Paeds - cardio Flashcards
what can cause a congenital left to right shunt?
how would they present?
ventricular septal defect
persistent arterial duct
atrial septal defect
they present as breathless
what can cause right to lefts shunts?
how would they present?
tetralogy of Fallot
transposition of the great arteries
they would present as blue
what are the circulatory changes at birth?
first breath = a fall in resistance to pulmonary blood flow
- -> blood flow through the lungs increases six fold
- ->this results in a rise in left atrial pressure.
- -> mean while the blood returning to right atrium falls as the placenta is excluded from the circulation
- -> the changes in the pressure difference causes the foramen ovale to close
- the DA will normally close within the first few hours or days of life
what are the symptoms and signs of heart failure?
symptoms - breathlessness (particularly on feeding or exertion), sweating, poor feeding, recurrent chest infection
signs: poor weight gain or faltering growth, tachypnoea, tachycardia, heart murmur, gallop rhythm, enlarged heart, hepatomegaly, cold peripheries.
what usually causes heart failure in the first week of life?
left heart obstruction e.g. coarctation of the aorta
*if obstruction is very severe then arterial perfusion may be predominantly by right to left flow of the blood via the ductus arteriosus (duct dependant circulation) - closure of the duct = severe acidosis, collapse and death unless duct patency is restored
after the first week of life what is the most common cause of progressive heart failure?
left to right shunt
this eventually causes pulmonary oedema and breathlessness due to decreased pulmonary vascular resistance
what happens if a left to right shunt is left untreated?
children will develop Eisenmenger syndrome which is irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary arterial pressure and flow. this lead the shunt reversing to a right to left shunt and results in a blue child. If this develops the only surgical option is a heart-lung transplant if available.
what might cyanosis in the new-born infant in the new born be due to?
- cardiac disorders - cyanotic congenital heart disease
- resp disorders - surfactant deficiency, meconium aspiration, pulmonary hypoplasia
- persistent pulmonary hypertension of the new born - failure of the pulmonary vascular resistance to fall after birth
- infection - septicaemia from group B streptococcus and other organisms
- metabolic disease - metabolic acidosis and shock
what are the different types of ASD?
secundum ASD
partial/primum ASD
what are the symptoms of ASD?
often asymptomatic
may have recurrent chest infections
from the fourth decade they may develop arrhythmias
what are the signs of ASD?
an ejection systolic murmur best heard at the upper left sternal edge - due to increased flow across the pulmonary valve because of the left-to-right shunt
a fixed and widely split second heart sound
what investigations would you perform for ASD?
CXR - cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular markings
ECG - right bundle branch block
Echo
how do you manage ASD?
if the defect is large enough to cause ventricular dilation treatment will be required.
For secundum ASD this is by cardiac catheterisation with insertion of an occlusive devise
Partial AVSD - surgical correction is needed - usually at about 3-5 years of age to prevent heart failure and arrhythmias
what is a small VSD?
smaller than the aortic valve in diameter - up to 3mm
how would a small VSD present ?
what would you hear when auscultating a small VSD?
what investigations would you perform and how would you manage one?
often asymptomatic
a loud pan-systolic murmur at lower left sternal edge (loud murmur = smaller defect)
quiet pulmonary second sound
CXR and ECG will be normal
echo will demonstrate precise anatomy of defect
management - often will close spontaneously
what is a large VSD?
how would a large VSD present ?
what would you hear when auscultating a large VSD?
what investigations would you perform and how would you manage one?
- bigger than the size of the aortic valve
- symptoms of heart failure with breathlessness and FTT after 1 week old, recurrent chest infections, tachypnoea, tachycardia and enlarged liver from heart failure, active precordium.
- there may be a soft or no murmur = large defect
- there will be a loud pulmonary second sound from raised pulmonary pressure
- CXR - cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema
- ECG - biventricular hypertrophy by 2 months of age
- ECHO - demonstrated anatomy of defect
- Management: diuretic for heart failure (furosemide) often combined with captopril.
Surgery should be performed at age 3-6 month
what is patent ductus arteriosus?
a form of congenital heart defect
generally classed as ‘acyanotic’. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis.
connection between the pulmonary trunk and descending aorta
usually the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance
more common in premature babies, born at high altitude or maternal rubella infection in the first trimester
what are the signs of patent ductus arteriosus?
left subclavicular thrill continuous 'machinery' murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat
how do you manage a PDA?`
1st line - IV indomethacin (inhibits prostaglandin synthesis ) - closes the duct in the majority of the cases
*if it is associated with another congenital heart defect amenable to surgery then prostaglandin E1 is usefull to keep the duct open until after surgical repair
use a percutaneous catheter device closure
if baby too small or large duct then use surgical ligation
what test can be used in cyanosed infants to test for heart disease?
the hypoxia (nitrogen washout) test place them in 100% oxygen for 10 mins and then if right radial artery PaO2 from blood gas remains low then it Is cyanotic congenital heart disease
what are the 4 cardinal anatomical features of tetralogy of Fallot?
ventricular septal defect (VSD)
- right ventricular hypertrophy
- right ventricular outflow tract obstruction
- pulmonary stenosis
- overriding aorta
what are the symptoms of TOF?
cyanosis - hypercyanotic episodes
a loud harsh ejection systolic murmur
in older children there may be clubbing
may have tachypnoea
what investigations would you perform for TOF and what would they show?
CXR - boot shaped heart
ECG - will show right ventricular hypertrophy when older
ECHO - will show the cardinal features.
how do you manage TOF?
complete surgical repair to close VSD and relieve RV outflow obstruction
what is transposition of the great arteries?
the aorta is connected to the right ventricle and the pulmonary arteries are connected to the left ventricle - this means there is two parallel circulation
*incompatible with life unless there is a mixing of blood - often there is naturally occurring VSD, ASD, PDA which can achieve mixing short term
leads to right to left shunt
what are the symptoms of transposition of the great arteries?
neonatal cyanosis
usually appears on day 2 when ductal closure leads to reduced mixing of desaturated and saturated blood
often a loud, single heart sound
how do you manage transposition of the great arteries?
maintain ductus arteriosus with prostaglandin infusion
a balloon atrial septostomy
all patients will require surgery, arteria switch procedure in the neonatal period
what is and AVSD?
how does it present
how is it managed?
atrioventricular septal defect
common in down-syndrome
may be complete or partial
it will present on antenatal USS screening
cyanosis at birth or heart failure at 2-3 weeks of life
manage heart failure and fix surgically at 3-6 months
what is aortic stenosis?
when the aortic valve leaflets are partly fused together, giving a restrictive exit form the left ventricle
often associated with mitral valve stenosis and coarctation of the aorta
what are the symptoms and signs of aortic stenosis?
asymptomatic murmur
severe stenosis - may lead to exercise tolerance, chest pain on exertion or syncope
small volume, slow rising pulse
carotid thrill
ejection systolic murmur that radiates to the neck
delayed and soft aortic second sound
apical ejection click
how do you manage aortic stenosis and pulmonary stenosis ?
balloon dilatation
what are the clinical features of pulmonary stenosis?
what would investigations show?
mainly asymptomatic
small number of neonates have critical pulmonary stenosis and have duct- dependant circulation
ejection systolic murmur best heard at the upper left sternal edge
ejection click
there may be a ventricular heave when stenosis is severe
CXR - normal or post stenotic dilation of the pulmonary artey
ECG - evidence of right ventricular hypertrophy
what is coarctation of the aorta?
uncommon
narrowing of the aorta distal to the left subclavian artery adjacent to the insertion of the arterial duct.
what are the clinical features of coarctation of the aorta?
often asymptomatic sometimes when the duct closes the aorta also constricts, causing severe obstruction of the LV outflow systemic hypertension ejection systolic murmur radiofemoral delay
what would you see on investigations for coarctation of the aorta?
CXR - rib notching due to the development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction, 3 sign - visible notch in the descending aorta at the sit of the coarctation
ECGH - LV hypertrophy
how do you manage coarctation of the aorta?
insertion of a stent
sometimes surgical repair is required
what is interruption of the aortic arch?
Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta.
usually VSD is present
presentation is with shock in the neonatal period - circulatory collapse with absent femoral pulses and absent left brachial pulse
how do you manage an interruption of the aortic arch?
maintain ABC
prostaglandin infusion
complete correction with closure of VSD and repair of the aortic arch is usually performed within the first few days of life
what is hypoplastic left heart syndrome?
underdevelopment of the left side of the heart
small or atretic mitral valve
left ventricle is diminutive and there is usually aortic valve atresia
small ascending aorta
circulatory collapse - all peripheral pulses absent
sometimes detected antenatally
no flow through the left side of the heart, so ductal constriction leads to profound acidosis and rapid CV collapse
how do you manage hypoplastic left heart syndrome?
maintain ABC and prostaglandin infusion
Norwood procedure then at 67 months the Glenn or hemi-Fontan procedure and again the Fontan procedure about 3 years
what is a sinus arrhythmia?
it is normal in children and it detectable as a cyclical change in heart rate with respiration (acceleration during inspiration)
what is the most common childhood arrhythmia?
supraventricular tachycardia
what are the clinical features of supraventricular tachycardia?
rapid HR between 250-300BPM
can cause poor CO and pulmonary oedema
it will typically present as heart failure
what will an ECG show in supraventricular tachycardia?
narrow complex tachycardia
management of supraventricular tachycardia
circulatory and respiratory support vagal stimulation manoeuvres e.g. carotid sinus massage IV adenosine electrical cardioversion flecainide
what is congenital complete heart block?
rare condition
usually related to the presence of anti-RO or anit-LA antibodies in maternal serum - this mothers will either manifest or latent connective tissue disorders
the antiboides appear to prevent the normal development of the conduction system in the developing heart with atrophy and fibrosis of the AV node
all children with symptoms will require the insertion of an endocardial pacemaker
hoe does long QT syndrome present in children?
associated with sudden loss of consciousness during exercise, stress or emotion usually in late childhood
may be mistakenly diagnoses as epilepsy
inheritance is AD
what is rheumatic fever?
there is an abnormal immune response to a streptococcal infection
usually develops 2-6 weeks following the infection
it is usually a pharyngeal infection - then polyarthritis, mild fever and malaise develop
what is the evidence for a recent streptococcal infection?
raised ASO titre or other streptococcal antibodies
positive throat swabs
positive rapid group A strapt antigen test
what are the major and minor criteria for rheumatic fever?
Two major or one major and two minor
MAJOR: erythema marginatum - uncommon early manifestation rash on trunk and limbs, Sydenham’s chorea - a late feature - involuntary movements and emotional liability, polyarthritis, pancarditis (endocarditis, myocarditis, pericarditis), subcutaneous nodules
MINOR: fever, raised ESR or CRP, arthralgia, prolongued PR interval
what are the symptoms of chronic rheumatic fever?
the most common long-term damage is mitral stenosis from scarring and fibrosis.
how should you manage rheumatic fever?
acute management is with bed rest and anti-inflammatory agents
if there is evidence of active myocarditis then bed rest and limitation of exercise are essential
aspirin good for supressing inflammatory response in joints and heart - it needs to be given in high dose so needs to be monitored
if fever and inflammation do not resolve rapidly then corticosteroids will be required
if there is persistent infections then antibiotics should be given
following resolution of an acute infection, recurrence should be prevented with monthly injections of zathine penicillin
what are risk factors for infective endocarditis?
congenital heart disease
rheumatic fever valve disease
prosthetic valves
IVDU
when should you suspect infective endocarditis?
it should be suspected in any child or adult with fever, malaise, raised ESR, unexplained anaemia or haematuria
what are the most common causes of infective bacterial endocarditis?
streptococcus viridans - linked to poor dental hygiene staphylococcus aureus (common in IVDU)
what are the clinical signs of infective endocarditis?
fever anaemia and pallor splinter haemorrhages in nailbed clubbing necrotic skin lesions changing cardiac signs splenomegaly neuro signs from cerebral infarction retinal infarcts arthritis/arthralgia haematuria
how should you diagnose infective bacterial endocarditis ?
blood cultures should be taken before starting Abx
detailed cross-section echo may confirm diagnosis but will not exclude diagnosis
acute phase reactants are raised
how should you treat bacterial endocarditis?
high dose IV Abx - penicllin/vancomycin for a min of 4-6 weeks
what are some causes of heart failure in children?
large left to right shunt left sided obstructive lesion cardiomyopathy myocarditis endocarditis Kawasaki disease tachyarrhythmias
what are the clinical features of heart failure in children ?
sweating breathless, tachypnoea, coughing, lung creps poor feeding, poor weight gain and FFT hepatomegaly cardiomegaly tachycardia, gallop heart rhythm
what are some causes of acyanotic heart disease?
VSD ASD PDA pulmonary valve disease coarctation of the aorta aortic stenosis hypoplastic left heart syndrome hypertrophic obstructive cardiomyopathy dextrocardia
what are some causes of cyanotic heart disease?
tetralogy of fallot
transposition of the great arteries
tricuspid atresia
what are the principle causes of pericardial inflammation?
- Infections (coxsackie B , EBV, strept, TB, toxoplasmosis
- connective tissue (RA, rheumatic fever, SLE, sarcoidosis
- metabolic - hyperuricaemia, hypothyroidism
- malignancy
- radiotherapy
what would an ECG show when there is pericarditis?
low voltage QRS complex
what are the clinical features of pericarditis?
typically sharp pain which is exacerbated and exaggerated by lying down and relieved by sitting or leaning forward. Pain is often referred to left shoulder
how is pericarditis managed?
analgesia
anti-inflammatory drugs
pericardiocentesis for pericardial effusion
what might myocarditis be due to ?
infections
Kawasaki
drugs - Adriamycin
connective tissue disease: SLE, RA, rheumatic fever, sarcoidosis
what are the classifications of cardiomyopathy?
hypertrophic
dilated
restrictive
what are the causes of dilated cardiomyopathy?
inherited
secondary to metabolic disease
direct result from a viral infection
