB MOA Left (higher pressure) -\> Right (lower pressure) shunt Types - Ostium secondum is failure of foramen ovale to close - Ostum primum is congenital, when it involves the AV valve (common in downs) ECG: - Superior axis deviation - Incomplete RBBB Clinical: - asymptomatic - splitting of 2nd heart sound (because you've got more blood going into pulmonary circulation so pulmonary valve shuts slightly later than aortic valve) - pulmonary flow murmur - diastolic rumble across tricuspid valve - no lung changes ECG - Small uncomplicated - normal - First degree heart block (prolongued PR interval) - R axis deviation - Complete RBBB (wide QRS with rsR')

Answer 47

Sx - Onset depends on severity of pulmonary stenosis; cyanosis may appear in infancy (2 to 6 months of age) or in childhood; other symptoms include hypercyanotic 'Tet' spells (when crying) or decreased exercise tolerance Exam - Central cyanosis; clubbing of nail beds; - grade 3 or 4 long systolic ejection murmur heard at ULSB; systolic thrill at ULSB; single S2 ECG - Increased right ventricular forces as evidenced by tall R waves in V1. - Additionally, right atrial enlargement is manifested by prominent P waves in V1 (\*). - Right ventricular hypertrophy is demonstrated by a rightward deviated axis. CXR - small boot shaped heart

Answer 48

Sx - Usually asymptomatic but may have symptoms secondary to pulmonary congestion Clinical - Systolic ejection murmur (grade 2 to 5) heard best at ULSB radiating to infraclavicular regions, axillae, and back - Nnormal or loud S1; variable S2 - Systolic ejection click may be heard at left sternal border and may vary with respiration

Answer 49

Sx - Newborns and infants may present with CHF; older children are usually asymptomatic or may have leg pain or weakness Clinical - Systolic ejection murmur best heard over interscapular region; normal S1 and S2; decreased or delayed femoral pulse; may have increased left ventricular impulse

Answer 50

Sx -Usually asymptomatic; symptoms may include dyspnea, easy fatigue, chest pain, or syncope; newborns and infants may present with CHF Clinical - Systolic ejection murmur (grade 2 to 5) best heard at URSE with radiation to carotid arteries/neck; left ventricular heave; thrill at ULSB or suprasternal notch CXR: prominent LV +/- post-stenotic aortic dilatation ECG - findings of L hypertrophy/L heart strain - The S wave in V1 is deep - the R wave in V4 is high Often some ST depression can be seen in leads V5-V6, which is in this setting is called a left ventricular strain pattern

Answer 51

Sx - Variable presentation depending on type; may include cyanosis or CHF in first week of life Exam - Cyanosis; clubbing of nail beds; single S2; murmur may be absent or grade 1 or 2 nonspecific systolic ejection murmur; may have a grade 3 or 4 holosystolic murmur at LLSB and mid-diastolic murmur at apex

Answer 52

Sx - Early-onset cyanosis or CHF within the first month of life Exam - Cyanosis; clubbing of nail beds; normal pulses; single S2; holosystolic murmur at LLSB or midsternal border; murmur may be absent; mid-diastolic flow murmur at apex may be present

Answer 53

Sx -May be asymptomatic at birth, with circulatory collapse, shock (met acidosis), tachypnoea, cyanosis, and CHF developing with duct closure (d1-3) Exam - Hyperdynamic precordium; single S2; nonspecific grade 1 or 2 systolic ejection murmur along left sternal border, decr peripheral pulses

Answer 54

- Onset of CHF in first few weeks of life; minimal cyanosis Exam - Increased cardiac impulses; holosystolic murmur (ventricular septal defect); mid-diastolic rumble

Answer 55

Sat MV = (3xsat SVC + 1xsat IVC / 4

Answer 56

VO2 / (Pvsat - Pasat) x Hb x 1.36 Assume vo2 120 if not given express sats as frac (85% = 0.85)

Answer 57

pressure difference across lungs / cardiac output (Qp)

Answer 58

ASD #1 AVSD VSD PDA TOF

Answer 59

1. Incr O2 is the biggest factor that influences closure 2. Decr prostaglandins (from placenta)

Answer 60

1. Ductus arteriosus (pulm a to aorta) 2. Ductus venosus (umbi vein to IVC so bypass liver) 3. PFO (R atrium to L atrium)

Answer 61

Release of stored Ca frmo sarcoplasmic reticulum Ca binds to troponin to get tropomysin heads to move away so acitn and myosin can bind together then ATP can bind -\> hydrolysed -\> 'power stroke' = muscle contraction

Answer 62

Loud second heart sound

Answer 63

Separation between aortic and pulm valve closure on inspiration (aortic closes before pulmonary due to greater systemic pressure)

Answer 64

Fixed splitting - splitting of 2nd heart sound with both inspiration AND expiration = ASD

Answer 65

Secondum - 75% (hole in fossa ovalis = ostium secondum) Primum - assoc w cleft mitral valve or AVSD

Answer 66

Bicuspid aortic valve (two cusps instead of three) - 16% Coarctation (narrowing) of aorta - 11% Other Aortic arch anomalies

Answer 67

Pulmonary valve stenosis (39%) Hypertrophic cardiomyopathy (10%) Atrial septal defect (8%) Tetralogy of Fallot (4%)

Answer 68

**Ventricular septal defect** (VSD) Atrial septal defects Tetralogy of Fallot

Answer 69

Conotruncal defects: - TOF - Truncus arteriosus * Interrupted aortic arch

Answer 70

Hypokalaemia Hypocalcaemia Congenital Long QT sydnroem Myocarditis Malnutrition Medications (antipsychotics, antiarrhythmics, antidepressants, antibiotics, ondansetron)

Answer 71

Peaked T waves Prolongued QRS (\>3 squares) Prolongued PR interval (\>5 sq) Conduction blocks

Answer 72

BRoad QRS with delta wave Short PR interval ST depression Risk of re-entrant SVT

Answer 73

3-5 small boxes (120-200ms) → 160ms in children → 180ms in adolescents

Answer 74

**2nd degreee Mobitz type 1** (benign) - progressive lengthening of PR interval with ultimate **dropped P wave** **2nd degreee Mobitz type 2** (pathological) - pr interval LONG (fixed) with intermittent **dropped QRS**

Answer 75

Systolic BP drop of \>10mmhg with inspiration Main cause is cardiac tamponade asthma PE pericarditis hypovolaemia

Answer 76

Second degree AV block = intermittent failure of conduction to the ventricles type I - Wenckebach = progressive lengthening of the PR interval followed by a non-conducted P wave (dropped QRS). The PR interval then shortens and the cycle is repeated. type II - intermittent non-conduction of P waves without progressive prolongation of the PR interval. It is due to a failure to conduct below the AV node (His-Purkinje system) and is more likely to be related to structural damage to the conducting system. Can progress to third degree/complete heart block.

Answer 77

Flattened p waves Depressed ST segment U waves T wave inversion

Answer 78

**Brugada syndrome** is due to a **mutation** in the cardiac **sodium channel gene** **Predisposes** to VT, VF Often fhx sudden death or may present as sudden collapse (may be unmasked by fever/infection, drugs, ischaemia etc) Brugada sign = Coved **ST segment elevation** \>2mm in \>1 of **V1-V3** followed by a **negative T wave.**

Answer 79

RA enlargement

Answer 80

= Complete heart block P waves and QRS complexes are completely dissociated

Answer 81

second degree Mobitz type II and third degree/complete heart block

Answer 82

left atrial enlargement

Answer 83

Look at leads I, II, AVF Normal axis (0 to +90deg): Positive I, II, aVF LAD (0 to -90): Positive I, Negative aVF (II + or neutral) RAD (90 to 180): Negative I, Positive II and aVF Extreme AD (-90 to -180): Neg I, Neg II, Neg AVF

Answer 84

Look at V1 and V6 first Neonates: V1 the R wave is dominant, with S wave dominance in V6 (RV dominance) Children \>3yo: S wave dominance in V1 and R wave dominance in V6 (LV dominance) In between: R wave is dominant in both V1 and V6

Answer 85

RV: tall R waves in lead V1 and deep S waves in lead V6. LV: Deep S waves V1; tall R waves V6

Answer 86

\<1 week and \>10yo: upright 1week-10yo: inverted (if upright, suggests RV hypertrophy)

Answer 87

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)

Answer 88

congenitally corrected transposition of the great arteries

Answer 89

120ms (three small squares) Prolongation points to abnormal ventricular depolarization for which there are several causes 1. bundle branch block (RBBB RsR' more common than LBBB in kids) 2. an ectopic ventricular origin of cardiac activation (ventricular ectopy or pacing) 3. electrolyte disturbances 4. drug toxicity

Answer 90

RBBB- WIDE QRS and RsR'. common post TOF repair. vs Incomplete RBBB has normal QRS interval (\<120ms or 3 small sq) with RsR' . Usually benign, but may be associated with atrial septal defect

Answer 91

a shift (elevation/depression) of up to 1 mm may be normal in limb leads, whereas up to 2 mm is normal in left praecordial leads attributed to early repolarization of the heart.

Answer 92

QTc = QT / sqroot(RR) \<= 440ms

Answer 93

Rate \>200bpm Normal QRS complex Lack of discernible p waves

Answer 94

BROAD Qrs QRS complexes can be regular = monomorphic VT arising from single focus OR QRS irregular complexes - can have multiple foci. ex: Torsades de Pointes (can be sugg of underlying long QT)

Answer 95

Torsades de pointes Can be sign of underlying long QT syndrome

Answer 96

Cardiac outflow tract anomalies that include: * tetralogy of Fallot, pulmonary atresia with ventricular septal defect * double-outlet right ventricle (DORV) * double-outlet left ventricle * truncus arteriosus * transposition of the great arteries (TGA) Frequently found in patients with 22q11.2 deletion syndrome .

Answer 97

PR interval is prolonged This in itself is not problematic, but may progress to higher degrees of AV block

Answer 98

Compication of or most severe form of pulm htn/CHD = Pulmonary hypertension at systemic level due to high PVR with reversed or bidirectional blood flow through a septal defect (PVR so high that it trumps systemic vascular resistance and causes reversal of shunt from L-\>R to R-\>L)

Answer 99

Clinical: - Right ventricular lift/tap - S2= loud and single - Ejection click and early diastolic decrescendo murmur of PR - Holosystolic murmur of TR may be audible LLSB - Signs of RHF ECG: RAD and RVH +/- strain pattern

Answer 100

Corrective sx for underlying CAD ## Footnote Treatment of other underling diseases – such as CF, asthma, pneumonia bronchopulmonary dysplasia Anticoagulation and antiplatelet Pharmacologic treatment of Pulm HTN For ‘responders’ – pulmonary vasodilators are used in ‘responders’ - \> Nifedipine – Ca channel blocking agent - \> Prostacyclins – epoprostenol - \> Endothelin receptor antoagonists – bosentan, sitaxsenton, sildenafil, NO inhalation For ‘non responders’: - \> NO inhalation - \> Atrial septectomy - \> Lung or heart transplant

Answer 101

Genetic disorder of ventricular repolarization characterized by a prolonged QT interval on the ECG and ventricular arrythmia èusually torsades de pointes that may result in SCD **Pathophys** * Long QT interval = abnormally long repolarisation phase * Channel defect prolongs the myocardial recovery from excitation à prolonged potassium efflux * Leads to increased period of refractoriness * This predisposes re-entry pathway (wave of excitation wandering around the ventricle) à VT * Types * Type 1 - 35%; excessive K efflux. arrhythmia is classically triggered by exercise or emotional events * Type 2 - 25%; excessive K efflux. Arrhythmia is classically triggered during sleep/rest * Type 3 - minimal Na influx

Answer 102

Repaired TOF

Answer 103

-\> RA to RV connected to aorta, LA connected to PA. Survival depends on a shunt to allow for mixing, otherwise would not have oxygenation. Levo-TGA = ventricles on wrong side -\> RA to LV to aorta. LA to RV to pulm circ (usually asymptomatic)

Answer 104

Pathological in V1

Answer 105

Ventricular tachycardia

Answer 106

Deep S wave V1,2 Tall R wave V5,6 T wave inversion V5-6 LAD

Answer 107

Tall R wave in V1 Deep S wave in V6 ABnormal t wave direction in V1 (should be upright in newborns and \>age of 10, otherwise inverted)

Answer 108

Sit pt upright O2 Diuretics - furosemide, spironolactone to reduce pre and afterload Inotropes (PO digoxin or IV dobutamine/dopamine if severe) Vasodilators - captorpil, hyralazine to reduce afterload I+V if needed

Answer 109

Williams syndrome Congenital rubella syndrome Alagille syndrome CHD (ASD, VSD, PDA, TOF, supravalv AS)

Answer 110

PRogressive bile duct dilatation TOF BUtterfly vertebrae Npehritis Facialfeautres POsterior embrotoxon

Answer 111

Myocarditis PDA Micropthalmia Cataracts Deafness

Answer 112

Supravalvular AS HyperCa Mental retardation Elfin facies

Answer 113

Sodium valproate (ASD, VSD most commonly) Lithium (ebstein anomaly) Phenytoin Alcohol Rubella SLE/Srogrens Diabetes

Answer 114

Left (right if pulm HTN)

Answer 115

Normally close spontaenously during first few days of life Severe sx (heart failure, recurrent chest infections etc) w FTT pulm HTN aortic regurg persistent shunting \> 10yo

Answer 116

Seccundum (defect in atrial septum only) - RAD, incomplete RBBB +/- RVH Primum (failure of development of septum primum diving mitral and tricuspid valves and cleft in anterior leaflet of mitral valve; assoc w Downs syndrome) - Get mitral regurg. - LAD + incomplete RBBB, RVH

Answer 117

primum - always need surgical repair secondum - usually close spontantously; surgical repair if symptomatic or at 3-5yrs if perisistent

Answer 118

Assoc w downs syndrome involves mitral and tricuspid valves and atria Ventricles Sx - severe w ealry onset heart failure, recurrent pneumonia, FTT, pulmonary HTN Large L-\> R shunt across atria and ventricles ECG - Left/extreme axis w biventricular hypertrophy and incomplete RBBB (narrow QRS) CXR - cardiomegaly, pulm plethora Mx - surgical repair within 6 mo to prevent pulm HTN developing

Answer 119

Preterm infants - systolic murmur LSE older - MAchinery murmur below L clavicle

Answer 120

Fluid restriction Indomethacin if \<34/40 and within 3/52 of birth Surgical ligation if failure of medical management

Answer 121

LVH (right if pulm htn present)

Answer 122

Noonan syndrome

Answer 123

Balloon dilatation or surgical valvotomy if: - If pressure gradient across valve is \> 50mmHg - Severe pulm valve thickening - critical neonates (duct dependent circulation w cyanosis)

Answer 124

Balloon or surgical valvotomy if: - Neonate (followed by vlave repalcement later on) - Older children if pressure gradient across Aortic valve is \>50mmHg

Answer 125

Constriction of descending aorta REsults in LV outflow tract obstruction Duct dependent for circulation Presenation - circulatory collapse in first week of life when duct closes OR asymptomatic murmur (ejection systolic) HTN in upper limbs only, weak pulse in legs (do 4 limb BPs, pulses) Radiofemoral delay Heart failure ECG - RVH in noenates (bc RV is systemic) and LVH in older kids CXR: normal +/- prominent LV Cardiomegaly w increased pulmonary vascular markings Rib notching (collaterals forming beneath ribs) if \>8yo

Answer 126

PGE2 infusion Ventilation and inotropes as needed

Answer 127

1. COLLAPSE due to duct-dependent sytstemic circulation = L ventricle/aortic outflow obstruction a) Coarctation of aorta b) Hypoplastic L heart syndrome c) Critical aortic stenosis d) Interrupted aortic arch

Answer 128

Cyanosis in first week (anything with RV or pulm artery outflow obstruction) a) Transposition of great arteries b) Pulmonary atresia w VSD c) Critical pulmonary stenosis d) Pulmonary atresia w no VSD e) Tetralogy of fallow f) Tricuspid atresia g) TAPVD w obstruction h) Ebstein anomaly -

Answer 129

Pulmonary stenosis - note severity of cyanosis depends on severity of PS VSD Overriding aorta RV hypertrophy (Secondary to pulmonary stenosis) \*Note - no pulmonary HTN as pulm stenosis is protective\*

Answer 130

Features: 1. Absent tricuspid valve (no passage between RA and RV) 2. ASD (so blood can get to LA) 3. VSD (so mixed blood from LA -\> LV can get to RV -\> lungs) 4. Small, non-functional RV (as no blood flow to R ventricle) CLinical findings: Cyanosis at birth and increases with age as pulmonary flow decreases Systolic murmur at LSE Single S2 ECG: LAD or superior axis CXR: small haert w pulm oligaemia

Answer 131

O2 IV fluids Morphine Propanol IV (decr peroph vasc resistance) Bicarb (if acidotic) +/- IPPV (paralysed to decr O2 demand) +/- Noradenaline (decr systemic vasc resist so incr pulm blood flow) Surgery (high mortality if not corrected) - at 4-12 months of age close VSD and relieve RVOT obstruction

Answer 132

DOwn sydnrome 22q microdeletion (DiGeorge) syndrome CHARGE syndrome VACTERL

Answer 133

Right -\> Left shunt (due to pulmonary stenosis)

Answer 134

Both atria empty into single ventricle Both PA and aorta arise frmo this same ventricle Features depend on hte pulm blood flow 1. High pulm blood flow - pink w severe heart failure and eventually eisenmenger reaction 2. Low pulm blood flow - cyanosis w no heart failure CXR - cardiomegaly w pulm plethora (HF) or oligaemia (cyanotic)

Answer 135

1. initial 'palliation' with Blalock-Taussing shunt (anastamosis of subclavian artery to pulmonary artery) 2. Definitve palliatoin at 2-5yo with Fontan procedure (SVC and IVC connected directly to pulmonary artery.

Answer 136

1. ABnormal tricuspid valve ( leaflets adherent to ventricle wall and displaced distally) - \> results in RVOT obstruction 2. ASD 3. Small, underformed RV 4. WPW syndrome type B 5. Functional pulmonary atresia Clinical - cyanosis - FTT - SVT, extrasystoles - asymptomatic

Answer 137

Pulmonary vasodilation (O2, prostacycln, NO) or PGE2 to open duct Mx cardiac failure Tricuspid repait/replacement Closure of ASD Ablation of WPW pathway

Answer 138

WHat is it - Small L ventricle - Small mitral valve and aortic valve atresia - SMall asc aorta Clinical ft - Collapse/impalpable peripheral pulses/acidosis

Answer 139

Features - Complete absence of pulmonary valve + VSD and PDA (or collateral vessels) - Complete absece of pulmonary valve, NO VSD, a PDA, hypoplastic R heart, normal pulmonary arteries (Supplied by PDA). COmpletely duct dependent for pulmonary circulation. Note: PDA supplies mixed blood from aorta directly to pulmonary artery -\> lungs Clinical - Early neonatal cyanosis - Continuous murmur at the back due to collaterals with VSD (not heard at front) - Single S2 ECG: RA and RVH CXR: prominent RA, pulmonary oligaemia +/- R soded aortic arch and small boot shaped heart if VSD present

Answer 140

Mx - PGE2 if duct dependent - Surgery

Answer 141

SUpracardiac - pulm veins drain into SVC rather than LA. 'snowman' appearance on cxr with supracardiac shadow. Cardiac - pulm veins drain into r atrium rather than LA Infracardiac - pulm veins drain into IVC, ductus venosus, portal vein rather than LA. This is associated with obstruction to pulmonary venous return. hazy lung fields/'white out' on CXR REquires PFO, PDA or ASD to allow mixing of bloods between R and L sides of heart

Answer 142

Supracardiac TAPVD (no obstruction) - mild cyanosis - cardiac failure - recurrent chest infx - pulm htn (incr blood flow RA -\> RV)

Answer 143

Obstructed infracardiac TAPVD Sx - severe cyanosis, resp distress, hepatomegaly, no murmurs

Answer 144

JLN: AR, long QT + congenital deafness trigger: fear, excitement, exercise Romano-WArd: AD, isolated long QT

Answer 145

Cause is Group A beta-haemolytic strep infection (same strep that causes strep throat/scarlet fever) Strep throat infection (supported by ASOT or throat cultures) is followed 2-6 weeks later by polyarthritis, fever, malaise and cardiac sx Need 2 major criteria or 1 major and minor for diagnosis Major : - Carditis - polyarthritis (large joints, migratory) - sydenham chorea (involuntary movement) - erythema marginatum (pale red rings in recurrent crops, not itchy) - Subcut nodules on extensor surfaces (hard, painless, pea-like) Minor - Fver - Arhtralgia - Long PR interval or elevated ST - Raised ESR or CRP - Leucocytosis - Previous Rh fever

Answer 146

Bed rest (if active fever, carditis or arthritis) High dose aspirin Steroids for carditis Treat haeart failure Benzylpenicillin IM or oral penicillin Then lifelong penicillin prophylaxis

Answer 147

Mitral stenosis +/- aortic stenosis

Answer 148

Always think of this as a ddx in pt w known cardiac defect who is unwell - sustained fever, night sweats, malaise - new cardiac murmur - persistence of fever after acute illness - splenomegaly, splenic rub (common) - Arthritis of large joints (common) - splinter haemorrhages - roth spots (retinal haemorrhages) - Janeway lesions (erythematous macules on thenar and hypothenar eminences) - Osler's nodes (hard swellings on toes, fingers, soles, palms) - EMbolic phenomena (cerebral, pulm, coronary, peripheral) - Renal lesions-\> haematuria, fsgs, renal failure - Clubbing (late sign)

Answer 149

4-6 weeks antimicrobial tx (2 weeks IV) Abx prophylaxis for future proceudres Surgery if extensive valve damage/cardiac failure, vegetations or embolisaiton

Answer 150

Infection of endocardium, particularly predisposed by CHD or previously damaged/abnormal or prosthetic valves

Answer 151

Inflammation of heart with necrosis and fibrosis causing weakening of heart muscle with cardiac and resp failure. Can become chronic Causes 1. infectious - viral (coxsackie, adeno) - bacterial - dipheria, ricketsia - fungal - parasitic 2. toxic (related to pnuemonia, sepsis, drugs) 3. connective tissue disease 4. Idiopathic

Answer 152

CLinical ft - cardiac failure (tachycardia, weak pulses, resp distress) - arrhytmia - sudden death - can be asympatomic (eg in teens) CXR - cardiomehgaly (pericardial effusion), pulm plethora ECG; Arrhythmias Bloods - elevated CK, LDH, troponin

Answer 153

Supportive tx for cardiac failure, arrhythmas ECMO may be needed Transplant if chronic/refractory heart failure and irreversible damage to heart

Answer 154

Jervell-Lang- Neilson syndrome

Answer 155

Lung disease Caardiac disease PPHN Methaemoglobinaemia

Answer 156

Nitrogen washout test - baby is given \>90% oxygen to breathe for 10 min If paO2 rises to above \>100mmHg, the cause is resp or central disorder If paO2 doesnt change (or small rise but remains \<100), cause it cardiac or PPHN

Answer 157

MOA - ductal smooth muscle relaxant. keeps duct open SE: hypotension fever apnoea jitteriness

Answer 158

Methaemoglobinaemia - Iron in Hb is in the ferric (Rather than ferrous) form, non-functional and brown in colour - diagnose this on spectrophtometry of blood (Reveals metHb) - treat with reducing agents (ascorbic acid or methylene blue) or exchange transfusion

Answer 159

Pericarditis Raditation to L shoulder tip/neck it due to innervation of phrenic nerve C3,4,5

Answer 160

Coxsackie vairus is 1/3 of cases Staph, Haemophilus influenzae, Tb Rh fever Malignancy (Hodgkin disease)

Answer 161

ST elevation (saddle) T wave inversion

Answer 162

All cases of acute pericarditis eventually develop a pericardial effusion ECG- small voltages CXR - large globular heart Echo is diagnostic

Answer 163

Decr cardiac output Impalpable apex beat Soft heart sounds Friction rub Pulses paradoxicus (BP incr on respiration) Hypotension Kausmal's sign (neck veins distended on inspiration Raised JVP w Friedrich's sign (steep y descent)

Answer 164

Cardiac failure - dyspnoea, sweating, hepatosplenomegaly, ascites, peripheral oedema Decr ventricular filling - pulsus paradoxus, kusmaul's sign, friedrich's sgn ``` AF Pericardial knock (loud 3rd heart sound) ```

Answer 165

tb Acute pericarditis Haemopericardium

Answer 166

Systolic EF = reduced (less than 50-70%) Diastolic EF = preserved EF (because preload or filling capacity is also reduced)

Answer 167

Systolic (pumping - most common) cuased by: - IHD - Long-standing HTN (Arterial pressure ++ -\> harder to pump against -\> L sided hypertrophy -\> this increases O2 demand of myocardium but also reduces supply as the coronary arteries are squished/narrowed) - Dilated cardiomyopathy (chamber grows which increase filling/preload but musclular walls thin over time which reduces their strength for contractions) Diastolic (filling) caused by: 1. Concentric cardiomyopathy (incr muscle wall mass results in less room in chamber for filling) - long-standing HTN - Aortic stenosis - hypertrophic cardiomyopathy (genetic) 2. Restrictive cardiomyopathy (heart muscle stiffer and less compliant so less able to stretch and fill)

Answer 168

Activation of RAS system due to decr CO and decr perfusion of kideys LEads to Na nad water retention in order to increase preload/filling and thus contractility and CO

Answer 169

Pulmonary oedema -\> dyspnoea nad orthopnoea; inspiratory creps on ausculation

Answer 170

OFten caused by L sided HF (-\> biventricular HF) L -\> R cardiac shunt (ASD or VSD) -\> incr fluid volume on R side leading to concentric hypertrophy which elads to ischaemia (and thus systolic failure) and reduced filling/preload (and thus diastolic failure) Chronic lung disese -\> hypoxia -\> pulm arteriole constriction -\> increased pulm BP = pulmonary HTN -\> Cor pulmonale = R sided heart failure (as harder for R ventr to pump against)

Answer 171

Pitting oedema Ascites Hepatomegaly Incr JVP

Answer 172

BACKGROUND - Only a single arterial trunk with a truncal valve leaves the heart and gives rise to the pulmonary, systemic, and coronary circulations. - A large perimembranous infundibular VSD - The truncal valve may be bicuspid, tricuspid, or quadricuspid, and it is often incompetent. CLN FEATURES - Cyanotic (may be seen immediately after birth.  Worsens as PVR increases with L to right shunting) - Ejection click - Ejection systolic murmur at R and L USB - Signs of left and right heart failure (develop several weeks after birth)

Answer 173

1. Size of ASD 2. Relative compliance of ventricles - \> In infants the right ventricular wall is thick and poorly compliant (due to high inutero periph vasc resistance) – this LIMITS the left to right shunt - \> As the infant becomes older and pulmonary vascular resistance drops, the right ventricular wall becomes thinner (ie. more and the L to R shunt across the ASD increases - \> This is why signs of ASD occur in children 3-4 years of age (if previously undiagnosed) because degree of shunting is much greater

Answer 174

Clinical ft - ACYANOTIC Continuous murmur all over precordium Murmur more likely to be present at birth as not as reliant on fall of PVR Patients are usually asymptomatic. However, CHF may develop if the shunt through the fistula is large. Mx - Except for the very small coronary to PA fistulas, elective surgery is indicated for most fistulas as soon as the diagnosis is made to prevent complications such as subacute bacterial endocarditis (SBE), fistula rupture, myocardial infarction, and possible sudden

Answer 175

What is it? High-pitched sounds that occur at the moment of maximal opening of the aortic or pulmonary valves. They are heard just after the first heart sound What causes it? - Dilated aorta or pulmonary artery - systemic HTN, pulm HTN, TOF - The presence of a bicuspid or flexible stenotic aortic or pulmonary valve

Answer 176

Very close to aortic valve Can lead to aortic regurg - \> decr exercise toleratnce

Answer 177

LEvo (congenitally corrected) TGA - RA -\> LV -\> pulmonary artery (acyanotic) Dextro TGA - RA -\> RV -\> aorta (cyanotic)

Answer 178

L coronary artery arises abnormally from pulm artery instead of from aorta Results in inadequate perfusion of L pulm artery -\> lateral ischeamia, infarction, fibrosis of lateral leads Get ST elevation in lateral leads of ECG +/- RV/LVH Results in heart failure -\> death in first 6 months of life if untreated

Answer 179

Tetralogy of fallow

Answer 180

Transposition of great arteries TGA

Answer 181

Crying/defecation/hypotension -\> decr in systemic vascular resistance -\> incr R to left shunting of blood across VSD -\> leads to reduced blood flow across pulmonary valve (where RVOT is) -\> softer murmur

Answer 182

Di GEorge syndrome q11.2 deletion frmo chromosome 22

Answer 183

T21 DiGoerge/22q11 Turners syndrome

Answer 184

Noonans syndrome

Answer 185

Marfans Ehlos danlos syndrome

Answer 186

AD / denovo mutations Similar features to Marfans - incr armspan:height ratio CARDIC: dilated aortic root -\> aortic aneurysm or dissection - widely spaced eyes (hypertelorism) - bifid uvula - Scoliosis, joint laxity, arachnodactyly (abnormally long/slender finers and toes)

Answer 187

22q11 deletion Craniofcaial - Prominent nose, Small mouth, Small ears Palalte -bifid uvula, cleft palate (but NOT lip) Cardiac - Conotruncal abnormalities (TOF, Truncus arteriosus, interrupted aortic arch, VSD) Immune - Thymic hypoplasia -\> impaired cell fxn Endocrine: parathyroid deficiency + hypocalcaemia; GH deficiency Developmental delay, behavioural problems, ID

Answer 188

Is only recommended prior to: 1. Dental procedures 2. Invasive respiratory procedures (T&As, bx, incisions) 3. Invasive GU procedures In high risk patients: 1. Unrepaired cyanotic heart defects 2. Previous IE 3. Prosthetic valves/material (conduits, shunts) 4. Cardiac transplant recipients with valvulopathy 5. RHD in indigenous Australians

Answer 189

= VT Mx Lidocaine, amiodarone, procainamide Propranolol Cardioversion

Answer 190

Sick sinus syndrome A disease characterized by abnormal sinus node functioning with resultant bradycardia and cardiac insufficiency. Sx result from decr CO and decr end-organ perfusion Syncope, pre-syncope Dizziness Palpiatations Mx: Severe bradycardia = atropine Pacing = temporary followed by permanent Remove causative agents (ie digoxin, Ca blockers, beta blockers)

Answer 191

Long QT syndrome BEta blockers

Answer 192

SVT Vagal maneuver IV adenosine Oral flecainide Cardioversion if acutely ill Do NOT give digoxin

Answer 193

Atrial flutter (saw tooth, rate \>300, variable blocks) Digoxin, sotalol Cardioversion

Answer 194

Need a minimum of 3 of the following: Vertebral anomalies Anal malformations (imperf) Cardiac (TOF most common, VSD, ASD, Truncus, TGA) Tracheo-esophageal fistula Renal - incomplete formation of 1 or both kidneys +/- single umbilical artery Limb anomalies - poly/syndactyly, displaced or radial thumb

Answer 195

Tuberous sclerosis

Answer 196

maternal SLE (Lupus) - maternal ab against connective tissue crosses placenta and targets neonatal myocardium -\> test for ENA = extractable nuclear antigens (Anti-rho and anti-La ) maternal Sjogrens tx - medical is atropine or isoproterenol; pacemaker

Answer 197

* Refers to the presence of a congenital accessory pathway (called the **Bundle of Kent**, or atrioventricular bypass tract) and episodes of tachyarrhythmias * Sinus rhythm: * Type A: ***positive delta wave*** in all precordial leads with prominent R \> 1 in V₁ * Type B: prominent S wave in V1 and ***negative delta wave*** in leads V₁ and V₂ * AVRT can be **orthodromic** or **antidromic** conduction * orthodromic: narrow QRS, no discernible p waves, indistinguishable from AV-nodal re-entry tachycardia (AVNRT) * antidromic: wide QRS, resembles VT (which is less common in children)

Answer 198

Type A sinus rhythm pattern WPW * Sinus rhythm with a very short PR interval (\< 120 ms) * Broad QRS complexes with a slurred upstroke to the QRS complex — the [delta wave](https://litfl.com/delta-wave-ecg-library/) * **Dominant R wave in V1** — this pattern is known as “**Type A” WPW** and is associated with a **left-sided accessory pathway**

Answer 199

Type B WPW SR * Negative delta waves in leads III and aVF simulate the Q waves of prior [inferior MI](https://litfl.com/inferior-stemi-ecg-library/) (= **pseudo-infarction** pattern)

Answer 200

**Orthodromic atrioventricular re-entry tachycardia (AVRT), WPW** * Regular, narrow complex tachycardia at 225 bpm * No discernible P-waves * The QRS complexes are narrow because impulses are being transmitted in an orthodromic direction (A -\> V) via the AV node * This rhythm is indistinguishable from AV-nodal re-entry tachycardia (AVNRT)

Answer 201

**Antidromic AVRT in a 5-year old boy with WPW:** * There is a regular, broad complex tachycardia at ~280 bpm; this would be very difficult to distinguish from VT * However, given the child’s age, VT is very unlikely: \> 95% of broad complex tachycardias in children are actually some form of SVT with aberrancy

Answer 202

**Atrial fibrillation in a patient with WPW:** * Rapid, irregular, broad complex tachycardia (overall rate ~ 200 bpm) with a [LBBB](https://litfl.com/left-bundle-branch-block-lbbb-ecg-library/) morphology (dominant S wave in V1) * This could easily be mistaken for AF with LBBB * However, the morphology is not typical of LBBB, the rate is too rapid (up to 300 bpm in places, i.e. too rapid to be conducted via the AV node) and there is a subtle beat-to-beat variation in the QRS width which is more typical of WPW (LBBB usually has fixed width QRS complexes)

Answer 203

[**Monomorphic VT**](https://litfl.com/ventricular-tachycardia-monomorphic-ecg-library/) * Although there is a broad complex tachycardia (HR \> 100, QRS \> 120), the appearance in V1 is more suggestive of SVT with aberrancy, given that the the complexes are not *that* broad (\< 160 ms) and the right rabbit ear is taller than the left. * However, on closer inspection there are signs of AV dissociation, with superimposed P waves visible in V1 * Also, the presence of a northwest axis and an **R/S ratio \< 1 in V6** (tiny R wave, deep S wave) indicate that this is VT * This patient had a completely different QRS axis and morphology on his baseline ECG.

Answer 204

v1-6, placed on pt's chest

Answer 205

A Ventricular Assist Device (VAD) is a mechanical pump used to provide adequate cardiac output when heart failure is resistant to medical therapy (assists ventricles in pumping blood to the body) used for patients w ventricular dysfunction due to an acute but potentially reversible process (ex: acute myocarditis) CI: biventricular dysfunction OR pulmonary HTN, active infection (sepsis)

Answer 206

**“atrial gallop**,” occurs just before S1 when the atria contract to force blood into the left ventricle. Note- normally it is inaudible, only occurs with noncompliant L ventricle

Answer 207

“ventricular gallop,” occurs just after S2 when the mitral valve opens, allowing passive filling of the left ventricle = rapid ventricular filling

Answer 208

Closure of aortic and pulmonary valves = isovolumetric relaxation physiological splitting occurs during inspiration (aortic valve closes first, followed by delay in closure of pulmonary valve) paradoxical splitting occurs during expiration and can be caused by anything that delays the closure of the aortic valve including AS, HOCM or in the presence of a LBBB A fixed split S2 (ie during inspiration AND expiration) is pathognomonic for the presence of an ASD

Answer 209

closing of the mitral and tricuspid valves = isovolumetric contraction RBBB or PVCs can cause splitting of S1 (or can be physiological in 40-70% of ppl)

Answer 210

AS, HOCM or in the presence of a LBBB.

Answer 211

* 1st week of life: 125 * 1-2 months: 150 * 5-7 years: 100

Answer 212

1. RAD = RVH 2. LAD = not LVH. Abnormal activation of L muscle mass/abnormal position of conduction system ex: tricuspid atresia, complete AVSD, primum ASD, double outlet R ventricle, HOCM, Noonan syndrome associated PVS or HOCM

Answer 213

Divide number of large squares between QRS into 300 (note - rhythm must be regular)

Answer 214

Leads I and aVF

Answer 215

Conduction or heart block abnormalities Coronary artery dilatation (Kawasai like illness) Elevated troponin, BNP, ventriclar dysfunction

Answer 216

TOF repair RBBB after transatrial repair of tetralogy of Fallot is usually produced by **infundibular resection**, but not by VSD closure, and is associated with delayed activation of the pulmonary outflow tract and base of the right ventricle which results from damage to portions of the right ventricular conduction system.

Answer 217

LBBB → ‘William’ * QRS duration \> 120ms * Dominant S wave in V1 * Broad monophasic R wave in lateral leads (I, aVL, V5-6) * Absence of Q waves in lateral leads * Prolonged R wave peak time \> 60ms in leads V5-6

Answer 218

Look at leads II and V1 Right atrial enlargement: p amplitude \> 3mm in V1 and lead II (almost 1 small square) LA enlargement: terminal deflection in V1 is wider and deeper than 1 small square

Answer 219

\>8 days - 8 years: inverted t waves in R precordium (V1-4) * if not the case then RVH is present (IE upright T in V1 up to 8yrs is sign of RVH) * After 8years get progressive change to upright T waves from L → R (V1 the last to change to upright, often persists inverted until late teens)

Answer 220

U waves Flattened T waves ST depression QT prolongation

Answer 221

RVH Because in utero RV has been pushing blood through PDA into desc aorta → RVH

Answer 222

RVH ***Axis***: RAD for the patients age ***Voltages***: * Tall R waves (greater than limits for patient’s age) in right-sided leads V4R and V1 * Deep S waves (greater than limits for patient’s age) in left-sided leads V5 and V6 ***R/S ratio***: Abnormal R/S ratio in favour of RVH. * Increased R/S ratio (greater than upper limits for child’s age) in V1-2 * R/S ratio \< 1 in V6 (after one month of age) ***Abnormal T waves:*** Upright T waves in V1 and V4R in children 3 days to 6 years (provided that T waves are normal elsewhere, i.e. upright in V6). This is evidence alone of significant RVH. ***Abnormal Q waves**:* qR pattern in V1 (small Q wave, tall R wave) = highly specific for RVH.

Answer 223

LVH ***Axis***: LAD for the patients age (marked LAD is rare with LVH). ***Voltages:*** * Tall R waves in the left-sided leads V5 and V6 (greater than limits for patient’s age) * Deep S waves in the right-sided leads V4R and V1 (greater than limits for patient’s age) ***R/S ratio***: * Abnormal R/S ratio in favour of LV * Decreased R/S ratio in V1-2 (less than upper limits for child’s age) ***Abnormal Q waves*** in V5 and V6 ***Inverted T waves*** in I and aVL (LV strain pattern)

Answer 224

**Re-entry** * Mechanism: Uni-directional block * Initiating trigger * ex PAC, PVC * inducible and over drivable by pacing * Cardiovertable **Automatic** * NOT inducible or over drivable by pacing * NOT cardiovertable * Warm up/cool down (rate varies widely) **Triggered** * Mixed features

Answer 225

Supraventricular → Atrial → Atrioventricular Ventricular -only require tissue **below the bifurcation of bundle of his** for their continuum

Answer 226

Atrial flutter ‘sawtooth’ pattern Can be first presentation of chaotic atrial tachycardia

Answer 227

VF Some polymorphic VT In other cases with a regular well defined QRS complex, should be SYNCHRONISED w the QRS complex to avoid inducing VF

Answer 228

4 J/kg Then CPR for 2 min then assess rhythm

Answer 229

Monomorphic VT

Answer 230

ECG of AVSD Features: RBBB Left axis Peaked P waves (right atrial hypertrophy) Prolonged PR interval (prolongation of the atrioventricular conduction time) RVH (or Biventricular hypertrophy)

Answer 231

It is a specific form of PVT occurring in the context of QT prolongation — it has a characteristic morphology in which the QRS complexes “twist” around the isoelectric line. * For TdP to be diagnosed, the patient must have evidence of both PVT *and* QT prolongation Causes * Drug-induced * Electrolyte abnormalities (Low K or Mg) * Congenital long QT syndrome.

Answer 232

Torsades de pointes

Answer 233

TOF 0.7/1000

Answer 234

* Oxygen (acts as a pulmonary vasodilator and a systemic vasoconstrictor, though maybe limited effect as primary problem is reduced pulmonary blood flow) - high flow via mask * Squat position (knee-chest) * Increases systemic venous return and thus pulmonary blood flow * If not getting better * Morphine (to suppress the respiratory centre and abolish hyperpnoea) * IV beta blocker (propranolol) * Relaxation of the RVOT with improved pulmonary blood flow * Reduces the frequency/severity of spells (but usually surgical referral is required at this stage) * Bicarb if acidotic

Answer 235

* Any event that ↓ SVR suddenly (i.e. crying, defecation, tachycardia, hypovolemia or activity) produces a large right to left shunt that may initiate a hypoxic spell as pulmonary blood flow reduces * The resulting drop in Pa0₂ and PaCO₂ and reduced pH stimulate respiratory centres causing hyperpnoea * This increases venous return to the RV which causes further 0₂ desaturation, and repeats the cycle * You see worsening cyanosis and _disappearance of the pulmonary stenosis murmur_ * The baseline arterial sats (75-80%) are the best indicator of whether cyanotic episodes while crying are actually Tet spells * May be worse in children with mild cyanosis (aren’t used to hypoxemia)

Answer 236

Plateau in myocyte AP allows INFLUX OF Ca from SR (=depolisation) → stimulates muscle contraction * extracellular Ca enters via L-type Ca channels * Higher intracellular Ca triggers release of more Ca from SR through ryanodine receptors (=Ca induced Ca release) * Ca attaches to troponin C → tropomyosin moves → actin-myosin cross bridges form → contraction Cross bridges last as long as Ca occupies tropninin * tension in proportional to intracellular Ca concentration IC Ca is removed (back into SR) to induce relaxation via: * Ca ATPase * Na/Ca ATP exchanger Calcium-induced calcium release from the sarcoplasmic reticulum must occur under normal excitation-contraction coupling to cause contraction.

Answer 237

Pathological splitting is caused by prolonged opening/delayed closure of pulmonary valve * Pulm stenosis (R→L shunt) * RBBB (delayed RV contraction → RV emptying → delayed pulm valve closure) * ASD (RV volume overload → delayed pulm valve closure) - note this is characteristically ‘**fixed splitting**’ ie in insp AND exp Paradoxical (splitting on expiration) * severe AS * HOCM * LBBB

Answer 238

During fetal development, the primitive truncus does not divide into the pulmonary artery and aorta, instead resulting in a single, large, arterial trunk that overlies a large, malalignment type ventricular septal defect. Blood mixing occurs in ventricles and enters pulmonary and systemic circulation Sx: Mild cyanosis and symptoms and signs of congestive heart failure (eg, tachypnea, poor feeding, diaphoresis, enlarged liver) in the first few weeks of life. * Hyperdynamic precordium * Increased pulse pressure with bounding pulses * Loud and single 2nd heart sound (S2), and an ejection click. * A grade 2 to 4/6 systolic murmur is audible along the left sternal border (see table [_Heart Murmur Intensity_](https://www.msdmanuals.com/professional/cardiovascular-disorders/approach-to-the-cardiac-patient/cardiac-auscultation#v27888211)).

Answer 239

33% - CATCH 22 33% - 22q11 deletion/Di George (conotruncal defect)

Answer 240

Pericarditis ECG features widespread STE and PR depression with reciprocal changes in aVR (occurs during the first two weeks)

Answer 241

1. Prostin to keep duct open 2. Norwood procedure - first few weeks of life with aim to make the RV the player to pump systemic blood around body (Join aorta to RV, close PDA, make ASD bigger and create Blaylock-Taussig-Thomas shunt to shunt blood from aorta to lungs) 3. Glenn procedure - 4-6mo old (remove BT shunt and disconnect SVC and reconnect directly to PA) 4. Fontan procedure - 18-36mo (disconnect IVC and reconnect directly to PA so all systemic blood now goes directly to lungs)

Answer 242

A [_Hypoplastic left heart syndrome_](https://kidshealth.org/en/parents/hypoplastic-heart.html) _(no LV outflow)_ [_Double outlet right ventricle_](https://kidshealth.org/en/parents/dorv.html) _(both aorta and PA connected to RV, no LV outflow)_ [_Tricuspid atresia_](https://kidshealth.org/en/parents/tricuspid-atresia.html) _(no RV/PA outflow)_

Answer 243

TALL R wave in V1 Deep S wave in V6 Upright T-waves in V1 (this is abnormal in children age 8days -8yo, should be inverted!)

Answer 244

Deep S wave in V1 (daggers like LBBB) Tall R wave in V6 Inverted T-waves in lead I and IVL

Answer 245

Deep S wave in V1 (daggers like LBBB) Tall R wave in V6 Inverted T-waves in lead I and IVL

Answer 246

Qp:Qs \> 2:1 indicates large L→R shunt

Answer 247

Woods units \> 12

Answer 248

Atrial tachycardia. * ‘incessant’ AT = rare chronic arrhythmia in children and young adults. Can be difficult to manage * Rate ~ 100 - \>200 beats/min. * Often resembles sinus tachycardia. * narrow complex tachycardia * Each QRS complex is preceded by an abnormal P wave * p wave morphology is consistent throughout * Diagnosis is important as it may lead to dilated cardiomyopathy if and left ventricular dysfunction if not properly managed * First line digoxin -\> beta blocker -\> class 1 antiarrhythmic

Answer 249

Systolic murmur loudest at the lower left sternal edge Large/Severe VSDs are often accompanied on auscultation by a mid diastolic rumble indicating functional mitral valve stenosis

Answer 250

The magnitude of the left-to-right shunt is most influenced by the relative right and left ventricular compliances, with changes in the right ventricular compliance having the dominant effect. The relative vascular resistance in the pulmonary and systemic circulation also contribute to a lesser extent. In the newborn period high PVR is accompanied by a relatively thick, poorly compliant RV. During this period flow may be bidirectional with minimal net flow through the defect. Throughout infancy, PVR drops and the increasingly complicant RV is able to accept more blood volume, resulting in increased L to R shunting through the atrial shunt.

Answer 251

Pulmonary valve stenosis in 50% Less commonly: aortic stenosis, aortic coarctation, atrial septal defects (ASD), ventricular septal defects (VSD), and hypertrophic cardiomyopathy (HCM)

Answer 252

Peripheral pulmonary stenosis

Answer 253

Valvular pulmonary stenosis HOCM

Answer 254

VSD is #1 ASD TOF

Answer 255

supravalvular aortic stenosis (70% of cases)

Answer 256

Dilated cardiomyopathy Cardiac dysfunction is a frequent manifestation of Duchenne muscular dystrophy and a common cause of death for individuals with this condition. Early diastolic dysfunction and focal fibrosis proceed to dilated cardiomyopathy, complicated by heart failure and arrhythmia in most patients

Answer 257

Dilated cardiomyopathy Cardiac dysfunction is a frequent manifestation of Duchenne muscular dystrophy and a common cause of death for individuals with this condition. Early diastolic dysfunction and focal fibrosis proceed to dilated cardiomyopathy, complicated by heart failure and arrhythmia in most patients

Answer 258

PVCs Usually benign except in cases of prolonged QTc → can lead to torsades de pointes, or in case of WPW can trigger tachydysrhythmias

Answer 259

Premature atrial ectopics BEnign except in case of WPW/ischaemia etc when can trigger re-entrant tachydysrhythmias

Answer 260

Premature junctional complex Usually benign PJCs have the following features: * Narrow QRS complex, either (1) without a preceding P wave or (2) with a retrograde P wave which may appear *before, during, or after* the QRS complex. If before, there is a short PR interval of \< 120 ms and the “*retrograde*” P waves are usually inverted in leads II, III and aVF. * Occurs sooner than would be expected for the next sinus impulse. * Followed by a compensatory pause. * PJCs that arrive early in the cycle may be conducted aberrantly, most commonly with a RBBB morphology.