Child health- Congenital heart disease Flashcards
describe transposition of the great arteries
the aorta and pulmonary artery are transposed, so the aorta is supplying the right atrium and the PA is supplying left atrium
how would Transposition of the Great Arteries be diagnosed
echocardiogram
what are the four aspects of the tetralogy of Fallot
Stenotic pulmonary valve
Ventricular septal defect
Overriding Aorta
Right ventricular hypertrophy
what does Spells refer to in ToF?
physiological stress causes cyanosis of the patient, causing them to have a ‘blue spell’
how are spells treated in ToF?
using morphine to relax the infundibulum muscle
what is the main risk of ToF
acidosis
describe hypoplastic left heart syndrome
life limiting condition, often detected antenatally, in which the left ventricle is underdeveloped, causing poor systemic blood flow.
which conditions are acyanotic heart diseases?
Atrial septal defect
ventricular septal defect
persistent ductus arteriosus
describe ASD and VSD
hole between either the atria or ventricles
where is the most common location for a hole in ASD
where the FO was previously. failure to close
what are the risks of ASD and VSD
heart failure, more common in VSD.
when would a loud murmur be heard?
in smaller VSD as there is more turbulent blood flow
how does heart failure present in children
right sided more common: hepatic oedema
how is the ductus arteriosus kept open?
prostaglandins eg. PGE1
how is the ductus arteriosus closed?
prostaglandin inhibitors
describe coarctation of the aorta
there is a narrowing of the aorta, often presents with a child collapsing around day 3-5 after birth (the closing of the ductus arteriosus reduces the compensation)
how is the coarctation of the aorta detected
in the new baby examination, femoral pulses will be different.
what treatment is given if a coarctation is detected
prostaglandins, stent in the aorta.
describe aortic stenosis
describe pulmonary stenosis
which conditions are cyanotic heart diseases
tetralogy of fallot
transposition of the great arties
total anomalous pulmonary venous return
hypoplastic left heart syndrome
describe eisenmenger syndrome
when there is a right to left shunt eg ASD, VSD, PDA the pressure in the left should be greater to avoid blood coming into the left side of the heart.
Eisenmenger’s is when the right sided pressure increases to bypass this affect, allowing for cyanosis.
pulmonary pressure is greater than systemic pressure- allows right to left movement.
which population more commonly have Patent DA?
premature babies
where is prostaglandin E2 produced?
in the placenta
what is the function of prostaglandin E2 during pregnancy?
keeps ductus arteriosus open
what type of shunt does a PDA cause?
pressure in the aorta is higher than the pulomonary vessels so blood flows from aorta to pulmonary artery.
this is a left to right shunt.
what are the complications of a persistant DA?
additional blood creates more pressure in pulmonary vessels
causes strain on right ventricle.
pulmonary hypertension and right sided heart strain lead to right ventricular hypertrophy
there is an increased volume of blood returning from the lungs to the left side of the heart- this causes left ventricular hypertrophy
how does a larger PDA present on examination
continuous crescendo- decrescendo machinery murmur
heard loudest below the clavicle
normal first heart sound, second sound is difficult to hear over the murmur
how can a PDA present with symptoms
SOB
difficulty feeding
poor weight gain
LRTI
how is PDA managed?
echocardiogram confirms the diagnosis
medical closure using indomethacin, ibuprofen or paracetamol attempted in preterm infants- work by inhibiting prostaglandins
what are surgical interventions for PDA?
endovascular procedures
open surgery
what else may need to be managed in PDA?
heart failure
diuretics may be used to remove the water
what are the types of atrial septal defect
patent foramen ovale (not strictly classified as ASD)
Ostium secundum (septum secundum fails to close
ostium primum (septum primum fails to close)
what are the complications of ASD
stroke in the context of venous thromboembolism
atrial fibrillation or atrial flutter
pulmonary hypertension and right sided heart failure
eisenmenger syndrome
when can a DVT cause a stroke rather than a pulmonary embolism?
if there is a history of ASD
the clot can bypass the pulmonary circulation and enter the systemic
when are ASD detected?
antenatal scans or newborn examinations
it may be asymptomatic in childhood and present in adulthood with dyspnoea, heart failure, stroke.
what are childhood symptoms of ASD?
SOB
Difficulty feeding
poor weight gain
lower resp tract infections
which ASD is linked to migraine with aura?
patent foramen ovale
no recommendations for testing or treating patiets with migraine as a potential PFO
how is ASD managed
echocardiogram confirms diagnosis
paediatric cardiologist manages patients with ASD
-active monitoring
-percutaneous transvenous catheter closure
-open heart surgery
which syndromes might VSD be associated with?
turners
downs
describe the presentation of a VSD
initially symptomless, can present in adulthood
can be detected in antenatal scans or newborn baby check
what are the typical symptoms of VSD
poor feeding
dyspnoea
tachypnoea
failure to thrive
what would a VSD sound like on auscultation
pan systolic murmur
heard loudest at the left lower sternal border in the third and fourth intercostal spaces
systolic thrill on palpation
how is a VSD managed
echocardiogram confirms the diagnosis
A paediatric cardiologist will manage patients with a VSD. Management options include:
-Active monitoring (small defects may -close on their own)
-Percutaneous transvenous catheter closure (via the femoral vein)
-Open-heart surgery
what does VSD increase the risk of
infective endocarditis
what would be found on examining a patient with Eisenmengers syndrome
signs of pulmonary hypertension
-right ventricular heave
- loud P2 (forceful closing of pulmonary valve)
- raised JVP
-peripheral oedema
murmurs relating to the underlying septal defect (ASD,VSD, PDA)
which clinical findings can be caused by right to left shunt and chronic hypoxia
cyanosis
finger clubbing
dyspnoea
plethoric complexion
what is the definitive treatment for Eisenmengers syndrome
heart-lung transplant
which complications need to be managed in Eisenmengers syndrome
SOB- use oxygen
pulmonary htn- use sildenafil and bosentan
oedema- diuretics
arrhythmia
polycythaemia- venesection
thrombosis - anti coagulation
infective endocarditis - antibiotics
what is the prognosis of Eisenmenger syndrome
reduces life expectancy by around 20 years compared with healthy individuals
main causes of death- heart failure, infection, thromboembolism and haemorrhage
mortality up to 50% in pregnancy
where is a coarctation in the aorta usually found
near the ductus arteriosus
which genetic condition is coarctation of the aorta particularly associated with?
turners
what are signs other than reduced femoral pulses in coarctation of the aorta for infants?
-Raised respiratory rate
-Increased work of breathing
-Poor feeding
-Heart failure
-Shock
Additional signs may develop over time:
Left ventricular heave due to left ventricular hypertrophy
Underdeveloped left arm where there is reduced flow to the left subclavian artery
Underdevelopment of the legs
what is the management for coarctation of the aorta?
depends on severity
mild may not require surgical intervention
severe may require emergency surgery shortly after birth
describe hypertrophic obstructive cardiomyopathy
thickening of the left ventricle muscle, especially the ventricular septum
thickening reduces space in the ventricle, blocking blood flow to the aorta
known as left ventricular outflow tract obstruction
what is HOCM associated with
increased risk of heart failure, myocardial infarction, arrythmia and sudden cardiac death
is HOCM a genetic conditionq
yes!
autosomal dominant resulting from a defect in the genes for the sarcomere proteins
what is the prevelance of hocm
1 in 500 people
is family history relevant in hocm?
yes, there is usually someone who died suddenly.
HOWEVER it can also be a de novo mutation (new)
what is the usual presentation of hypertrophic OCM?
most patients asymptomatic
non specific presentation of:
-SOB
-Fatigue
-Dizziness
-syncope
-chest pain
-palpitations
SEVERE cases may present with symptoms of heart failure
what would be found on examination of a patient with HOCM
ejection systolic murmur
fourth heart sound
thrill at the lower left sternal border
how is HOCM diagnosed
echocardiogram or cardiac MRI
genetic testing may be considered to establish the affected genes
how is HOCM managed?
beta blockers
surgical myectomy
implantable cardiocerter defibrillator
heart transplant
what are patients advised to avoid?
intense exercise, heavy lifting and dehydration
AVOID ACE inhibitors and nitrates, as they can worsen LVOT obstruction
what are possible complications of HOCM
arrhythmia
mitral regurgitation
heart failure
sudden cardiac death
