Childhood Cardiac Conditions

Lydia Burland

By the end of the session you should; Recognise common heart murmurs present in

childhood

Be able to list the risk factors for cardiac disease in childhood

Be able to define innocent murmurs and explain to parents

Be able to answer exam-based questions

Learning Outcomes

A 5 year old girl attends A+E with a 2 day history of watery eyes, cough and runny nose

She is also pulling at her left ear intermittently and is off her food and drinks

She has no relevant medical history, though there is a family history of epilepsy

Case 1

Observations: HR 124, RR 28, Sats 97%, T 37.9

On examination:Red, watery eyes and coryzal, inflamed left TM HS I + II + systolic murmurChest: good AE with transmitted sounds and mild wheeze bilaterallyAbdo: SNT, no masses or organomegaly

What do you think about the observations?What else would you want to ask/examine?

Case 1

Murmur loudest at the upper left sternal edge, no radiation, thrills or heaves

Brachial and femoral pulses present, with good volume

No other stigmata of cardiac disease

What are your differential diagnoses?Do you want any further investigations or F/U?

Case 1

Innocent murmurs are common in childhood

They are;SystolicSoft (or musical)Localised with no radiationAlter with changes in position and respiration

As there is no underlying cardiac abnormality there are no other associated symptoms

Innocent Murmurs

Flow murmur: HR and blood flow within the heart increase in response to

increased oxygen demand Turbulent blood flow results in an audible murmur Loudest at the left sternal edge

Venous hum: Around 20% of cardiac output flows to the brain, which in

turn drains into the internal jugular veins The flow of blood results in ‘vibration’ of the vessel walls,

resulting in an audible ‘hum’ Loudest beneath the clavicle, and obliterated on lying flat

Innocent Murmurs

No investigation is needed in a well child with otherwise normal examination/observations

Follow up should be arranged in 6-8 weeks when the child is well to review the murmur

If still present reassure parents

Echo if any doubt regarding murmur/red flags

Innocent Murmurs

A 6 month old attends paediatric outpatients with failure to thrive and recurrent LRTIs

He was born on the 50th centile, and now sits below the 2nd

His intake is adequate for his age and he is otherwise developing normally

Case 2

On examination: Bright, good colour and tone, mild tachypnoea

Small, but no evidence of dysmorphism

HS I + II + continuous murmur loudest in the infraclavicular area

Bounding brachial and femoral pulses

Chest: good air entry with no added sounds

Abdo: SNT, 2cm liver edge palpable

Case 2

In utero the ductus allows diversion of blood away from the lungs (pulmonary artery to aorta)

It usually closes on day 1-2 of life, and disappears by week 3

Risk factors:Female sex Downs syndromeCongenital rubella Maternal valproate exposurePrematurity

Patent Ductus Arteriosus

Small PDAs are usually asymptomatic

Large PDAs present with failure to thrive and recurrent LRTIs in childhood

Continuous ‘machinery murmur’ in the infraclavicular area or upper left sternal edge

Associated systolic thrill and bounding pulses

Echo confirms diagnosis and shunt size

Patent Ductus Arteriosus

Symptomatic patients: Preterm: ibuprofen or indometacin

Diuretics for heart failure

Surgical ligation

Asymptomatic patients: Regular echo review and catheter closure if still

patent at 1 year

Patent Ductus Arteriosus

A 2 year old girl is referred to paediatric outpatients with a heart murmur

It was found on routine examination by her GP

She is otherwise well and developing normally

Mum’s only concerns is that she is much shorter than her nursery friends and siblings

Case 3

On examination:Short, with low set earsPink and well perfused, CRT <2 secsHS I + II + systolic murmur loudest in L infraclavicular

area and radiating into the backFemoral pulses are present, but weak

Is there anything else you want to check?What is the most likely diagnosis?

Case 3

Narrowing of the aortic arch

Usually distal to left subclavian artery, near the ductus arteriosus

Results in proximal hypertension, ventricular hypertrophy and eventually heart failure

Risk factors:Males Positive family historyTurner’s syndrome

Aortic Coarctation

Investigation includes:CXR ECG MRIEcho U+E +/- cardiac catheter

Management depends on presentation:Critical stenosis in neonates – prostaglandinHeart failure – diureticsHypertension – anti-hypertensives

Definitive management is surgical

Aortic Coarctation

A 6 week old boy is referred with poor feeding, failure to thrive and increased WOB

Mum did not attend antenatal clinics, but reports no pregnancy problems other than her ‘age’ (42)

He was born by normal vaginal delivery, did not require resuscitation and has been well since

There is no family history of note

Case 4

On examination:Pink and active, mild hypotonia and low set earsCRT <2 secs, RR 62, sats 95%, pulse normalHS I + II + pansystolic murmur at lower LSELeft parasternal heave, no thrillsChest clear, abdo SNT

What are your differential diagnoses?

Case 4

Most common form of congenital heart disease

One or more defects in the interventricular septum

Most VSDs occur in the perimembranous area

Risk factors;The trisomies (13/18/21) Turners syndrome (45XO)Maternal diabetes Fetal alcohol syndrome

VSD

Presentation depends on; Size of VSD Right/left ventricular pressures Size of shunt across defect

Small: asymptomatic, murmur on examination (pansystolic, loudest at LSE)

Moderate: SOB on feeding from 5-6 weeks of life, increased WOB and poor weight gain

Large: as above, but may lead to irreversible pulmonary hypertension and cyanosis

VSD

Diagnosis confirmed on echo

Many small VSDs close spontaneously <2yrs

Management if symptomatic; Medical: diuretics and high-calorie feeds Surgical: open-heart surgery or catheter closure

VSD

Congenital (acyanotic); ASD AVSD (Downs syndrome)

Congenital (cyanotic); Tetralogy of Fallot Transposition of the Great Arteries

Acquired; Coronary artery aneuryms (Kawasaki disease) Carditis/mitral valve disease (rheumatic fever)

Other Conditions

Murmur in an asymptomatic child is most likely innocent

Innocent murmurs do not need investigating, and family should be reassured

Congenital heart disease may present with cyanosis, heart failure, feeding issues and respiratory distress

Echo is the key investigation, and acute management should follow an ABCDE approach

Key Learning Points

Practice Questions

1. The most common form of congenital heart disease is...a. Atrial septal defectb. Atrioventricular septal defectc. Ventricular septal defectd. Pulmonary stenosis

2. Which of the following presents with cyanosis?a. VSD b. ASDc. Tetralogy of fallot d. Coarctation of the aorta

MCQs

3. Which of the following are risk factors for congenital heart disease?a. Maternal diabetes in pregnancyb. Congenital rubella infectionc. Down’s syndromed. All of the above

4. Which of the following is associated with coronary artery aneurysm?a. Rheumatic fever b. Type 1 diabetes mellitusc. Kawasaki disease d. Downs syndrome

MCQs

a. Venous hum b. Flow murmurc. Patent ductus arteriosus d. VSDe. Tetralogy of fallot f. Aortic stenosis

5. A 28 weeker has had several failed attempts at extubation on NNU. On examination he has a continuous murmur in the left infraclavicular area.

6. A 5 year old presents to her GP with an URTI. She is found to have a systolic murmur at the lower LSE. She is pink and well perfused, pulses are normal and there is no other evidence of cardiac disease.

EMQs

a. Venous hum b. Flow murmurc. Patent ductus arteriosus d. VSDe. Tetralogy of fallot f. Aortic stenosis

7. A 14 year old presents with repeated collapses on exertion. There is an ejection systolic murmur at the LSE on examination.

8. A 4 year old is noted to frequently ‘squat’ when running around with friends. Her mum thinks she looks ‘blue’ sometimes when she does this.

EMQs

a. Downs syndrome b. Turners syndromec. Rheumatic fever d. Patau syndromee. Kawasaki disease f. Congenital rubella

9. A 15 year old presents with delayed puberty, short stature and a murmur radiating to her back.

10. A 11 month old is found to have a murmur. On echo he is diagnosed with an AVSD.

EMQs

a. Downs syndrome b. Turners syndromec. Rheumatic fever d. Patau syndromee. Kawasaki disease f. Congenital rubella

11. A premature infant has evidence of IUGR, microcephaly and a continuous machinery murmur.

12. A 2 year presents with 6 days of fever, red lips, cervical lymphadenopathy and a new murmur.

EMQs

13. This baby presents with cyanosis.

a. What does the image show?

b. How does it improve the cyanosis?

c. What is the underlying diagnosis?

Clinical Image

Answers

1. The most common form of congenital heart disease is...a. Atrial septal defectb. Atrioventricular septal defectc. Ventricular septal defectd. Pulmonary stenosis

2. Which of the following presents with cyanosis?a. VSD b. ASDc. Tetralogy of fallot d. Coarctation of the aorta

MCQs

1. The most common form of congenital heart disease is...a. Atrial septal defectb. Atrioventricular septal defectc. Ventricular septal defectd. Pulmonary stenosis

2. Which of the following presents with cyanosis?a. VSD b. ASDc. Tetralogy of fallot d. Coarctation of the aorta

MCQs

3. Which of the following are risk factors for congenital heart disease?a. Maternal diabetes in pregnancyb. Congenital rubella infectionc. Down’s syndromed. All of the above

4. Which of the following is associated with coronary artery aneurysm?a. Rheumatic fever b. Type 1 diabetes mellitusc. Kawasaki disease d. Downs syndrome

MCQs

3. Which of the following are risk factors for congenital heart disease?a. Maternal diabetes in pregnancyb. Congenital rubella infectionc. Down’s syndromed. All of the above

4. Which of the following is associated with coronary artery aneurysm?a. Rheumatic fever b. Type 1 diabetes mellitusc. Kawasaki disease d. Downs syndrome

MCQs

a. Venous hum b. Flow murmurc. Patent ductus arteriosus d. VSDe. Tetralogy of fallot f. Aortic stenosis

5. A 28 weeker has had several failed attempts at extubation on NNU. On examination he has a continuous murmur in the left infraclavicular area.

6. A 5 year old presents to her GP with an URTI. She is found to have a systolic murmur at the lower LSE. She is pink and well perfused, pulses are normal and there is no other evidence of cardiac disease.

EMQs

a. Venous hum b. Flow murmurc. Patent ductus arteriosus d. VSDe. Tetralogy of fallot f. Aortic stenosis

5. A 28 weeker has had several failed attempts at extubation on NNU. On examination he has a continuous murmur in the left infraclavicular area.

6. A 5 year old presents to her GP with an URTI. She is found to have a systolic murmur at the lower LSE. She is pink and well perfused, pulses are normal and there is no other evidence of cardiac disease.

EMQs

a. Venous hum b. Flow murmurc. Patent ductus arteriosus d. VSDe. Tetralogy of fallot f. Aortic stenosis

7. A 14 year old presents with repeated collapses on exertion. There is an ejection systolic murmur at the LSE on examination.

8. A 4 year old is noted to frequently ‘squat’ when running around with friends. Her mum thinks she looks ‘blue’ sometimes when she does this.

EMQs

a. Venous hum b. Flow murmurc. Patent ductus arteriosus d. VSDe. Tetralogy of fallot f. Aortic stenosis

7. A 14 year old presents with repeated collapses on exertion. There is an ejection systolic murmur at the LSE on examination.

8. A 4 year old is noted to frequently ‘squat’ when running around with friends. Her mum thinks she looks ‘blue’ sometimes when she does this.

EMQs

a. Downs syndrome b. Turners syndromec. Rheumatic fever d. Patau syndromee. Kawasaki disease f. Congenital rubella

9. A 15 year old presents with delayed puberty, short stature and a murmur radiating to her back.

10. A 11 month old is found to have a murmur. On echo he is diagnosed with an AVSD.

EMQs

a. Downs syndrome b. Turners syndromec. Rheumatic fever d. Patau syndromee. Kawasaki disease f. Congenital rubella

9. A 15 year old presents with delayed puberty, short stature and a murmur radiating to her back.

10. A 11 month old is found to have a murmur. On echo he is diagnosed with an AVSD.

EMQs

a. Downs syndrome b. Turners syndromec. Rheumatic fever d. Patau syndromee. Kawasaki disease f. Congenital rubella

11. A premature infant has evidence of IUGR, microcephaly and a continuous machinery murmur.

12. A 2 year presents with 6 days of fever, red lips, cervical lymphadenopathy and a new murmur.

EMQs

a. Downs syndrome b. Turners syndromec. Rheumatic fever d. Patau syndromee. Kawasaki disease f. Congenital rubella

11. A premature infant has evidence of IUGR, microcephaly and a continuous machinery murmur.

12. A 2 year presents with 6 days of fever, red lips, cervical lymphadenopathy and a new murmur.

EMQs

13. This baby presents with cyanosis.

a. What does the image show? Child being placed in knee-to-chest position

b. How does it improve the cyanosis? Increases venous return to the heart

c. What is the underlying diagnosis? Tetralogy of fallot (tet spell)

Clinical Image

Get Ahead! Specialities

Masterpass SBAs and EMQs in Paediatrics for Medical Students

Masterpass SBAs and EMQs in Obstetrics and Gynaecology for Medical Students

Pastest OSCEs for Medical Students Vol 1/2/3

Macleod’s Clinical OSCEs (available May 15th)

Exam Resources

Any Questions?