Cyanosis

Index conditions 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 Physiology of ventilation, perfusion and oxygenation (including function of haemoglobin and

oxygen dissociation) 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

COPDChronic bronchitis and emphysemaCough and sputum for more than 3 months in 2 consecutive years

Pathology EpidemiologyPersistent 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 COPDAffects mainly over 40s, more common with

increasing ageMen > women

Risk factors HistologySMOKINGAir pollutionPoor working conditionsGenetic risk (alpha-1-antitrypsin deficiency 1/100

COPD sufferers)Airway hypersensitivityIV drug use (“% IVDUs)Immunodeficiency syndromes – HIVVasculitis syndromeConnective 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 continuesSputumRecurrent chest infectionsVague symptoms – weight loss, tiredness, ankle

swellingChest pain and coughing up blood are not

common featuresSMOKER

Normal respiratory examination in early or mild disease

ClubbingWheezingHyperinflationDiffusely decreased breath soundsHyperresonance on percussionProlonged expirationCoarse cracklesObeseDistant heart sounds

Complications: Investigation:Cor pulmonaleRespiratory 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

CT2D echocardiogram6-minute walking distanceElectrocardiogramRight sided heart catheterizationABGAlpha1-antitrypsin measurement (normal 3-

7mmol/L)

Management:Smoking cessationBronchodilatorsManagement of inflammation – systemic and inhaled corticosteroidsManage infection – common infections include S. Pneumonia H. Influenza and M. CatarrhalisManagement of sputum – mucolytic agents eg N-acetylcysteinePPI for exacerbations and the common coldOxygen therapy and hypoxaemiaFlu vaccinationsLong term monitoring and end-of-life care

Prognosis4th leading cause of death

Cystic FibrosisMost 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 EpidemiologyDefects 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 – 1/3,200Hispanics – 1/9,200African Americans – 1/15,000Asian Americans – 1/31,000Females show greater deterioration of pulmonary function than men causing younger age at death

Risk factors HistologyCFTR gene mutation (1604 mutations recognised)

Thickened epithelial fluid lining.

Classification: Aetiology:CFTR gene mutation

History: Examination:Median age of diagnosis – 6-8monthsNeonates – meconium ileus and anasarcaInfancts – wheezing, coughing, recurrent RTIs, pneumonia. Steatorrhoea, failure to thriveChildren – pancreatic insufficiency, chronic cough, sputum production.

Nose – rhinitis, nasal polypsRespiratory – tachypnoea, respiratory distress, wheezes, crackles, cough, increased antero-posterior 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 chloriseImaging – 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.PhysiotherapyInvolvement of – surgeons, otolaryngologist, endocrinologist, cardiologist, transplant teamAssisted ventilationPancreatic enzymes, vitamins, bronchodilators, mucolytic agents, antibioticsMay need lung transplantation

PrognosisMedian survival is 36.9 years80% should reachg adulthood with treatment

PneumoniaInflammation of the lung tissue, usually more serious than bronchitis. Sometimes bronchitis and pneumonia occur together – bronchopneumonia

Pathology EpidemiologyViral 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 HistologyAge – young or elderlyLow immunityComorbidities

Pathogen invasion leased to cell death or apoptosis, causing an inflammatory immune response.

Classification:Aspiration – inhalation of oropharyngeal or gastric contentsBacterial - 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 sputumThoracentesis

Management:ThoracentesisOxygen and ventilation AntibioticsCorticosteroid therapy

CURB-65 – useful for determining need for hospital administration:C – confusionU – ureal > 7mmol/lR – Respiratory rate > 30B – Bp SBP <90mmHg, DBP <60mmHg65 – aged >65

PrognosisDependant on the underlying cause, comorbidities and complications.25% usually require hospitalisation.

BronchiolitisAcute inflammatory response injury of the bronchioles usually caused by a viral infection

Pathology EpidemiologyVery 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 AetiologyClose 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 noneIn severe disease – respiratory failure

Diagnosis mainly on clinical findingsSeptic screenViral testingPulso oximetryNasopharyngeal aspirateImagingChest X-ray

Management:May require oxygenBronchodilators - Salbutamol (nebs, inhaler or IV)Maintain adequate hydration

PrognosisInfectious self limiting disease

Pulmonary oedema

PathologyCardiogenic – 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, non-haemorrhagic 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 deathNeurogenic – 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.

PrognosisCardiogenic – hospital mortality rates can be as high as 20%, associated with MI with a mortality rate

of 40%. Mortality rate approaches 80% if hypotensiveNeurogenic – pulmonary oedema usually resolved within 48-72 hrs in the majority of patients...

early stage cardiogenic – interstitial pulmonary oedema, cardiomegaly, left pleural effusion.

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

Neurogenic mechanism

Cyanotic congenital heart diseaseHeart defect present at birth resulting in low blood oxygen levels. There may be more than one defect.

PathologyCoarctation of the aortaCritical pulmonary valvular stenosisEbstein’s anomalyHypoplastic left heart syndromeInterrupted aortic archPulmonary valve atresiaPulmonic stenosis with an atrial or ventricular septal defectSome forms of total anomalous pulmonary returnTetraolgy of FallotTotal anomalous pulmonary venous returnTransposition of the great vesslesTricuspid atresiaTruncus arteriosus

Risk factorsChemical exposureGenetic and chromosomal syndromes (eg Downs, trisomy 13, Turners, Marfans, Noonan syndrome,

Ellis-van Creveld)Infections (eg rubella) during pregnancyPoorly controlled blood sugar during pregnancyMedications taken during pregnancy

Aetiology:The normal blood flow through the heart and lungs in altered (right to left shunt).

History: Examination:CyanosisDyspnoeaAnxiety, hyperventilation, sudden increase in

cyanosis, fatigue, sweating, syncope, feeding problems / reduced appetite, puffy eyes or face

CyanosisClubbingAbnormal heary soundsHeart murmurLung crackles

Complications: Investigation:ArrhythmiasBrain abscessHeart failureHaemoptysisImpaired growthInfectious endocarditisPolycythemiaPulmonary hypertensionstroke

CXRFBCPulse oxABGECGEcho-DopplerTransoesophageal echocardiogramMRI heart

Management:Get rid of excess fluidHelp the heart pump harderMaintain blood vessel patencyTreat abnormal heartbeats or rhythms

PrognosisDependant on defectSome cause sudden death

Congenital heart defectsPresentation – dependant on the size of the defect. Ease to fatigue, recurrent respiratory chest

infections, exertional dyspnoeaSigns 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 murmurRisks in alcohol use and illicit drug use, some have genetic predispositionsLeft 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 maleIncomplete adhgesion between the flap

valve associated with the foramen ovale and the septum secundum after birth

15-20% of ASDa female 2:1 maleIncomplete fusion of the septum primum with the

endocardial cushion

Atrial septal defect – sinus venosus

Coronary sinus ASD

5-10% ASDa female 2:1 maleAbnormal fusion between the embryologic

sinus venosus and the atrium

May occur on a familial basisUnroofed coronary sinus and persistent left superior

vena cava which drains into the left atrium

Aortopulmonary septal defect Ventricular septal defect:UncommonDeficiency 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 vesselsPersistent 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 syndromeRare 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 aortaThe tricuspid valve is missing or abnormally

developed., blocking blood flow from the right strium to the right ventricle.

Fallot’s tetralogyOne of the most common congenital heart disordersOften associated with trisomy 21 (Down’s syndrome)

Pathology Epidemiology1. Right ventricular outflow tract

obstruction (infundibular stenosis)2. Ventricular septal defect3. Aorta dextroposition4. Right ventricular hypertrophy

A few children also have atrial septal defect

10% of congenital heart defects3-6 / 10,000 births

Risk factorsMaternal rubella and other infections, poor prenatal nutrition, maternal alcohol, older maternal age, maternal phenylketonuria, maternal diabetes.

Aetiology:UnknownGenetic 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 palpationMay have a bulging left hemithoraxAortic ejection clickSquatting position (compensatory mechanism)Scoliosis (common)

Retinal engorgementHaemoptysis

Complications: Investigation:Worsened by acidosis, stress, infection, posture, exercise, beta-adrenergic agonists, dehydration, closure of the ductus arteriosus.

Complications from surgery – heart block, residual entricular septal defects, ventricular arrhythmias, late postoperative death.

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

Management:Oxygen – don’t provoke during cyanotic episodeIV propanolol in severe episode to relax muscle spasm.Surgery

PrognosisWithout surgery – mortality rates 30% age 2, 50% age 6Sudden death in 1-5%Most who survive develop congestive heart failure by age 30With surgery – 40% reduction in death.Tetralogy of Fallot with pulmonary atresia – 50% survive to age 1, 8% survive to 10 years.

PneumothoraxPresence of air or gas in the pleural cavity.

Pathology EpidemiologySpontaneous – 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 factorsPrimary spontaneous – tall young people without lung disease, smoking, Marfan syndrome,

pregnancy, familialSecondary 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 spontaneousTensionPneumomediastinum

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:MisdiagnosisHypoxic 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, hypoxaemiaCXR – 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 neonatesChest 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 -

PrognosisPrimary, 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 pneumothoraxTensionOneumomediastinum

Pleural effusionClinical importance ranges from incidental manifestations of cardiopulmonary diseases to symptomatic inflammatory or malignant disease.

Pathology EpidemiologyNormal 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 soundsDullness to percussionDecreased tactile fremitusEgophony (E-to-A change)Pleural friction rubMediastinal shift away from the effusion.

Complications: Investigation:Permanent lung damageInfection 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, CTDiagnostic 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 defectsDiet – restrict fatMonitoring

PrognosisVaries depending of underlying causeMalignant 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 EpidemiologyEndothelial 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 factorsHypertension, diabetes mellitus, smoking, CRP, elevated fibrinogen, familial hypercholesterolaemia,

History: Examination:Dependant on vascularture affectedCNS – 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.HyperlipidaemiaCVD – 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 HDLBlood glucose and HbA1C – raisedUSS – evaluation of arteries showing intima-media thickeningMRI – thickening of blood vessel wall and characterise plaque composition.

Management:Control risk factors.Dietary and pharmacologic treatment of hypertensionControl DMTreatment of familial hypercholesterolaemia

PrognosisDependant on systemic durden of disease, vascular bed(s) involved, degree of flow limitation.

Idiopathic pulmonary fibrosisSpecific form of chronic, progressive fibrosing interstitial pneumonia of known cause.

Pathology EpidemiologyCurrent 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 yearFemales – 7.4/100,000 persons per year

Prevalence:Males – 20/100,000Females – 13/100,000

Aetiology: HistologyUndefinedPossibly 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 historyExertional dysponoea, non productive coughSystemic symptoms include weight loss, low-

grade fever, fatigue, arthralgia or myalgiasSymptoms for 1-2 years before diagnosis is made5% have no presenting symptomes

Fine bibasilar inspiratory (Velcro) crackles.Digital clubbing (25-50%)Pulmonary hypertension

Complications: Investigation:Pulmonary hypertensionAcute exacerbation of pulmonary fibrosisRespiratory infectionAcute coronary syndromeThromboembolic disease

Routine labs are non-specific, can be used to rule out other causes of interstitial lung disease

CRP and ESR may be elevatedChronic hypoxaemiaImaging – CXR (peripheral reticular opacities at

Adverse medication effectsLung 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 smokingOxygen therapyVaccination against influenza and pneumococcalLung transplantationDiet – maintain ideal bodyweightRemain active

PrognosisPoor prognosis – 2-5 year from time of diagnosisDeath rates increase with age, higher in men than women, worse during winter60% dies from their idiopathic fibrosis as opposed to dying with it.

Asthma

Pathology EpidemiologyCompolex 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 HistologyFH 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 asthmaSerious respiratory infections eg pneumoniaPneumothoraxRespiratory failureStatus asthmaticus (severe asthma attacks that

do not respond to treatments)Death

Labs: blood and sputum eosinophils, elevated IgECXR : may be normal, hyperinflationCT: 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 tachycardiaAllergen skin testSpirometry – reduced peak flow and FEV1.

Management:(see below)

1. Occasional used of relief bronchodilators2. Regular inhaled anti-inflammatory agents3. High dose inhaled steroids or low dose inhaled steroids plus long acting beta-agonist

bronchodilator4. High does inhaled steroids and regular bronchodilators5. Addition of regular steroid tablets

PrognosisMortality 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 therapyUseful website: http://www.homeoxygen.nhs.uk/2.php

Provides advise on smokingUseful 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

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

• 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?

• There is a genetic predisposition to allergic conditions e.g. asthma, emphysema

Respiratory System Examination

Wash your hands 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

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 CO2 retention) whilst simultaneously assessing respiratory 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)

Palpation Check trachea and apex beat for deviation Assess chest expansion anteriorly (normal >5cm,

definitely abnormal <2cm) Assess tactile vocal fremitus

Percussion Starting at the apices, percuss from side to side

anteriorly

Auscultation Starting at the apices, auscultate from side to

side anteriorly and laterally with open mouthed breathing (clavicle to 6th rib, midclavicular line; Axilla to 8th 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 HistoryPresenting complaints include: Chest pain Dyspnoea (SoB) Orthopnoea (SoB on lying flat that is relieved by

sitting up) Paroxysmal nocturnal dyspnoea (acute dyspnoea

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

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 obesityChest 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’ 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)

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

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

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

Drug history and allergies Antihypertensives, all cardiac drugs Drugs with cardia side effects, eg

corticosteroids, salbutamol, theophylline, nifedipine, thyroxine

Social History Occupation, smoking, alcohol (AF and

cardiomyopathy), diet, stress

Family HistoryFH of IHD or CVA before the age of 65.

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:

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 the 3rd/4th intercostal space

Sit the patient back and auscultate the carotids for bruits or a transmitted systolic murmur.

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.

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 – HbMet-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: