Congenital Cardiovascular Disease

Question 1

what is congenital heart disease?

Answer 1

heart disease the patient is born with

Question 2

what are the causes of of congenital heart disease?

Answer 2

• genetic defects
• chromosomal abnormalities
• intrauterine infection
• drugs
• maternal alcohol
• maternal diabetes

Question 3

what are common classes of congenital cardiovascular abnormalities?

Answer 3

failure of septation, failure of development, failure to or incorrect rotation, abnormalities of the great vessels, failure of closure

Question 4

what congenital defects result from failure to develop?

Answer 4

obstruction- tricuspid, pulmonary atresia, pulmonary stenosis
coarctation
hypoplasia- hypoplastic left heart

Question 5

what congenital abnormalities arise from failure or incorrect rotation?

Answer 5

transposition of the great arteries TGA
congenitally corrected transposition of the great vessels
dextrocardia

Question 6

what ways can congenital cardiovascular disease present?

Answer 6

incidental- lots result in heart murmurs which can be heard during auscultation
heart failure
central cyanosis
pulmonary hypertension

Question 7

what symptoms could a patient with congenital heart disease present with?

Answer 7

normal heart failure symptoms
difficulty feeding
failure to thrive 
tachypnoea 
cyanosis 
clubbing 
squatting=tetralogy of fallot 
syncope

Question 8

what congenital heart defects can a patient have if they are acyanotic and have shunts?

Answer 8

atrial or ventricular septal defects

persistent ductus arteriosus

Question 9

what congenital defects are indicated by a patient who is acyanotic and has no shunts?

Answer 9

pulmonary stenosis
aortic coarctation
aortic/left heart obstruction

Question 10

what congenital heart defects could a patient have if they are cyanotic and have shunts?

Answer 10

all with pulmonary hypertension
transposition of the great vessels
fallots tetralogy

Question 11

what congenital heart defects could a patient have is they are cyanotic and have no shunts?

Answer 11

hypo plastic left heart
severe pulmonary stenosis
pulmonary/tricuspid atresia with or without infarct septum

Question 12

what preventative measure can be taken for congenital cardiovascular disease?

Answer 12

foetal echocardiography

Question 13

what palliative treatment can be provided for congenital cardiovascular disease?

Answer 13

allow growth for definitive treatment
maintain arterial duct
PA bonding
arterial septostomy

Question 14

what are the methods of long term treatment for congenital cardiovascular disease?

Answer 14

creation of systemic to pulmonary shunt: subclavian, central pulmonary artery to aorta
-idea is constrict blood flow to pulmonary artery to reduce blood flow in through lungs

Question 15

what definitive treatment is there available for congenital cardiovascular disease?

Answer 15

radical correction of TGA, tetralogy of fallot

percutaneous closure devices for: persistent ductus arteriosus, atrial and ventricular septal defects

Question 16

what transplants can be carried out to treat congenital cardiovascular disease?

Answer 16

heart

heart and lungs for conditions complicated by pulmonary hypertension

Question 17

what are the functional consequences of pulmonary hypertension? how does it arise?

Answer 17

• it makes radical correction pointless or dangerous, left to right shunt reversed to a right to left shunt leading to cyanosis
• arises as are ult of high flow through pulmonary bed and genetic influences

Question 18

what are the 2 types of atrial septal defects?

Answer 18

ostium secundum

ostium primum

Question 19

what are the functional complications of ostium secundum and primum ASDs?

Answer 19

secundum- partial right bundle branch block, right axis

primum- partial right BBB, left axis

Question 20

how are atrial septal defects managed?

Answer 20

nothing done

percutaneous closure

Question 21

how do patients with ASDs present? explain how patients with secundum and primum present

Answer 21

uncomplicated= heart failure or failure to thrive
secundum is usually uncomplicated
primum is complicated by other lesions= mitral valve clefts, anomalous venous drainage

Question 22

what are the functional complications of Ventricular septal defects?

Answer 22

perimembranous is most common
can range fro trivial to large when associated with other lesions
small VSDs restrict shunt to trivial levels
large shunts- have a hydrostatic murmur

Question 23

how are large shunt VSDs treated?

Answer 23

palliated until definitive treatment

Question 24

what are the functional complications of PDA? explain the complications of both small and large

Answer 24

• if larger will present as heart failure with continuous murmur and wide pulse pressure
• can cause pulmonary hypertension and only show lower body cyanosis

-small causes characteristic continuous murmur with normal pulse pressure

Question 25

how is PDA treated?

Answer 25

mainly closed percutaneously

in infancy prostaglandin inhibition may close duct

Question 26

what is coarctation of the aorta?

Answer 26

large range of narrowing from complete interruption to small low grade stenosis

Question 27

what are the functional complications of coarctation of aorta ?

Answer 27

• causes systemic hypertension in adults
• associated with intracranial aneurysms
• absent or delayed femoral pulses

Question 28

what is the treatment for coarctation of the aorta?

Answer 28

balloon dilation

surgery

Question 29

how does TGA present?

Answer 29

presents rapidly after closure of duct with blue and failing body if septum intact

Question 30

how is TGA diagnosed?

Answer 30

echo diagnosis

Question 31

what palliative treatment is available for TGA?

Answer 31

pharmacologic maintenance of arterial duct
atrial septostomy
radical switch procedure
Mustard procedure

Question 32

what are the 4 features of tetralogy of Fallot?

Answer 32

pulmonary infundibular stenosis
overriding aorta
ventricular septal defect
right ventricular hypertrophy

Question 33

what are the functional complications of tetralogy of fallot?

Answer 33

cause of cyanotic heart disease
severity determined by degree of RV outflow obstruction
squatting is characteristic since it raises systemic pressure

Question 34

what complicates tetralogy of fallot?

Answer 34

pulmonary regurgitation

if severe causes RV dilation and venous thrombosis

Question 35

what are the treatment options for tetralogy of fallot?

Answer 35

corrective surgery if possible