Which one of the following best describes the actions of ACE?
ACE is produced by vascular endothelial cells in the pulmonary vasculature (and systemic vascular endothelium). It converts angiotensin I to the active angiotensin II and promotes the production of bradykinin. An ACE inhibitor blocks the formation of angiotensin II and provides survival benefit in patients with LV systolic dysfunction. Angiotensin II stimulates the production of aldosterone and norepinephrine.
Which one of the following is not a contraindication to an ACE inhibitor?
TTE suggests moderate aortic stenosis, not severe AS, which is not a contraindication to starting an ACE inhibitor. All other answers are contraindications to starting an ACE inhibitor.
A 40-year-old man with known hypertrophic cardiomyopathy presents to the outpatient clinic with a history of increased breathlessness. He has noted a marked reduction in his exercise tolerance over the last 6 months and it is now limited to 100 yards despite his being commenced on bisoprolol. Clinical examination demonstrates a forceful apex and a mid-systolic murmur. There is no evidence of fluid overload. Echocardiography demonstrates asymmetric left ventricular hypertrophy with a septal thickness of 20 mm. There is a resting left ventricular outflow tract gradient of 60 mmHg.
What is the most appropriate management for this patient?
Septal ablation should be considered for relieving outflow tract obstruction with hypertrophic cardiomyopathy in patients with marked breathlessness (NYHA class III or IV) refractory to medical therapy. Although a change in medical therapy may help (e.g. addition of disopyramide), patients with a peak resting or exercise-induced gradient ≥50 mmHg should be referred to a specialist centre. Although there is some data to support the use of dual-chamber pacing, the overall consensus is that this should only be considered in certain subgroups, e.g. elderly patients or those at high operative risk. ICD implantation is used only to reduce the risk of sudden cardiac death in high risk patients.
A 25-year-old woman is referred with a mid-systolic murmur. Echocardiography demonstrates asymmetric left ventricular hypertrophy with good left ventricular systolic function. The septal thickness is 17 mm with a small left ventricular outflow tract gradient. She is symptom free.
Which one of the following statements is not true?
. Young patients with no symptoms and no risk factors for sudden cardiac death do not require medical therapy. They should however be risk stratified with annual exercise testing to look for evidence of exercise induced arrhythmia (VT or AF) and a <20mmHg rise in blood pressure. Annual 24 hour Holter monitoring should also be performed to look for VT (>3 beats at a rate of >120bpm) or AF. AF is more common in the HCM population and should be treated medically (beta-blockers, verapamil or Amiodarone) and formal anticoagulation. Pregnancy is safe in patients without symptoms and usually culminates in a normal vaginal delivery. However, care should be delivered in a joint cardiac obstetric clinic. First degree relatives should be screened with ECG and echocardiography. Due to cases of late presentation of HCM, these tests should be repeated if normal.
A 53-year-old man presents to the outpatient clinic with symptoms of lethargy and tiredness. Clinical examination reveals him to be pale with a blood pressure of 110/70 mmHg, a JVP of +8 cmH2O, and oedema to his mid-calf. His 12-lead ECG demonstrates a PR interval of 200 ms, a QRS duration of 145 ms, and poor R-wave progression. A subsequent echocardiogram was technically challenging, but demonstrated a thickened ventricle with a septal thickness of 15 mm. Overall systolic function is reported as normal. An E/A ratio was estimated to be 1.4 with tissue Doppler giving an E/E' ratio of 12.
Which one of the following investigations is most likely to help make the diagnosis?
. Clinical features of right heart failure and a thickened left ventricle with evidence of restrictive filling (reversed E/A ratio and raised E/E’) is suggestive of a restrictive cardiomyopathy. The presence of conduction disease and small QRS complexes in the context of echocardiographic evidence of left ventricular hypertrophy suggests amyloid deposition. Although demonstrating a plasma cell dyscrasia increases the likelihood of AL amyloid, the presence of cardiac amyloid can be demonstrated by endomyocardial biopsy. Myocardial perfusion imaging only demonstrates the presence of coronary artery disease. A left and right heart catheter can show restrictive physiology but not its aetiology
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