Cardio cram Flashcards
Syndromes associated with ASD
Holt Oram
Fetal alcohol syndrome
Widely split fixed S2
ESM left upper sternal border
Mid-diastolic rumble
Mild RVH or RBBB
= ASD
- ESM (relative PS) and mid-diastolic rumble (relative TS)
Primum ASD = superior axis
Secundum ASD = RAD
Syndromes associated with VSD
T21
DiGeorge
Turner
Holosystolic murmur LLSB +/- thrill
Apical mid-diastolic murmur
Early decrescendo murmur
LVH or LAH
= VSD
- Early decrescendo murmur (AR with infundibular VSD)
- outlet associated with aortic insufficiency/LVOT
LVH -> LAH -> RVH
BVH if large shunt
Syndromes associated with AVSD
T21
Apical holosystolic murmur
Superior axis
RVH or RBBB
Prolonged PR interval
= AVSD
Syndromes associated with PDA?
Maternal rubella
Prematurity
Bounding pulses, wide pulse pressure, hyperactive praecordium
Continuous machinery murmur
LVH or LAH
= PDA
Systolic murmur LUSB, mid-diastolic rumble, RVH or RBBB (or normal ECG)
= PAPVR
Syndromes associated with pulmonary stenosis
Rubella = PS and PPS
Alagille = PPS
Williams = PPS
Noonan = dysplastic PV
ESM LUSB radiating to back
A2 and P2 widely split
Prominent a wave
RAD, RAH and RVH strain
Pulmonary stenosis
Syndromes associated with aortic stenosis
Supravalvular - Williams, familial hyperchol
Valvular - Turner (bicuspid AV)
Harsh ESM RUSB
Early decrescendo LLSB
S2 splits normally or narrowly
LVH with strain
Aortic stenosis
Syndromes associated with coarctation
Turner
Kabuki
PHACES
Single S2, S3 gallop, reduced femoral pulses
Nonspecific ejection systolic
Low post ductal sats
Normal or RAD
RVH and RBBB, progresses to LVH by 2 years
= coarctation of the aorta
Inter AA
Extreme form of CoA in which the aortic arch is atretic or a segment of the arch is absent
Load S1 at apex, narrow split S2, low frequency mid-diastolic rumble
LAH, RVH +/- LAD
= MS
May have opening snap (rheumatic)
LAD associated with pulmonary hypertension
Syndrome associated with MR
MPS (Hurler) - most common cardiac lesion
Most common murmur in KD and ARF
S1 normal/reduced, loud S3, pansystolic murmur at apex
LVH +/- LAH, ECG can also be normal
= MR
Loud S3 due to increased blood flow across MV
May have apical mid-diastolic murmur of relative TS
Syndromes associated with mitral valve prolapse?
Marfan
EDS
OI
Stickler
PCKD (adults)
Klinefelter
Mid-systolic click +/- late systolic murmur
Enhanced by expiration
May have 1st degree AV block
= mitral valve prolapse
Syndromes associated with aortic regurgitation?
Overall rare, but associated with dilated aortic root (Marfan, EDS)
MPS (Hurler)
High pitched diastolic decrescendo murmur at LLSB, hyperdynamic praecordium, bounding pulse, wide pulse pressure
LVH if severe
Aortic regurgitation
Conditions associated with TOF?
22q11
T21
Alagille
Harsh ESM, systolic thrill, continuous murmur, single S2
RAD, RAH, RVH
= TOF
ESM - right ventricle outlet obstruction
Continuous murmur - PDA
Single S2 - pulmonary component not audible
Variations of TOF
TOF + PA: usually no murmur, S1 ejection click, S2 loud and single
TOF + absent PV: PS and PR ‘to and fro’ murmur
Conditions associated with tricuspid atresia
Pulmonary stenosis
TGA - common!!!
Coarctation
Cyanosis, single S2, LV impulse, holosystolic murmur LLSB
Superior axis, RAH +/- LAH, LVH
= TA
VSD causing holosystolic murmur LLSB is most common
Conditions associated with Ebstein anomaly
Maternal lithium use
Cardiac associations - PS, PA, TOF, VSD
Mild to severe cyanosis, quadruple rhythm (split S1, S3 and S4), holosystolic or early systolic murmur of TR at LLSB, hepatomegaly
Superior axis, RBBB, RAH, 1st degree AV block, WPW
= Ebstein anomaly
May also have SVT/other atrial tachycardias
Cyanosis, single and loud S2, usually no murmur (but can be present)
Upright T wave in V1
= dTGA
Can have VSD holosystolic murmur or outflow tract murmur
May have BVH, RAH or LAH on ECG
L-TGA associated with what conditions?
VSD = 80%
PS = 50%
TR = 40%
Dextrocardia in 50%
Absence of q waves in V5/6, presence of Q waves in V1, upright T waves across praecordium, varying AV block, atrial arrhythmia +/- WPW
= L-TGA
Hyperactive RV impulse, widely split S2, systolic murmur LUSB, mid-diastolic murmur LLSB
RV overload (rsR’ in V1) and RVH
= TAPVR (without obstruction)
Cyanosis, marked tachypnoea, single S2, gallop rhythm, no murmur, pulmonary crackles, hepatomegaly
RAD, RVH
= TAPVR (with obstruction)
Associations with truncus arteriosus
22q11 (30%)
VSD always present, with complete mixing
May also have coronary artery abnormality or interrupted aortic arch/right aortic arch
Varying cyanosis, hyperactive praecordium, single S2, ejection systolic click LUSB, systolic murmur
BVH in majority +/- LAH
= truncus arteriosus
Can have varying signs of CHF
Systolic murmur due to VSD
Associations with double inlet left ventricle
TGA 85%
PS/PA 50%
Associations with hypoplastic left heart?
Turner
T18, T13
Jacobson’s syndrome
22q11
Cardiac - ASD/PFO, VSD, coarctation (75%)
CNS abnormalities in 30%
Shock and acidosis, pulses weak/absent, loud and single S2, no murmur, grey colour
RVH
= hypoplastic left heart
5 most common cyanotic lesions
- Truncus arteriosus
- TGA
- Tricuspid atresia
- TOF (most common)
- TAPVR
DDx for cyanosis and respiratory distress
= increased pulmonary blood flow: TGA, truncus arteriosus, obstructed TAPVR
DDx for cyanosis without respiratory distress
= reduced pulmonary blood flow: TA, Ebstein, PA, PS, TOF
Lesions presenting with shock
Hypoplastic left heart
Critical AS
Critical CoA
Interrupted aortic arch
Lesions presenting with very early cyanosis?
TGA
Pulmonary atresia
Ebstein malformation
Lesions associated with respiratory distress
At birth - truncus arteriosus, TAPVR, PDA in preterm infants
At 6 weeks - VSD, PDA
Causes of single S2 related to abnormal valve position
Truncus arteriosus
TOF
Tricuspid atresia
HLH
TGA
L-TGA
Causes of single S2 related to abnormal valve
Severe AS/PS/aortic atresia
PA
Eisenmenger
Large VSD
Other causes of single S2
Pulmonary hypertension
Characteristics of TOF
- Large VSD
- RVOT/PS (subvalvular)
- Overriding aorta
- RVH
Characteristics of TA
Tricuspid valve absent - no connection between RA and RV, hypoplastic RV
Requires inter-atrial communication, systemic blood flow to cross to LA via ASD or PFO
Characteristics of PA and intact ventricular septum
Absent PV - majority have a diaphragm like membrane PV
Inter-atrial communication required for systemic blood flow
Characteristics of Ebstein anomaly
Downward displacement of TV into RV cavity - atrialised RV, hypoplasia of RV
Inter-atrial communication always present (PFO, ASD)
Characteristics of D-TGA?
Aorta is located anterior and to the right of the PA (dextro = ‘d’)
Requires mixing lesion
Characteristics of L-TGA?
Congenitally adjusted TGA:
RA -> LV -> PA
LA -> RV -> aorta
(i.e. wrong ventricles but blood moving the direction it should)
Characteristics of TAPVR
No direct communication between PV and LA
PV drain to alternative sites:
supra cardiac 50%
infra cardiac 20%
cardiac 20%
mixed 10%
ASD/PFO required for survival
Characteristics of truncus arteriosus?
Common PA and aorta (single arterial trunk)
VSD always present
Complete mixing of ventricles
Characteristics of double inlet left ventricle (single ventricle)
Both AV valves connected to a main, single chamber
Main chamber connected to a rudimentary chamber via the BVF
One great artery arises from each chamber
Characteristics of hypoplastic left heart syndrome
- Hypoplasia of LV - completely non-functional
- Atresia or critical stenosis of AV/MV
- Hypoplasia of ascending aorta and aortic arch
Indications for Fontan procedure
Dominant RV - HLHS
Dominant LV - DILV, PA and intact VSD, tricuspid atresia
Other - unbalanced AVSD, complicated DORV
Indications for Fontan procedure
Dominant RV - HLHS
Dominant LV - DILV, PA and intact VSD, tricuspid atresia
Other - unbalanced AVSD, complicated DORV
Phase 0 of cardiac action potential
Contractile cells are depolarised to threshold potential by the influx of Na and K through gap junctions.
When threshold potential is reached (-70mV) sodium channels open to allow rapid depolarisation through influx of sodium into cell
Phase 1 of cardiac action potential
Sodium channels begin to close, therefore reduced influx of sodium
Rapid open and closure of potassium channels, allows efflux of potassium
Combined effect = brief depolarisation of the cell
Phase 2 of cardiac action potential
Potassium channels open again allowing efflux of potassium from the cell
L-type calcium channels (activated by Na in phase 0) allow influx of calcium ions
Depolarising potassium is electrically balanced by depolarising calcium and tapering sodium currents, results in a plateau (distinguishes cardiac AP from nerve and skeletal muscle)
Influx of calcium triggers calcium release from sarcoplasmic reticulum, the intracellular calcium is responsible for cardiac myocyte contraction
Phase 3 of cardiac action potential
Rapid depolarisation occurs through closure of L-type calcium channels whilst potassium channels remain open, allowing more potassium to leave the cell and resulting in a net negative current
Potassium channels close when membrane potential is restored to -85-90mV.
Ionic pumps restore concentrations to levels from prior to AP
Phase 4 of cardiac action potential
Resting membrane potential of -90mV maintained through ionic pumps until stimulated to threshold potential.
