self-assessment

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.

http://dx.doi.org/10.1016/j.paed.2013.10.002

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



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


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



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 aneurysm
Trauma

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.


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:



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


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.



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