Congenital Heart Disease Flashcards
What is congenital heart disease?
Possible outcomes of congenital HD
CHD - Abnormalities of the heart and or great vessel present from birth
Result in stillbirth, present at birth, or remain undetected until later
When do anomalies arise?
What are the causes of conenital heart disease?
3-8 Weeks of embryogenesis
Causes:
Genetic: Trisomies 13,15,18,21; Turner (45,XO), Monogenic disorders
Environmental - Infection, etc.
Idiopathic - 90%
Final changes of embryogenesis
Umbilical arteries involute - forms ligament
Ductus Venosus - Turns to ligament
Ductus Arteriosus - Turns to ligament
Foramen Ovale - Closes
What is a shunt?
Abnormal communication between chambers or vessels
Can be structural or functional
What does a Left to Right shunt do?
What diseases are associated with it?
Late cyanotic
Atrial Septal Defect (ASD), Ventricular (VSD), Atrioventricular Septal (AVSD), Patent Ductus Arteriosus (PDA)
What does a Right to Left shunt do?
What diseases are associated with it?
Cyanotic
Tetralogy (of Fallot), Transposition, Truncus, TV Artesia, TAPVR
How does L-R cyanosis develop?
Originally no cyanosis because oxygenated blood is dumped into right side
Increases pulmonary blood flow beyond capacity
Pulmonary HTN and RVH
Switches to R-L shunt due to pressure gradient: Late cyanosis
What does an atrial septal defect cause?
Where are they typically located?
What associated pathology?
What is the likelihood of progression?
Increased pulmonary blood flow which is well tolerated by pulmonary vessels
90% - Fossa ovalis, 5% - Primum: Adjacent to AV valves, 5% - Sinus venosus: Near SVC entrance
Paradoxical embolism
Can be asymptomatic until adulthood, < 10% lead to pulmonary hypertension, eventual RVH, Dilated Atria
ASD vs PFO
- Prevalence
- Size of defect
- Shunting
- Complications
- Rare vs 1/3 people
- Fenestrated or deficient fossa ovale vs Small remnant opening
- L to R shunting vs No shunting due to back pressure
- Pul HTN/RHF vs Paradoxical emboli, decompression sickness, migraines
What is a ventricular septal defect?
What does it cause?
Where is it located?
What is it associated with?
Interventricular opening between LV and RV
Pulmonary HTN
90% at septum (membranous VSD)
Usually associated with other anomalies (only 30% in isolation)
What is the most common congenital heart anomaly?
VSD
What is the size of (peri)membranous VSD?
What can spontaneously close it?
What is the treatment plan?
Large defect
Spontaneous closure by septal TV leaflet in <10%
Requires surgical closure around 1 year of age
What is the size of muscular VSD?
What can spontaneously close it?
What is treatment plan?
What is a swiss cheese septum?
Defect is usually small
Spontaneous closure > 60% of cases by 1 yr of age due to fibrous adhesions
Most do not need surgery
Multiple muscular VSDs
When does the ductus arteriosus normally close?
What can cause it to close later?
What is the classic finding of a PDA?
How often is a PDA seen in isolation?
When is a PDA necessary for survival?
Typically close functionally 12 hours,
Structurally 3 months
Delayed by PGE, closes later in preemies and at high altitude
Harsh continuous machinery-like murmur
Usually in isolation (90%)
Necessary for survival in AV or PV atresia, others
What is an AV septal defect?
What are the two types?
What is a common association with AVSD?
What is the treatment plan?
Deficient AV septum, associated with MV and TV anomalies
Types: Partial - Primum ASD & Cleft MV with Regurg
Complete: AVSD & common AV valve
Down syndrome (40%) with complete AVS
Needs early surgical correction