SELF-ASSESSMENT
Fig
PAE
Self-assessment
Questions
Case 1
A 14-year-old boy presented to his GP with a 48 hour his-
c) Left upper lobe collapse
d) Mediastinal lymphadenopathy
e) Cardiomegaly with pulmonary oedema
f) Pneumothorax
tory of sore throat, cough and breathlessness at night.He had been born in Zimbabwe and his mother recalled
that he had been born ‘blue’ with a hole in his heart. He had
received some medication, and by three years of age the
hole had apparently closed and he had no further medical
input. Immunization history was unclear.
He was commenced on amoxicillin by the GP and a chest
X-ray performed.
His chest X-ray is shown in Figure 1.
ure 1 Chest X-ray performed in view of persistent cough.
1. What does this chest X-ray suggest? Select ONE answer
only.
a) Isolated cardiomegaly
b) Left lower lobe collapse
Nancy J Bostock BSc MBCHB MRCPCH DTM&H is an ST2 in Paediatrics at
Addenbrooke’s Hospital, Cambridge, UK. Conflict of interest: none.
Elena Cattaneo MD PhD MRCPCH is an ST6 in Paediatrics at Adden-
brooke’s Hospital, Cambridge, UK. Conflict of interest: none.
Viktoria Dixon is a Consultant Paediatrician at Hinchinbrooke Hos-
pital, Huntingdon, Cambridgeshire, UK. Conflict of interest: none.
DIATRICS AND CHILD HEALTH 23:12 553
g) Pulmonary tuberculosis
The patient was referred to a paediatric inpatient ward.
Examination revealed a loud 3e4/6 pansystolic murmur,
displaced apex beat to the mid-axillary line and a 3e4 cm
liver edge, increased jugular venous pressure with large V
waves and no peripheral oedema.
An ECG was performed and is shown in Figure 2.
2. What does this ECG show? Select ONE answer only.
a) Complete left bundle branch block with P pulmonale
b) Incomplete left bundle branch block with P mitrale.
c) Complete right bundle branch block with P pulmonale.
d) Incomplete rightbundlebranchblockwithPpulmonale.
e) Complete right bundle branch block with normal P
mitrale.
f) Incomplete right bundle branch block with P mitrale.
An urgent echo was performed and showed a grossly
enlarged right atrium and ventricle and tricuspid regurgi-
tation of 3 millilitres.
3. What is the likely diagnosis? Select ONE answer only.
a) Ebstein’s anomaly
b) Tricuspid atresia
c) Congenital tricuspid regurgitation with secondary
dilated cardiomyopathy
d) Idiopathic cardiomyopathy
e) Chagas disease
f) HIV related cardiomyopathy
Case 2
A 16-year-old girl presented to A&E with severe retrosternal
pain radiating to the left shoulder and pyrexia. She reported
a history of 3 days of back pain moving 2 days earlier to the
chest and shoulder. On examination the girl looked unwell,
pale, sweaty, with an increased work of breathing,
tachypnoea and tachycardia, BP 110/68 mmHg, saturation
in air 100%. No past medical history of note, no regular
medications. She had been seen by the GP the day before
and blood results revealed a CRP of 16 mg/litre and WCC of
11, FBC and U&E otherwise unremarkable. Repeat bloods in
A&E showed a raised CRP to 160 mg/litre.
1. Which is the investigation that you would most likely
give you the diagnosis?
a) Chest X-ray
b) ECG
c) CT chest
d) Cardiac enzymes
e) Echocardiography
� 2013 Elsevier Ltd. All rights reserved.
I
II
III
II
↓ aVR ↓ V1 ↓ V4
↓ aVL ↓ V2 ↓ V5
↓ aVF ↓ V3 ↓ V6
Figure 2
SELF-ASSESSMENT
2. What abnormalities can you identify on theECG(Figure3)?
a) Low voltage complexes
b) Prolonged PR
c) Elevated ST
d) Abnormal QRS complexes
e) Ischaemic changes
Following the findings on the ECG a chest X-ray was
performed.
3. What abnormalities can you describe in this chest X-ray
(Figure 4)?
a) Increased cardiothoracic ratio
b) Pleural effusion
c) Consolidation
d) Mediastinal mass
Answers
Case 1
1. The chest X-ray shows isolated cardiomegaly. Car-
diomegaly is defined when there is a cardiothoracic ratio
(CTR) exceeding 0.5 on a PA X-ray. This is sometimes
difficult to determine as most X-rays in the paediatric
population are taken AP. However, massive car-
diomegaly is usually obvious.
Causes of cardiomegaly include ventricular hypertrophy,
myocarditis, congenital cardiac disease, dilated cardiomy-
opathy, pericardial effusion and congestive cardiac failure.
PAEDIATRICS AND CHILD HEALTH 23:12 554
2. The ECG shows incomplete right bundle branch block
(RBBB) with P pulmonale. Right bundle branch block
may be a normal variant. It is also present in cor pul-
monale, ASD, myocarditis, pulmonary embolism, pul-
monary hypertension, pulmonary stenosis and right
ventricular hypertrophy. To diagnose RBBB on an ECG:
1) The heart rhythm must originate in the sinoatrial
node, atria or atrioventricular node.
2) The QRS duration must be:
a) Incomplete block >100 ms
b) Complete block >120 ms
1) A terminal R wave should be present in lead V1.
2) A slurred S wave is seen in leads I and V6.
3) The T wave should be deflected in the opposite
direction of the QRS complex.
A commonly used mnemonic to remember the ECG
changes in bundle branch block is WiLLiaM MaRRoW. In
left bundle branch block there is a W in V1 and M in V6 and
in RBBB there is an M in V1 and a W in V6.
A peaked P wave is seen in right atrial hypertrophy and
is called P pulmonale. A peaked P wave is also seen in
hypokalaemia where it is called pseudo P pulmonale. P
mitrale is a bifid P wave which is seen in left atrial hyper-
trophy.
3. The diagnosis for this boy is congenital Tricuspid
Regurgitation (TR) with secondary hypertrophic
� 2013 Elsevier Ltd. All rights reserved.
I
II
III
II
↓ aVR ↓ V1 ↓ V4
↓ aVL ↓ V2 ↓ V5
↓ aVF ↓ V3 ↓ V6
Figure 3
SELF-ASSESSMENT
cardiomyopathy. Surgical repair of the valve was per-
formed with good outcome.
TR is most commonly congenital in adolescents and
young people, and is often asymptomatic. It can be
Figure 4
PAEDIATRICS AND CHILD HEALTH 23:12 555
secondary to right ventricular hypertrophy of any cause (in
adults often secondary to left ventricular failure). Acquired
causes include endocarditis, rheumatic fever, SLE and
rheumatoid arthritis.
Tricuspid regurgitation is often asymptomatic, and
symptoms and signs are usually those of right ventricular
failure: raised jugular venous pressure, oedema, ascites and
hepatomegaly. Due to the relatively low pressure on the
right side of the heart, a murmur is not always present, but
a pansystolic murmur of low frequency, loudest at the
lower left sternal edge is sometimes heard. The murmur
increases with inspiration and decreases with expiration.
Definitive diagnosis is, of course, with Echo.
Treatment of the underlying cause of the left ventricular
hypertrophy (e.g. mitral stenosis repair with balloon val-
votomy) often results in improvement of degree of TR.
Severe TR causes right ventricular dilatation which in
turn may worsen TR, leading to progressive right ventric-
ular dysfunction. Medical therapy is often of limited use,
especially if the primary problem is intrinsic tricuspid valve
disease. There is limited evidence for when surgical inter-
vention is indicated, but the 2006 ACC/AHA valvular
guidelines recommend that isolated tricuspid valve surgery
is reasonable for patients with severe primary TR if symp-
tomatic, but recommend against surgery in asymptomatic
patients if pulmonary artery systolic pressure is <60 mmHg
� 2013 Elsevier Ltd. All rights reserved.
Idiopathic
Infections
C Viral: Coxsackievirus, echovirus, adenovirus, EBV, CMV,
influenza, varicella, rubella, HIV, hepatitis B, mumps, parvo-
virus B19, vaccina (smallpox vaccination)C Bacterial: Staphylococcus, Streptococcus, pneumococcus,
Haemophilus, Neisseria (gonorrhoeae or meningitidis), Chla-
mydia (psittaci or trachomatis), Legionella, tuberculosis, Sal-
monella, Lyme diseaseC MycoplasmaC Fungal: Histoplasmosis, aspergillosis, blastomycosis, coccidio-
domycosis, actinomycosis, nocardia, candidaC Parasitic: Echinococcus, amebiasis, toxoplasmosis
Radiations
Neoplasm
C Metastatic: Lung or breast cancer, Hodgkin’s disease,
leukaemia, melanomaC Primary: Rhabdomyosarcoma, teratoma, fibroma, lipoma,
leiomyoma, angiomaC Paraneoplastic
Cardiac
C Early infarctionC Late postcardiac injury syndrome
SELF-ASSESSMENT
in the presence of a normal mitral valve. Repair of the valve
is generally preferred to replacement where possible.
The following is an explanation of the differential
diagnoses:
a) Ebstein’s anomaly: Ebstein’s anomaly is a congenital
heart defect where the tricuspid valve is displaced to-
wards the apex of the right ventricle, and so a large part
of the right heart is atrialized. ECG would show large,
‘Himalayan’ P waves, 50% have Wolff-Parkinson-White
syndrome.
b) Tricuspid atresia alone would not have the signs of right
sided heart failure (cough and breathlessness).
d) In Idiopathic cardiomyopathy you would expect the
whole of the heart to be dilated, not just the right side.
e) Chagas disease is American trypanosomiasis, a parasitic
disease caused by the protozoan Trypanosoma cruzi. It
is transmitted to humans via the blood-sucking redu-
viidae bug. Chronic infection can cause many symptoms
including dilated cardiomyopathy. This is very unlikely
in this case as there is no history of the patient having
travelled to the Americas.
f) HIV related cardiomyopathy. This is unlikely in a boy
with no other symptoms or signs of HIV.
Further reading
ACC/AHA 2006. Guidelines for the management of patients
with valvular heart disease: executive summary. Circulation
2006; 114: 450e527.
Gill GV, Beeching NJ. Lecture notes on tropical medicine.
Blackwell Science Ltd, 2009.
Kumar P, ClarkM. Clinicalmedicine. Elsevier Saunders, 2012.
Schelvan C, CopemanA,Davis J, JeanesA, Young J. Paediatric
radiology for MRCPCH and FRCR. Royal Society of Medicine
Press, 2010.
C MyocarditisC Dissecting aortic aneurysmTrauma
C BluntC PenetratingC Iatrogenic
Autoimmune
C Rheumatic disease: Including lupus, rheumatoid arthritis,
vasculitis, scleroderma, mixed connective diseaseC Others: Granulomatosis with polyangiitis (Wegener’s), poly-
arteritis nodosa, sarcoidosis, inflammatory bowel disease
(Crohn’s, ulcerative colitis), Whipple’s, giant cell arteritis,
Behcet’s disease, rheumatic fever
Drugs
C Procainamide, isonazid, hydralazineC Other: Cromolyn sodium, dantrolene, methysergide, anticoag-
ulants, thrombolytics, phenytoin, penicillin, phenylbutazone,
doxorubicin
Metabolic
C HypothyroidismC UremiaC Ovarian hyperstimulation syndrome
Case 2
1. b
2. c
3. a
Differential diagnosis of acute chest pain in young adult
includes:
� Myocardial infarction
� Pulmonary embolism
� Aortic dissection
� Musculoskeletal pain
� Gastrointestinal reflex
� Non-organic cause
Thorough history and physical examination in
conjunction with selected investigations should identify
serious causes and rule out non-organic aetiology. Most of
the life threatening options can be diagnosed by ECG, and
ECG should be part of the initial evaluation of all the chil-
dren presenting with chest pain. In this case ECG showed
clear evidence of pericarditis.
Pericarditis is a rare but serious cause of chest pain in
children. Identification and treatment of pericarditis and
pericardial effusion can be lifesaving.
PAEDIATRICS AND CHILD HEALTH 23:12 556
Diagnostic signs of pericarditis are:
� Typical pleuritic chest pain (typically sharp and
improving by sitting or leaning forward)
� Pericardial friction rub (best heard over left sternal
border)
� Suggestive ECG changes (ST elevation and PR depression)
� New or worsening pericardial effusion
Aetiology of pericardial disease:
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SELF-ASSESSMENT
ECG changes in pericarditis:
Stage 1: upwardly concave ST segment elevation in all leads
(except aVR); PR segment depression or elevation
(PR segment deviates opposite to the polarity of P
wave). At stage 1, ECG changes are very similar to
those of the early depolarization.
Stage 2: diffuse ST segment elevation disappears. Flattening
of the T wave may be observed.
Stage 3: diffuse T wave negativity. At this stage ECG may
resemble extensive myocardial ischaemia.
Stage 4: ECG may become normal or T wave abnormalities
may persist.
Treatment of pericarditis is usually aspirin and/or
non-steroidal anti-inflammatory drugs (NSDAI). Treat-
ment with steroids can be considered in patients not
responding to first line treatments, although an increased
risk of recurrences has been suggested in patients treated
with steroids. Colchicine has been used with success in
case of failure of the conventional treatment or in
PAEDIATRICS AND CHILD HEALTH 23:12 557
recurrent pericarditis. Furthermore colchicine used from
the first episode appears to reduce the incidence of
recurrences.
Approximately 15e30% of patient with pericarditis have
recurrent or persistent disease, frequently presenting with
pericardial effusions. The response to aspirin or non-ste-
roidal anti-inflammatory drug can be an indication of the
risk of recurrence.
Further reading
Jindal A, Singhi S. Acute chest pain. Indian J Pediatr 2011 Oct;
78 (10): 1262e7. Epub 2011 May 4.
Ratnapalan S, Brown K, Benson L. Children presenting with
acute pericarditis to the emergency department. Pediatr
Emerg Care 2011; 27: 581.
Shabetai R. Diseases of the pericardium. In: Schlant RC,
Alexander RW et al., eds. Hurst’s the heart. 8th Edn, 1995.
� 2013 Elsevier Ltd. All rights reserved.