Congenital Cardiovascular Disease

Question 1

Congenital cardiovascular defects can involve what parts of the heart?

Answer 1

Inferior walls of heart, valves inside the heart and arteries and veins that carry blood to the heart or body

Question 2

What is a rough statistic of the number of congenital heart defects?

Answer 2

About 8 out of 1000 newborns. Most common type of birth defect. Many are simple conditions that do not require treatment, some are easy to fix and others are rather complex.

Question 3

List the Non-cyanotic congenital heart diseases

Answer 3

• Ventricular septal defect, - Atrial septal defect, - Patent ductus arteriosus, - Coarctation of aorta, - Pulmonary stenosis, - Aortic stenosis - Atrioventrivcular canal, - Mild Ebstein’s anomaly

Question 4

List the cyanotic congenital heart defects

Answer 4

• Tetralogy of Fallot, - Total anomalous pulmonary venous return, - Transposition of great vessels, - Tricuspid atresia, - Truncus arteriosus (these are the 5 Ts). - Hypoplastic left heart, -Pulmonary atresia, - Severe Ebstein’s anomaly

Question 5

Draw table with the acyanotic and cyanotic defects what are with and without shunts

Answer 5

Add photo at end

Question 6

Draw diagram showing which Acyanotic and cyanotic defects cause increased/decreased pulmonary BF and which obstruct BF from ventricles, which caused mixed BF

Answer 6

Question 7

What other anomalies can occur with congenital heart defects?

Answer 7

Extracardiac anomalies which are most frequently in musculoskeletal system and associated with specific syndromes. This can increase mortality. Occurs in 25% of infants

Question 8

Name some causes of congenital heart disease

Answer 8

Drugs such as retinoic acid, chemicals, alcohol and infections (rubella). Poorly controlled blood sugar in diabetic mothers can link to increased risk.

Question 9

What are some of the signs of heart failure?

Answer 9

Cyanosis, rapid breathing, extra work of breathing and grunting, tachycardia (for newborns its above 180bpm), fatigue, faltering growth, sweating, enlarged livers and murmurs.

Question 10

What are some of the complications of congenital heart disease

Answer 10

Faltering growth, paradoxical embolus, bacterial endocarditis, pulmonary hypertension, polycythaemia (high conc of RBCs), Haemoptysis and arrhythmias.

Question 11

Describe features ventricular septal defects?

Answer 11

They can be small to large defects but small defects don’t cause as many problems and likely to close on their own. Medium sized defects are less likely to close. Large can allow a lot of blood flow from the left to right ventricles which can increase pressure in right side heart and lungs.

Question 12

What occurs with large ventricular septal defects?

Answer 12

The extra workload on the heart can cause heart failure and poor growth. If the isn’t close then the high blood pressure can scar the arteries in the lungs which lead to pulmonary hypertension

Question 13

Describe features of atrial septal defects (small, medium and large)

Answer 13

Similar to VSD, they can be small to large. Small ASD allow a small amount of blood through atria, it doesn’t effect how the heart works and mostly close on their own. Medium and large ASDs allows for more blood to pass through the atria and less likely to close on their own and need repaired using catheter or open heart surgery.

Question 14

What occurs in ASD if there is longstanding significant blood flow traveling from left to right shunt?

Answer 14

The increased blood flow through the lungs can scar the arteries which can lead to complications of pulmonary hypertension/shunt reversal and eisenmenger’s syndrome.

Question 15

What is patent ductus arteriosis?

Answer 15

Persistent communication between thoracic aorta and pulmonary artery due to failure to close dutus arteriosus

Question 16

What treatment is given for PDA?

Answer 16

IV indomethacin or ibuprofen- effective in closure of PDA if given in first 10-14 days. Other surgical options are catheter closure and surgical ligation

Question 17

What is different about foetal circulation?

Answer 17

Foetal - Mixing of oxygenated and deoxygenated blood Adult - No mixture

Question 18

What is a patent foramen ovale? and describe features of it

Answer 18

It is failed closure of the foramen ovale. Not an ASP as no IA septum missing. Can be unproblematic and about 25% of people have one and it can be functionally closed, however if the pressure in the right side of the heart increases then it can force it open. Risk of paradoxical emboli

Question 19

What is coarctation of the aorta?

Answer 19

Narrowing or constriction of the aorta which is commonly associated with bicuspid aortic valve and VDS.

Question 20

Where is the most common site for coarctation of the aorta?

Answer 20

Thoracic aorta distal to origin of left subclavian artery about level of ductal structure.

Question 21

What can occur as a result of coarctation of the aorta?

Answer 21

Imposes significant afterload on left ventricle which increases wall stresses and results in compensatory ventricular hypertrophy. This can occur acutely in neonates following closure of ductus arteriosus with severe coarctation, resulting in congestive HR and shock.

Question 22

What is the clinical presentation of coarctation of the aorta?

Answer 22

Depends of degree of stenosis so patients can be asymptomatic but often patients present with angina pectoris and leg claudication. There may be diminished femoral pulses

Question 23

Describe the treatment and prognosis of coarctation of the aorta?

Answer 23

Urgency depends of presence of congestive HR. It can be treated by primary surgical repair with excision of the coarctation and end-to-end anastamosis or balloon angioplasty.

Question 24

What are some congenital heart diseases affecting the valves?

Answer 24

Atresia - valve doesn’t form correctly and lacks hole for blood to pass through. Stenosis - Occurs if valve flaps have thickened, stiffened or fused. Regurgitation - Occurs if valve doesn’t close tightly so blood leaks

Question 25

What is pulmonary stenosis? What occurs as a result and how is it treated?

Answer 25

Narrowing of pulmonary valve that can be valvar, subvalvar or supravalvar. While it is normally mind, babies with severe cases can mean the RV gets very overworked as it tries to pump blood to pulmonary artery. It is treated with a catheter procedure

Question 26

What does aortic valve stenosis cause?

Answer 26

Severe obstruction of the left ventricular outflow which can be associated with left heart obstruction lesions, left heart hypoplasia or extracardiac malformations.

Question 27

What is the treatment for aortic valve stenosis?

Answer 27

Balloon aortic valvuoplasty is first line treatment as surgical repair or replacement of aortic valve is primarily reserved for patients where valvuoplasty failed.

Question 28

Describe features of the tetralogy of Fallot heart defect

Answer 28

Combo of four defects; Pulmonary valve stenosis, a large VSD, overriding aorta (aorta located between left and right ventricles directly over VDS meaning deoxygenated blood can flow through aorta) and right ventricular hypertrophy. Appears as boot shape on CXR

Question 29

Describe tet spells

Answer 29

Some children with unrepaired tetralogy of fallot have sudden episodes of cyanosis and hypoxia (TET SPELL). Can be triggered by things that decrease oxygen saturation or systemic vascular resistant. Children go into the knee chest position

Question 30

Describe the Palliative surgery used for treatment of TOF

Answer 30

Blalock-Taussig Shunt - Forms a side to end anastamosis with pulmonary artery, this redirects the partially oxygenated blood into the lungs increasing flow through pulmonary circuit. This greatly relieves symptoms in patients. However was not curative. Now curative surgery requires open heart surgery

Question 31

What is Hypoplastic left heart syndrome? (HLHS) When does it present?

Answer 31

Where the left ventricle doesn't develop properly so it is much smaller. It presents when the ductus arteriosus closes as this means that the aorta receives much less blood.

Question 32

How is hypoplastic left heart syndrome treated?

Answer 32

A series of surgeries or heart transplant. Newborns are put on IV prostaglandin E1 to prevent closure of ductus arteriosus until surgeries. They made need balloon septostomy after birth to widen foramen ovale if there is not sufficient blood flow.

Question 33

What is transposition of the great arteries? and explain the only way the infant can survive

Answer 33

Where the pulmonary arteries are supplied by LV and aorta supplied by RV. Infants can only survive if there is a shunt between two sides of the heart and an atrial septal defect needs to be enlarged to allow mixing of blood

Question 34

Describe what truncus arteriosus is and what is the tratment

Answer 34

Where a single vessel (truncus arteriosis) comes out for, the left and right ventricles, meaning blood mixes. The aorta and pulmonary arteries arise from this 'trunk' (ductus arteriosis). Treatment is sewing a flexible tube with its own valve from RV to PA.

Question 35

What is ebstein's anomaly?

Answer 35

Anomaly of tricuspid valve that means when the right ventricle contracts, a large amount of blood flows backwards into the RA. It may be asymptomatic but surgery can sometimes be required if the valve is very incompetent.

Question 36

Describe features of total anomalous pulmonary venous drainage

Answer 36

Where pulmonary veins are not connected to the LA. Instead they are connected to one of the main veins that drain into the RA. Means mixing of blood. Only blood reaching LA is that through a ASD. Needs surgical intervention

Question 37

Describe features of the atrioventricular septal defect

Answer 37

Holes between the chambers of the right and left sides of the heart. Valves that control BF may not be formed correctly. This is common in babies with trisomy 21

Question 38

Name the causes of sudden unexplained collapse in the first week of life

Answer 38

Duct dependant CHD, sepsis, hypoglycaemia, congenital adrenal hyperplasia and inborn errors of metabolism.

Question 39

Briefly describe eisenmenger's syndrom

Answer 39

It is caused by longstanding left to right cardiac shunt due to a defect. Patient can develop pulmonary hypertension causing pressure in right side of heart to exceed the left resulting in shunt reversal and development of cyanosis. Has poor prognosis. Supportive treatment but lung or heart transplant may be an option.