Paediatric - Cardiology

Question 1

What are the four characteristic features of Tetralogy of Fallot?

Answer 1

• ventricular septal defect (VSD)
• right ventricular hypertrophy
• pulmonary stenosis - right ventricular outflow tract obstruction
• overriding aorta

Question 2

What pulse abnormality on examination is most associated with Patent Ductus Arteriosus?

Answer 2

Collapsing pulse

Question 3

What drug should be given to a neonate with Patent Ductus Arteriosus?

Answer 3

Indomethacin (inhibits prostaglandin synthesis and, in the majority of cases, closes the defect)

Question 4

In ASD, blood moves from which atrium to the other?

What syndrome = reversal of this?

Answer 4

Blood moves from the left atrium to the right atrium because the pressure in the left atrium is higher than the pressure in the right atrium.

• Eisenmenger syndrome is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic

Question 5

What is Eisenmenger syndrome?

Answer 5

Eisenmenger syndrome. This is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic

Question 6

Name one of the 3 common Atrial Septal Defects

Answer 6

• Ostium secondum, where the septum secondum fails to fully close, leaving a hole in the wall.
• Patent foramen ovale, where the foramen ovale fails to close (although this not strictly classified as an ASD).
• Ostium primum, where the septum primum fails to fully close, leaving a hole in the wall. This tends to lead to atrioventricular valve defects making it an atrioventricular septal defect.

Question 7

Name a complication of ASD’s

Answer 7

Stroke in the context of venous thromboembolism
Atrial fibrillation or atrial flutter
Pulmonary hypertension and right sided heart failure
Eisenmenger syndrome

Question 8

What murmur do ASD’s cause?

Answer 8

mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border

Question 9

Which syndromes are VSD’s associated with?

Answer 9

Down’s and Turner’s

Question 10

In VSD, blood moves from which ventricle to the other?

What syndrome = reversal of this?

Answer 10

Blood moves from the left ventricle to the right because the pressure in the left ventricle is higher than the pressure in the right ventricle.

• Eisenmenger syndrome is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic

Question 11

What murmur do VSD’s cause?

Answer 11

pan-systolic murmur more prominently heard at the left lower sternal border in the third and fourth intercostal spaces (systolic thrill)

Question 12

Name a risk factor for heart failure

Answer 12

CHD, MI, valve disease, HT, palpitations, DM, meds like NSAIDs, Alcohol use, Sleep apnea, smoking, obesity, thiamine deficiency

Question 13

What 2 proteins might be raised in heart failure?

Answer 13

BNP and NT-pro-BNP

Question 14

What are 3 typical signs of Heart Failure?

Answer 14

breathlessness (dyspnoea on exertion / Orthopnea - lying down), fatigue, ankle swelling

Question 15

Why might BNP be raised (other than HF) and why might it be reduced?

Answer 15

Raised:
Left ventricular hypertrophy.
Ischaemia.
Tachycardia.
Hypoxaemia.
Renal dysfunction (eGFR <60).
Age over 70.
Liver cirrhosis.
Sepsis.
COPD.
Diabetes.

Reduced:
Obesity.
African-Caribbean origin.
Medication - diuretics, ACE inhibitors, angiotensin-II receptor blockers, beta-blockers, mineralocorticoid receptor antagonists

Question 16

What are the 4 stages of HF by NYHA

Answer 16

Class I: no symptoms on ordinary physical activity.
Class II: slight limitation of physical activity by symptoms.
Class III: less than ordinary activity leads to symptoms.
Class IV: inability to carry out any activity without symptoms.

Question 17

Cyanotic vs Acyanotic congenital heart defects

Answer 17

Cyanotic:

• Tetralogy of Fallot.
• Transposition of the great vessels.
• Pulmonary atresia.
• Total anomalous pulmonary venous return.
• Truncus arteriosus.
• Hypoplastic left heart syndrome.
• Tricuspid valve abnormalities.

Acyanotic:

• Ventricular septal defect (VSD).
• Atrial septal defect (ASD).
• Atrioventricular septal defect.
• Patent ductus arteriosus (PDA).
• Pulmonary valve stenosis.
• Aortic valve stenosis.
• Coarctation of the aorta.

Question 18

What heart murmur is heard for atrial septal defect?

Answer 18

mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border, with a fixed split second heart sound

Question 19

Which heart defects might cause a pan-systolic murmur?

Answer 19

• Mitral regurgitation heard at the mitral area (fifth intercostal space, mid-clavicular line)
• Tricuspid regurgitation heard at the tricuspid area (fifth intercostal space, left sternal border)
• Ventricular septal defect heard at the left lower sternal border

Question 20

What heart defect is a cause of stroke in patients with a previous DVT?

Answer 20

In patients with an ASD the clot is able to travel from the right atrium to the left atrium across the ASD. This means the clot can travel to the left ventricle, aorta and up to the brain, causing a large stroke

Question 21

What procedure is used to correct a VSD?

Answer 21

transvenous catheter closure via the femoral vein or open heart surgery

Question 22

What 3 lesions can result in Eisenmenger Syndrome?

Answer 22

Atrial septal defect, Ventricular septal defect, Patent ductus arteriosus

Question 23

How might bone marrow react to Eisenmenger Syndrome / what haem condition is associated?

Answer 23

• Bone marrow will respond to low oxygen sats by producing more red blood cells and haemoglobin to increase oxygen carrying capacity of the blood.
• This leads to polycythaemia, which is a high concentration of haemoglobin in the blood. Polycythaemia gives patients a plethoric complexion

Question 24

Examination findings associated with pulmonary hypertension?

Answer 24

• Right ventricular heave: contracts forcefully against increased pressure in the lungs
• Loud P2: loud second heart sound due to forceful shutting of the pulmonary valve
• Raised JVP
• Peripheral oedema

Question 25

What is used to keep the ductus arteriosus open while awaiting surgery shortly after birth?
(+ what is used to close PDA?)

Answer 25

Prostaglandin E2
(Indometacin closes PDA)

Question 26

Risk factors for Tetralogy of Fallot?

Answer 26

• Rubella infection
• Increased age of the mother (over 40 years)
• Alcohol consumption in pregnancy
• Diabetic mother

Question 27

What is a ‘Tet spell’ and what body position may be adopted to help with symptoms?

Answer 27

• “Tet Spells” are intermittent symptomatic periods where the right to left shunt becomes temporarily worsened, precipitating a cyanotic episode (pulmonary vascular resistance increases or the systemic resistance decreases)
• children may squat when a tet spell occurs. Younger children can be positioned with their knees to their chest. Squatting increases the systemic vascular resistance. This encourages blood to enter the pulmonary vessels

Question 28

Acute management options for a tet spell

Answer 28

• Supplementary oxygen is essential in hypoxic children as hypoxia can be fatal.
• Beta blockers can relax the right ventricle and improve flow to the pulmonary vessels.
• IV fluids can increase pre-load, increasing the volume of blood flowing to the pulmonary vessels.
• Morphine can decrease respiratory drive, resulting in more effective breathing.
• Sodium bicarbonate can buffer any metabolic acidosis that occurs.
• Phenylephrine infusion can increase systemic vascular resistance.

Question 29

What is Ebstein’s anomaly, what can be seen on ECG trace and what is the diagnostic investigation?

Answer 29

• Ebstein’s anomaly is a congenital condition where the tricuspid valve is set lower in the right side of the heart causing a bigger right atrium and a smaller right ventricle (associated with right to left shunt via ASD => cyanosis)
• It is associated with Wolff-Parkinson-White syndrome (short PR, wide QRS)
• Ix = Echocardiogram to assess severity