Cardiology Flashcards
Eisenmenger Syndrome
Increased pulmonary pressures leads to reversal of L-R shunt, causing R-L shunt and cyanosis.
Can occur with many different underlying defects including PDA, ASD, and VSD.
Onset typically 2nd-3rd decade
Management of Tet Spell
Increase SVR
- squatting
- bring knees to chest
Decrease PVR
- calm child
- O2
Slow HR to allow RV filling
- beta blocker
- morphine
4 components of Tetralogy of Fallot
- RVOT
- VSD
- Overriding aorta (dextroposition of aortas with overriding of the ventricular septum)
- RVH
Genetic conditions associated with TOF
DiGeorge syndrome
Trisomy 21, 13 and 18
Alagille Syndrome
CHARGE syndrome
What is transposition of great arteries
Aorta connected to RV and pulmonary artery connected to LV
Most mixing occurs through PFO, then PDA, and 50% will also have a VSD
Presentation of TGA
Early severe cyanosis
OR
If also have a VSD, will present with HF and cyanosis at 3-4wks
Single, loud S2
Egg shaped heart on CXR
Persistently positive T waves in V1
Congenital heart lesions with LAD?
- Ostium primum ASD
- Complete AV canal defect
- Tricuspid atresia
Types of TAPVR
- Supracardiac (50%): pulmonary venous blood drains into left vertical vein -> L innominate vein -> SVC
- Infracardiac (~23%): pulmonary venous blood drains via a descending vein below the diaphragm -> ductus venosus -> IVC. This type is most likely to have severe obstruction to pulmonary venous return
- Cardiac (~20%): connects to RA (usually via the coronary sinus)
- Mixed type
Features of obstructed TAPVD
Profound cyanosis
Shock
Respiratory distress
Get elevated PA pressure and reduced systemic output
Features of RA isomerism
Bilateral “right-sidedness”
- Bilateral Right atria and right lungs (3-lobes)
- Horizontal liver with equal-sized lobes
- Bilateral morphologic RA, each with an SA node
- Asplenia
- Bowel malrotations common
- Complex CHD common (90%) - complex cyanotic CHD and anomalous pulmonary venous return
Features of LA isomerism
Bilateral “left-sidedness”
- 2-30 equal-sized spleens (polysplenia) which often function abnormally
- Bilateral left atria and left lungs (2 lobes each)
- Central transverse liver
- Risk of bowel malrotation
- CHD (50%) - simple acyanotic + abnormal rhythms (including complete heart block)
Situs inversus
Mirror image configuration include arrangement of GIT
- 3 lobed lung on L, 2 lobes on R
- Liver on L
- The atria are switched
- Risk of primary ciliary dyskinesia (25%), no increased risk of cardiac anomalies
CXR findings in CoA
- Cardiomegaly
- ‘3’ or ‘reverse E’ (notching of aortic isthmus in L superior mediastinum)
- Rib notching due to erosion from large collaterals
What is Ebstein anomaly
Rare anomaly, associated with maternal lithium use
Leaflets of tricuspid valve are displaced downwards to RV wall, forming a large RA.
Will see RA enlargement on ECG and CXR with wall-to-wall enlargement
Aortic stenosis murmur
Systolic ejection murmur at RUSE with radiation to the neck
Suprasternal thrill with more severe stenosis
Can have ejection click at apex
HCM Murmur
Harsh, systolic crescendo-decrescendo murmur heard best at LLSE
May also have S3 and S4
Quieter with squatting (increased venous return)
Louder with Valsalva (reduced venous return)
ie opposite to AS
Pulmonary artery sling
Left PA arises from Right PA and runs between the trachea and oesophagus, leaving an ANTERIOR indentation on the oesophagus
Double aortic arch
Persistence of both R and L 4th embryonic arches
Encircling of trachea and oesophagus, with indentation of the R and L sides of the trachea and oesophagus
Right aortic arch
Common with TOF and TA
Usually only symptomatic if there is an aberrant L subclavian artery which the ductus arteriosus can arise from and create a ring
Most common cardiac cause of cyanosis in first 24hrs?
TGA
ECG features of VSD
LVH if moderate defect
Biventricular hypertrophy if large defect
Murmur of VSD
Harsh holosystolic heard best at LLSE
Stimulus for closure of PDA
Increased O2 tension and decreased prostaglandin after birth
Murmur of PDA
Continuous “rumbling” murmur heard throughout systole and diastole. Heard best below L clavicle
Will also have wide pulse pressure and bounding pulses
Fixed split S2 suggests which congenital cardiac pathology?
ASD
(increased blood volume into RV -> delayed closure of pulmonary valve)
Murmur in ASD?
Murmur occurs due to increased flow across RVOT and pulm valve
= systolic ejection crescendo-decrescendo
Can also get an early or mid diastolic murmur due to increased flow across tricuspid valve
Narrowly split or single S2?
Pulmonary hypertension
Single loud S2 can also occur with TGA, HLHS, Truncus arteriosis
Medications which reduce PVR?
- Phosphodiesterase 5 (PDE5) inhibitors eg Sildenafil
- Endothelin receptor antagonists eg bosanten
- Prostacyclin (PGI2)
- Nitric oxide
Pulmonary stenosis murmur
Systolic ejection click followed by a harsh systolic crescendo-decrescendo murmur heard best at LUSE and radiations to below L clavicle
ECG changes in PS
- RAE (tall p waves)
- RAD
- RVH with very tall R waves in V1 and V2
“Egg on a string” CXR
TGA
Immediate management of TGA
Prostaglandin to keep the duct open
Balloon atrial septostomy to create an ASD
Later (by 2-3wks), arterial switch surgery
Snowman or figure 8 on CXR
TAPVD
ECG features of aortic stenosis
- LVH
- LV strain (ST depression in left lateral leads)
How common are bicuspid aortic valves?
1-2% of the population! May develop stenosis over months- decades
Most common cardiac defect seen in Williams Syndrome
Supravalvular AS
- narrowing just above the level of the coronary arteries
Wall-to-wall heart on CXR
Ebstein anomaly
- downwards displacement of the valve leaflets of the triscupid valve -> large RA, TR, PS or functional atresia
- associated with maternal lithium use
Management of HCM
Beta blockers
Restriction from competitive activity
Some need ICD
CCB +/- amiodarone used in some patients
ECG features of HCM
- LVH, prominent septal Q waves, abnormal depolarisation or strain (negative T waves in V6)
What is ALCAPA?
Anomalous origin of the L Coronary Artery from the Pulmonary Artery
- instead of coming off the aortic sinus, the L coronary artery comes off the Pulm Artery
- After birth, when PA pressure falls, blood can flow from RCA to LCA through collaterals (mini R-L shunt) and back to lungs rather than to myocardium -> anterolateral ischaemia
ECG features of ALCAPA
Anterolateral ischaemia
- deep Q waves in I, aVL, V5, V6; poor R wave progression in praecordial elads
- ST and T waves changes can occur
Which type of vascular ring causes anterior indentation of the oesophagus?
Anomalous L pulmonary artery aka pulmonary sling
Which type of vascular ring causes indentation on both sides of the oesophagus and trachea?
Double aortic arch
Jones criteria for RF?
- Polyarthritis (migratory)
- Carditis
- Syndenhams chorea
- Subcutaneous nodules
- Erythema marginatum
Need evidence of recent or concurrent GAS infection + 2major or 1 major + 2minor
Most common valve pathology in Acute rheumatic fever?
Mitral regurgitation
(2nd = aortic regurgitation)
Pericardial friction rub is pathognmonic for…
Pericarditis
Muffled heart sounds suggests…
Pericardial effusion
What is pulsus paradoxes and what causes it?
Larger change in BP with inspiration that occurs with cardiac tamponade
(usually drops 4-10mmHg with inspiration, with pulsus paradoxus it drops >10-15mmHg)
Beck triad for cardiac tamponade
- Rising JVP
- Quiet, muffled heart sounds
- Dropping systolic BPs
Which drugs are contraindicated in WPW?
Digoxin
Verapamil
Which CHD commonly causes Wolff Parkinson White?
Ebstein anomaly
Triggers in congenital long QT syndrome types 1, 2 and 3?
Type 1= exercise, commonly swimming
Type 2= arousal eg. loud noises, emotions
Type 3= rest/sleeping
Most common type of congenital long QT syndrome and its genetic mutation?
Type 1 congenital Long QT syndrome, caused by KCNQ1 gene mutation (~35%)
Type 2 (KCNH2) ~30%
Type 3 (SCN5a) ~10%
Management of congenital long QT syndrome?
Avoid contraindicated medications
Avoid excessive exertion + no swimming (type 1)
Beta blockers - nadolol or propranolol
Some may need ICD for secondary prevention
Defect in the gene for the cardiac ryanodine receptor (RyR2) channel can lead to what condition?
Catecholaminergic Polymorphic VT (CPVT)
This channel is particularly sensitive to adrenaline
Loss of function in the SCN5A gene encoding cardiac action potential sodium channel?
Brugada syndrome
(Congenital Long QT 3 caused by gain of function mutation in same gene)
Most common cardiac defect in Alagille syndrome?
Branch/ peripheral pulmonary stenosis
Acyanotic congenital heart diseases with increased pulmonary blood flow and LVH or combined ventricular hypertrophy?
VSD
PDA
AVSD
Acyanotic congenital heart diseases with increased pulmonary blood flow and RV hypertrophy?
ASD
partial anomalous pulmonary venous drainage
Acyanotic heart disease with normal pulmonary blood flow and LVH?
Aortic stenosis
Mitral regurgitation
Aortic regurgitation
CoArctation of the aorta
Acyanotic heart disease with normal pulmonary blood flow and RVH?
Pulmonary stenosis
Coarctation of the aorta in infants
Mitral stenosis
Most common ECG finding in acute rheumatic fever?
Prolonged PR interval
Acyanotic heart defects which can cause LVH/ LAE on ECG and CXR
- PDA
- VSD
(unlike ASD which usually causes RVH/RAE)
Neonate with cyanosis with LAD, LVH on ECG?
Tricuspid atresia
(TAPVD, TOF and TGA cause RAD and RVH)
What causes S3?
Rapid (passive) ventricular filling - deceleration of blood moving from LA to LV
Low frequency brief vibration heard early in diastole
What causes S4?
S4 is heard during atrial contraction, just before S1 and is due to “stiff” ventricles (LVH or reduced ventricular compliance)
Most common type of SVT in infants?
AV re-entrant tachycardia (orthodromic more common than antidromic)
Management of atrial flutter?
Synchronised DC cardioversion
or
Oesophageal overdrive pacing
(don’t give flecainide as can convert to 1:1 conduction)
ECG differences between orthodromic and antidromic AVRT?
Orthodromic will have narrow QRS complex, and usually retrograde p waves
Antidromic will have wide QRS complex
