Cardiac - Paediatric heart conditions Flashcards
Pathophysiology of heart failure
Heart failure = pathophysiologic state in which the heart fails to pump blood at a rate to meet the demand of the
body, i.e. 2 components:
1) Dysfunction of heart (pump failure) – commonly in adults (e.g. ischemic heart disease, impaired contraction of LV)
2) Cardiac overload (pressure or volume overload with preserved heart function) – more common in children
Imbalanced supply and demand causes S/S of HF
Systemic signs of children with heart failure
Breathlessness (SOB), especially on exertion/feeding
Poor feeding (longer time required to finish milk feed)
Excessive sweating during feeding
Failure to thrive (poor growth)
Recurrent chest infection
Exercise incapacity in older children
Signs of venous congestion
Pulmonary
Tachypnea
Subcostal in-sucking
Wheezing (in infants)
Systemic
Hepatomegaly
Distension of neck veins (not obvious in small children)
Peripheral edema (rare because without cardiac dysfunction
Decompensation:
Low pulse volume
Cool extremities
Prolonged capillary refill
Compensatory mechanism to venous congestion
Tachycardia
Cardiomegaly
Causes of heart failure in neonates
1) Left ventricular outflow tract obstruction
- Coarctation of aorta
- Aortic stenosis
- Aortic atresia/ hypoplastic left heart syndrome
2) Myocardial dysfunction
- Transient myocardial ischemia
- Myocarditis
- Cardiomyopathy
3) Abnormal HR/ rhythm
- Supraventricular tachycardia
- Complete heart block
4) Extra- cardiac causes
- Systemic disturbance causing ventricular dysfunction:
o Sepsis
o Asphyxia
o Hypocalcaemia
- Anaemia (high-output cardiac failure)
Coarctation of aorta in neonate
- Associated syndrome
- Structural abnormalities in heart
- Pathophysiology
- Presentation
Syndrome: Turner’s syndrome with familial clustering (although majority sporadic)
Associations: hypoplasia of transverse aortic arch, VSD, bicuspid aortic valve, berry aneurysms
Pathophysiology: discrete narrowing of descending aorta at insertion of ductus
- RV supplies descending aorta via persistent arterial duct
- impaired flow from proximal to distal part of aorta
- Organs depend on supply from pulmonary artery through PDA to descending aorta»_space; Low perfusion to organs
Presentation
- LV pressure overload with systemic hypertension (duct-independent CoA)
- acute heart failure and renal failure with shock (duct-dependent CoA)
Coarctation of aorta
- Clinical signs for duct-dependant and non-duct dependent CoA
General/ both:
- Compensatory LVH
- Systemic arterial insufficiency»_space; enlargement of intercostal arteries as collaterals with rib notching
- Systolic HTN in UL due to outflow obstruction
Duct-dependent:
- Weak LL pulses
- RV strong impulse
- Soft ESM at LUSB
- Collapse, shock, oliguria after ductal closure
Duct-independent:
- Weak LL pulse with radiofemoral delay*
- LV impulse (heaving apex)
- ESM at LUSB radiating to left interscapular region
- soft continuous murmur throughout chest
Coarctation of aorta
First line investigations and typical finding
□ Bloods: severe metabolic acidosis due to ischaemic colitis and AKI upon duct closure
□ CXR:
→ Cardiomegaly and ↑pulm vascular markings in infants/neonates with HF
→ Cardinal ‘figure of sign at site of coarctation with pre- and post-stenotic dilatation
→ Notching of undersurface of posterior ribs due to erosion by collaterals
□ ECG: RVH if neonatal HF, LVH in others
□ Echo: demonstrate site and severity of coarctation
□ MRI: demonstrates length and severity of coarctation
Critical aortic stenosis in neonate
- Pathophysiology
- Effect
- Signs
Pathophysiology: Critical AS Impedes blood flow from LV to ascending aorta
- Congenital valve defect or rheumatic fever
Effect: LV Pressure overload causes low cardiac output
Signs:
- Low pulses in both upper and lower limbs
- Pulsus parvus et tardus/ anacrotic pulse (slow, notched upstroke with sustained peak)
- LV volume overload: sustained parasternal heave
- Systolic thrill in aortic area
- Ejection systolic murmur at aortic area +/- radiation to bilateral neck
Aortic atresia/ hypoplastic left heart syndrome in neonates
- Pathophysiology
- Associated syndrome
- Effect
- Pulse
Pathophysiology
No left heart function: Complete atresia of aortic valve, mitral valve atresia and left ventricular hypoplasia
Blood returning to lungs then to LA is channeled through patent foramen ovale/ atrial communication into right atrium and right ventricle
From RV through pulmonary trunk, supply lungs and aorta through persistent ductus arteriosus
A/w Turner’s syndrome
Effect:
Right heart supports both pulmonary circulation and systemic circulation
Pulse:
Lower pulse in both upper and lower limbs
Myocardial dysfunction in neonate
- Causes
Transient myocardial ischaemia (due to perinatal asphyxia and hypoxemia»_space; transient disturbance of ventricular contraction»_space; metabolic acidosis)
Myocarditis (uncommon; acquired infection from mother)
Cardiomyopathy (usually in older children)
Abnormal heart rate/ arrhythmia in neonates
Causes
Supraventricular tachycardia (more common): Associated with accessory pathways like in Wolff-Parkinson-White
Complete heart block: Maternal SLE with anti-Ro and anti-La destroys fetal heart conduction tissue
Extra-cardiac causes of neonatal heart failure
Systemic disturbance causing ventricular dysfunction:
o Sepsis
o Asphyxia
o Hypocalcaemia
Anaemia (high-output cardiac failure)
Causes of heart failure in infants
Due to large left-to-right shunts
- Ventricular septal defect (VSD)
- Atrioventricular septal defect (AVSD)
- Persistent arterial duct (patent ductus arteriosus, PDA)
- Atrial septal defect (less common)
Ventricular septal defect (VSD) in infants
- Pathophysiology
- Clinical signs
- Associated syndrome
Pathophysiology:
Defect in ventricular septum - severe Left to right shunt
High pulmonary flow - pulmonary hypertension
Increased pulmonary venous return - LV volume overload
Clinical signs:
- Pansystolic murmur at tricuspid area, widely radiating with thrills
- Pulmonary arterial congestion»_space; respiratory distress
- ± RV pressure overload (parasternal heave) at LATE stage
- LV volume overload (displaced, thrusting apex)
- Pulmonary hypertension (loud P2 or single S2)
Syndromes:
DiGeorge
Down
Eisenmenger
Atrioventricular septal defect (AVSD) in infants
Pathophysiology
Most commonly associated syndrome
Clinical signs
Pathophysiology:
AV septum devoid of partition due to valve formation defect
Blood flows from LA to RA and LV to RV
Particularly common in Down syndrome
Clinical signs:
- Pulmonary arterial congestion»_space; respiratory distress
- Volume overload of LA»_space; Parasternal heave
- LV dilatation»_space; Displaced apex, strong LV impulse
Persistent arterial duct (patent ductus arteriosus, PDA) in infants
Pathophysiology
- Clinical signs
Pathophysiology
Ductus arteriosus connects aorta to pulmonary artery
Aortic pressure is much greater than pulmonary artery pressure
L to R shunt of blood from aorta to pulmonary artery
Clinical signs:
- Differential clubbing (toes only)
- Collapsing pulse/ Corrigan’s sign
- Continuous murmur at L infraclav/LUSB
- LV volume overload (displaced thrusting apex)
- Pulmonary arterial congestion»_space; respiratory distress
- Volume overload of LA»_space; Parasternal heave
- RV dilatation
ASD in infants
- Pathophysiology
- Effect
- Signs
Pathophysiology:
- Atrial septum formed by Septum primum with foramina primum and secundum, and Septum secundum with foramen ovale
- At birth, High pulmonary venous return increases LA pressure
→ septum primum pressed against foramen ovale for closure + gradual fusion
- Failure to close foramen secundum → secundum ASD
- Failure to close foramen primum → primum ASD
Effect:
- chronic L-to-R shunt
- RV volume overload (cf other L-to-R shunt usu a/w LV overload)
Signs: RV volume overload (parasternal heave) Wide, fixed splitting of S2 (characteristic) ESM at LUSB due to ↑PV flow MDM at LLSB due to ↑TV flow
Causes of heart failure in older children
1) Myocardial disease (acquired):
Myocarditis (usually viral)
Cardiomyopathy (primary/ secondary) (mutation of sarcomeric proteins, e.g. actin, myosin, troponin, etc.)
2) Unoperated structural heart defects
3) Certain repaired/ palliated congenital heart defects (ventricular dysfunction +/- valvular insufficiencies)
General management options for paediatric heart failure
1) Identify the cause and precipitating factors, e.g.
Surgical intervention (e.g. VSD, large ASD)
Catheter intervention (e.g. large ASD)
Tackle precipitating factors (infection, metabolic disturbance…)
2) Supportive treatment (optimize nutrition)
3) Medical therapy
4) Mechanical circulatory support:
a) Extracorporeal membrane oxygenation:
b) Ventricular assist device
5) Heart transplantation (e.g. terminal dilated cardiomyopathy)
Medical therapy options for paediatric heart failure
Medical therapy of heart failure:
Diuretics (corrects preload because HF activates RAAS for fluid retention)
ACEI (symptoms)
Carvedilol (= beta blockers for dilated cardiomyopathies)
Digoxin
Indications for Mechanical circulatory support in paediatric heart failure
Extracorporeal membrane oxygenation:
- Viral myocarditis
- Terminal dilated cardiomyopathy (bridge to heart transplant)
Ventricular assist device
- Bridge to heart transplant
Treatment for duct-dependent systemic circulation in infants e.g. coarctation of aorta, interrupted aortic arch, PDA
1) Initial stabilization by prostaglandin E1/ E2
- maintain ductal patency/ open stenosed arterial ducts to ensure adequate blood flow to systemic circulation
2) Corrective surgery/ catheter intervention (balloon dilation)
Treatment of paediatric heart conditions with severe left to right shunts
Surgical closure of septum defects:
VSD (patch repair/ catheter delivery to occlude the defect)
Large PDA (ductal ligation (main choice)/ device closure)
ASD (device closure)
