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53 Cards in this Set
- Front
- Back
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In utero what direction does the PDA shunt? And why?
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From Right to left because of high pulmonary presures
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What percentage of blood flows into the lungs via PA in utero?
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10%
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Name 3 common CHD that present in the first few hours?
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Pulmonary or aortic atresia, critical stenosis, Hypoplastic L) heart
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Describe the ion movement in pacemaker cells
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Ca2+ influx and K+ efflux
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Describe the ion movement in myocyte cells
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Na influx (phase 0), Ca2++ influx (K+ efflux) (phase 2) and K+ efflux (phase 3)
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What channel is affected in Brugada syndrome?
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Na channel - AD defect
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What ion is key for myocardial contraction and what does it do?
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Ca++ release from SER binds to Tn-C - inhibits Tn-1 causing a conformational change in tropomyosin, exposing the active site between actin and myosin enabling contraction
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Name the four components of Tetralogy of Fallot
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VSD, over-riding aorta, RVOT, RVH
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What ECG changes do you find with TOF?
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R) axis deviation
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At what age does the PDA close?
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Between 7 and 10 days
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Describe the common heart findings in hypoplastic left heart.
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Hypoplastic LV, atresia or critical stenosis of aortic or mitral valve
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What is the initial operation performed for hypoplastic L) heart?
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Atrial septostomy
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Describe the stages of Hypoplastic left heart repair
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1st - Norwood - links the R) ventricle to aorta, PA divided - prox to Aorta, conduit RV to PA, and distal segment to desc via MBTS
2nd - Bidirectional cavopulmonary shunt (12 -18mths) 3rd - Fontan +/- fenestration |
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Which condition is associated with truncus arteriosus and interrupted aortic arch and what is the genetic finding?
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Di George and 22q11 deletion
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What are the congenital anomalies associated with Noonan syndrome?
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HOCM (20%), ASD and PS
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Which condition is associated with supravalvular AS?
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Williams
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What is the most common heart defect found in Down's syndrome?
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AVSD. 1/3 Atrioventricular canal and 1/3 VSD
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Name two congenital conditions in which you find VSD.
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VACTERL association (vertebral, anorectal, cardiac (can also have aortic dilatation), TOF, esophageal atresia, rectal, limb) and Down's.
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What cardiovascular complication is associated with muscular dystrophy?
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Dilated cardiomyopathy.
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What type of CHD do you find in CHARGE - coloboma of eye, heart defects, atresia of choanal, retardation of growth and dev, genitourinary and ears.
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65% TOF, truncus arteriosus, aortic arch abnormalities (vascular ring, interrupted aortic arch), VSD
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Name 3 characteristics of innocent murmurs.
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1. Grade 1-2/6
2. Not ejection 3. Not diastolic Pathological > 3/6, Harsh, evident over LSE |
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What syndrome is associated with ASD?
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Holt-Oram syndrome
AD Hypoplastic or absent radii 1st degree heart block + ASD |
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Name the three types of ASDs? Which is more common and what are the typical findings on ECG? What is the murmur you find?
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1. Primum ASD - more extensive involves AV canal or endocardial cushion defect - usually involving tricuspid or mitral valve
2. Secundum ASD - most common 3. Sinus ASD ECG - incomplete RBBB and L) axis deviation Systolic murmur - increased flow across the normal pulmonary valve |
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Which arrythmia do you find in Ebstein's anomaly?
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WPW - upsloping of R wave
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When do children with VSD present and why?
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At 6-7 weeks due to a decrease in PVR?
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Why can you get a diastolic murmur with VSD?
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Due to increased flow across the mitral valve.
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What condition is associated with sensorineural deafness and prolonged QT syndrome?
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Jervell-Lange-Neilson AR
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What is the inherited defect in prolonged QT syndrome?
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malfunction in K+ channels (inadequate potassium efflux) or in sodium channels (excess inflow of sodium)
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Name two conditions associated with prolonged QT
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Romano-ward AD
Jervell-Lange-Neilson AR |
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What is a common disorder is found in the mother of infant's with congential heart block?
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CT disease SLE anti-Ro antibodies
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What does lengthening of repolarisation do in prolonged QT syndrome?
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Delays inactivation of calcium channels leading to "early after depolarisations" - progress to ventricular arrhythmias
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In what condition do you find a hamartoma?
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Tuberous sclerosis
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Which two anti-arrhythmics do you not give in WPW and why?
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Digoxin and verapamil - increase conduction velocity and can increase risk of VT
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What are "warning signs" in a history of syncope?
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1. In response to loud noise, fright or exercise or emotional stress
2. during exercise 3. whilst supine 4. FHx 5. If immediately lucid after 6. "odd" description |
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What arrhythmia can present with syncope?
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Long QT
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What congenital heart defects are more commonly associated wtih SVT re-entry?
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Ebstein's anomaly
CCTGA Single ventricle |
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Name 4 features more common to SVT than sinus tachycardia
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1. Rate > 160bpm
2. Fixed (or almost fixed) RR interval 3. abnormal P waves or axis or absent P waves 4. little changes with respiration or crying |
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What are the three types of SVT and which is the most common?
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1. Ectopic focus
2. Re-entry with accessory pathway - most common 3. Re-entry withough accessory pathway (AV re-entry) |
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Name three functional murmurs and their characteristics
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1. Venous hum - blood coursing through systemic large veins - blowing continuous murmur below clavicles, changes with respiration + position
2. Pulmonary flow murmur - soft ejection systolic murmur in pulmonary area 3. Vibratory murmur (Still's murmur) short buzzing murmur over the L) sternal edge, changes with posture and usually disappears by puberty 2. |
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What is the murmur usually heard post surgical correction?
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Pulmonary or aortic stenosis
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What operations are related to a R) thoracotomy scar?
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Blalock-Taussing scar
PDA ligation |
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What operations are related to a L) thoracotomy scar?
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Blalock-Taussig shunt (feel for absent pulses in the ipsilateral arm)
PDA ligation Coarctation of the aorta repair Pulmonary artery banding (listen for murmur) Lung biopsy |
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How does LCAPA present and at what age?
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Tachypnoea and grey with feeds. Usually after 6/52 with reduced PVR.
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What is Ebstein's anomaly?
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• During embryology the leaflets lift off from the endocardial surface (delamination) failure to do this leads to Ebstein’s – displacement of hinge-part of septal leaflet.
• Atrialised RV ( i.e a portion of the RV is incorporated into the RA) – functional hypoplasia of RV results. Presence of inter-arterial communication |
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Describe the difference in location of murmurs and the clinical significance of these.
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Max. above the nipple - ESM - AS and PS (think if post-op + AR/PR)
Below the nipple - VSD (lower L sternal edge) or MR/Mstenosis apex |
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What is the normal closing pattern of the second heart sound?
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Aortic closes before pulmonary, increases inspiration - increa return to R) side of heart
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What are the causes of widened splitting of the second heart sound?
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RBBB and pulmonary stenosis and fixed atrial septal deect
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What are the causes of reversed splitting of second heart sound?
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LBBB, severe aortic stenosis and left ventricular failure
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What causes narrow splitting of the second heart sound?
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pulmonary hypertension and aortic stenosis
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What causes a loud pulmonary component of the second heart sound?
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Pulmonary hypertension
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Name 5 causes of a single second heart sound.
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1. TOF
2. pulmonary atresia 3. Single ventricle 4. Aortic atresia 5. Pulmonary atresia |
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What is Kawasaki disease? and diagnostic criteria?
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Acute systemic febrile vasculitis of unknown aetiology.
Fever 5/7 + 1. Acute cervical lymphadenopathy >1.5cm 2. Strawberry tongue, mucosal hyperamia, dry,red cracked lips 3. Non-purulent conunctivitis, 4. Polymorphous rash 5.Peripheral oedema, hyperaemia followed by sequamination |
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Mainstay of Tx for Kawasaki disease
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IVIG and high dose aspirin
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