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Flashcards in Congenital Heart Disease Deck (30):
1

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

2

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%

3

Final changes of embryogenesis

Umbilical arteries involute - forms ligament
Ductus Venosus - Turns to ligament
Ductus Arteriosus - Turns to ligament
Foramen Ovale - Closes

4

What is a shunt?

Abnormal communication between chambers or vessels
Can be structural or functional

5

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)

6

What does a Right to Left shunt do?
What diseases are associated with it?

Cyanotic
Tetralogy (of Fallot), Transposition, Truncus, TV Artesia, TAPVR

7

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

8

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

9

ASD vs PFO
1. Prevalence
2. Size of defect
3. Shunting
4. Complications

1. Rare vs 1/3 people
2. Fenestrated or deficient fossa ovale vs Small remnant opening
3. L to R shunting vs No shunting due to back pressure
4. Pul HTN/RHF vs Paradoxical emboli, decompression sickness, migraines

10

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)

11

What is the most common congenital heart anomaly?

VSD

12

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

13

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

14

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

15

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

16

What can R-L shunts cause?
What symptoms?

Decreased pulmonary blood flow causing early cyanosis
Paradoxical emboli and decompression sickness can occur when they are not filtered by the lungs and pass directly into the systemic circulation
Symptoms include cyanosis, digital clubbing, polycythemia

17

What is Tetraology of Fallot?
What does anteriosuperior displacement of the infundibular septum lead to?
What is the clinical outcome depend on?

Most common form of cyanotic congenital HD
VSD, subpulmonary stenosis, overriding aorta, RVH
Subpulmonary stenosis

18

What is the appearance of the heart in Tetraology of Fallot?
Does the R-L shunt damage the lung? Why?
What occurs with age?
What is the treatment plan?

Boot shaped due to RVH
No, due to subpulmonary stenosis restricting pulmonary blood flow
Pulmonary outlet does not grow with child so worse with age
Surgery is required

19

What is transposition of the great arteries?
What are the sequelae?
What are the two types?

Aorta from RV, pulmonary from LV
Aorta lies anterior and to the right of the pulmonary artery, separate pulmonary and systemic circulations,RVH develops, Pulmonary HTN develops
Intact ventricular septum (65%): Unstable needs prompt surgical intervention With VSD (35%): Stable

20

What is truncus arteriosus?
What is the etiology?
What is the clinical sequelae?
What variations are there?
What genetic association?
 

Definition: Origin of aorta & pulmonary artery from truncal artery
Most have large VSD
Etiology: Developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery
Clinical sequelae: Mixing of blood and increased pulmonary blood flow & pulmonary HTN
Variations: Patterns in PA origin, truncal valve cusps
Associated with DiGeorge syndrome (field defect)

21

What is tricuspid atresia?
What causes it?
What coexisting morbidities must exist?
What complications can it lead to?

Complete occlusion of the tricuspid valve orifice
Results from unequal division of the AV canal - Mitral valve enlargement
Needs coexisting ASD/PFO and VSD
Causes right ventricular hypoplasia, symptomatic with high mortality

22

What is the total anomalous pulmonary venous return?
What is the physiological result?
What is the developmental cause?

Pulmonary veins do not drain directly into left atrium instead to left innominate vein or coronary sinus
Resulting left atrial hypoplasia, ASD/PFO allows oxygenated blood to enter psystemic circulation
Occurs when common pulmonary vein fails to develop or regress

23

What diseases are classified as obstructive?

Pulm Stenosis
Aortic Stenosis
Coarctation

24

What is aortic coarctation?
What types of aortic coarctation are there?
What is the clinical presentation?

Definition - Constricting/narrowing of the aorta

Types - Preductal/infantile - Tubular hypoplasia with PDA
Postductal/adult - Ridgelike infolding at ligament without PDA

Presents with Bicuspid AV, preductally: lower body cyanosis (requires surgery as neonate), postductally: symptoms depend on degree of narrowing, surgically treatable HTN, upper > lower, rib notching on CXR

25

What is pulmonary stenosis?
How does isolated PV stenosis present?
How does PV atresia with intact VS present?
What are long term changes?

Definition: PV obstruction due to hypoplasia, dysplasia, or abnormal number of cusps
Isolated: RV dilation and hypertrophy, post-stenotic injury to PA, may be asymptomatic until adulthood
Atresia: Hypoplastic RV and TV, PDA needed to get blood to lungs
Long term: Pulmonary artery dilation, RVH/RA dilation

26

What is aortic stenosis?
How does isolated AV stenosis present?

Definition: AV obstruction due to hypoplasia, dysplasia, oir abnormal number of cusps
Isolated: LVH, LA dilation, AS may range from mild to critical, Systolic murmur

27

What is hypoplastic left heart syndrome?
What does it present with?
What is necessary for survival?
What treatment?

Aortic valve atresia with intact VS
Hypoplastic mitral valve and left ventricle
Dependent on PDA for survival
Requires staged surgical correction

28

What is Ebstein anomaly of tricuspid valve?
What are the secondary effects?
What are the other features?

Defintion: Inferiorly displaced and adherent septal and posterior, redundant leaflet, and dilated annulus with tricuspid regurg
Secondary effects: RV and RA dilation
Other: Arrhythmias including WPW syndrome
May be asymptomatic until adulthood

29

What are surgical risks?
What are transplant risks?

Surgical: Bleeding, infection, death, stroke, long term arteriosclerosis and fibrosis
Transplant: Same, plus rejection, infection, post-transplant lymphoproliferative disease, other malignancies

30

What are the late effects of CHD?

Endocarditis
Hyperviscosity
Pulmonary HTN, shunt reversal (Eisenmeiger complex)
Childbearing risk
Residual post-surgical pathology