Congenital heart diseases Flashcards
Increased PBF clinical presentation
Failure to thrive Chest deformity Cyanotic/acyanotic Shortness of breath Sweating Poor feeding CCF Cardiomegaly Congest
Cyanotic
Increased PBF
TGA Persistent truncus arteriosus TAPVD HLHS Eisenmenger syndrome
Cyanotic
Decreased PBF
TOF Tricuspid atresia Pulmonary atresia Critical pulmonary stenosis Ebstein's anomaly
Acyanotic
Increased PBF
LR shunt ASD VSD AVSD PDA
Acyanotic
Normal PBF
Aortic stenosis Coarctation Pulmonary stenosis Tricuspid regurgitation Mitral regurgitation
Aetiology
Diabetes Rubella SLE Warfarin Alcohol Chromosomal Genetic syndromes
What is the clinical presentation of an acyanotic normal pulmonary blood flow congenital heart lesion?
Asx
Ventricle hypertrophy (displaced apex, LPH)
Low CO
What is the clinical presentation of an acyanotic increased pulmonary blood flow congenital heart lesion?
Pulmonary oedema Chest deformities Cardiomegaly CCF PHT FTT
What is the clinical presentation of a cyanotic increased pulmonary blood flow congenital heart lesion?
Pulmonary oedema Chest deformities Cardiomegaly CCF PHT FTT
What is the clinical presentation of a cyanotic decreased pulmonary blood flow congenital heart lesion?
Severe cyanosis
Stunting
When will cyanosis become apparent?
Sats <85%
Name the causes of cyanosis in an infant
- Airway
- choanal atresia
- laryngomalacia
- vocal cord paralysis
- tracheal stenosis
- vascular ring
- external mass - Breathing
- pneumonia
- bronchiolitis
- congenital lung abnormality
- congenital diaphragmatic hernia - Circulation
- O2 carrying
- congenital cardiac lesions
- PPHN
Which syndrome is associated with Ebstein anomaly?
Wolff Parkinson White (supraventricular tachycardia)
What is the optimal gestational age for screening cardiac defects?
18-22w
When should urgent echo be performed in a child 1-2 days old?
R hand or legs <90% sats
OR
R hand or legs 90-95%/3% difference on 3 different occasions
Ebstein’s anomaly is linked with which drug?
Lithium
What are the features of Ebstein’s anomaly?
Tricuspid regurg
RV atrialization (hypertrophy)
Assoc w/ PFO, ASD
PSM at LLSB
Which congenital heart defect improves with age?
Ebstein’s anomaly (pulmonary pressure decreases)
What are the features of tricuspid atresia?
Absent tricuspid valve
RV hypoplasia
Assoc w ASD, VSD
RA dilation
PSM at LLSB
Left axis deviation (LVH)
Jugular venous distention with a prominent A wave
What are the features of persistent truncus arteriosus?
VSD
Single S2
PSM at LLSB w/wo MDM
Which syndrome is associated with persistent truncus arteriosus?
DiGeorge Syndrome
What are the features of ToF?
Pulmonary infindibular stenosis
Overriding aorta
VSD
RVH
ESM LLSB
Which syndromes are associated with ToF?
DiGeorge syndrome
Down’s syndrome
How do you manage a hypercyanotic spell?
Knee chest position 100% Oxygen (usually has minimal effect) Morphine 0.1 mg/kg - calm child - relax infindibulum Fluid bolus 10 - 20 ml/kg crystaloid /colloid - incr preload, incr SVR and LV pressure Sodium Bicarb - severe acidosis B blocker (esmolol 0.5 mg/kg stat, then 0.1 mg/kg/min) - incr CO Phenylephrine - incr SVR Emergency surgery
What are the features of pulmonary atresia?
PDA
ASD
VSD
How do you manage all PDA-dependent lesions?
PGE1 IVI
PGE2 orally
Treat metabolic acidosis
Refer
At what age are cyanotic incr PBF lesions common?
<3-6mo
What are the features of transposition of the great vessels?
PFO, ASD
PDA
VSD
What is an intrauterine risk factor for transposition of the great vessels?
Infant to diabetic mother
What are the features of TAPVD?
ASD
PDA
RVH
What syndromes are associated with HLHS?
Trisomy 13
Trisomy 18
Jacobsen syndrome
Turner syndrome
What are the features of HLHS?
ASD
PDA
Name complications of cyanosis
Polycythaemia
CVS events
Clotting risk
Bleeding risk
What is the most common congenital heart defect?
VSD
What murmur is caused by a VSD?
Pansystolic at LLSB
-> pressure LV > RV in systole, no difference in diastole
What changes occur with a VSD?
Left heart dilation -> PA pressure rises -> RH hypertrophy -> PA pressure rises higher -> RH hypertrophy increases -> PA pressure rises -> pressures balance and PSM disappears -> shunt reverses (Eisenmenger syndrome)
How do you manage a VSD?
Manage CCF
IE prophylaxis
Closure - surgery/cath lab
What murmur is caused by a PDA?
Continuous murmur at ULSB
-> pressure during both systole and diastole AO > PA
What is the importance of Eisenmenger syndrome in PDA?
Cyanotic only in feet!
Aortic arch perfuses upper body
How do you manage a PDA?
Indomethacin/aspirin
Manage CCF
IE prophylaxis
Closure - surgery/cath lab
What murmur is heard in an AVSD?
None (big defects balance quickly)
OR
Pansystolic (TR LLSB/MR Apex)
What is the importance of Eisenmenger syndrome in AVSD?
Double shunt -> happens much faster
How do you manage an AVSD?
Manage CCF
IE prophylaxis
Closure - surgery/cath lab
What murmur is heard in an ASD?
Ejection systolic ULSB
What changes occur with an ASD?
Right heart dilation -> RV dilates
How do you manage an ASD?
Manage CCF
Watch for arrythmias
Closure - surgery if large/cath lab
What are features of an aortic stenosis?
Reduced CO -> syncope
PDA dependent if tight
LVH
What are features of coarctation?
Reduced/absent lower limb pulse, BP, sats
PDA dependent if tight
LVH
Hypertension
What murmur is heard in mitral regurgitation?
Pansystolic at apex
What are features of a pulmonary stenosis?
RVH
PDA-dependent if tight
Soft S2
What murmur is heard in an aortic stenosis?
Ejection systolic at RUSB
What murmur is heard in a pulmonary stenosis?
Ejection systolic at LUSB
What murmur is heard in tricuspid regurgitation?
Pansystolic at LLSB
What is a sign of tricuspid regurgitation on examination?
Pulsatile liver
Which syndrome is associated with AVSD?
Down’s syndrome
What arrhythmia is associated with ASD?
Atrial fibrillation
Which axis does conduction in neonates favour?
Right axis
What are the features of a tricuspid atresia?
ASD
VSD - PDA if no VSD
Single 1st heart sound
Left axis deviation on ECG
What is the differential diagnosis for a left axis deviation on ecg in a congenital defect?
Cyanotic = tricuspid atresia Acyanotic = AVSD
Which cardiac defect causes a “wall to wall heart” on CXR?
Ebstein’s anomaly
PA without VSD
What murmur is heard in a ToF?
EJM at LUSB
You don’t hear the VSD murmur because PS -> high RV pressure -> pressures are balanced -> no flow over the VSD
What are the clinical features of hypercyanotic spells?
Acidotic breathing Hyperinflated Cyanosis Limpness Convulsions
How can pulmonary atresia appear on CXR?
No VSD = wall to wall heart
VSD = boot shaped heart
What kind of congenital heart defect is a ToF?
Cyanotic, decr PBF
What kind of congenital heart defect is a persistent truncus?
Cyanotic incr PBF
What kind of congenital heart defect is a pulmonary atresia?
Cyanotic, decr PBF
What kind of congenital heart defect is an aortic stenosis?
Acyanotic, normal PBF
What kind of congenital heart defect is a PDA?
Acyanotic, incr PBF
What kind of congenital heart defect is a TGA?
Cyanotic, incr PBF
What kind of congenital heart defect is a TAPVD?
Cyanotic, incr PBF
What kind of congenital heart defect is a pulmonary stenosis?
Acyanotic, normal PBF
What kind of congenital heart defect is a mitral regurg?
Acyanotic, normal PBF
What kind of congenital heart defect is a VSD?
Acyanotic, incr PBF
What kind of congenital heart defect is a tricuspid atresia?
Cyanotic, decr PBF
What kind of congenital heart defect is a critical pulmonary stenosis?
Cyanotic, decr PBF
What kind of congenital heart defect is a tricuspid regurg?
Acyanotic, normal PBF
What kind of congenital heart defect is a PDA?
Acyanotic, incr PBF
What kind of congenital heart defect is a HLHS?
Cyanotic, incr PBF
What kind of congenital heart defect is an Ebstein’s anomaly?
Cyanotic, decr PBF
What kind of congenital heart defect is a coarctation?
Acyanotic, normal PBF
What kind of congenital heart defect is an AVSD?
Acyanotic, incr PBF
What complication do children with persistent truncus arteriosus present with very quickly?
Pulmonary hypertension
What are the 3 locations in TAPVD?
Supracardiac - SVC
Infracardiac - ICV
Cardiac - RA
Which murmurs are heard in a TAPVD?
ESM LUSB (PS) MDM LLSB (TS)
What is the dose of prostin?
0.05ug/kg/min - 0.1ug/kg/min
What are common side effects of prostin?
Fever Apnoea Wheezing Diarrhoea Elevated neutrophils Transient feeding difficulties Abdominal distension Elevated K+ level Bartter syndrome
What types of polycythaemia are there?
Absolute (incr RBC mass)
- primary (rubra vera)
- secondary
Relative (decr plasma volume)
Name causes of secondary polycythaemia
CCHD
High altitudes
Tumours
Smoking
What are clinical features of polycythaemia?
Purple appearance (mucosa, lips, limbs) Dyspnoea Headache Hypertension Thromboembolism Epistaxis
At what Hct is there an exponential increase in blood viscosity?
Hct >65
Why do children with polycythaemia get thromboembolism and epistaxis?
Thromboembolism - hypercoagulable
Epistaxis - platelet clumping
What are complications of polycythaemia?
Stroke (hypercoaguable, microcytic RBC)
How do you manage unoperated CCHD to decrease polycythaemia risk?
Iron + folic acid supplementation
Partial exchange transfusion