Cyanosis
Index conditions
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Chronic pulmonary disease (COPD, fibrosis)
Acute pulmonary disease (e.g. pneumonia, bronchiolitis)
Pulmonary oedema
Cyanotic congenital heart disease.
Other causes of shunting.
Clinical Skills
 Aware of use of home O2 therapy
 Provides advise on smoking
 Able to take focussed history to elicit underlying causes
 Able to perform cardiac and respiratory examination
 Able to recognise peripheral signs of chronic lung disease
 Able to differentiate central from peripheral cyanosis
 Able to distinguish main causes
 Able to select appropriate initial investigations (CXR, blood gases, ECG, pulmonary function
tests, echocardiography lung scan)
 Able to eludidate clinical findings and investigations
 Can initiate treatment for acute ad acute cardiac failure
 Can monitor O2 therapy
Professional behaviour
 Appropriate approach to Down’s syndrome baby with congenital heart disease
Practical skills
 Can perform ECG, spirometry and PEFR
 Can take atrial blood gases
 Can explain invasive procedures (bronchoscopy, cardiac catheterisation, imaging)
 Can administer controlled oxygen therapy
 Can explain various forms of assisted ventilation
 Recognises and initiated acute management in severe cardiac and respiratory failure while
calling for expert assistance
Basic Medical Sciences
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Physiology of ventilation, perfusion and oxygenation (including function of haemoglobin and
oxygen dissociation)
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Role of erthropoietinses. Abnormal Haemoglobins. Met and Sulf – Hb
Development of the heart and circulation
Clinical Sciences
 Pathophysiology of chronic lung disease and respiratory failure
 Pathophysiology of cyanotic congenital heart disease
 Differences between central and peripheral cyanosis.
 Causes of peripheral cyanosis.
 Causes of Reynauds.
Population Health Sciences
 Epidemiology of COPD and occupational lung disease
COPD
Chronic bronchitis and emphysema
Cough and sputum for more than 3 months in 2 consecutive years
Pathology
Epidemiology
Persistent inflammation of the bronchi
(bronchitis) and damage of the smaller airways
and alveoli (emphysema). Narrowing of the
airways resulting in obstructive airflow.
3 million people in the UK suffer COPD
Affects mainly over 40s, more common with
increasing age
Men > women
Risk factors
Histology
SMOKING
Air pollution
Poor working conditions
Genetic risk (alpha-1-antitrypsin deficiency
1/100 COPD sufferers)
Airway hypersensitivity
IV drug use (“% IVDUs)
Immunodeficiency syndromes – HIV
Vasculitis syndrome
Connective tissue disorders
Bronchitis – mucous gland hyperplasia, atrophy,
squamouse metaplasia, ciliary abnormalities,
variable amounts of smooth muscle
hyperplasia, inflammation and bronchial wall
thickening.
Emphysema – focal destruction limited to the
bronchioles and central portion of the acini.
History:
Examination:
Cough – 3 months for 2 years, productive,
‘smokers cough’
Short of breath and wheeze – on exertion,
worsening over time if smoking continues
Sputum
Recurrent chest infections
Vague symptoms – weight loss, tiredness, ankle
swelling
Chest pain and coughing up blood are not
common features
SMOKER
Normal respiratory examination in early or mild
disease
Clubbing
Wheezing
Hyperinflation
Diffusely decreased breath sounds
Hyperresonance on percussion
Prolonged expiration
Coarse crackles
Obese
Distant heart sounds
Complications:
Investigation:
Cor pulmonale
Respiratory failure
Spirometry – reduced FEV1 (severity dependant)
mild:>80%, moderate 50-79%, severe 30-49%,
very severe <30% predicted
Chest x-ray – hyper inflation, thickened inflamed
scarred airways
CT
2D echocardiogram
6-minute walking distance
Electrocardiogram
Right sided heart catheterization
ABG
Alpha1-antitrypsin measurement (normal 3-
7mmol/L)
Management:
Smoking cessation
Bronchodilators
Management of inflammation – systemic and inhaled corticosteroids
Manage infection – common infections include S. Pneumonia H. Influenza and M. Catarrhalis
Management of sputum – mucolytic agents eg N-acetylcysteine
PPI for exacerbations and the common cold
Oxygen therapy and hypoxaemia
Flu vaccinations
Long term monitoring and end-of-life care
Prognosis
4th leading cause of death
Cystic Fibrosis
Most common lethal inherited disease in white people.
Disease of the exocrine gland function that involves multiple organ systems, but chiefly respiratory
infections, pancreatic enzyme insufficiency and associated complications in untreated patients.
Pathology
Epidemiology
Defects in the CFTR gene. Results in decreased
secretion of the chloride ion and increased
reabsorption of sodium and water across
epithelial cells. Results in reduced height of the
epithelial lining fluid and decreased hydration of
mucus, thicker mucus promoting infection and
inflammation.
Increase in viscosity of secretions in the
respiratory tract, pancreas, GI tract, sweat glands
and other exocrine tissues.
Whites of northern European origin – 1/3,200
Hispanics – 1/9,200
African Americans – 1/15,000
Asian Americans – 1/31,000
Females show greater deterioration of
pulmonary function than men causing younger
age at death
Risk factors
Histology
CFTR gene mutation (1604 mutations
recognised)
Thickened epithelial fluid lining.
Classification:
Aetiology:
CFTR gene mutation
History:
Examination:
Median age of diagnosis – 6-8months
Neonates – meconium ileus and anasarca
Infancts – wheezing, coughing, recurrent RTIs,
pneumonia. Steatorrhoea, failure to thrive
Children – pancreatic insufficiency, chronic
cough, sputum production.
Nose – rhinitis, nasal polyps
Respiratory – tachypnoea, respiratory distress,
wheezes, crackles, cough, increased anteroposterior diameter of chest, clubbing, cyanosis,
hyperresonant chest upon percussion
GI – abdo distension, hepatosplenomegaly,
rectal prolapse, dry skin (vita deficiency),
cheilosis (vitB complex deficiency)
Other – scoliosis, kyphosis, submandibular and
parotid gland swelling, aquagenic wrinkling of
the palms.
Complications:
Investigation:
Respiratory – pneumothorax, massive recurrent
or persistent haemoptysis, nasal polyps,
persistent and chronic sinusitis
GI - meconium ileus, interssusception,
gastrostomy tube placement for supplemental
feeding, rectal prolapse
Guthrie testing.
Sweat test – high chlorise
Imaging – pulmonary nodules resulting from
abscess, infiltrated with or without lobar
atelectasis, marked hyperinfiltration with
flattened domes of the diaphragm, thoracic
kyphosis, bowing of the sternum
Pulmonary function testing
Management:
Diet and exercise – normal diet with additional energy and unrestricted fat intake. High energy, high
fat diet, with additional supplemental vitamins (especially fat soluble) and minerals.
Physiotherapy
Involvement of – surgeons, otolaryngologist, endocrinologist, cardiologist, transplant team
Assisted ventilation
Pancreatic enzymes, vitamins, bronchodilators, mucolytic agents, antibiotics
May need lung transplantation
Prognosis
Median survival is 36.9 years
80% should reachg adulthood with treatment
Pneumonia
Inflammation of the lung tissue, usually more serious than bronchitis. Sometimes bronchitis and
pneumonia occur together – bronchopneumonia
Pathology
Epidemiology
Viral or bacterial infection of the lungs.
Inflammatory condition of the lungs,
particularly the alveoli.
Spread through droplets.
Most common in children less than 5 and over
75.
Risk factors
Histology
Age – young or elderly
Low immunity
Comorbidities
Pathogen invasion leased to cell death or
apoptosis, causing an inflammatory immune
response.
Classification:
Aspiration – inhalation of oropharyngeal or gastric contents
Bacterial - Strep pneumonia, pseudomonas aeruginosa.
Viral – largest proportion of aediatric pneumonias (influenza, RSV, adenovirus, parainfluenza))
Community acquired – usually mycoplasmal. Also strep pneumonia (penicillin sensitive and
resistant), H influenza, M catarrhalis (these 3 accound for 85%)
Congenital – true congenital (established at birth, transmission through haematogenous, ascending
or aspiration), intrapartum (during passage in the birth canal), postnatal (in first 24hrs after birth)
Fungal – usually endemic (eg H capsulatum, C immitis) or opportunistic (eg Candida and Aspergillus)
Chlamydial – pneuminiae (adolescents or young adults), psittaci(following bird exposure) or
trachomatis ~(cause of STDs, pneumonia in infants and young children).
History:
Examination:
Cough, fever, sweats, rigor, poor appetite,
generally unwell.
Headaches, aches and pains.
Yellow/green sputum, may be bloodstained.
May be breathless, tachypnoeaic and tight chest
pain if plauritic infection
Temperature, tachycardia, respiratory distress,
rales, signs of consolidation, pleural rub.
Extrapulminary findings may include: meningitis,
skin lesions, rheumatologic and allergic
reactions.
Complications:
Investigation:
Sepsis and shock
Bloods Imaging –
Cultures – blood and sputum
Thoracentesis
Management:
Thoracentesis
Oxygen and ventilation
Antibiotics
Corticosteroid therapy
CURB-65 – useful for determining need for hospital
administration:
C – confusion
U – ureal > 7mmol/l
R – Respiratory rate > 30
B – Bp SBP <90mmHg, DBP <60mmHg
65 – aged >65
Prognosis
Dependant on the underlying cause, comorbidities and complications.
25% usually require hospitalisation.
Bronchiolitis
Acute inflammatory response injury of the bronchioles usually caused by a viral infection
Pathology
Epidemiology
Very contagious. Virus spread by airborne
droplets.
Increased nasal secretions, bronchial obstruction
and constriction, alveolar death, mucus debris,
viral invasion, air trapping, atelectasis, reduced
ventilation leading to ventilation/perfusion
mismatch, labored breathing
1/9 infants contracts bronchioloitis in the first
year of life during fall and winter, 10% require
hospital admission.
Low socioeconomic status can adversely affect
outcome.
Higher incidence in boys.
Risk factors
Aetiology
Close contact with infection
90% caused by RSV
Others – parainfluenze virus types 1, 2 and 3,
influenza B, echovirus, rhinovirus, adenovirus (1,
2 and 5 in particular), mycoplasma (mainly in
school aged children)
History:
Examination:
Cough, dyspnoea, wheezing, poor feeding,
hypothermia or hyperthermia.
Hypo/hyperthermia, otitis media, tachpnoea,
nasal flaring, intercostal retraction, subcostal
retraction, irritability, fine rales, wheezing,
hypoxia
Complications:
Investigation:
Usually none
In severe disease – respiratory failure
Diagnosis mainly on clinical findings
Septic screen
Viral testing
Pulso oximetry
Nasopharyngeal aspirate
Imaging
Chest X-ray
Management:
May require oxygen
Bronchodilators - Salbutamol (nebs, inhaler or IV)
Maintain adequate hydration
Prognosis
Infectious self limiting disease
Pulmonary oedema
Pathology
Cardiogenic – increased pulmonary capillary pressure, decreased plasma oncotic pressure, increated
negative intersitinal pressure, damage to the alveolar-capillary barrier, lymphatic obstruction or
idiopathic mechanism.
Neurogenic – relatively rare, increased pulmonary interstitial and alveolar fluid, develops within a
few hours of neurologic insult.
Classification:
Aetiology:
Cardiogenic – extravasation of fluid from the
pulmonary vasculature into the interstitium
and alveoli of the lung.
Neurogenic – follows neurological insult
Cardiogenic – mitral stenosis, atrial myxoma,
thrombosis of a prosthetic valve, cor
tritriatum, acute MI or ischemia, salty diet,
non compliance with diuretics, severe
anaemia, sepsis, thyrotoxicosis, myocarditis,
chronic valve disease
Neurogenic – SAH, cerebral haemorrhage,
epileptic seizure, head injury, MS, nonhaemorrhagic stroke, bulbar poliomyelitis, air
embolism, brain tumour, ECT, bacterial
meningitis, cervical spinal cord injury.
History:
Examination:
Cardiogenic – symptoms of left heart failure,
sudden onset breathlessness, anxiety, feeling
of drowning, profuse diaphoresis, 24hr onset
dyspnea
Neurogenic – follows CNS insult, dyspnoea, mild
haemoptysis
Cardiogenic - hypoxia, increased sympathetic
tone, tachypnoea, tachycardia, agitated,
confused, hypertension, fine creps or wheezes,
jugular venous distension, aortic stenosis,
aortic regurge, mutral regurge,
Neurological – tachypnoea, tachycardia, bibasilar
crackles, respiratory distress, normal jugular
venous pressue, fever.
Complications:
Investigation:
Cardiogenic – respiratory fatigue and failure,
sudden cardiac death
Neurogenic – prolonger hypoxic respiratory
failure, haemodynamic instability, nosocomial
infections, death.
Cardiogenic – bloods (anaemia, sepsis,U+E),
imaging (enlarged heart, inverted blood flow),
ABG, SpO2, ECG (LV hypertrophy)
Neurogenic – no specific lab study, CXR shows
bilateral alveolar filling with normal heart size,
Management:
Cardiogenic – Oxygen, ventilator support, preload reduction (NTG, loop diuretics), afterload
reduction (ACE-i, angiotensin II receptor blockers), surgery (intra-aortic balloon pumping,
ultrafiltration), diet (low salt)
Neurogenic – focus on the underlying cause.may need O2 therapy.
Prognosis
Cardiogenic – hospital mortality rates can be as high as 20%, associated with MI with a mortality rate
of 40%. Mortality rate approaches 80% if hypotensive
Neurogenic – pulmonary oedema usually resolved within 48-72 hrs in the majority of patients...
early stage cardiogenic – interstitial
pulmonary oedema, cardiomegaly, left pleural
effusion.
Neurogenic mechanism
Neurogenic - bilateral alveolar filling process
and a normal-sized heart. This may mimic
congestive heart failure with cephalization of
blood flow, although other features of heart
failure, such as septal Kerley B lines, are
usually not evident
Cyanotic congenital heart disease
Heart defect present at birth resulting in low blood oxygen levels. There may be more than one
defect.
Pathology
Coarctation of the aorta
Critical pulmonary valvular stenosis
Ebstein’s anomaly
Hypoplastic left heart syndrome
Interrupted aortic arch
Pulmonary valve atresia
Pulmonic stenosis with an atrial or ventricular septal defect
Some forms of total anomalous pulmonary return
Tetraolgy of Fallot
Total anomalous pulmonary venous return
Transposition of the great vessles
Tricuspid atresia
Truncus arteriosus
Risk factors
Chemical exposure
Genetic and chromosomal syndromes (eg Downs, trisomy 13, Turners, Marfans, Noonan syndrome,
Ellis-van Creveld)
Infections (eg rubella) during pregnancy
Poorly controlled blood sugar during pregnancy
Medications taken during pregnancy
Aetiology:
The normal blood flow through the heart and lungs in altered (right to left shunt).
History:
Examination:
Cyanosis
Dyspnoea
Anxiety, hyperventilation, sudden increase in
cyanosis, fatigue, sweating, syncope, feeding
problems / reduced appetite, puffy eyes or
face
Cyanosis
Clubbing
Abnormal heary sounds
Heart murmur
Lung crackles
Complications:
Investigation:
Arrhythmias
Brain abscess
Heart failure
Haemoptysis
Impaired growth
Infectious endocarditis
Polycythemia
Pulmonary hypertension
stroke
CXR
FBC
Pulse ox
ABG
ECG
Echo-Doppler
Transoesophageal echocardiogram
MRI heart
Management:
Get rid of excess fluid
Help the heart pump harder
Maintain blood vessel patency
Treat abnormal heartbeats or rhythms
Prognosis
Dependant on defect
Some cause sudden death
Congenital heart defects
Presentation – dependant on the size of the defect. Ease to fatigue, recurrent respiratory chest
infections, exertional dyspnoea
Signs of pulmonary arterial hypertension, atrial arrhythmias, mitral valve disease.
Palpable pulsation of the pulmonary artery, ejection click, S1 may be split, S2 often widely split, may
have rumbling middiastolic murmur
Risks in alcohol use and illicit drug use, some have genetic predispositions
Left to right shunt with developed pulmonary hypertension and cyanosis: Eisenmenger’s syndrome
Atrial septal defect: Ostium
secundum
Atrial septal defect – ostium primum
75% of ASDs female 2:1 male
Incomplete adhgesion between the flap
valve associated with the foramen ovale
and the septum secundum after birth
15-20% of ASDa female 2:1 male
Incomplete fusion of the septum primum with the
endocardial cushion
Atrial septal defect – sinus
venosus
Coronary sinus ASD
5-10% ASDa female 2:1 male
Abnormal fusion between the embryologic
sinus venosus and the atrium
May occur on a familial basis
Unroofed coronary sinus and persistent left superior
vena cava which drains into the left atrium
Aortopulmonary septal defect
Ventricular septal defect:
Uncommon
Deficiency in the septim between the aorta
and pulmonary artery
Deficiency of growth or a failure of alignment or fusion
of component parts of the ventricular septum.
Patent ductus arteriosus
Transposition of the great vessels
Persistent communication between the
descending thoracic aorta and the
pulmonary artery.
Fairly common and usually spontaneously
close
The aorta and the pulmonary vein are switched
Ebstein’s anomaly
Hypoplastic left heart syndrome
Rare defect involving the tricuspid valve.
Deep lying valves leaflets which are larger
than normal
Parts of the left side of the heart (mitral valve, left
ventricle, aortic valve and aorta) do not develop
completely
Truncus arteriosus
Total anomalous pulmonary venous
return
A single blood vessel comes out of the left
and right vntricle instead of the normal
two (pulmonary artery and the aorta)
None of the four pulmonary veins is attached to the
left artrium (usually returned to the right atrium), a
septal defect must exist for the infant to survive
Tricuspid atresia
Coarctation of the aorta
The tricuspid valve is missing or abnormally
developed., blocking blood flow from
the right strium to the right ventricle.
Fallot’s tetralogy
One of the most common congenital heart disorders
Often associated with trisomy 21 (Down’s syndrome)
Pathology
Epidemiology
1. Right ventricular outflow tract
obstruction (infundibular stenosis)
2. Ventricular septal defect
3. Aorta dextroposition
4. Right ventricular hypertrophy
A few children also have atrial septal defect
10% of congenital heart defects
3-6 / 10,000 births
Risk factors
Maternal rubella and other infections, poor prenatal nutrition, maternal alcohol, older maternal age,
maternal phenylketonuria, maternal diabetes.
Aetiology:
Unknown
Genetic studies suggest multifactorial aetiology
History:
Examination:
Cyanosis (during feeding, crying, etc), exertional
dyspnoea worsening with age, haemoptysis due
to rupture of the bronchial collaterals in older
children
Smaller than expected for age. Finger and toe
clubbing from 3-6 months.
Systolic thrill usually present anteriorly along the
left sterna border. Harsh systolic ejection
murmur over pulmonic area and left sterna
border. S2 is usually single.
Murmurs disappear during cyanotic episodes.
RV predominance on palpation
May have a bulging left hemithorax
Aortic ejection click
Squatting position (compensatory mechanism)
Scoliosis (common)
Retinal engorgement
Haemoptysis
Complications:
Investigation:
Worsened by acidosis, stress, infection, posture,
exercise, beta-adrenergic agonists, dehydration,
closure of the ductus arteriosus.
Haematological studies – raised haemoglobin
and haematocrit. SpO2 65-70%, hyperviscosity
and coagulopathy
ABG and pulse ox – vary O2 sats, pH and pCO2 are
normal.
Radiological studies – CXR: classic boot-shaped
appearance
ECG – right axis deviation, RV enlargement,
partial or complete RBB block.
Cardiac catheterization and angiography
Complications from surgery – heart block,
residual entricular septal defects, ventricular
arrhythmias, late postoperative death.
Management:
Oxygen – don’t provoke during cyanotic episode
IV propanolol in severe episode to relax muscle spasm.
Surgery
Prognosis
Without surgery – mortality rates 30% age 2, 50% age 6
Sudden death in 1-5%
Most who survive develop congestive heart failure by age 30
With surgery – 40% reduction in death.
Tetralogy of Fallot with pulmonary atresia – 50% survive to age 1, 8% survive to 10 years.
Pneumothorax
Presence of air or gas in the pleural cavity.
Pathology
Epidemiology
Spontaneous – rupture of the blebs and bullae.
Primary spontaneous has no underlying disease
usually related to increased shear forces in the
apex
Tension – disruption of the visceral plaura,
parietal pleura or the tracheobronchial tree.
Rupture forms a one way valve.
Pneumomediastinum – excessive intra-alveolar
pressure leading to rupture of the perivascular
alveoli. Air escapes into the surrounding
connective tissue and dissects into the
mediastinum.
Primary, secondary and recurring spontaneous
pneumothorax – incidence underestimated,
10% are asymptomatic. 20-30.
7.4-18 / 100,000 (age dependant)
COPD 26/100,000 persons
Iatroenic and traumatic – 5-7/10,000 ospital
admissions. 1-2% all neonates.
Tension – complication in 1-2% idiopathic
spontaneous pneumothorax. 1.4% all patients
with TB.
Pneumomediastinum – young healthy patients
without serious underlying pulmonary disease.
4th decade of life. 1/100,000 hospital
admissions.
Risk factors
Primary spontaneous – tall young people without lung disease, smoking, Marfan syndrome,
pregnancy, familial
Secondary spontaneous – COPD, asthma, immunodeficiency, necrotizing pneumonia, TB, sarcoidosis,
CF, bronchogenic carcinoma, metastatic malignancy, idiopathic pulmonary fibrosis, inhalation and
IVDU, interstitial lung diseases.
Iatrogenic and traumatic – needle aspiration biopsy, transbronchial or pleural biopsy, throacentesis,
thacheostomy etc.
Tension – blunt or penetrating trauma, barotraumas, pneumoperitoneum, acupuncture, etc.
Pneumomediastinum – asthma, RTI, parturition, emesis, severe cough, mechanical ventilation,
trauma, athletic competition.
Classification:
Spontaneous / primary spontaneous
Tension
Pneumomediastinum
History:
Examination:
Spontenous and iatrogenic – usually develops at
rest. Not associated with trauma. No clinical
signs before pneumothorax. Acute onset chest
pain and SOB. Chest pain severe and stabbing,
radiated to the ipsilateral shoulder, worsens on
inspiration
Tension – chest pain, dyspnoea, anxiety, fatigue,
acute epigastric pain.
Pneumomediastinum – 67% chest pain, 42%
persistent cough, 25% sore throat, 8%
dysphagia, SOB, vomiting and nausea.
Respiratory – respiratory distress, tacypnoea,
asymmetrical lung expansion, distant or
absent breath sounds, transmitted lung
sounds, hyperressonant percussion, decreased
tactile fremitus, adventitious lung sounds
(crackles, wheezes, ipsilateral finding)
Cardiovascular – tachycardia, pulsus paradoxus,
hypotension, jugular venous distension,
cardiac apical displacement.
Spontaneous / iatrogenic – tachycardia,
tachypnoea and hypoxia
Complications:
Investigation:
Misdiagnosis
Hypoxic respiratoy failure, respiratory or cardiac
arrest, haemopneumothorax,
bronchpulmonary fistula, pulmonary oedema,
empyema, pneumomediastinum,
pneumopericardium pneumoperitoneum,
pyopneumothorax.
Complications of surgical procedures – failure to
cure the problem, acute respiratory distress
or failure, infection of the pleural space,
cutaneous or systemic infection, persistent
air leak, reexpansion pulmonary oedema, pair
at the site of the chest tube insertion,
prolonged tube drainage and hospital stay.
ABG - acidaemia, hypercarbia, hypoxaemia
CXR – linear shadow of visceral pleura, ipsilateral
lung edge parallel to chest wall, deep sulcus
sign, small pleural effusion, mediastinal shift,
airway or parechymal abnormalities
Transillumination – in neonates
Chest CT – distinguish between bullae and
pneumothorax, underlying emphysema,
determine exact size
Ultrasonography -
Management:
Prehospital – ABC, give O2. Needle decompression in tension pneumothorax.
Hospital – ABCs, primary and secondary spontaneous; needle aspiration if small, pigtail catheter if
larger. Pleurodesis decreases risk of recurrence. Iatrogenic and traumatic; aspiration, recurrence
not usually a factor. In small pneumothorax no intervention may be needed – give oxygen.
Tension; life-threatening – needle decompression
Thoracotomy -
Prognosis
Primary, secondary and recurring spontaneous pneumothorax – complete resolution takes
approximated 10 days. Typically benign and often resolves without medical attention.
Recurrences usually strike within 6 months to 3 years. 5 year recurrence rate is 28-32% for primary,
43% for secondary. Recurrences more common in those who smoke.
Tension – rapidly progresses to respiratory insufficiency, cardiovascular collapse and death is
unrecognised and untreated. Prognosis depends of associated injuries and morbidities.
Pneumomediastinum – generally benign self-limiting condition. Malignant or tension
pneumomediastinum have comorbid conditions often related to trauma. No reports of fatal
outcome with spontaneous pneumomediastinum. Mortality rate 70% in patients with
pneumomediastinum secondary to Boerhaave syndrome even with surgical intervention.
Spontaneous pneumothorax
Tension
Oneumomediastinum
Pleural effusion
Clinical importance ranges from incidental manifestations of cardiopulmonary diseases to
symptomatic inflammatory or malignant disease.
Pathology
Epidemiology
Normal pleural space contains approximately
1ml fluid representing the balance between
hydrostatic and oncotic forces in the visceral and
parietal pleural vessels and extensive lymphatic
drainage. Pleural effusion result from disruption
of this balance.
1 million cases per year in the US.
324/100,000 in industrialised countries.
Aetiology:
Transudates: congestive heart failure, cirrhosis, atelectasis, hypoalbuminaemia, nephritic syndrome,
peritoneal dialysis, myxoedema, constrictive pericarditis.
Exudates: parapneumonic causes, malignancy (carcinoma, lymphoma, mesothelioma), pulmonary
embolism, collagen vascular conditions (RA, SLE), TB, asbestos exposure, pancreatitis, trauma,
drug use, sarcoidosis.
History:
Examination:
Dyspnoea related to distortion of the diaphragm
and chest wall more than hypoxaemia.
Signs of underlying lung or heart disease.
Less commonly – mild non-productive cough or
chest pain.
Constant chest pain suggests invasive cancers.
Productive cough suggests pneumonia.
Pleuritic chest pain suggests embolism or
inflammatory pleural process.
Persistent systemic toxicity evidenced by fever,
weight loss and inanition suggests empyema.
Normal examination til 300 ml effusion.
Decreased breath sounds
Dullness to percussion
Decreased tactile fremitus
Egophony (E-to-A change)
Pleural friction rub
Mediastinal shift away from the effusion.
Complications:
Investigation:
Permanent lung damage
Infection causing abscess or empyema
Frankly purulent fluid indicates empyema, putrid
odour suggests anaerobic empyema, milky
Pneumothorax
opalescent fluid suggests chylothorax, bloody
fluid suggests trauma, malignancy or asbestos
related syndromes.
Exudate if: pleural fluid to serum protein ratio
>0.5, fluid to serum LDH >0.6, pleural fluid LDH
> 2/3 the upper limit of normal serum value.
Transudate: pleural fluid LDH >0.45 upper limit
normal serum level, pleural fluid cholesterol >
45 mg/dl, pleural fluid protein >2.9 g/dl.
Imaging: CXR, USS, CT
Diagnostic thoracentesis, therapeutic
thoractentesis. Tube thoracostomy,
pleurodesis or pleural sclerosis
Management:
Treat the underlying cause.
Drainage
Surgical intervention: video assisten thoracoscopy, pleural sclerosis, implanted shunts, surgery to
close defects
Diet – restrict fat
Monitoring
Prognosis
Varies depending of underlying cause
Malignant disease have a poor prognosis – median survival 4 months, mean survival less than 1 year.
Peripheral vascular disease (atherosclerotic
disease)
Disease affecting the large and medium sized arteries.
Pathology
Epidemiology
Endothelial dysfunction, vascular inflammation,
uild up of lipids, cholesterol, calcium and cellular
debris within the intima of the vessel wall. Build
up results in plaque formation, vascular
remodelling, acute and chronic luminal
obstruction, abnormalieies of blood flow and
diminished blood supply to target organs.
Men > women
>40 years
Risk factors
Hypertension, diabetes mellitus, smoking, CRP, elevated fibrinogen, familial hypercholesterolaemia,
History:
Examination:
Dependant on vascularture affected
CNS – stroke, reversible ischarmic neurologic
deficit, transient ischaemic attack.
Peripheral – intermittent claudication,
impotence, nonhealing ulceration and
infection of extremities.
Visceral ischarmia – signs and symptoms of
target organ failure.
Mesenteric angina – epigastric or periumbilical
postprandial pain, haematemesis,
haematochezia, melena, diarrhoea,
nutritional deficiencies, weight loss.
AAA
Mild disease appears clinically normal.
Hyperlipidaemia
CVD – diminished carotid pulses, carotid artery
bruits, focal neurological defecits.
PVD – decreased peripheral pulses, peripheral
arterial bruits, pallor, peripheral cyanosis,
gangrene and ulceration.
AAA – pulsatile abdominal mass, peripheral
embolism, circulatory collapse
Atheroembolism - livedo reticularis, gangrene,
cyanosis, ulceration, digital necrosis, GI bleed,
rectal ischaemia, cerebral infarction, renal
failure.
Investigation:
Lipid profile – elevated LDL, high triglycerides, low HDL
Blood glucose and HbA1C – raised
USS – evaluation of arteries showing intima-media thickening
MRI – thickening of blood vessel wall and characterise plaque composition.
Management:
Control risk factors.
Dietary and pharmacologic treatment of hypertension
Control DM
Treatment of familial hypercholesterolaemia
Prognosis
Dependant on systemic durden of disease, vascular bed(s) involved, degree of flow limitation.
Idiopathic pulmonary fibrosis
Specific form of chronic, progressive fibrosing interstitial pneumonia of known cause.
Pathology
Epidemiology
Current hypothesis: exposure to inciting agent
(eg smoke, environmental pollutants,
environmental dust, viral infections, GORD,
chronic aspiration) causing aberrant
activation of alveolar epithelial cells provokes
migration, proliferation and activation of
mesenchymal cells with the formation of
fibroblastic/ myofibroblastic foci, leading to
the exaggerated accumulation of extracellular
matrix with the irreversible destruction of the
lung paraenchyma.
Activated alveolar epithelial cells release potent
fibrogenic cytokines and growth factors.
Normal wound healing doesn’t occur. Excess
apoptosis and fibroblast resistence to
apoptosis contributes to fibroproliferation
and idiopathic pulmonary fibrosis.
No large scale studies are available.
Incidence:
Males – 10.7/100,000 persons per year
Females – 7.4/100,000 persons per year
Prevalence:
Males – 20/100,000
Females – 13/100,000
Aetiology:
Histology
Undefined
Possibly exposue to inciting agents
Heterogenous, variegates appearance with
alternating areas of healthy lung, interstitial
inflammation, fibrosis and honeycomb change
resulting in a patchwork appearance.
History:
Examination:
Non-specific history
Exertional dysponoea, non productive cough
Systemic symptoms include weight loss, lowgrade fever, fatigue, arthralgia or myalgias
Symptoms for 1-2 years before diagnosis is made
5% have no presenting symptomes
Fine bibasilar inspiratory (Velcro) crackles.
Digital clubbing (25-50%)
Pulmonary hypertension
Complications:
Investigation:
Pulmonary hypertension
Acute exacerbation of pulmonary fibrosis
Respiratory infection
Acute coronary syndrome
Thromboembolic disease
Routine labs are non-specific, can be used to rule
out other causes of interstitial lung disease
CRP and ESR may be elevated
Chronic hypoxaemia
Imaging – CXR (peripheral reticular opacities at
Adverse medication effects
Lung cancer
lung bases, honeycombing, lower lobe volume
loss). CT (subpleural honeycombing)
4 criteria: subpleural basal predominance,
reticular abnormality, honeycombing with or
without traction bronchiectasis, absence of
inconsistent features.
Lung function tests
Management:
Stop smoking
Oxygen therapy
Vaccination against influenza and pneumococcal
Lung transplantation
Diet – maintain ideal bodyweight
Remain active
Prognosis
Poor prognosis – 2-5 year from time of diagnosis
Death rates increase with age, higher in men than women, worse during winter
60% dies from their idiopathic fibrosis as opposed to dying with it.
Asthma
Pathology
Epidemiology
Compolex pathophysiology involving airways
inflammation, intermittent flow obstruction
and bronchial hyperresponsiveness. The
mechanism of inflammation may be acute,
subacute or chronic with the presence of
airway oedema and musuc secretion
contributing to airway obstruction and
brochial reactivity.
5-10% of the population.
Overall prevalence rate of exercise induced
bronchospasm is 3-10% in people without
asthma or allergies, 12-15% in the whole
population.
In the UK 1,200 deaths from asthma in the UK,
40 were under 40 (average of 3 deaths a day)
Risk factors
Histology
FH asthma, allergy, sinusitis, rhinitis, eczema,
nasal polyps.
Social factors – smoking, workplace, education
level.
Mononuclear cell and eosinophil infiltration,
mucus hypersecretion, desquamation of the
epithelium, smooth muscle hyperplasia and
airway remodelling.
Aetiology:
Environmental allergens (dustmites, animal allergens. Cockroach allergens, fungi), Viral RTIs, GORD,
chronic sinusitis or rhinitis, aspirin or NSAID hypersensitivity, use of beta-adrenergic receptor
blockers, obesity, environmental pollutants, tobacco smoke, occupational exposure, irritants
(sprays, paint fumes), emotional factors, stress, perinatal factors.
History:
Examination:
Wheezing, cough, recurrent bronchitis,
bronchiolitis, pneumonia, persistent cough
and coryza, croup, chest rattling, chest
tightness, recurrent chest congestion.
May be exercise induced.
Mild: breathless, talk in sentences, can lie down,
increased resp rate, accessory muscles not
really used, HR <100, SpO2 >95% in air.
Moderate: RR increased, accessory muscle used,
supraclavicular and intercostals muscle
recession, nasal flaring, HR 100-120bpm, loud
expiratory wheeze, SpO2 91-95% in air,
difficulty talking or feeding, may sit straight.
Severe: breathless at rest, not eating, sit upright,
broken sentences, agitated, RR>30, accessory
muscles used, suprasternal retraction,
HR>120, loud biphasic wheeze, SpO2<91 in air,
hunched position (tripod)
Imminent respiratory arrest: severe episode +
drowsy and confused, severe hypoxaemia,
may have a bradycardia.
Complications:
Investigation:
COPD in severe asthma
Serious respiratory infections eg pneumonia
Pneumothorax
Respiratory failure
Status asthmaticus (severe asthma attacks that
do not respond to treatments)
Death
Labs: blood and sputum eosinophils, elevated IgE
CXR : may be normal, hyperinflation
CT: bronchial wall thickening, bronchial
dilatation, cylindrical and varicose
bronchiectasis, reduced luminal area, mucoid
impaction of the bronchi, centrilobar opacities,
linear opacities, airtrapping, mosaic lung
attenuation.
ECG: sinus tachycardia
Allergen skin test
Spirometry – reduced peak flow and FEV1.
Management:
(see below)
1. Occasional used of relief bronchodilators
2. Regular inhaled anti-inflammatory agents
3. High dose inhaled steroids or low dose inhaled steroids plus long acting beta-agonist
bronchodilator
4. High does inhaled steroids and regular bronchodilators
5. Addition of regular steroid tablets
Prognosis
Mortality as high as 0.86 / 100,000 interntationally.
Mortality related to lung function with an 8-fold increase in the lowest quartile.
Mortality also linked to management failure, smoking and comorbidities.
High-resolution CT scan of the thorax obtained during inspiration demonstrates airtrapping in a
patient with asthma. Inspiratory findings are normal.
High-resolution CT scan of the thorax obtained during expiration demonstrates a mosaic pattern of
lung attenuation in a patient with asthma. Lucent areas (arrows) represent areas of airtrapping.
Clinical Skills notes
Aware of use of home O2 therapy
Useful website: http://www.homeoxygen.nhs.uk/2.php
Provides advise on smoking
Useful website: http://smokefree.nhs.uk/
They can provide a QuitKit with practical tools and advice
Respiratory history and exam:
Respiratory System
Examples of presenting complaints
Breathlessness
•
How is the patient normally? (Is this acute / chronic / acute or
chronic?)
•
Onset, timing, duration, variability
•
Exacerbating factors e.g. allergic triggers, exertion, cold air
•
Relieving factors e.g. rest, medication
•
Associated symptoms e.g. cough, sputum, haemoptysis,
pain, wheeze, night sweats, weight loss, oedema
•
Severity e.g. at rest? Only on exertion? Limiting ADLs?
Cough
•
Onset, timing, duration, variation (e.g. changed chronic
cough), diurnal variation
•
Productive / unproductive?
Sputum
•
Onset, timing, duration, variation, diurnal variation
•
Colour
•
Consistency (viscous (fluid), mucous, purulent, frothy)
•
Quantity (teaspoon, cupful etc.)
•
Odour (fetid suggests bronchiectasis or a lung abscess)
Haemoptysis
•
Origin (differentiate haemoptysis from haematemesis, sure it
was coughed up?)
•
Onset, timing, duration, variation
•
Quantity
•
Colour (fresh blood or dark altered blood)
•
Consistency (liquid, clots, mixed with sputum)
•
Chest pain
•
Recent trauma to chest or elsewhere?
•
Recent / current DVT?
•
Weight loss, fever, night sweats?
•
Breathlessness?
•
Bleeding or bruising elsewhere?
Respiratory System
Examination

Wash your hands
Past Medical History
Specific risk factors include:
•
Previous respiratory problems
o
Pneumonia can lead to bronchiectasis or
pulmonary fibrosis
o
Tuberculosis can reactivate
o
Severe measles or whooping cough can lead to
bronchiectasis
o
Asthma
•
Recent surgery
o
Dental surgery can lead to aspiration of purulent
material or fragments of tooth
o
Abdominal, pelvic or orthopaedic surgery are risk
factors for DVT and possible pulmonary embolus
•
Cardiac disease may lead to pulmonary oedema
•
Immunocompromise
(e.g.
HIV,
immunosuppression
post‐transplant surgery) may predispose to atypical
infections
Drug history and allergies
•
Inhalers, steroids, antibiotics, ACE inhibitors (may cause
cough), oxygen therapy
•
Social history
•
Occupation (industrial hazards e.g. dusts, asbestos)
•
Smoking (pack years)
•
Pets
•
Overseas travel
•
Living conditions e.g. damp
•
Alcohol
•
Exercise, activities of daily living, independence
Family history
•
Infections may be transmitted between family members
•
There is a genetic predisposition to allergic conditions e.g.
asthma, emphysema
Palpation

Check trachea and apex beat for deviation

Assess chest expansion anteriorly (normal >5cm, definitely
abnormal <2cm)

Assess tactile vocal fremitus



Introduction, identification and consent
General inspection of the bed area e.g. inhalers, nebuliser,
oxygen mask, sputum pot
General observation of the patient (colour, breathing,
comfort, position, purse‐lipped breathing, nutritional state
Percussion

Starting at the apices, percuss from side to side anteriorly
Auscultation

Starting at the apices, auscultate from side to side anteriorly
th
and laterally with open mouthed breathing (clavicle to 6 rib,
Inspection

Inspect the hands for clubbing

Look for tremor (flapping asterixis in respiratory failure, fine
with beta‐agonists e.g. salbutamol)

Assess pulse rate, rhythm and character (e.g. bounding in
CO retention) whilst simultaneously assessing respiratory
th
2
rate, rhythm, pattern and effort.

Check for raised JVP (cor pulmonale)

Look for respiratory disease in the eyes:
o
Horner’s syndrome
o
Chemosis (conjunctival oedema may be seen with
hypercapnia 2° to COPD)

Look for respiratory disease in the face and mouth:
o
Dental caries (may cause lung abscess by inhalation of
debris)
o
Central cyanosis
Expose the chest and inspect for:

Shape
o
Barrel chest (hyperinflated in emphysema)
o
Severe kyphoscoliosis
o
Severe pectus excavatum (funnel chest)
o
Pectus carinatum (pigeon chest) +/‐ Harrison’s sulci

Symmetry

Scars

Muscle wasting

Chest versus abdominal (diaphragmatic) breathing

Use of accessory muscles

Recession (more common in children, but can be seen in
adults with partial laryngeal/tracheal obstruction)

midclavicular line; Axilla to 8 rib, mid axillary line). Note the
presence of:
o
Vesicular (normal) breath sounds
o
Bronchial breathing
o
Rhonchi (wheezes)
o
Crepitations
o
Pleural rub
o
Assess for any change in these sounds after coughing
(crepitations due to secretions will alter after coughing
whereas those in fibrotic conditions won’t)
Assess vocal resonance +/‐ whispering pectoriloquy (whisper
222)
Repeat inspection, palpation, percussion, and auscultation
(spine of scapula to 11th rib) on the back with the patient sitting
forward

This is a convenient time to palpate the cervical lymph nodes.

Check sputum pot (volume, consistency, colour, odour, any
haemoptysis)

Assess peak flow

Thank the patient

Wash your hands
Cardiac history and exam:
Cardiac History
Presenting complaints include:

Chest pain

Dyspnoea (SoB)

Orthopnoea (SoB on lying flat that is relieved by sitting up)

Paroxysmal nocturnal dyspnoea (acute dyspnoea that wakes
patient from sleep)

Ankle oedema

Cough, sputum, haemoptysis

Dizziness

Lightheadedness

Presyncope and syncope

Palpitations

Nausea and sweating

Claudication

Systemic symptoms (eg fatigue, weightloss, anorexia, fever)
Ischaemic heart disease risk factors:

Age,
smoking,
hypertension,
DM,
FH,
Hypercholesterolaemia, inactivity and obesity
Chest pain

Site – central substernal, across mid-thorax anteriorly,
in both arms / shoulders, neck, cheeks, teeth, forarms,
fingers, intrascapular region

Onset – Acute

Character – crushing, tight, constricting, squeezing,
burning, ‘heaviness’
SoB


Onset – acute, chronic, acute – on – chronic
Associated symptoms 0 sweating, nausea, pain, cough,
sputum, peripheral oedema, palps, nocturnal
micturition, rapid weight gain

Timing – on exercion? At rest? Constant? At night?

Exacerbating factors – position (number of pillows –
orthopnoea?)

Alleviating factors – rest, medications, oxygen, sitting
up straight

Severity – how debilitating, effect of ADLs, excess
tolerance

Any respiratory diseases?
Palpitations

Ever had palpitations? Been aware the heart is racing?

Anything provoke it?

Sudden or gradual build up / stopping? How long does it
last?

Any other symptoms?

Tap the rhythm – regular, irregular, irregularly irregular?
Fast / slow?
Syncope

Sudden or brief loss of consciousness, with deficit of
postural tone, spontaneous recovery

Usually due to sudden transient hypotension,  poor
cerebral profusion

Presyncope – feeling of imminent syncope eg faintness




Radiation – neck, jaw, left arm
Associations – sweating, nausea, SoB, palpitations
Timing – on exertion? At reast?
Exacerbations = exercise, excitement, stress, cold
weather, after meals, smoking
Alleviations – rest, medications, oxygen
Severity – pain scale (1-10)
Past Medical History

Similar episodes, previous diagnoses, treatments and
responses to them

Previous cardiac surgery

Hypertension, hypercholesterolaemia, anaemia, DM,
angina, MI, CVA, TIA, peripheral vascular disease,
cardiac failure, rheumatic fever
Factors against cardiac ischaemia as the cause of pain

Character of pain: knife like, sharp, stabbing,
aggravated by respiration

Location of pain: left submammary area, left
hemithorax
Exacerbating factors: pain after exercise, specific body motion
Drug history and allergies

Antihypertensives, all cardiac drugs

Drugs with cardia side effects, eg corticosteroids,
salbutamol, theophylline, nifedipine, thyroxine


Cardiac Examination












Wash hands
Introduction, identification and consent
Inspect the bedspace for clues e.g. GTN spray
Inspect the general appearance of the patient (colour,
breathing, comfort, position, build)
Inspect the patients hands for cardiac signs:
o
Nicotine staining
o
Vasodilatation/constriction, temperature
o
Sweating (suggests increased sympathetic drive)
o
Pallor of palmar creases
o
Peripheral cyanosis
o
Clubbing
o
Splinter haemorrhages
o
Osler’s nodes
o
Tendon xanthomas
Check the presence of both radial pulses simultaneously.
Assess rate and rhythm in one radial pulse (usually the right).
Assess the character and volume of the brachial pulse
(normal, slow rising, collapsing). Ask the patient if they have
pain
in
their
arm
before
checking
for
collapsing/waterhammer pulse (aortic regurgitation)
Assess the character and volume of the carotid pulse (one
side at a time)
Look for cardiac signs in the eyes:
o
Subconjunctival pallor
o
Corneal arcus
Look for cardiac signs in the face:
o
Malar flush (mitral stenosis)
Look for cardiac signs in the mouth/lips:
o
Central cyanosis (under tongue or on mucous
membranes inside lips)
o
High‐arched palate (Marfan’s)
o
Dental caries (may predispose to infective
endocarditis)
Position the patient at 45° and check for raised JVP (normal =
2 ‐ 4cm above sternal angle). Check for low JVP using
hepatojugular reflux by compressing the liver and observing
the JVP (will rise). Check for high JVP by sitting patient
upright and looking near the ear lobes for venous pulsation.
Identify the two main waves by palpating the carotid.
Social History

Occupation,
smoking,
alcohol
cardiomyopathy), diet, stress
(AF
and
Family History
FH of IHD or CVA before the age of 65.

Expose the patients chest and inspect the precordium
o
sternotomy scar
o
severe pectus excavatum
o
severe kyphoscoliosis
o
visible cardiac pulsation

Palpate for the apex beat and parasternal heaves (outward
displacement of the palpating hand by cardiac contraction
e.g. in left ventricular hypertrophy) and thrills (palpable
murmurs)

Auscultate aortic, pulmonary, tricuspid and mitral valve areas

Auscultate left axilla for mitral incompetence

If extra sounds are heard, palpate the carotid pulse to time
them with the first and second heart sounds

Switch to the bell and auscultate the apex with the patient
rolled 45° to the left (for mitral stenosis).

Switch back to the diaphragm, sit the patient forward and
auscultate at the 4th/5th intercostal space to the left of the
sternum on held expiration (aortic regurgitation)

Auscultate lung bases, assess for sacral oedema. If
coarctation is suspected, auscultate to the left of the spine in
rd
th
the 3 /4 intercostal space

Sit the patient back and auscultate the carotids for bruits or a
transmitted systolic murmur.

Lay the patient flat, if they can tolerate it, and palpate for
hepatomegaly. If the liver is enlarged, feel for pulsation
(tricuspid regurgitation).

Check the femoral pulses. Check synchrony with the radial
pulse (radiofemoral delay in coarctation).

Check for pitting oedema at the ankles.

Check BP in both arms and lying and standing in one arm.

Perform opthalmoscopy for hypertensive retinopathy
Thank the patient and wash your hands.
Basic Sciences
Physiology of ventilation, perfusion and oxygenation (including function of
haemoglobin and oxygen dissociation)
Role of erthropoietinses. Abnormal Haemoglobins. Met and Sulf – Hb
Met-haemoglobin: the iron heme group must initially be in the Fe2+ state to support oxygen and
other gases binding and transport. It switches to the ferric (Fe3+) when bound to oxygen, forming
methaemoglobin which can’t bind to more oxygen.
Sulf-haemoglobin- haemoglobin bound to sulphun monoxide (SO) which bind iron in the heme
without changing its oxidation state, they inhibit oxgen binding can cause toxicity (can also bind to
cyanide (CN-), nitric oxice (NO) and sulphide (S2-).
Haemoglobin: