A 40-year-old man is undergoing investigation for acromegaly. MRI of the
pituitary fossa is normal, but a routine chest X-ray reveals a large centrally based mass. he patient is a non-smo!er. he most li!ely type of lung tumour is" Options A. #$uamous cell B. #mall cell C. %arcinoid D. &arge cell E. Adenocarcinoma Teaching Notes for Question 1 Theme: Lung Tumours A central based mass in a non-smo!er sho'ing clinical evidence of neuroendocrine cell origin is consistent 'ith a carcinoid. It represents the 'ell differentiated spectrum of small cell lung cancer and surgery in non- metastatic disease confers (0) survival at * years. Question 2 A patient is diagnosed 'ith primary adenocarcinoma of the left upper lobe of the lung. he +,-. is ../ 010) predicted2 and the staging % scan sho's only ipsilateral hilar lymphadenopathy. he ne3t step for the patient is" Options A. %hemotherapy B. Radiotherapy and chemotherapy C. &obectomy D. Mediastinoscopy and lobectomy E. 4est supportive care Teaching Notes for Question 2 Theme: Lung Tumours he best chance of cure for someone 'ith primary non-small cell lung cancer is surgery. % is not al'ays able to e3clude mediastinal node involvement and mediastinoscopy is al'ays re$uired before surgery. Question 3 A 5/-year-old builder is admitted 'ith sudden onset high fever, left-sided pleuritic chest pain and confusion. his presentation is most suggestive of pneumonia caused by" Options A. 6aemophilus influen7ae B. Mycoplasma pneumoniae C. #treptococcus pneumoniae D. Influen7a type A E. &egionella pneumophila Teaching Notes for Question 3 Theme: Communit Ac!uire" #neumonia #treptococcus pneumoniae typically presents 'ith acute onset, high fever and pleuritic chest pain. ,lderly and patients 'ith co-morbidity are at increased ris!. +emale se3, diabetes mellitus, %89: and alcoholism are associated 'ith bacteraemia Question $ A 54-year-old man is referred for investigation of the cause of chronic sputum production and haemoptysis. 6e is a non-smo!er. he most appropriate ne3t investigation is" Options A. Ig; subclass measurement B. ,lectron microscopy 0,M2 of a nasal biopsy C. #'eat test D. Aspergillus Ig; and Ig, levels E. 6igh resolution computer tomography 06R%2 of the lungs Teaching Notes for Question $ Theme: Chronic %uppurati&e Lung Disease %hronic production of sputum and haemoptysis is very consistent 'ith underlying bronchiectasis. 6R% is diagnostic of the disease. 8nce the diagnosis is established the cause must be found. Question ' A *5-year-old man 'ith history of smo!ing presents 'ith breathlessness. #pirometry sho's an obstructive pattern. he most appropriate first line of treatment is" Options A. Inhaled steroid B. 6igh dose inhaled steroid to prevent disease progression C. #albutamol D. Ipratropium bromide E. #almeterol Teaching Notes for Question ' Theme: Chronic O(structi&e Air)as Disease Anticholinergics are more effective than b5 agonists in %89: and are thus the treatment of choice during the initiation of therapy. #teroids do not alter the progression of the disease 0unli!e asthma2 and are reserved for patients 'ho demonstrate steroid responsiveness or very severe disease. Question * <hich of the follo'ing patterns of lung function tests 'ould best fit someone 'ith alpha-. antitrypsin deficiency= Options A. 8bstructive spirometry 'ith lo' >%8 and MM genotype B. 8bstructive spirometry, mar!edly decreased >%8 and basal emphysema C. 8bstructive spirometry, mar!edly decreased >%8 and upper lobe emphysema D. 8bstructive spirometry, normal >%8 and basal emphysema E. &o' >%8 and normal residual volume Teaching Notes for Question * Theme: Chronic O(structi&e Air)as Disease Although %89: due to alpha-. antitrypsin can present in any of the described 'ays the classic presentation is of predominantly basal emphysema and is pan-acinar 0obstructive spirometry and lo' >%82. he 9i?? genotype leads to the 'orst form of the disease. Question + he most important investigation to confirm the adult respiratory distress syndrome in an adult 'ith refractory hypo3aemia is" Options A. %hest X-ray B. ,chocardiogram C. % thora3 D. 9ulmonary artery catheter 'ith 'edge pressure measurement E. 4roncho-alveolar lavage Teaching Notes for Question + Theme: A"u,t -espirator Distress %n"rome he radiological investigations mimic heart failure in the adult respiratory distress syndrome 0AR:#2 and it is important to confirm that the pulmonary capillary 'edge pressure 09%<92 is less than or e$ual to ./mm6g 0thus no evidence of left ventricular failure2. Question . A patient 'ith rheumatoid arthritis develops a progressive fall in the +,-.. he residual volume is increased by 5l and the measurements of diffusion are normal. he patient is a smo!er. he most li!ely diagnosis is" Options A. 8rganising pneumonia B. 4ronchiolitis obliterans C. %aplan@s syndrome D. Rheumatoid associated lung fibrosis E. %hronic obstructive pulmonary disease Teaching Notes for Question . Theme: -heumato,og an" the Lung Although all of the possible options can occur in rheumatoid arthritis, a progressive and relentless fall in the +,-. indicates bronchiolitis obliterans. Inflammation in the small distal air'ays leads to obstructive spirometry and this is relentlessly progressive. Air trapping occurs as a conse$uence leading to increased lung volumes. Question / A A4-year-old man is rescued from a burning building and brought to hospital. 6e has nausea, vomiting, diarrhoea and abdominal pain. 6e is confused. he carbo3y-haemoglobin 0%86b2 level is 5*). <hich of the follo'ing is the best treatment for this patient= Options A. 6yperbaric o3ygen B. 6igh flo' o3ygen via a facial mas! C. % brain and lumbar puncture D. Intravenous fluid and o3ygen via nasal prongs E. Brgent senior surgical opinion to e3clude bo'el obstruction Teaching Notes for Question / Theme: #hsio,og %arbo3y-haemoglobin 0%86b2 levels do not correlate 'ell 'ith the clinical picture. he indications for hyperbaric o3ygen are neurological or psychiatric symptoms, cardiac complications, %86b levels of .40) and pregnant 'omen Question 10 A 50-year-old college student is referred 'ith breathlessness. ,3ercise-induced asthma is the most li!ely diagnosis if" Options A. Inhaled steroid abolishes the symptoms B. here is no history of atopy C. he symptoms usually occur at the end of strenuous e3ercise D. he symptoms re-occur on immediate repeat e3ercise E. &eu!otriene receptor antagonist abolishes the symptoms Teaching Notes for Question 10 Theme: Asthma ,3ercise-induced asthma is due to cold air drying the mucosa and affecting the periciliary fluid osmotics. his leads to inflammatory release and symptoms occur 'ithin *C.0min of e3ercise and lasts up to .h. here is usually a refractory period follo'ing this of up to 5C4h. %ysteinyl leu!otrienes are !ey players here and leu!otriene receptor antagonists are used to prevent e3ercise-induced bronchospasm. #teroids are not helpful. Question 11 A 4(-year-old miner develops smear positive Mycobacterium tuberculosis. <hich of the follo'ing dusts is most li!ely to have increased the ris! of this infection in this patient= Options A. %oal dust B. Asbestos C. %admium D. #ilica E. 4eryllium Teaching Notes for Question 11 Theme: 1ranu,omatous Lung Disease #ilica is to3ic to macrophages and impairs their function. hus there is an increased ris! of Mycobacterium tuberculosis in slate 'or!ers, stone masons, fettlers and miners 0drilling through $uart7 strata2. Question 12 A 40-year-old 'indo' cleaner 'ants to $uit smo!ing. 6e is on aspirin, a steroid inhaler and phenytoin. he best treatment for this man is" Options A. %ounselling B. 4upropion 0?ybanD2 C. Eicotine patches D. %ounselling and nicotine patches E. +luo3etine Teaching Notes for Question 12 Theme: Therapeutics an" the Lung 4upropion 0?ybanD2 is far more effective than nicotine patches in smo!ing cessation. 6o'ever it is contra- indicated in patients 'ith eating disorder and those 'ith a history of sei7ures. here is no advantage in combining nicotine patches 'ith bupropion. Question 13 A */-year-old, obese, heavy smo!er presents 'ith impotence, nocturia and depression. 6e is hypo3ic at rest on air and has an!le oedema. he most appropriate investigation to determine the aetiology is" Options A. Arterial blood gas B. %hest X-ray C. -entilation-perfusion scan D. hyroid function test E. #leep study Teaching Notes for Question 13 Theme: -espirator 2ai,ure %or pulmonale secondary to diurnal respiratory failure occurs in patients 'ith severe obstructive sleep apnoea 08#A2. Most patients 'ho develop this complication have lo'er air'ay obstruction 0from smo!ing2, gross obesity or respiratory muscle 'ea!ness. 6ypercapnia out of proportion to the degree of lung disease should suggest 8#A as a possible diagnosis. Question 1$ A 4*-year-old 'oman is breathless. he &%8 is very lo' but the >%8 is .(0) predicted. he most li!ely diagnosis is" Options A. Eeuromuscular chest 'all disorder B. 9rimary pulmonary hypertension 09962 C. A patient 'ith the ?? genotype for alpha-. antitrypsin D. #cleroderma E. 6ereditary haemorrhagic telangiectasia Teaching Notes for Question 1$ Theme: #hsio,og 9atients 'ith e3tra-pulmonary restriction 0e.g. neuromuscular chest 'all disorders2 the lungs cannot fully inflate. hus the surface area available for gaseous e3change is decreased 0lo' &%82. 6o'ever, the cardiac output is unchanged so that a higher density of blood per unit volume is obtained resulting in a raised >%8. Question 1' A A/-year-old 6I--positive man presents 'ith life threatening haemoptysis. <hich one of the follo'ing features 'ould be consistent 'ith >aposi@s sarcoma as the cause= Options A. 9leural effusion B. &ac! of systemic symptoms C. ;eneralised lymphadenopathy D. 6I- ac$uired through I- drug abuse E. %avity on chest X-ray Teaching Notes for Question 1' Theme: 345 an" the Lung >aposi@s sarcoma 0>#2 is a very vascular tumour and a cause of life threatening haemoptysis. 9leural effusion is commonly involved 'ith lung malignancy and in A0) of cases of >#. #ystemic symptoms are common. he finding of generalised lymphadenopathy and cavitation is non-specific in 6I-. Question 1 A A/-year-old man presents 'ith severe arthralgia of the an!les and red s'ellings. he %XR sho's bilateral hilar lymphadenopathy. he treatment of choice is" Options A. Analgesia B. 6igh dose prednisolone C. 6igh dose prednisolone and a7athioprine D. 8bservation E. Inhaled steroid Teaching Notes for Question 1 Theme: 1ranu,omatous Lung Disease &ofgren@s syndrome. his is also !no'n as ,rythema Eodosum syndrome and is characterised by the abrupt onset of fever, arthralgia and erythema nodosum. here is bilateral hilar lymphadenopathy on the %XR and no other real lung involvement. his form of sarcoid is usually self-limiting and has an e3cellent prognosis 'ith recurrence and any pulmonary involvement being rare. Eo steroid treatment is usually re$uired, unless systemic symptoms are severe. Question 2 A A4-year-old 'oman presents 'ith a prolonged history of epista3is and rapidly progressive, shortness of breath. he >%8 and eosinophil count are raised. he most li!ely diagnosis is" Options A. ;oodpasture@s syndrome B. Microscopic polyangiitis C. %hurg-#trauss syndrome D. <egener@s granulomatosis E. Alveolar proteinosis Teaching Notes for Question 2 Theme: %stemic Disease an" the Lung A patient 'ith breathlessness and a raised >%8 has alveolar haemorrhage till proven other'ise. A prolonged history of epista3is or sinusitis is commonly found in <egener@s granulomatosis, 'hich in some patients is also associated 'ith an eosinophilia. A history of asthma must usually be present to diagnose the %hurg- #trauss syndrome. Question 3 In the adult respiratory distress syndrome the most important determinant of a good prognosis is" Options A. Bse of steroids B. 6igh 9,,9 settings to overcome decreased pulmonary compliance C. Bse of inotropes for cardiovascular support D. he underlying cause of the syndrome E. Eutrition Teaching Notes for Question 3 Theme: A"u,t -espirator Distress %n"rome he management of AR:# is supportive and although this does effect prognosis, the overall survival rate is dependent on the cause. Question $ he most appropriate treatment regimen for a Russian man 'ith smear positive mycobacterial disease is" Options A. Isolation and rifampicin and ethambutol for .5 months B. 4%; vaccination C. Rifampicin and isonia7id for 5 months and revie' sensitivity D. Rifampicin, pyra7inamide,ethambutol for .5 months E. Rifampicin, isonia7id, pyra7inamide, ethambutol for 5 months and revie' sensitivity Teaching Notes for Question $ Theme: Therapeutics an" the Lung Multi-drug resistant 0M:R2-4 is of concern in anyone 'ho is from a part of the 'orld 'here M:R-4 is prevalent and also in those 'ho have had previous partially treated disease. hus a 4 drug regime until sensitivity is available is usual practice. Question ' A AF-year-old hairdresser 'ho has been 6I--positive for .0 years presents 'ith progressive shortness of breath on e3ercise. he chest X-ray is normal e3cept for prominent pulmonary arteries. 9ulse o3imetry sho's he desaturates on e3ercise. he most li!ely diagnosis is" Options A. 9neumocystis carinii pneumonia B. 9rimary pulmonary hypertension C. Intracardiac shunt across an atrial septal defect D. 9ulmonary embolic disease E. Anaemia Teaching Notes for Question ' Theme: Circu,ation of the Lung ,nlarged pulmonary central vasculature but other'ise normal chest X-ray suggests pulmonary arterial hypertension, a primary form of 'hich is associated 'ith chronic 6I- infection. 9ulmonary arterial hypertension should be suggested by enlargement of the central elastic arteries and pruning of the peripheral arteries. he pulmonary artery systemic pressure is usually .A0mm6g. 9ulmonary oligaemia 0the pulmonary trun! is small or inapparent 'ith small peripheral vessels2 usually indicates right ventricular outflo' obstruction 'ith a right to left shunt. Bneven vascularity on the %XR is e3pected 'ith embolic disease. Question * An airline pilot presents 'ith cough, 'hee7e and bloody sputum. he %XR sho's upper lobe infiltrates and the eosinophil count is ..5. he most important diagnostic step is" Options A. #putum for acid-fast-bacilli B. #tool for ova, cysts and parasites C. Aspergillus Ig, and Ig; D. 4ronchoscopy E. 6I- testing Teaching Notes for Question * Theme: Eosinophi,ia an" the Lung A49A is associated 'ith eosinophilia, high aspergillus Ig, 0leads to positive s!in pric! tests2, fre$uently positive aspergillus Ig; 0precipitins2 and eosinophilic consolidation of the lung that is characteristically flitting in nature. Asthma and pro3imal bronchiectasis are clinical complications. Question + A 54-year-old patient presents 'ith cough, 'hee7e and lo'-grade fever. he eosinophil count is raised. <hich of the follo'ing is the least li!ely diagnosis= Options A. ,3trinsic allergic alveolitis B. #alicylate abuse C. ropical pulmonary eosinophilia D. %hurg-#trauss syndrome E. Allergic bronchopulmonary mycosis Teaching Notes for Question + Theme: Eosinophi,ia an" the Lung ,3trinsic Gallergic@ alveolitis is not associated 'ith 'hee7ing but 'ith fever, coughing and dyspnoea. In addition, eosinophilia is not a feature. All the rest cause eosinophilia. Question . A 4.-year-old man presents un'ell. 6e is noted to have a microcytic anaemia and pulse o3imetry sho's saturations of /() on air. 4/h later he suffers a dense left hemiplegia. here is a strong family history of stro!e and several other members of his immediate family have been anaemic. he most li!ely diagnosis is" Options A. 6ereditary haemorrhagic telangiectasia B. -asculitis C. 9ulmonary arterio-venous malformation D. 9atent foramen ovale E. -entricular septal defect Teaching Notes for Question . Theme: Circu,ation of the Lung 6ypo3ia and a history of stro!e should suggest a right to left shunt. he history of anaemia and family history 'ould be consistent 'ith hereditary haemorrhagic telangiectasia 0662 as they bleed from both cerebral and gut arterio-venous malformations. Question / &ong term o3ygen therapy for an e3-smo!er 'ith ype II respiratory failure 'ould best" Options A. Improve lung function of the patient B. Improve the $uality of life of the patient C. 9rolong survival of the patient D. 6ave no effect on the patient@s haemoglobin level E. 6ave the most benefit if used for episodes of shortness of breath only Teaching Notes for Question / Theme: -espirator 2ai,ure &ong term o3ygen therapy 0&82 in %89: leads to" H Improved survival H Improved polycythaemia H #lo'ing of any further increases in pulmonary artery pressure H Eo improvement in lung function occurs H Iuality of life remains unchanged he benefit of &8 is proportional to the number of hours per day the o3ygen is 'orn by the patient. Question 10 he most important finding to confirm the diagnosis of asthma is" Options A. 6igh total blood Ig, level B. :emonstration of air'ay reversibility C. 6igh levels of interleu!in-* and granulocyte macrophage colony stimulating factor 0;M-%#+2 in blood D. +amily history of asthma E. A high &%8 and >%8 Teaching Notes for Question 10 Theme: Asthma Asthma by definition must demonstrate variability in airflo' limitation. he other investigations 'ould suggest asthma but can also occur 'ith other respiratory diseases. Question 11 <hich of the follo'ing additional features 'ould be most suggestive of a diagnosis of cystic fibrosis in a young 'oman 'ith recurrent chest infections= Options A. A prolonged prothrombin time and high s'eat sodium B. A high s'eat sodium C. <eight loss D. Infertility E. 6aemoptysis Teaching Notes for Question 11 Theme: Chronic %uppurati&e Lung Disease A prolonged prothrombin time 'ould suggest both malabsorption of -itamin > and cirrhosis, both specific complications of cystic fibrosis 0%+2. he other findings are also found in %+ but are not specific to the disease. Question 12 <hich one of the follo'ing patients carries the highest ris! of developing lymphoma of the lung= Options A. A heavy smo!er 'ith recurrent chest infections B. A 'oman 'ith a / year history of dry eyes and mouth 'ith nucleolar anti- nuclear antibody C. A patient 'ith recurrent scarring alopecia D. A man 'ith seronegative spondyloarthropathy E. A smo!er 'ith cough productive of copious $uantities of sputum Teaching Notes for Question 12 Theme: -heumato,og an" the Lung 9rimary #JKgren@s syndrome is associated 'ith an increased incidence of lymphoid lung malignancy. :ry mouth and dry eyes is a classic symptom of this condition, and patients are AEA positive 'ith a nucleolar pattern. Question 13 A *0-year-old patient 'ith asthma is prescribed a leu!otriene inhibitor. 6e presents 'ith severe abdominal pain and a pleural effusion. he most li!ely cause of the effusion is" Options A. %arcinoma B. 9ancreatitis C. 9neumonia D. 6eart failure E. %hurg-#trauss syndrome Teaching Notes for Question 13 Theme: %stemic Disease an" the Lung %hurg-#trauss syndrome has a predilection for serosal surfaces and therefore can cause both pleural and pericardial effusions and peritonitis. %ytological analysis of this fluid 'ould confirm an eosinophilia. &eu!otriene inhibitor use is associated 'ith an increased incidence of the disease. Question 1$ A patient 'ith pneumocystis carinii pneumonia 09%92 has the follo'ing blood gases" 9a85 1.(!9a and 9a%85 A.*!9a. he most important prognostic step is" Options A. Easal ventilation B. 9hysiotherapy C. Intravenous steroids D. Brgent commencement of retroviral therapy E. %ontrolled o3ygen therapy Teaching Notes for Question 1$ Theme: 345 an" the Lung In pneumocystis carinii pneumonia, steroids decrease the ris! of respiratory failure by *0), and the ris! of death by AA). #teroids are indicated if the arterial o3ygen tension is less than or e$ual to (.A!9a on air. Question 1' A A4-year-old plumber has Just started treatment for a community ac$uired pneumonia but is noticed to have developed anaemia 'ith a fall in haemoglobin of AgLdl over F days. he M%- is .05fl. he most useful investigation is" Options A. Mycoplasma serology B. Reticulocyte count C. 6aematinic measurements D. &iver function tests E. ,ndoscopy Cardiology Question 1 he follo'ing drugs are matched correctly 'ith their action" Options A. :igo3in stimulates EaL> A9ase B. #imvastatin inhibits 6M;%oA reductase C. #otalol has class I and class III properties D. Amiodarone shortens the action potential E. #trepto!inase increases fibrinogen levels Teaching Notes for Question 1 Theme: Car"io&ascu,ar #harmaco,og #otalol has class II 0b-bloc!er2 and class III 0Amiodarone2 li!e actions. Amiodarone prolongs the action potential and the I interval. 4oth #otalol and Amiodarone carry the ris! of precipitating ventricular arrhythmias. After thrombolysis, fibrinogen levels are lo', due to consumption during thrombosis. Question 2 A 41-year-old man 'ith a history of high alcohol inta!e presents 'ith breathlessness and peripheral oedema. <hich of the follo'ing feature 'ould not be characteristic of alcoholic cardiomyopathy= Options A. :ilated cardiomyopathy B. Mitral regurgitation C. 9ericardial effusion D. Increased ris! of arrhythmias E. Improvement 'ith thiamine replacement Teaching Notes for Question 2 Theme: 6ocar"ia, Disease %hronic alcohol abuse results in myocyte mitochondrial damage and a dilated cardiomyopathy. An elevated g- ; or M%- 'ould be suggestive of ethanol to3icity as a cause. his particular cardiomyopathy may be improved 'ith thiamine. As 'ith other dilated cardiomyopathies, mitral regurgitation and arrhythmias are common. Question 3 %auses of a reversed split second heart sound include" Options A. Right bundle branch bloc! 0R4442 B. &eft bundle branch bloc! 0&4442 C. Right atrial pacing D. Mild aortic stenosis E. -entricular septal defect Teaching Notes for Question 3 Theme: 75#8 #u,se8 an" 3eart %oun"s #5 is reversed split if at rest 95 occurs before A5. hen 'ith inspiration, as 95 is delayed, the gap bet'een the t'o components of #5 gets smaller. his may occur 'ith delay in A5, as 'ith severe aortic stenosis 0not mild2, left bundle branch bloc! and right ventricular pacing. Question $ A F5-year-old 'oman is admitted to hospital 'ith sudden onset right arm and leg 'ea!ness. #he 'as previously 'ell and independent ta!ing only bendroflua7ide for hypertension diagnosed 5 years previously. ,3amination reveals a right hemiparesis 'ith bris! refle3es and an e3tensor plantar on the right. #he has a soft mid systolic clic! on auscultation of her chest. here are * splinter haemorrhages on her left hand, and she has a cold 'hite left middle finger. %XR sho's cardiomegaly and is suggestive of left atrial enlargement. BM,s, +4% and clotting are normal but the ,#R is F0mmLhr. An urgent ,%68 reveals a mass in the left atrium. <hich of the follo'ing clinical features is not e3plained by this finding= Options A. ,mbolic %-A on brain % B. he mid-systolic clic! C. #ystemic emboli D. &eft atrial dilatation E. Raised ,#R Teaching Notes for Question $ Theme: Other Car"io,og My3omas in the heart ma!e up *0) of primary cardiac tumours. hey are most common in the left atrium arising from a pedicle on the fossa ovalis. 8n third present 'ith emboli, a third 'ith systemic inflammation 0,#R elevated in .LA2 and a third are asymptomatic 'hen detected. hey can intermittently flop through the mitral valve, and cause a mid-diastolic clic! 0tumour plop2 'hen they stop moving. ,levated left atrial pressures cause dilatation. #yncope can occur due to obstruction. hey are more common in 'omen. Question ' he follo'ing drugs enhance the effects of 'arfarin" Options A. 9henytoin B. Rifampicin C. %arbama7epine D. %imetidine E. 9ravastatin Teaching Notes for Question ' Theme: Car"io&ascu,ar #harmaco,og 9ravastatin does not interact 'ith 'arfarin, 'hereas simvastatin can theoretically raise the IER slightly by inhibiting the metabolism of 'arfarin. %imetidine, being a 94*0 en7yme inhibitor potentiates 'arfarin greatly. 9henytoin, rifampicin and carbama7epine are en7yme inducers and so reduce the effects of 'arfarin. Question * %oarctation of the aorta" Options A. Is more common in 'omen B. Is associated 'ith rib notching all .5 ribs on the left C. Is commonly associated 'ith pulmonary stenosis D. Rarely causes problems in paediatric life E. Is associated 'ith berry aneurysms Teaching Notes for Question * Theme: B,oo" 5esse, Disease %oarctation can cause heart failure in the neonate and hypertension in the adult. It is associated 'ith urner@s syndrome, neurofibromatosis, bicuspid aortic valve, and more 'ea!ly 'ith -#: and A#:. #tro!e may result from hypertension or from associated berry aneurysms. As 'ith all left heart obstructions, it is more common in males. Eotching of ribs AC/ is seen. Eotching or ribs . and 5 suggests inferior vena cava obstruction, a 4laloc! shunt or hypertrophied nerves. Question + A A5-year-old man is noted to have a systolic murmur at an insurance medical. 4lood pressure is normal. An echocardiogram sho's a bicuspid aortic valve. <hich of the follo'ing are recognised associations= Options A. %alcification of the valve in childhood B. riple vessel coronary disease in adulthood C. %oarctation of the aorta D. Marfan@s syndrome E. &eft main stem anomalous origin in 5) of cases Teaching Notes for Question + Theme: Congenita, 5a,&u,ar Disease 4icuspid aortic valve occurs in .C5) of the population. It may remain asymptomatic but can become stenotic or regurgitant 'ith time. It is associated 'ith a left dominant coronary circulation 0i.e. the posterior descending artery arises from the left circumfle3 rather than the right coronary artery2. *) of cases demonstrate significant coarctation of the aorta. %alcification does not occur until adult life, and unli!e calcific aortic stenosis of the old, coronary disease is very rare. Question . A 5/-year-old 'oman is 5A 'ee!s pregnant and referred for evaluation of a heart murmur. #he is asymptomatic. 8n e3amination she has triphalangeal thumbs. here is a prominent systolic murmur at the upper left sternal edge. ,%; sho's right a3is deviation, and incomplete R444. An echocardiogram sho's an A#:. <hich of the follo'ing statements is true= Options A. here is a recognised association bet'een A#: and triphalangeal thumbs B. #he should be advised to see! antibiotic prophyla3is for dental procedures C. he murmur is produced by associated pulmonary stenosis D. #he has a lo' ris! of developing pulmonary hypertension no' she is an adult E. R444 'ith a normal a3is is the usual ,%; finding Teaching Notes for Question . Theme: Congenita, 3eart Disease A#:s are the second commonest congenital heart disease seen in adults. hey are not benign, 'ith *0) death rate at age *0. %omplications rarely occur in childhood, but prolonged shunting leads to pulmonary hypertension, atrial arrhythmias, but not the ris! of endocarditis. A 9rimum defect causes R444 and &A:, 'hilst #ecundum causes R444 and RA: on the ,%;. he only murmur heard is a pulmonary flo' murmur, 'hich is not as pronounced as the murmur of pulmonary stenosis. #ecundum A#: is associated 'ith 6olt- 8ram syndrome, 'ith tri-phalangeal thumb and radial abnormalities. Question / he follo'ing are of aetiological significance in defining the cause of a cardiomyopathy" Options A. -'ave flattening in the inferior ,%; leads in a 10-year-old man B. he presence of a soft pan-systolic murmur in the mitral area C. A# of *0IBLl 'ith a bilirubin of .5mmolLl in a *0-year-old lady D. he presence of sinus tachycardia, 'ith 49 .40L/0 E. he presence of diabetes mellitus in a tanned patient Teaching Notes for Question / Theme: 6ocar"ia, Disease he commonest form of cardiomyopathy is dilated cardiomyopathy, 'ith dilatation of the left ventricle, and reduction in the eJection fraction. Eo cause is often found, although rarely, it can be familial. A cause should be loo!ed for in most cases. he commonest causes are ischaemic heart disease 0I-'aves on the ,%;, history of MI2, and hypertension. Eon-specific ,%; changes and atrial fibrillation are common and do not point to a specific cause. Minor abnormalities of liver function result from liver congestion. More mar!ed abnormalities and diabetes in a pigmented patient 'ould suggest haemochromatosis. Mitral regurgitation and arrhythmias are common in dilated cardiomyopathy of any cause. Question 10 Aortic regurgitation may be found in all the follo'ing e3cept" Options A. 4icuspid aortic valve B. 9rimary syphilis C. An!ylosing spondylitis D. %oarctation of the aorta E. <illiam@s syndrome Teaching Notes for Question 10 Theme: Ac!uire" 5a,&u,ar Disease %hronic aortic regurgitation 0AR2 can occur 'ith leaflet problems 0bicuspid aortic valve, calcific degeneration, <illiam@s syndrome2, or aortic root problems 0seronegative spondyloarthritidies, connective tissue diseases, aortitis2. Acute syphilis does not cause an aortitis and is not associated 'ith AR. Question 11 In hyperlipidaemia" Options A. riglycerides 0;2 .AmmolLl give a ris! of pancreatitis B. 9olygenic disorders are more common than monogenic C. #tatins are the agent of choice in secondary hyperlipidaemia D. &ipoprotein lipase 0&9&2 deficiency elevated &:& more than ; E. he liver function tests need to be measured monthly on statin therapy Teaching Notes for Question 11 Theme: Other Car"io,og A triglyceride level ..*mmolLl increases the ris! of pancreatitis. &o'er levels probably increase coronary ris! in association 'ith lo' 6:& levels. #econdary hyperlipidaemias need treatment of their cause 0e.g. nephrotic syndrome2 as 'ell as treatment of the lipid abnormality. <hen first mar!eted, there 'as concern about both rhabdomyolysis and hepatic dysfunction 'ith statin therapy. Routine measurements of %> or &+@s are not called for. &9& deficiency is a cause of elevated triglycerides more than of cholesterol, but polygenic disorders are much more common than monogenic ones. Question 12 In patients 'ith ischaemic heart disease 0I6:2 all the follo'ing are true e3cept" Options A. A fall in blood pressure on e3ercise testing suggests severe coronary disease B. he only finding on e3amination may be a fourth heart sound during an acute attac! C. :yspnoea after an attac! of angina is the hallmar! of poor left ventricular 0&-2 function D. Angiotensin converting en7yme inhibitor 0A%,-I2 therapy reduces myocardial infarction rates E. A positive troponin- 'ithout creatinine !inase 0%>2 rise diagnoses non-I 'ave myocardial infarction Teaching Notes for Question 12 Theme: E&i"ence Base" Car"io,og Ischaemic heart disease is often manifesting only 'ith the symptoms of angina. ,3amination may find predisposing factors 0e.g. hypertension2, or complications 0e.g. heart failure2, but in the absence of these, a fourth heart sound during an attac! of angina may be the only finding. ,ven before systolic &- dysfunction occurs, cardiac ischaemia produces diastolic dysfunction, causing impaired rela3ation and a stiff ventricle, creating the conditions for an #4 'ith atrial systole. he myocardium can become Gstunned@ 'ith ischaemia, so causing temporary &- systolic impairment and dyspnoea despite good resting &- function. he use of A%,- I is associated 'ith reduced future MI rates as sho'n in the 689, trial. he definition of non-I 'ave myocardial infarction has e3panded to include those patients 'ith unstable angina 'ho have had a rise in troponin- only. heir prognosis is as bad 'hether or not %> is also elevated. Question 13 A 10-year-old diabetic man undergoes an elective coronary angioplasty to relieve a 10) stenosis in his right coronary artery. A Amm by .*mm stent is inserted to produce an optimal angiographic result. <hich of the follo'ing adJunctive therapies 'ould reduced the ris! of restenosis= Options A. Angiotensin converting en7yme inhibitors B. 9ravastatin C. %lopidogrel D. &o'-molecular 'eight heparin E. Abci3imab in diabetic patients Teaching Notes for Question 13 Theme: Car"io&ascu,ar #harmaco,og Restenosis after 9%A is a problem in up to .LA of cases. Eo medication appears to eliminate the ris!, but there is some data to suggest that the use of the anti-platelet glycoprotein IIbLIIIa receptor bloc!er abci3imab 0Reo9roD2 may reduce the ris! or restenosis in diabetic patients. he use of stents definitely reduces the rate of restenosis. %lopidogrel reduces acute thrombosis ris!, but not restenosis. Question 1$ A A(-year-old 'oman presents 'ith breathlessness. 8n e3amination she is noted to have a 'ide split and fi3ed second heart sound. <hich of the follo'ing is the most li!ely diagnosis= Options A. +allot@s tetralogy B. %onstrictive pericarditis C. Aortic stenosis D. R444 E. #ecundum atrial septal defect Teaching Notes for Question 1$ Theme: 75#8 #u,se8 an" 3eart %oun"s he second heart sound is made up of the closure of the aortic valve 0A52, follo'ed by the closure of the pulmonary valve 0952. 95 is delayed on inspiration as there is more blood flo'ing into the right heart, hence physiological splitting. 95 is delayed even on e3piration if there is R444, getting 'ider still on inspiration, unless the R444 is accompanied by a connection bet'een the t'o atria, as in an atrial septal defect, hence 'ide fi3ed splitting. Question 1' -entricular septal defects" Options A. %ause heart failure on the first day of life B. :o not re$uire antibiotic prophyla3is if small C. Are associated 'ith aortic regurgitation D. Are associated 'ith a loud systolic murmur once ,isenmenger@s syndrome has occurred E. Are associated 'ith lithium e3posure in utero Teaching Notes for Question 1' Theme: Congenita, 3eart Disease -#:s are the commonest adult congenital heart disease. hey may close spontaneously, and if small cause loud systolic murmurs. his murmur disappears 'ith the onset of ,isenmenger@s syndrome as pressures e$ualise. <ith high pulmonary artery 09A2 pressures at birth, physical signs and the shunt are not pronounced. 6eart failure occurs 'ith the lo'ering of 9A pressure after a fe' 'ee!s. #ub-aortic -#:s are associated 'ith aortic regurgitation. &ithium e3posure during development is associated 'ith ,bstein@s anomaly.