Congenital Heart Disease

Question 1

Congenital Heart Disease Includes abnormalities of the heart and _______.

Answer 1

great vessels

Question 2

Congenital Heart Disease malformations- Obstruction

list three

Answer 2

Aortic stenosis and atresia
Pulmonic stenosis and atresia
Coarctation of the aorta

Question 3

Congenital Heart Disease malformations- shunts

list two

Answer 3

Left-to-right cardiac shunt

Right-to-left cardiac shunt

Question 4

A cardiac shunt is a communication between _____

Answer 4

heart chambers

Question 5

A cardiac shunt Allows blood flow in an abnormal direction, depending on _______ in the respective chambers

Answer 5

pressures

Question 6

Left-to-Right Shunts Result in _____ months to years after birth

Answer 6

cyanosis

Question 7

______ Shunts Increases right ventricular pressure resulting in right ventricular hypertrophy, eventual right heart failure

Answer 7

Left-to-Right

Question 8

How do you assess Left-to-Right Shunts?

Answer 8

Assessed with echocardiography, ultrasound, Doppler ultrasound

Question 9

_________ defects are due to a defect located at the oval fossa (remnant of the foramen ovale)

Answer 9

Atrial septal defects

Question 10

Atrial septal defects Results in blood flow from ___ to ___ atrium

Answer 10

blood flow from left to right atrium

Question 11

in Atrial septal defects, there is a _____ murmur as a result of excessive flow over the pulmonic valve

Answer 11

Systolic

Question 12

Atrial septal defects lead to heart failure with pulmonary ______, or may be well tolerated, depending on shunt size

Answer 12

hypertension

Question 13

_______ defects are Due to incomplete closure of the ventricular septum

Answer 13

Ventricular septal

Question 14

In Ventricular septal defects, Effect depends on?

Answer 14

size of defect and presence of other anomalies

Question 15

____________ eventually causes pulmonary hypertension due to high pulmonary artery pressure/flow, also right ventricular hypertrophy and right heart failure

Answer 15

Ventricular septal defects

Question 16

what kind of murmur is heard with Ventricular septal defects?

Answer 16

Pansystolic murmur

Question 17

in Atrial-ventricular septal defects, there is Incomplete closure of the entire ______, and inadequate tricuspid and mitral valves

Answer 17

AV septum

Question 18

Atrial-ventricular septal defects

are Often associated with ______

Answer 18

Down syndrome

Question 19

in Ductus arteriosus , there is A connection between the _________ and the aorta that diverts blood away from the pulmonary bed due to lower interuterine peripheral pressure

Answer 19

main pulmonary artery

Question 20

Ductus arteriosus Normally closes within ____ after birth due to increased oxygen and reduction of vasodilatory PGE2, results in increased pulmonary blood flow

Answer 20

10 – 15 hr

Question 21

Patent ductus arteriosis Eventually causes pulmonary hypertension and right heart failure due to _______

Answer 21

high pulmonary artery pressure/flow

Question 22

is Patent ductus arteriosis present after birth?

Answer 22

yes

Question 23

how does Patent ductus arteriosis present?

Answer 23

Presents with continuous harsh murmur, no cyanosis

Question 24

_______ Shunts Result in cyanosis immediately after birth due to unoxygenated blood being sent into the left atrium or ventricle

Answer 24

Right-to-Left

Question 25

how do you access Right-to-Left Shunts?

Answer 25

with echocardiography, ultrasound, Doppler ultrasound

Question 26

four features of Tetralogy of Fallot

Answer 26

1. Ventricular septal defect 2. Subpulmonary stenosis 3. Aorta overriding the ventricular septal defect (one-way valve) 4. Right ventricular hypertrophy

Question 27

Tetralogy of Fallot Severity depends on severity of __________ (greater right-to-left shunting with decreased outflow through pulmonary artery)

Answer 27

subpulmonary stenosis

Question 28

Aorta arises from the right ventricle and the pulmonary artery from the _____.

Answer 28

left ventricle

Question 29

in Right-to-Left Shunts, Aorta arises anterior to _________ (normally posterior)

Answer 29

pulmonary artery

Question 30

in Right-to-Left Shunts, Aorta arises anterior to pulmonary artery (normally posterior). this Results in separation of _____ and ______circulations

Answer 30

systemic and pulmonary circulations

Question 31

Right-to-Left Shunts are Incompatible with postnatal life unless ______ exists

Answer 31

stable shunt

Question 32

Normal physiology of foramen ovale In utero, the foramen ovale is a flap-like opening between right and left atria (right-to-left shunt) which allows ________ blood (from placenta) to flow from the right to the left atrium (for systemic oxygenation)

Answer 32

oxygenated

Question 33

At birth, the foramen ovale flap closure occurs due to:

Answer 33

1. Oxygen filling the alveoli causes the pulmonary arterioles to open (reduction in right heart pressure and pulmonary vascular resistance) 2. Increasing amount of blood returning from the pulmonary circulation raises left atrial pressure

Question 34

foramen ovale normally fully fuses by__ years of age

Answer 34

2

Question 35

the foramen ovale normally fully fuses by 2 years of age in what percentage of children?

Answer 35

70 to 75 percent of children

Question 36

Pathophysiology of Patent Foramen Ovale: Foramen ovale doesn’t fully close, allowing blood flow between ____ Flow may go from right-left or left-right

Answer 36

atria

Question 37

Patient foramen ovale found in ____ percent of individuals and frequently persists into adulthood

Answer 37

25 to 30 %

Question 38

are most patients with Patient foramen ovale symptomatic?

Answer 38

no, they are asymptomatic

Question 39

Patient foramen ovale May be associated with other congenital cardiac septal anomalies such as?

Answer 39

1. “Cryptogenic” stroke (no specific cause known, 20-40% ischemic strokes), presumably due to embolus formation 2. Migraine and vascular headache

Question 40

Obstructive Congenital Anomalies: | Obstruction often occurs at the level of ______ or great vessels

Answer 40

valves

Question 41

how are Obstructive Congenital Anomalies assessed?

Answer 41

echocardiography, ultrasound, Doppler ultrasound

Question 42

Aortic stenosis and atresia (narrowing) is Obstruction at the ______.

Answer 42

aortic valve

Question 43

Aortic stenosis and atresia May occur as _____(e.g., bicuspid aortic valve), ______ (e.g., subpulmonary stenosis), or _____.

Answer 43

May occur as valvular (e.g., bicuspid aortic valve), subvalvular (e.g., subpulmonary stenosis), or supravalvular

Question 44

If Aortic stenosis and/or atresia is severe, it may lead to hypoplasia of LV and ascending aorta; if less severe may result in ____

Answer 44

LVH

Question 45

how is the murmur in Aortic stenosis and atresia?

Answer 45

Systolic murmur, sometimes a thrill

Question 46

Pulmonary stenosis and atresia: | Obstruction at the _______, degree may vary from minimal to complete (complete commonly associated with ASD)

Answer 46

pulmonary valve

Question 47

What is Coarctation of the aorta?

Answer 47

Constriction of the aortic wall resulting in decreased peripheral flow

Question 48

Coarctation of the aorta May be proximal or distal to ______.

Answer 48

ductus arteriosis

Question 49

how is the murmur with Coarctation of the aorta?

Answer 49

holosystolic murmur

Question 50

Two forms of Coarctation of the aorta

Answer 50

1. With patent ductus arteriosus – manifests early in life, high mortality, produces lower extremity cyanosis (A) 2. Without patent ductus arteriosus – Upper extremity hypertension, lower extremity hypotension, symptoms of arterial insufficiency (B)