Congenital heart defects (Cyanotic heart diseases) Flashcards
what are cyanotic heart diseases?
Tricuspid atresia, Transposition of great arteries, AVSD, Tetrallogy of fallot and eisemonger syndrome
usually R to L shunts
present with cyanosed babies Imediately-TA few hours TGA few days AVSD longer -TOF, eisenmoger
What is tricuspid atresia? and ebsteins abnormality?
tricupsid atresia - tricupsid valve (from RA to RV) is blocked -blood cant pass
only L ventricule works
Most common complex cyanotic heart disease (but many other exist)
fatal immediately if not for VSD/ASD/PDA presence (often there)
usually antenatal screened
Ebsteins abnormality
tricuspid valve not working-severe regurgitation
ASSOCIATED WITH LITHIUM use in pregnancy
Signs and Sx of tricuspid atresia and ebsteins abnormality
TA-
VERY early cyanosis (10mins)-blue, breathless, etc
O2 sats low
ESM at left sternal angle
hypoplastic L heart
present like TOF but TOF is at 1-6months
MUST HAVE VSD/ASD to allow mixing (its always there) if not baby would be dead
ebsteins -less severe so not always cyanotic but can be
split 1st/2nd heart sound, cardiomegaly
Ix and management of tricuspid atresia
Ix -echocardiogram to confirm but mainly clinical
MUST HAVE VSD/ASD to allow mixing (its always there) if not baby would be dead
ecg-Left axis deviation with left ventricular dominance is seen in tricuspid atresia
Mx -set of surgeries
1st -maintain Ductus arteriosus with artificial one -
Blalock tussvig shunt -between subclavian and pulm arteries
Pulm artery banding to reduce blood flow if SOB
IMPORTANT -complete corrective surgery often impossible because only 1 ventricule (L) worsks
2nd-glenn -connect SVC to pulm artery
3rd -fontan-IVC to pulm artery
Ix and management of Ebsteins abnormality
echo to confirm
XRAY -SEVERE CARDIOMEGALY
prostaglandins to maintain PDA
surgical repair of Tricupsid valve
what is transposition of the great arteries
2 arteries coming out of the heart are switched -aorta and pulmonary
aorta connects to RV-and connects deoxygenated blood to body
Pulm connects to LV and brings oxygneated blood to lungs
fatal immediately if not for VSD/ASD/PDA presence (often there)
M>F
diabetic moms
Signs and Sx of Transposition of the great arteries
cyanosis within a few hours of birth (not few mins like TA) -within 24h
sometimes without SOB
Loud S2 but NO MURMUR
Ix and Mx of Transposition of the great arteries
CXR Narrow upper mediastinum causing heart to look like "egg on string" Increased lung markings Abg-Low o2 echo-definitive
Mx-immediate prostaglandin infusion for PDA patency
Ballon atrial sepoplasty-use baloon to tear atrial walls to allow mixing
surgical fixing at 3m/o
what is AVSD -RF
Atrioventricular septal defect
. In AVSD, the two valves are combined, resulting in a common atrioventricular opening -all ventricules and atria are connected
very associated with DOWNS syndrome–
Signs and Sx of AVSD, Ix and Mx
cyanotic around 3m/o- NO murmur
often found on routine echo after downs diagnosis
Mx -HF management (medical)-diuretics n stuff
then repair at 3m/o with surgery
What is Tetralogy of fallot? and RF
congenital malformation with 4 key characteristics - VSD
Overidding aorta, Outflow obstruction from R ventricule, Secondary R Hypertrophy
Commonest SEVERE Cyanotic CHD -
can present anytime
RF -
Trisomy
Di George
Fhx
Signs and Sx of Tetralogy of Fallot
Can present anytime -most commonly early days
Classic – Hypercyanotic episodes (babies turns blue after crying) associated with HARSH ESM at Lowersternal L border (from Pulm stenosis)
Clubbing
can have sublte cyanosis
tachypneoae
O2 sats low
Ix and Mx of tetralogy of fallot
Ix - o2 low
CXR -small heart, Boot shaped heart -but normal doesnt rule out
ECG -RVH
Echo -see the defects (VSD, aorta, RVH etc)
Mx -medical first then surgical 6 months later
Keep PDA open -prostaglandins
+ reverse severe cyanosis:
if mild -self limit in 15 mins
if severe/prolonged -morphine (pain), propanalol, Fluids+bicard (acidosis), msucle paralysis and ventilation
+ BT shunt from subclavian to pulm OR RV ballon dilation
then surgery 6months later
Complications and prognosis of tetralogy of fallot
mainly heart failure and other heart issues
untreated average -12 years
treated -still get issues, but live a lot longer
What is eisenmenger syndrome?
After a while of L->R shunts (ASD, VSD, PDA), thickened arteries cause pulmonary HTN develops causing reversal of the shunt -> become a cyanotic heart disease around 15 y/o (cyanotic teen-esp extremities
these people die at 40 from R heart failure
Mx-
early intervention for flow
identify defects early
Heart transplants
