Cardiology Flashcards
In paediatric basic life support, what is the ratio of compressions to breaths?
15:2
In cyanotic congenital heart diseases, what should be given to maintain a patent ductus arteriosus?
Prostaglandin E1
5 T’s of cyanotic congenital heart diseases:
Tetralogy of fallot Transposition of great vessels (TGA) Tricuspid atresia Total anomalous pulmonary venous return Truncus arteriosus
When does TGA generally present?
within hours to days of the neonate’s birth
Causes of congenital heart defects?
Mostly unknown
Down/Edward/Patau/Turner’s
FH > 3X risk
Maternal illness: rubella (PDA, PS), SLE (complete HB), DM (10X risk, if uncontrolled)
Maternal meds: warfarin (PDA, PS), FAS (ASD, VSD, ToF).
What are acyanotic heart defects?
L>R shunting
ASD, VSD, PDA
Breathless or asymptomatic.
What are cyanotic heart defects?
R>L
blood can’t get into lungs
R sided problem.
PTA, transposition of great arteries, tricuspid atresia, ToF, total anomalous pulmonary venous connection.
What is acrocyanosis?
healthy newborn periph cyanosis mouth + extremities. Benign vasomotor changes, vasoconstriction ↑tissue O2 extraction. May persist 24-48hrs.
Complications of congenital heart defects?
Can present from mins to days, postnatal, lots of still births have CHD.
Decompensation: engorged neck veins, tachycardia, brady poor sign, weak pulse, acidosis
Eisenmenger: pulmonary HTN, shunt reversal, R>L, cyanosis.
What is Eisenmenger syndrome?
describes the reversal of a left-to-right shunt in a congenital heart defect due to pulmonary hypertension.
This occurs when an uncorrected left-to-right leads to remodeling of the pulmonary microvasculature, eventually causing obstruction to pulmonary blood and pulmonary hypertension.
Associated with VSD, ASD, PDA
Features: original murmur may disappear cyanosis clubbing right ventricular failure haemoptysis, embolism
Management
- heart lung transplantation is required
Diagnosis of congenital heart defects?
Antenatal: anomaly scan, fetal ECHO
FBC
CXR
4 limb BP
Pre-post ductal SpO2 = O2 R hand (pre) vs foot (post). Any differences i.e. post-ductal SpO2 is low > cyanotic CHD
ECHO, cardiac cath
Cardiac MRI
Nitrogen washout test: hyperoxia test, differentiate cardiac from non-cardiac Infant given 100% O2 for 10 mins after which ABG taken. pO2 of <15 kPa cyanotic congenital heart disease
What are Ventricular septal defects?
most common cause of congenital heart disease.
They close spontaneously in around 50% of cases.
L>R shunt between ventricles, overload of R side, RVH.
Can be acquired following MI, congenital infections, ToF, FAS, Turner’s, Edward’s, Patuau, cri-du-chat + Downs
Small: restrictive, normal pressure between ventricles.
Moderate/large: non-restrictive, no pressure difference between ventricles.
Mostly failure of descending membranous septum coming down from endocardial cushion = normally fuses with ascending muscular
Features of VSD?
Asymptomatic IU
failure to thrive
features of heart failure: hepatomegaly tachypnoea tachycardia pallor
Small: asymptomatic, loud, harsh pansystolic blowing murmur left sternal edge/ tricuspid
Moderate-large: early HF Eisenmenger. Sweating, poor feeding/ FTT, resp infections. Murmur + systolic thrill + diastolic rumble mitral area. LA/LV dilation. Pul HTN.
Signs of congestive HF (dyspnoea, cough, pul vascular resistance, hepatomegaly, ↑HR/RR pallor). Displaced apex, RV heave > cardiomegaly
Murmur in VSD?
classically a pan-systolic murmur which is louder in smaller defects
Management of VSD?
clearly highly specialised
small VSDs which are asymptomatic often close spontaneously are simply require monitoring
moderate to large VSDs usually result in a degree of heart failure in the first few months:
nutritional support
medication for heart failure e.g. diuretics
surgical closure of the defect:
Patch closure
Transcatheter closure: mesh (higher risk)
Complications of VSD?
aortic regurgitation
aortic regurgitation is due to a poorly supported right coronary cusp resulting in cusp prolapse
infective endocarditis
Eisenmenger’s complex
due to prolonged pulmonary hypertension from the left-to-right shunt
results in right ventricular hypertrophy and increased right ventricular pressure. This eventually exceeds the left ventricular pressure resulting in reversal of blood flow
this is turn results in cyanosis and clubbing
Eisenmenger’s complex is an indication for a heart-lung transplant
right heart failure
pulmonary hypertension
pregnancy is contraindicated in women with pulmonary hypertension as it carries a 30-50% risk of mortality
Diagnosis of VSD?
CXR: LA enlarged, RVH, cardiomeg, LVH, pul a enlarged, pulmonary plethora.
Echocardiogram: location + size
MRI: if echo doesn’t diagnose
Cardiac cath: echo + MRI didn’t Dx but pul HTN. ↑O2 sats in RV
ECG: LVH, RVH (L/RA enlargement (Katz-Wachtel phenomenon). Ventricular strain
20 wk scan
What are atrial septal defects?
most likely congenital heart defect to be found in adulthood.
L>R shunt, blood through pulmonary circuit redundantly
Associated with FAS/ Down’s, F>M
They carry a significant mortality, with 50% of patients being dead at 50 years.
Two types of ASDs are recognised, ostium secundum and ostium primum. Ostium secundum are the most common
Ostium secundum - 70% of ASDs - associated with Holt-Oram syndrome (tri-phalangeal thumbs)
ECG: RBBB with RAD
Ostium primum - present earlier than ostium secundum defects
associated with abnormal AV valves
ECG: RBBB with LAD, prolonged PR interval
Features of atrial septal defects?
ejection systolic murmur, fixed splitting of S2
embolism may pass from venous system to left side of heart causing a stroke
Pul ejection/ systolic murmur, upper sternal edge, crescendo-decrescendo, RV heave
Infants + children: resp infections, FTT
Adults (before 40): palpitations, exercise intolerance, dyspnoea, fatigue, pul HTN, cyanosis, arrhythmia, haemoptysis + chest pain
↑JVP
Murmur in ASD?
ejection systolic murmur, fixed splitting of S2 (no change with breathing)
Complications of ASD?
Eisenmenger: murmur shortens + splitting disappears
Pul/ tricuspid regurg
Paradoxical emboli from DVT can cause stroke.
Pul HTN
Diagnosis of ASD?
CXR: r heart dilation, prominent pul vascularity, small aortic knuckle, pulmonary plethora, progressive atrial enlargement. Cardiomegaly, globular heart.
Transoesophageal echo: size/ location
Right heart cath: ↑O2 sat in: RA, RV + pul a, extra blood delay pul valve closure compared to aortic valve closure.
ECG: RBBB LAD + prolonged PR, or RAD. RVH.
Management of ASD?
Observation
Percut surgical closure
Adults: surgery in cases of RV enlargement, paradoxical embolism, R>L shunt
Pts need anticoags.
What is Patent Ductus Arteriosus?
DA stays open after birth. L>R shunt. Usually closes after 1st breaths, ↑pul flow, enhances prostaglandin clearance.
Congen rubella, premature babies, cytopathic damage to BVs, ischaemia to organs, prematurity, perinatal distress, hypoxia, born at high altitude, warfarin.
generally classed as ‘acyanotic’. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cyanosis
connection between the pulmonary trunk and descending aorta
Features of PDA?
left subclavicular thrill continuous 'machinery' murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat
Management of PDA?
indomethacin or ibuprofen
given to the neonate
inhibits prostaglandin synthesis
closes the connection in the majority of cases
if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair
Sx management: digoxin, furosemide
Surgery: closure, surgical ligation, cardiac catheterisation + coil.
Diagnosis of PDA?
Echo: dilated LA +LV
CXR: normal, cardiomegaly
ECG: LVH, LA enlargement
What is coarctation of the aorta?
describes a congenital narrowing of the descending aorta.
common at site of insertion of DA, distal to L subclavian a
↑blood flow in aortic branches before coarctation (upper extremities in head). ↓blood flow + pressure in lower extremities, kidneys activate RAAS, 2° HTN
more common in males (despite association with Turner’s syndrome)
Associations Turner's syndrome bicuspid aortic valve berry aneurysms neurofibromatosis
Features of coarctation of the aorta?
infancy: heart failure
PDA: gradual onset, infantile HF
DA closure: normal at birth, circulatory collapse at 2 days of life.
adult: hypertension
radio-femoral delay
mid systolic murmur, maximal over back
apical click from the aortic valve
notching of the inferior border of the ribs (due to collateral vessels) is not seen in young children
CP, cold extremities, claudication on exertion
Complications of coarctation of the aorta?
Aortic dissection
Berry aneurysm
HF
IE
Underdevelopment of limbs
DiGeorge: completely occluded, interruption of aortic arch, shock in neonatal period.
FTT/ poor feeding
2° HTN
Polycythaemia
Diagnosis/investigations of coarctation of the aorta?
Angiography: visualise narrowing
CXR: rib notching, 3 sign. Cardiomegaly.
ECHO: visualise location, size, blood turbulence
ECG: LVH, RVH, RA enlargement
Management of coarctation of the aorta?
Prostaglandin E: keep DA open. Alprostadil
Surgery: resection, end to end anastomosis, bypass graft across area of coarctation, stent/ surgery by 5
Patch aortoplasty, balloon angioplasty with stend, balloon dilation.
What is truncus arteriosus?
occurs when the blood vessel coming out of the heart in the developing baby fails to separate completely during development, leaving a connection between the aorta and pulmonary artery.
fails to divide into aorta/ pul a.
Single giant artery branching off from R+LV, oxygenated + deoxygenated blood mix > systemic circulation, cyanosis
DiGeorge syndrome, 22q11.2 deletion syndrome.
XS blood shunted to pul circuit, pul pressure < systemic circuit, fluid overload, HF.
RF: smoking, XS alcohol, teratogens, gestational DM, viral illness (rubella).
Features of trunks arteriosus?
Difficulty breathing
Pounding heart, weak pulse
impaired growth
Lethargy
Physical exertion: dizziness, fatigue, palpitations, dyspnoea
Loud systolic murmur along left sternal border, ↑flow across mitral
Diastolic flow murmur at apex when pul blood ↑
Cardiomegaly Pulmonary HTN, lung damage Resp problems Arrhythmia Valve regurg Poor feeding/ FTT,
Murmur of coarctation of the aorta?
mid systolic murmur, maximal over back
Murmur of truncus arteriosus?
Loud systolic murmur along left sternal border, ↑flow across mitral
Diastolic flow murmur at apex when pul blood ↑
Diagnosis/investigations of truncus arteriosus?
X-ray: heart size, lung abnormalities, XS fluid in lungs
ECHO: single large vessel from L+RV, abnormal valves Abnormal blood movement, volume of blood to lungs.
Newborn pulse ox: ↓O2 sats, diagnose before Sx develop
ECG: atrial enlargement (notching of P waves/ P mitrale), ventricular hypertrophy, abnormal T waves, right axis deviation.
Management of truncus arteriosus?
Diuretics
Inotropic agents: digoxin
Abx during dental/ other surgical procedures
Limit intense physical activity, lifelong monitoring
Surgery: vary depending on anatomy, close hole between R/LV separate large vessel into pul a + aorta.
What is transposition of the great arteries?
Cyanotic
It is caused by the failure of the aorticopulmonary septum to spiral during septation.
Basic anatomical changes
aorta leaves the right ventricle
pulmonary trunk leaves the left ventricle
2 small circuits of blood flow rather than 1 large. After birth, FO + DA close, no exchange between 2 circuits, cyanosis, death
Children of diabetic mothers are at an increased risk of TGA. Also rubella, poor nutrition, alcohol, >40
Features of transposition of the great arteries?
cyanosis tachypnoea loud single S2 prominent right ventricular impulse 'egg-on-side' appearance on chest x-ray
CV collapse at day 2-3 when duct closes
L-TGA - asymptomatic
Management of transposition of the great arteries?
maintenance of the ductus arteriosus with prostaglandins
surgical correction is the definite treatment.
Difference between D-TGA and L-TGA?
D-TGA, which presents with cyanosis early in life, and L-TGA, which on the other hand, may permit survival to adulthood without being diagnosed in childhood
d-TGA: complete (dextro = aorta on R)
I-TGA: congen corrected (levo = aorta on left). Ventricles + valves switched. Great vessels normal orientation, connected to wrong ventricle.
Diagnosis/ investigations of TGA?
Echo: eval heart function, structure
CXR: heart > egg on side, egg on string appearance, lung congestion, cardiomegaly
Angiogram: pre-surgery
ECG: R heart strain, RVH, axis deviation
Management of TGA?
Prostaglandin E: ST, keeps DA open
Balloon atrial septostomy: hole created in septum
Surgically switch vessels
What is tricuspid atresia?
a birth defect of the heart where the valve that controls blood flow from the right upper chamber of the heart to the right lower chamber of the heart doesn’t form at all
Cyanotic.
Tricuspid valve replaced by wall. ↓blood flow from RA to RV. Valve isn’t formed.
R heart small + underdeveloped.
Blood flows RA>LA through hole in wall, ASD, patent FO. Blood to lungs via DA, may also have VSD so LV>RV + pul a.
RF: rubella, alcohol, smoking, DM, parent with CHD, isotretinoin, bipolar/ antiseizure meds. Down’s.
Features of tricuspid atresia?
Tire easy esp during feeds Cyanosis Difficulty breathing Slow growth + weight gains HF
Murmur of tricuspid atresia?
A holosystolic murmur that may have a crescendo and decrescendo quality is present, signifying blood flow through the ventricular septal defect. A continuous murmur may be present.
Investigations and management of tricuspid atresia?
USS during gestation
ECHO: absence of tricuspid valve, irregular blood flow
Prostaglandin keep DA open
Atrial septosomy
Shunting: bypass from main BV leading out of heart to pul a,
Pul a band placement: large VSD, too much blood flowing to lungs, band ↓flow
Glenn procedure: 3-6mnths, connect SVC to pul a, blood flow directly to lungs, ↓workload on LV.
Fontan procedure: 2-5 y/o, path from IVC to pul a.
What is tetralogy of Fallot?
the most common cause of cyanotic congenital heart disease - however, at birth transposition of the great arteries is the more common lesion as patients with TOF generally present at around 1-2 months
typically presents at around 1-2 months, although may not be picked up until the baby is 6 months old
a result of anterior malalignment of the aorticopulmonary septum. The four characteristic features are:
ventricular septal defect (VSD)
right ventricular hypertrophy
right ventricular outflow tract obstruction, pulmonary stenosis
overriding aorta
The severity of the right ventricular outflow tract obstruction determines the degree of cyanosis and clinical severity
Alcohol, maternal >40y/o, poor nutrition, viral illness eg rubella in pregnancy, down’s, DiGeorge syndrome, FH of ToF
Features of Tetralogy of Fallot?
cyanosis
unrepaired TOF infants may develop episodic hypercyanotic ‘tet’ spells due to near occlusion of the right ventricular outflow tract
features of tet spells include tachypnoea and severe cyanosis that may occasionally result in loss of consciousness
they typically occur when an infant is upset, is in pain or has a fever
causes a right-to-left shunt
ejection systolic murmur due to pulmonary stenosis (the VSD doesn’t usually cause a murmur)
a right-sided aortic arch is seen in 25% of patients
Investigations for TOF?
chest x-ray shows a ‘boot-shaped’ heart
ECG shows right ventricular hypertrophy, RA enlargement, RBBB
Echo: pre/postnatal, degree of stenosis
Cardiac CT + MRI: info for planning surgery.
Compensatory polycythaemia
Management of TOF?
Prostaglandin E1
Tet spell: squat, bring knees to chest, kinks femoral a ↓cyanosis, ↑vascular resistance + systemic pressure, LV > RV, reverse shunt L>R, resolve cyanosis.
Surgery: VSD patch closure, 1st yr of life, RV outflow tract enlarged
O2, morphine to sedate child, relax pul outflow
BB
Phenylepinephrine
Murmur of TOF?
ejection systolic murmur due to pulmonary stenosis (the VSD doesn’t usually cause a murmur)
What is Total anomalous pulmonary venous congestion?
Both large veins (VC + pul vein) connect to RA, mixing of blood.
Need ASD for survival
In a baby with TAPVR, oxygen-rich blood does not return from the lungs to the left atrium. Instead, the oxygen-rich blood returns to the right side of the heart.
Features - cyanosis
CXR - snowman appearance
What is Epstein anomaly?
In this condition, your tricuspid valve is in the wrong position and the valve’s flaps (leaflets) are malformed.
Blood may leak back through valve
Tricuspid valve set lower in R side, bigger RA + smaller RV.
Poor flow from RA>RV>pul a
Patent FO/ASD in 80%, R>L shunt + cyanosis
RF: lithium, WPW
Features of Epstein anomaly?
Gallop rhythm
Cyanosis
SOB, tachypnoea
Poor feeding
Hepatomegaly
TR: pansystolic murmur, worse on inspiration
Widely split S1 + S2
Sx appear 48hrs as DA closes,
DA: blood aorta > pul a, to get oxygenated minimising cyanosis
HF
Cardiac arrest, collapse
Investigations and management of Epstein anomaly?
Prominent ‘a’ wave in distended JVP
ECG: arrhythmias, RAH, RBBB, LAD
CXR: cardiomegaly, RA enlargement, box in chest
ECHO
Treat arrhythmia + HF medically
Prophylactic Abx, prevent IE
Definitive Tx = surgery
RF for pulmonary stenosis in children?
Noonans
