his GP. He had a fatal cardiac arrest three weeks later. The post mortem showed a restrictive cardiomyopathy.
The first image shows cardiac histology stained with 'haematoxylin and eosin', the second stained with a specialized stain. Which of the following is the most likely diagnosis?
A : Hypertension
B : Hypertrophic obstructive cardiomyopathy
C : Ischaemic heart disease
D : Amyloidosis
E : Haemochromatosis.
Comment : The first image shows an amorphous eosinophilic substance within the specimen. The second image shows the classical apple green birefringence seen when the Congo red stain is viewed with polarised light. This is diagnostic of amyloid. Note the staining in the vessels as well as in the cardiac tissue. The man had no evidence of myeloma or lymphoma, but this was found to be an AL type of amyloid.The cardiac arrest was probably due to a dysrhythmia associated with amyloid rather than hypokalaemia. Coronary vessels were healthy for his age. D :
116. A 70-year-old man presents with shortness of breath that has developed over a few days.
He has no other symptoms. One week ago he returned on a transatlantic flight from a holiday in Florida. Which two investigations are most likely to reveal the diagnosis?
A : ECG
B : Ventilation-Perfusion isotope scanning
C : Echocardiogram
D : Serum troponin I
E : Chest radiograph
F : Spiral computed tomography scanning
G : Chest radiograph taken in expiration
H : Spirometry
I : Arterial blood gases
J : Ultrasound scanning of the leg veins.
Comment : The most likely diagnosis is pulmonary embolism, the two most specific tests for this being ventilation-perfusion isotope scanning and spiral computed tomography scanning.Ventilation scans are obtained using krypon-81m, technegas or xenon-133 and perfusion scans with intravenous 99m-Tc-labelled macroaggregates of albumin. Scans are reported as being normal or of low, medium or high probability, but it is very important to remember that reports must ALWAYS be interpreted in the light of the clinical context, and that a low probability scan does NOT mean that ‘pulmonary embolism is excluded’.Spiral computed tomography can detect intravascular clot from the pulmonary trunk down to the segmental arteries. It is the investigation of choice for patients with pre-existing lung disease, which renders the interpretation of ventilation-perfusion scans difficult or impossible. B : F:
117. A 39-year-old lady who who has a past history of treated hypertension is in her 3rd trimester of pregnancy and requires on-going hypertensive treatment. Which anti-hypertensive would you definitely not prescribe?
A : Hydralazine B : Labetalol C : Lisinopril D : Methyl-dopa E : Nifedipine.
Comment : Evidence underpinning the choice of anti-hypertensive therapy in pregnancy is inadequate to make firm recommendations. There are no reports of serious effects with methyl-dopa following long and extensive use. Calcium antagonists, labetalol and hydralazine are commonly used, particularly for resistant hypertension in the third trimester. However, angiotensin-converting enzyme (ACE)-inhibitors should be avoided because they may cause oligohydramnios, renal failure and intra-uterine death. C : 118.
A 28-year-old woman was referred for the investigation of hypertension. Routine examination demonstrated that she had absent pulses in the left arm. She was a non-smoker. An arch aortogram was performed (see image). Which of the following statements is correct?
A : She has coarctation of the aorta which is the likely cause of her hypertension.
B : The selective picture shows a severe left subclavian stenosis most probably due to atherosclerosis
C : The study is normal and she most likely has essential hypertension D : The selective picture shows a severe left subclavian stenosis most probably due to Takayasu's arteritis E : She has hypertension due to renovascular disease.
Comment : Hypertension in the young always needs investigation. The physical findings in this woman were highly suggestive of a large vessel disease process.The selective angio picture does not show coarctation but does reveal critical stenosis of the left subclavian artery,which is most likely to be due to Takayasu's arteritis in this case.Atherosclerosis would be unusual in a young non-smoker.Hypertension is probably driven by renal ischaemia due to either direct renal vessel involvement or indirectly by aortic narrowing (not seen on these pictures). Raised erythrocyte sedimentation rate (ESR) is typical of the active inflammatory phase of this illness; in Japan there have been reports of linkage with tuberculosis - steroids and other immunosuppressants can be effective. D :
119. A 20-year-old female student presents with central chest pain after four days of a ‘flu-like illness. The most likely diagnosis is:
A : Acute viral pericarditis
B : Gastro-oesophageal reflux
C : Acute myocardial infarction
D : Systemic lupus erythematosus
E : Pulmonary embolism.
Comment : The combination of a young patient and a ‘flu-like illness make acute viral pericarditis the most likely diagnosis in this case. The chest pain of pericarditis can be indistinguishable from that of myocardial infarction, excepting that sitting forward often eases it.
The key physical sign to elicit would be a pericardial rub, and the key initial investigation would be the ECG, looking for widespread ST segment elevation, concave upwards. A :
120. Which of the following is not associated with cocaine abuse?
A : Hypotension
B : Contraction band necrosis
C : Dilated cardiomyopathy
D : Acceleration of atherosclerosis
E : Aortic dissection.
MOHAMMED IS-HAG
Comment : Cocaine is associated with both acute and long-term cardiovascular disorders. Most commonly it is associated with myocardial ischaemia secondary to coronary spasm. Coronary spasm usually responds to nitrates, calcium antagonists or alpha blockers. Beta-blockers should be used with caution. A :
121. A 76-year-old lady is referred to the outpatient clinic with a 3-week history of new onset angina. In clinic she complains of chest pain at rest and is found on examination to have bilateral basal crepitations, a third heart sound and a pansystolic murmur. A 12 lead electrocardiogram shows ST depression of > 2 mm in her anterior leads. Which of the following are correct?
A : There is a higher risk of death with unstable angina than an acute myocardial infarction (MI) B : Short episodes of chest pain are markers of high risk unstable angina C : The finding of congestive cardiac failure has no prognostic signifigance D : Only ST depression of 2mm or more is of prognostic signifigance
E : A new or worsening MR murmur is a high risk factor for death from unstable angina.
Comment : In patients with unstable angina the risk of death is lower than with an acute MI.
Prolonged episodes of severe chest pain are important risk factors of high-risk unstable angina Any evidence on physical examination of acute congestive heart failure or a new or worsening MR murmur place the patient at increased risk of death.
Dynamic shifts in ST segment depression or elevation of >1mm or T wave inversions that resolve when symptoms are relieved are of prognostic significance. E :
122. A 70 year old woman presents with 8 hours of chest pain. Her pulse rate is 40/minute and blood pressure 105/85. The ECG shows complete heart block, ST segment elevation and Q waves in leads II, III and AVF. Which of the following statements is correct?
A : Atropine should be given immediately.
B : An isoprenaline infusion should be set up immediately.
C : Thrombolysis should be given immediately.
D : Thrombolysis should be avoided because she has completed her myocardial infarction.
E : Thrombolysis should be avoided because she may require a temporary pacing wire.
Comment : The top priority is to achieve myocardial reperfusion. The presence of chest pain and ST segment elevation, despite Q waves, on ECG indicate that thrombolysis is needed immediately. C : 123. A 37-year-old man presents with recurrent episode of atrial fibrillation. He is having
approximately three episodes each week, lasting for up to 5 hours. He has previously been treated with flecainide, sotalol and amiodarone. Which of the following is appropriate in his management?
A : Consider digoxin
B : Consider AV node ablation and pacemaker implantation
C : Consider him for an implantable cardioverter defibrillator (ICD) D : Consider radiofrequency ablation to isolate his pulmonary veins
E : None of the above.
Comment : He is spending a significant proportion of his time in atrial fibrillation (high atrial
fibrillation burden). However, it is paroxysmal and therefore digoxin is inappropriate and may increase the number of episodes.AV node ablation should not be considered in a young patient with paroxysmal symptoms. Generally this is reserved for patients in chronic atrial fibrillation resistant to drug treatment.
An atrial defibrillator may be appropriate but certainly not an ICD used to treat malignant ventricular arrhythmias.Pulmonary vein isolation for atrial fibrillation is an evolving technique and may be appropriate for this patient. D :
124. In a patient with resistant hypertension, which of the following would increase the degree of clinical suspicion for significant renovascular disease as aetiology?
A : Arterial bruits
B : Co-morbidity with diabetes
C : Concentric left ventricular hypertrophy
D : Grade III hypertensive change on fundoscopy
E : Palpable kidney.
Comment : The highest degree of clinical suspicion of renovascular disease will occur in patients with arterial bruits, discrepancy in renal size of >1.5 cm (on ultrasound) and co-morbid vascular disease. If a patient has none of these then renovascular disease is highly unlikely,
Evidence of end-organ damage is not helpful in differentiating aetiology. A :
125. A 60-year-old woman develops hypotension and a new systolic murmur 36 hours after being successfully thrombolysed for an anterior myocardial infarction. Which of the following statements is correct?
A : Acute mitral incompetence due to rupture of the posterior papillary muscle is the most likely diagnosis.
B : Acute mitral incompetence due to rupture of the anterior papillary muscle is the most likely diagnosis..
C : A basal ventricular septal defect (VSD) is the most likely diagnosis.
D : An apical ventricular septal defect is the most likely diagnosis.
E : The systolic murmur is likely to be due to mitral valve prolapse.
Comment : Anterior myocardial infarction is typically associated with apical VSDs whilst inferior myocardial infarctions are more commonly associated with basal VSD or posterior papillary muscle rupture. After confirmation of diagnosis by echocardiography or right heart catheter, which reveals a step up in oxygenation at ventricular level, urgent referral to a surgical centre is required, the outlook without surgical repair being extremely poor.Anterior myocardial infarction associated with apical VSD carries a better surgical outlook than inferior myocardial infarction associated with basal VSD. D : 126. A 72-year-old man on the coronary care unit has had a temporary transvenous pacing wire inserted. He suddenly becomes symptomatic with pre-syncope and a palpable pulse of 28 bpm.
The ECG monitor shows pacing spikes that are not related to the QRS complexes. Which of the following actions is appropriate?
Comment : If a patient with a temporary pacemaker becomes symptomatic it is important to check that the pacemaker is switched on and that the leads are still connected. The presence of pacemaker spikes on the ECG confirms that these are okay. It is likely that the tip of the pacing wire has migrated and that the threshold has increased. This may be overcome by increasing the voltage. If this is successful, the pacemaker wire will need to be repositioned. If inceasing the voltage is unsuccessful, external pacing should be substituted. E :
127. A 35-year-old lady with a long history of migraine presented with a mild right hemiparesis.
She made a full recovery after 4 days but subsequent investigation revealed a patent foramen ovale (PFO). Paradoxical thromboembolism through a PFO is an important cause of stroke. Which of the following statements regarding PFO and closure of PFO is incorrect?
A : Percutaneous closure of PFO is safe with a low incidence of recurrent neurological events.
B : Divers with decompression sickness may warrant PFO closure.
C : Spontaneous right to left shunting through a PFO indicates high risk.
D : Closure of PFO in patients with recurrent migraine with aura should be considered.
E : A three to five-fold higher prevalence of PFO is noted in patients with cryptogenic stroke.
Comment : PFO occurs in approximately 20-30% of the population. Percutaneous closure of a PFO using a variety of closure devices is now an accepted practice in certain populations and in certain centres. All of the above are correct apart from D. A number of studies have suggested a correlation between migraine and PFO. However, there is currently insufficient evidence to support closure in these patients. D :
128. You find a middle aged man on a path in a park. He has no pulse and is not breathing.
Which is the appropriate next step:
A : Give two rescue breaths and initiate CPR at a ratio of 5 compressions to 2 breaths B : Give two rescue breaths and initiate CPR at a ratio of 15 compressions to 2 breaths
C : Give a precordial thump
D : Give two rescue breaths and go for help.
E : Go to call 999 (emergency services) immediately.
Comment : The guidelines on basic life support identify the importance of early access to defibrillation in cardiac arrest. They therefore suggest that no CPR is commenced until a call for
emergency services has been made and the potential for early defibrillation is made possible. Once CPR begins the ratio of compressions to ventilations is 15 to 2. A precordial thump is not indicated in the unwitnessed collapse. E :
129. A 43-year-old woman with pulmonary hypertension attends clinic and asks to be prescribed bosentan. Which of the following is true?
A : It is licensed for use in all patients with pulmonary hypertension.
B : It inhibits the effects of endothelin-2 (ET-2).
C : It binds to both endothelin A (ET-A) and endothelin B (ET-B) receptors.
D : It is excreted primarily unchanged in the urine.
E : It is not effective in patients with scleroderma.
Comment : Bosentan is an antagonist of endothelin-1 binding to ET-A and ET-B receptors. It is licensed for the treatment of 'pulmonary arterial hypertension (PAH) to improve exercise capacity and symptoms in patients with grade III functional status'. It has been shown to be effective in patients with primary PAH and in those with PAH secondary to scleroderma. It is excreted in bile following metabolism by the cytochrome P450 enzymes and this is a potential source of interaction with drugs metabolised by the same isoenzyme (e.g. glibenclamide, ritonavir, ketoconazole, ciclosporin and itraconazole). C:
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130.
PLATE 14
A 62-year-old man presents with chest pain. What two features does his ECG show (see image)?
A : Posterior myocardial infarction
B : Atrial fibrillation
C : Anterior myocardial infarction
D : Right axis deviation
E : Unstable angina
F : Ventricular ectopic beats
G : Inferior myocardial infarction
H : Atrial ectopic beats
I : Left axis deviation
J : Left bundle branch block.
Comment : This ECG shows the classical appearances of acute myocardial infarction with ST segment elevation. This is seen in leads V1-V5, indicating that the infarct is anterior.
The rhythm strip shows a number of ectopics, of which there is a compensatory pause, indicating that they are ventricular in origin.C:F:
131.
This patient presented acutely unwell with the following ECG (see image). What is the correct diagnosis?
A : Hyperkalaemia
B : Ventricular tachycardia
C : Acute pericarditis
D : Acute anterolateral myocardial infarction
E : Digoxin toxicity.
Comment : The presence of tall, peaked T waves, flattened P waves, prolonged PR interval and wide QRS complexes are pathognomonic of hyperkalaemia. Give IV calcium immediately (10mls, 10% calcium gluconate) and call the renal team. A:
132.
A 30-year-old male with a heart murmur and breathlessness on effort is referred for transthoracic echocardiogram (see image). Which of the following is true of his condition?
A : Sudden death is very common.
B : Exercise testing is contraindicated.
C : Atenolol may help his symptoms.
D : Frusemide will help his shortness of breath.
E : The ECG is always abnormal.
F : It is very rare in elderly patients over 75 years of age.
G : The condition can be confidently diagnosed in patients over 10 years pf age.
H : Ischaemia may occur with a normal coronary arteriogram.
I : Endocarditis prophylaxis is not required.
J : Prophylactic amioderone is mandatory to prevent sudden death.
Comment : The above patient has hypertrophic cardiomyopathy with marked asymmetrical septal hypertrophy and a resting outflow tract gradient of 55mmHg.The ECG may be normal in 25% cases and morphological expression of the disease may not be completed until the end of puberty.Twenty -five per cent of all cases occur in those over 75.Sudden death occurs in 1% cases and those considered high risk should be referred for an implantable defibrillator.Exercise testing is extremely useful at identifying those patients with dynamic outflow tract gradients who would benefit from more aggressive
therapy.Surgical myectomy or percutaneous alcohol septal ablation is reserved for those with high (>
50mmHg) resting gradients with refractory symptoms despite optimal medical treatment. C:H:
133. A 72-year-old Caucasian woman in your outpatient clinic has uncomplicated essential hypertension. Her blood pressure is 162/102mmHg despite optimization of non-pharmacological therapy. Which one of the following treatments would you choose as the first-line agent for her?
A : Atenolol 50 od
B : Bendrofluazide 2.5mg od
C : Bendrofluazide 5mg od
D : Enalapril 5mg od
E : Ramipril 2.5mg od.
Comment : Hypertension is particularly common in those aged above 60 not least because of the steady rise in systolic blood pressure with age. These patients are at a high absolute risk of
cardiovascular complications. Furthermore, anti-hypertensive treatment may also reduce incidence of heart failure and possibly dementia.
Non-pharmacological therapy for hypertension should always be optimised prior to commencement of medication, whenever possible. Low-dose diuretics are accepted as the first-line treatment for
hypertension in the elderly and appear to confer greater benefit than Beta-adrenergic receptor
antagonists in this subgroup. 1. Treatment of isolated systolic hypertension in the elderly with the long-acting calcium channel blocker, nitrendipine, has been shown to reduce stroke and cardiovascular outcome. Therefore, calcium channel blockers may be suitable when diurectics are not tolerated, ineffective or contra-indicated.2 B:
MOHAMMED IS-HAG
134.
A 35-year-old man is admitted with chest pain. His ECG and cardiac enzymes are normal. Because a murmur is heard he has a transesophageal echocardiography (TOE) (see image). Which of the following is true
concerning the disorder shown? AoA is ascending aorta, AoD is descending aorta.
A : Medical treatment is the best long term option.
B : The most likely cause of the murmur is mitral regurgitation.
C : Methyldopa is contraindicated in the initial management.
D : The diagnosis can usually be made as easily with transthoracic echo.
E : CT scan of the chest is usually superior to TOE in making the diagnosis.
F : Pregnancy is protective from condition.
G : Atherosclerosis is the most likely underlying cause of the condition in this case.
H : Untreated overall prognosis is good.
I : Paraplegia is a recognized complication.
J : Persistent abdominal pain may be a worrying feature.
Comment : The TOE images demonstrate a Type A aortic dissection with an intimal flap seen in the ascending and descending aorta. The most likely cause for his murmur is aortic regurgitation or co-existent bicuspid aortic valve. Untreated surgically, mortality is high so early referral to a cardiothoracic unit is crucial. Type B dissections involving the descending aorta only are usually treated medically in the first instance as the complications of surgery are high.TOE,MRI and CTchest have similar sensitivities and specificities in making the diagnosis.Methyldopa may be used in the initial management to control blood pressure. When the descending aorta is involved persistent abdominal pain may indicate
involvement of the mesenteric arteries. Pregnancy is a rare but recognized cause. In young patients atherosclerotic disease is rarely the cause. I:J:
135.
PLATE 15