Congenital Heart Disease Flashcards
What are the examination features of ASD?
Recurrent chest infections
Arrhythmia as an adult (4th decade onwards)
- Ejection systolic murmur
- Upper left sternal edge
- Fixed and widely split S2
What are the investigation findings in ASD?
ECG
- Superior QRS (negative in AVF)
- Right axis deviation
- RBBB (may be normal in kids)
CXR
- Cardiomegaly
- Enlarged pulmonary arteries
- Increased pulmonary vascular markings
RBBB findings
Broad QRS > 120 ms
RSR’ pattern in V1-3 (‘M-shaped’ QRS complex)
Wide, slurred S wave in the lateral leads (I, aVL, V5-6)
Name the left to right shunts (Breathless)
ASD
VSD
PDA (as systemic pressures always > pulmonary in systole and diastole), Pulse pressure increased, collapsing or bounding pulse
What makes VSD large or small?
Relation to size of aortic valve diameter
Examination findings in small VSD
Pansystolic murmur at lower left sternal edge
Soft S2
Smaller VSD = Louder murmur
Investigation findings in small VSD
Normal CXR, ECG
Management small VSD
Watch and wait
Usually close spontaneously
Maintain good dental hygiene
Treatments of ASD/ AVSD
ASD significant enough to cause RV dilation will need closing with cardiac catheter and occlusion device
Partial AVSD will need surgical correction
Usually 3-5 years of age
Prevents arrhythmias/ failure later in life
Large VSD examination findings
Heart failure with breathlessness and faltering growth
Recurrent chest infections
Active percordium
Soft pansystolic mumur or no murmur
Apical mid-diastolic murmur
Loud P2 (soft P2 in small VSD)
Large VSD investigation findings
CXR - cardiomegaly, pulmonary oedema, increased vascular markings
ECG - bi-ventricular hypertrophy by 2 months
Large VSD management
Diuretics and Captopril to manage failure
Dietetics - increase calories
Surgery at 3-6 months of age
What are the signs of bi-ventricular hypertrophy on ECG and in which condition would you expect to see it?
Katz-Wachtel phenomenon: Tall diphasic QRS complexes (>50 mm in height) in the mid-precordial leads (leads V2, V3 or V4) typically associated with Biventricular Hypertrophy.
Seen in large VSD
What is the MOA and SE of Captopril?
ACE inhibitor
Suppresses RAAS
Reduced effect aldosterone
(Normally salt and water retention, loss of K)
(ACE - salt/ water loss, retention of K - hyperK)
SE - dry cough, GI upset, pruritis, taste impairment, hypotension
Name the right to left shunts (Blue)
Tetralogy of Fallot
Transposition of the great arteries
Presents with cyanosis in first week of life
What are the side effects of prostaglandin infusions?
Apnoea, jitteriness, seizures, flushing, vasodilation, hypotension
Explain and draw the clinical features of Tetralogy of Fallot
- Large VSD
- Overriding aorta
- (Sub) Pulmonary stenosis (infundibular septum) causing RVO
- Resulting RVH
Presentation of ToF
Antenatally diagnosed often
Murmur in first 2 months of life
May be cyanotic at this point, but tends to develop later
Tet spells (Hypercyanotic spells)
What are the findings in ToF?
ECG may be normal, progressing to RVH
CXR
Boot shaped heart (uptilting of apex) due to RVH
Pulmonary artery bay (earlier finding as no RVH yet)
Management of ToF
Will need surgical correction, usually 6 months
- Closure of VSD
- Correction right outflow tract obstruction
If very cyanosed, may need holding procedure
- balloon dilatation of right outflow tract
- Modified Blalock Taussig shunt
*shunt between right subclavian artery and pulmonary artery - increases pulmonary blood flow.
Management of Tet spell
Squatting Increases SVR/ Afterload... Increasing LA/ LV pressures.. Reverses R-L to L-R shunt... Less cyanosis, increased pulmonary flow
OR
- Morphine (venodilatation - reduced preload)
- Propranolol - increases peripheral arterial resistance and reduced the pulmonary stenosis/ infundibular spasm
- IV fluid bolus
- Bicarbonate for acidosis/ I&V to reduce metabolic demand
Explain TGA
Closed circulations
- Aorta connected to the RV
- Pulmonary artery connected to LV
Deoxygenated blood goes to body
Oxygenated blood to lungs
Requires mixing to be compatible with life
- ASD
- VSD
- PDA
Presentation of TGA
Cyanosis is predominant
- Usually day 2 when ducts close
- Usually no murmur, single S2
- May have systolic flow murmur
Investigation findings in TGA
CXR
Egg on the side
Narrow mediastinum
Increased pulmonary vascular markings
ECG
Usually normal
Management of TGA
Need to improve mixing!
- Maintain PDA
- Balloon atrial septostomy
Definitive
- Arterial switch in first few days of life
- Coronary arteries to be reconnected to new aorta
What are the common mixing defects?
Blue AND breathless
Complete AVSD Complex CHD (may be cyanotic/ failure dominant) - Tricuspid atresia*** most common - Double inlet left ventricle - Mitral atresia - Truncus arteriosus - Common arterial trunk
Draw and explain the features of AVSD
Large 5 leaflet common AV valve
Lower atrial and upper ventricular wall defect
Clinical features of AVSD?
Presents with cyanosis, heart failure 2-3 weeks of life Pulmonary hypertension Always superior axis on ECG No murmur Most often seen in T21
Management of AVSD?
Medically manage heart failure
Surgical correction at 3-6 months of age
Draw and explain tricuspid atresia
Issue - nonfunctional and small right ventricle. No tricuspid valve. Flow from RA is through an ASD, then VSD to reach the pulmonary artery.
- May have low flow to pulmonary artery - BT shunt
- May have high flow to pulmonary artery - PA banding
Describe the palliation of tricuspid atresia
Issue - small non-functioning right ventricle
relies on ASD/VSD
Left ventricle basically has to pump mixed blood to systemic circulation AND pulmonary circulation
Initially may have Blalock-Taussig shunt
- Subclavian and pulmonary artery
- basically bigger PDA
- Increases pulmonary flow
Then may have
- Glenn/ Hemi-fontan (6 months)
- Connects the SVC to the pulmonary artery - Fontan (3-5 years of age)
- Connects the IVC to the pulmonary artery
End effect - preload pressures drive all of the venous return to the pulmonary circulation passively
LEAVING the left ventricle to deal with the systemic circulation
What are the outflow tract obstructions in a well child?
These are murmurs in well children therefore usually have no symptoms. They are:
Aortic stenosis
Pulmonary stenosis
Adults type coarctation of the aorta
Clinical features and management of AS
Usually well child
If severe - reduced exercise tolerance, syncope, chest pain on exertion
If critical - would be duct dependent and present as neonate
OE: Slow rising pulse, ejection systolic murmur at upper right sternal edge, Apical ejection click, soft A2
THRILL ALWAYS PRESENT
ECG: May have LVH
OFTEN associated with Coarctation, other valve stenosis - should always be checked.
Management depends on symptoms:
- Balloon valvotomy (safer children, dangerous neos)
- Aortic valve replacement is definitive
Clinical features and management of PS
Most asymptomatic
May be duct dependent if critical
OE: Ejection systolic murmur at left upper sternal edge
Thrill may be present. Ejection click. May have heave.
ECG may show RVH
Managed by balloon dilatation when the pressure across the pulmonary valve becomes critical - about >64 mmHg
What are the outflow tract obstructions in the sick infant?
Coarctation of the aorta - most common
Interrupted aortic arch
Hypoplastic left heart
Critical AS
Presentation and management of coarctation
Sick, collapsed neonate (but well at birth)
Duct dependent
Day 2-3 of life
Absent femorals
Severe metabolic acidosis
CXR - cardimegally
ECG - normal
Prostaglandin to maintain duct. Surgical repair.
Presentation and management of hypoplastic left heart syndrome
Sickest of all collapsed neonates
Usually diagnosed antenatally
All of left heart is small
- nonfunctional left ventricle
- small or atretic mitral valve
- aortic valve atresia
- usually associated with coarctation
Sick. Metabolic acidosis. No pulses.
Management with prostaglandin
Surgical correction - Norwood
Describe the Norwood procedure and in which condition would it be indicated?
Hypoplastic Left Heart syndrome
Step 1: The Norwood procedure
- Aorta diverted from LV to RV
- Pulmonary artery detached, now pulmonary flow is through a BT shunt attached to the subclavian artery
- RV is driving both pulmonary and systemic mixed flow
Step 2: Glenn shunt, or semi-fontan
- SVC is diverted directly to the pulmonary artery
- BT shunt removed
Step 3: Fontan
- IVC is diverted directly to the pulmonary artery via a conduit. Pulmonary flow is now driven by venous return and preload pressures
Presentation and management of interrupted aortic arch
Presents as collapsed/ sick neonate Only right brachial pulse palpable Metabolic acidosis Duct dependent VSD usually present
Associated with DiGeorge syndrome
Cardiac defects and genetics of Williams syndrome?
Deletion in chromosome 7
Supravalvular aortic stenosis
Peripheral pulmonary artery stenosis
Cardiac defects and genetics of DiGeorge?
22q11.2
Truncus arteriosus
Tetralogy of Fallot
Aortic arch abnormalities
Cardiac defects and genetics of Noonans?
PTPN11
Pulmonary stenosis
Hypertrophic cardiomyopathy
Genetics and cardiac defects of Turners?
45XO
Aortic valve stenosis
Coarctation of the aorta
