paediatric cardiology pointers to pitfalls · rik de decker red cross children’s hospital...

36
Rik De Decker Red Cross Children’s Hospital PAEDIATRIC CARDIOLOGY pointers to pitfalls

Upload: dodiep

Post on 20-Jul-2018

223 views

Category:

Documents


0 download

TRANSCRIPT

Rik De DeckerRed Cross Children’s Hospital

PAEDIATRIC CARDIOLOGYpointers to pitfalls

QUESTION 5:

Bapendile is a 12-year-old girl referred to you from a local clinic with a heart murmur and tachycardia. You are the

attending medical officer at the hospital. Two years earlier (in 2013) she was diagnosed as having Sydenham’s chorea.

Her heart was normal at the time.

a) Define chorea.(2)

b) Which part of the brain is affected in chorea? (1)

c) List one of the three cardinal signs of Sydenham’s chorea (other than chorea). (1)

d) What was the most likely cause of the Sydenham’s chorea two years ago? (1)

e) What treatment should have been prescribed then (in 2013) and why? (4)

f) For how long should this treatment have been given and why? (4)

g) Suggest a good and sensitive marker for monitoring the response of Sydenham’s chorea to treatment. (1)

On examining Bapendile, you identify a tachycardia, cardiomegaly, hepatomegaly, a 3/6 pansystolic murmur at the

apex radiating to the left axilla, and a low pitched 1/4 diastolic murmur.

h) What cardiac lesion(s) does she have? (1)

i) How severe is the cardiac lesion? Explain. (4)

j) List two abnormal features that a chest x-ray is likely to show. (2)

k) What treatment should you start during this acute illness, and for how long? (4)

l) What will you do to ensure that this illness does not recur, i.e. what is your follow up plan? (4)

Later in the year Bapendile’s mother dies. Bapendile therefore moves in with an aunt who has no knowledge of

Bapendile’s medical history. Bapendile appears quite well to her aunt, so her treatment is discontinued. Seven months

later, she arrives at the hospital complaining of fever and joint pain.

m) What is the likely diagnosis now? (1)

n) Why has this illness occurred? (2)

o) Outline important issues that you will have to deal with in managing her now (as an adolescent patient). (5)

Following discharge, and a period of relative stability with good penicillin adherence, Bapendile’s condition deteriorates acutely once again.

p) Give THREE possible causes?

Examination for the Diploma of Child Health of the

College of Paediatricians of South Africa

25 August 2015

Spare a thought for the MO!• The MO is often the first port of call

• He may not have CXR and ECG immediately available

• He certainly does not have echocardiography on tap!

• Yet his clinical decisions may mean life or death to the patient

CME NOV/DEC

2011http://www.cmej.org.z

a

Scenarios

Neonates • haemodynamics

• cyanosis

• shock

Infants• acute heart failure

• post-op cyanosis

Children• tamponade

• syncope

• seizures

• warfarin

• severe cyanosis

Neonates

• You examine a newborn baby on day 2, before discharge from the nursery

• She gets a clean bill of health, and the happy family takes their new baby home

• At the routine 6-week check-up, you notice that she is failing to thrive, and that she is tachypnoeic and tachycardic

• You hear a loud ESM at the LSB and an MDM at the apex

• Your suspicion of a large VSD is confirmed on echo at RXH

• The parents now want to sue you for missing the VSD before discharge

Are they justified?

VSD

LA ENLARGEMENT

LV ENLARGEMENT

VSD

Pulmonary vascular resistance

Small: PSM only

Medium: ESM + MDM

Big: Loud P2

VSD size

Practice points

• The haemodynamics of VSDs are variable with time

• A murmur is not the only sign of heart disease

• You are asked to see a baby born earlier in the day by NVD

• His Apgars were 9 and 10

• His mom reports later that he’s not feeding well

• A nurse noted that he is dusky and his SaO2 is 40%

• You cannot hear any murmurs and his chest is clear. You think that the S2 is a bit loud.

What do you do?

TGAIVC

PDA

Rashkind atrial septostomy

Followed by:

Arterial switch OR

Mustard operation

Raises peripheral SaO2 by increasing pulmonary to systemic shunting

Practice points

• Cyanosis sneaks up on you

• Prostin restores and maintains ductal patency

• A baby is delivered by elective C/S

• Some time later, you are called to see the baby

• She is tachypnoeic, but looking alert. The SaO2 is 88% on the R thumb

• She has a soft ESM

• A blood gas reveals a metabolic acidosis

• U&E shows a rising K+ and urea and creatinine

• CXR shows mild cardiomegaly and a narrow mediastinum

• The sister comes to tell you that there has been no urine output for 3 hours

• You are concerned about neonatal septicaemia

Consider a differential diagnosis and early mx

Coarctation/Interrupted aortic arch

Practice points

• A shocked baby with bright eyes has an interruption

to systemic blood supply

• Prostin restores and maintains ductal patency

• A newborn baby is found to be moderately oedematous at birth

• Despite being quite distressed, her heart rate is only 44bpm

• She is pink and has a large hepar

• The parents say that everyone in the family is healthy

• Her ABG reveals a severe metabolic acidosis

Now what?

atrial rate = 110 bpm

ventricular rate = 36 bpm

Congenital heart blockPractice point

• Bradycardia can cause severe cardiac failure and

shock

Infants

• A 9 month old boy is brought to your rooms by his mother, who has been told he has asthma

• He’d previously been growing well, but has had a mild URTI over the past 2 days

• Now he is distressed, tachypnoeic and miserable

• A CXR shows a large heart and some diffuse parenchymal infiltrates

• He has a bilateral wheeze, and an 8cm hepatomegaly

• There are no murmurs, the heart sounds are soft, and you think you can hear a gallop rhythm

You reach for the nebulisers, then think again…

Acute cardiomyopathy

q waves!

Anomalous Left Coronary Artery from the Pulmonary Artery

(ALCAPA)

An 18mo old girl with a VSD.

What is happening?

• You are a GP in George

• An 11 month old boy is brought to your rooms

• He is tachypnoeic, but more concerning is his deep cyanosis

• His mother says that his face has been swollen for 3 days since his gastroenteritis started

• He has an old left lateral thoracotomy scar and a fresher median sternotomy scar

• You hear a short ESM, but little else

• His mother gives you a tatty discharge letter from Ward E1 RXH. You make out something like a Glenn shunt done 6 weeks previously

How do you make sense of all this?

Glenn shunt

Glenn shunt

Practice points

• Understand the possible late complications of

congenital cardiac surgery

• If in doubt, phone and ask a cardiologist!

Children

• It is a quiet Monday afternoon in casualty until an ambulance arrives from Knysna with a 7yr old boy, promised that am

• He is alert, but pyrexial, distressed and wasted

• His mother says that he has been “moeg” for 3 days, but has had a cough for a while

• Your quick examination reveals a weird arrythmia, a huge tender hepar, and a Mantoux, done that am, already reactive

• You hear no murmurs, and the apex is tricky to feel

• While you wait for the CXR, you decide to put up an IV line and take bloods

• As you do that, he slumps back and the tracing on the ECG monitor shows asystole

CARDIAC ARREST! What will you do next?

Pericardial tamponade

Practice point

• Not all funny pulse rhythms are arrhythmias!

• You are proud of your rooms just off the beach at Jeffries Bay

• Late one Wednesday afternoon, a beach life saver runs in with a toddler in his arms

• The boy and his dad were playing in the shallows, but then the little chap ran up towards where his mom was sitting on the beach.

• On the way, the boy collapses, then gets up but immediately collapses again and lies motionless in the sand

• A life saver witnesses all this and rushes over to help

• The boy is hardly breathing, and the life saver cannot find a pulse

• After only a minute or two of CPR, the boy comes round and the life saver brings him to you

• You worry about a seizure and that the CPR was probably uncalled for -but you decide to do an ECG anyway

The ECG looks a bit funny

Long QT syndrome

Practice point

• Not all syncope is neurological!

• A 7 yr old girl is referred to you in your rooms in St Elsewhere Hospital

• She is pyrexial, and has been so for 2 days

• She complains of general aches and pains

• She looks pale, but is pink and not clubbed

• She has a tachycardia and a 3/6 PSM, and the heart is a bit enlarged

• She has mild hepatosplenomegaly and haematuria

• Soon after you have examined her, she has a left sided focal seizure, lasting 3 minutes

What’s going on, and what will you do?

Endocarditis

Practice point

• Not all seizures are neurological!

• You are doing a locum night call in Paarl hospital’s emergency unit

• A 12 yr old boy is brought in by ambulance in severe respiratory distress, and you decide to intubate and ventilate him immediately. On intubation there are copious blood stained secretions from the ETT

• When the excitement is over, you recall that you had referred the boy to RXH 3 months earlier with severe rheumatic mitral valve disease, but you notice that he now has a new median sternotomy scar

• On examination he has a tachycardia, ice cold peripheries, a massive hepatomegaly, a loud apical PSM, and strange heart sounds

• His mother says that he is ”baie stout” and refuses to take his daily pink pill!

What’s gone wrong?

THROMBOSIS PREVENTING

VALVE LEAFLET MOBILITY

Practice points

• Understand the complications of valvular heart

disease

• Warfarin is a dangerous drug!

• You are called to see a 7 month old boy in ward B2 on a Saturday afternoon

• He is crying, tachypnoeic, distressed and markedly cyanosed

• He arrived late that morning from med reg, where he was pyrexial, and thought to have pneumonia and ?Tet

• The cardiologists have not seen him yet

• You cannot hear any murmurs, but the 2nd sound is single and not loud

• While you wonder what to do next, his SaO2 drops to 53%

What to do next?

TETRALOGY OF FALLOT

CYANOSIS

ACIDOSIS

Tachypnoea

NO ejection systolic murmur

Death

POSITIVE FEEDBACK

In conclusion…

• Neonatal haemodynamics are very variable

• A murmur is not the only sign of heart disease

• Prostin does not hurt (well, not much…)

• A shocked baby with bright eyes has an aortic

problem

• All children with respiratory distress deserve a

thorough cardiac examination

• Some causes of cardiomyopathy are surgically

correctable

• Understand the late complications of congenital

heart surgery

• Syncope may have a primary cardiac cause