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Cardiomyopathies

Total questions: 24

Worksheet time: 12mins

Name
Class
Date
1.

An 18-year-old man is evaluated for a medical fitness examination for entry to the college. He has no history of serious illness. Vital signs are within normal limits. Apart from a grade 3/6 systolic ejection crescendo-decrescendo murmur at the lower left sternal border, no other abnormality was detected. The murmur decreases in intensity on rapid squatting and increases when he performs the Valsalva manoeuvre. This patient is at increased risk for which of the following complications?

a)

Infective endocarditis

b)

Sudden cardiac death

c)

Paradoxical embolism

d)

Cerebral aneurysm

2.

A 19-year-old boy presented 20 minutes after collapsing while playing basketball. There is no personal or family history of serious illness. On arrival, there is no palpable pulse and no respiratory effort is seen. He is declared dead. The family agrees to an autopsy. Which of the following is most likely to be found in this patient?

a)

Interventricular septal hypertrophy

b)

Pericardial fluid collection

c)

Enlarged descending aorta

d)

Embolus in the pulmonary artery

3.

A previously healthy 25-year-old man presents 30 minutes after collapsing during soccer practice. His father died of sudden cardiac arrest at the age of 36 years. The patient has normal vital signs. Cardiac examination is shown. An ECG shows large R waves in the lateral leads and deep S waves in V1 and V2. Further evaluation is most likely to show which of the following?

a)

Aortic root dilatation

b)

Eccentric left ventricular dilation

c)

Aortic valve calcification

d)

Asymmetric septal hypertrophy

4.

A 65-year-old man is presented after A road traffic accident with loss of consciousness. Despite appropriate life-saving measures, he dies 2 hours later because of a severe intracranial haemorrhage. Autopsy of the heart shows a general thickening of the left ventricular wall with narrowing of the ventricular chamber. Which of the following conditions is the most likely underlying cause of the described cardiac changes?

a)

Aortic regurgitation

b)

Chronic heavy drinking

c)

Myocardial ischemia

d)

Chronic hypertension

5.

A 15_year_old boy is diagnosed with HOCM. Which of the following is the most appropriate treatment to reduce the risk of premature death in this patient?

a)

Cardiac pacemaker implantation

b)

Verapamil therapy

c)

Automated implantable cardioverter-defibrillator placement

d)

High-dose furosemide therapy

6.

A previously healthy 35-year-old man i presented 30 minutes after collapsing at home, with failed CPR. A photograph of sections of the heart obtained at autopsy is shown. The patient's condition is most likely associated with which of the following pathophysiologic changes?

a)

Severe narrowing of the left anterior descending artery

b)

Marked noncompliance of the left ventricular wall

c)

Fixed obstruction of the left ventricular outflow tract

d)

Significantly reduced myocardial contraction force

7.

A 20 year old man collaposed dead and diagnosed by autopsy as HOCM. Cardiac workup prior to this patient's death would most likely have shown which of the following findings?

a)

Systolic anterior motion of the mitral valve

b)

Narrowing of the left main coronary artery

c)

Bicuspid aortic valve

d)

Ventricular septum defect

8.

A 71-year-old man with hypertension is evaluated at a routine follow-up examination. Cardiovascular exam shows the point of maximal impulse to be in the mid-axillary line. A transthoracic echocardiogram shows concentric left ventricular hypertrophy with a normal right ventricle. Which of the following is the most likely underlying mechanism of this patient's ventricular hypertrophy?

a)

Infiltration of T lymphocytes

b)

Accumulation of sarcomeres in parallel

c)

Deposition of endomyocardial collagen

d)

Accumulation of glycogen

9.

A 65-year-old patient presents with increased shortness of breath for 3 months. His symptoms are particularly bad at night. Medical history includes long-standing hypertension and alcoholism. Examination shows a displaced apex beat and normal breath sounds. Cardiac auscultation shows an S3 gallop and a pan-systolic murmur radiating to the axilla. The chest X-ray is shown below. Which of the following is the most likely diagnosis?


Elimination tool

a)

Myocarditis

b)

Hypertrophic cardiomyopathy

c)

Dilated cardiomyopathy

d)

Infective endocarditis

10.

A 30-year-old man comes to the family medicine clinic for an annual physical. On auscultation, a systolic murmur is heard over the left lower sternal border. The murmur is accentuated when the patient stands up from a squatting position and with the valsava maneuver. The murmur decreases in intensity when the patient lays supine. The patient's pulmonary capillary wedge pressure is within normal limits. Which of the following diagnosis is most likely in this patient?

a)

Mitral regurgitation

b)

Hypertrophic cardiomyopathy

c)

Aortic stenosis

d)

Aortic dissection

11.

A 68-year-old woman presents with dyspnea on exertion, fatigue, and chest pain. Past medical history includes carcinoma of the breast, for which she received surgery and adjuvant radiation therapy. Her vitals were stable with a raised JVPand bilateral leg edema. Cardiac auscultation shows S3 and S4 heart sounds. Electrocardiogram (ECG) shows low amplitude signals. Which of the following is the most likely cause of this patient’s disease?

a)

Myocardial iron deposition

b)

Granulomatous disease

c)

Myocardial fibrosis

d)

Myocardial amyloid deposition

12.

A 27-year-old man presents after a syncopal episode. On inspection of the neck veins he has a prominent 'a' wave. On auscultation of the heart, there is a harsh crescendo-decrescendo systolic murmur that is heard best at the apex and lower left sternal border.


Which of the following medications should be avoided in this patient?

a)

ACE inhibitors

b)

Beta blockers

c)

Nondihydropyridine calcium channel blockers

d)

Warfarin

13.

A 25-year-old male who has arrived in cardiac arrest. He collapsed while playing football. His father died suddenly in his 30s and they were later told he had hypertrophic obstructive cardiomyopathy (HOCM).

Assuming the diagnosis is the same, which of the following is likely to confer the poorest prognosis?

a)

Increased blood pressure during exercise

b)

Atrial fibrillation on 24 hour holter monitoring

c)

Left ventricular wall thickness 25mm

d)

Genetic mutation in troponin T

14.

Which of the following is least associated with a poor prognosis in hypertrophic cardiomyopathy?

a)

Family history of sudden death

b)

Non-sustained ventricular tachycardia on 24 or 48-hour Holter monitoring

c)

Reduced left ventricular outflow gradient

d)

Syncope

15.

A 44-year-old man presents with 6 months history of episodes of palpitations associated with pre-syncopal symptoms. An ECG taken in clinic shows T wave inversion in leads V1-3 associated with a notch at the end of the QRS complex. He is known to have a family history of sudden cardiac death. What is the most likely diagnosis?

a)

Long QT syndrome

b)

Hypertrophic obstructive cardiomyopathy

c)

Arrhythmogenic right ventricular cardiomyopathy

d)

Brugada syndrome

16.

A 22-year-old man collapsed dead and is feeling that his heart beating very fast and then collapsed. During post mortem, the right ventricle was noticed to be thin-walled and histology of the right ventricle revealed fibro-fatty infiltration.


What is the most likely cause of death in this case?

a)

Hypertrophic cardiomyopathy

b)

Wolff-Parkinson-White syndrome

c)

Arrhythmogenic right ventricular cardiomyopathy

d)

Brugada syndrome

17.

Which one of the following cardiac conditions is most associated with a louder murmur following the Valsalva manoeuvre?

a)

Mitral stenosis

b)

Aortic stenosis

c)

Hypertrophic obstructive cardiomyopathy

d)

Aortic regurgitation

18.

A 34-year-old man presenrs with increasing dyspnoea and exercise-related syncope. His father died suddenly when at the age of 42-years-old. His ECG shows left ventricular hypertrophy with widespread T wave inversion. Given the likely diagnosis, what is the most appropriate next investigation?

a)

Transthoracic echo

b)

Transoesophageal echo

c)

24-hour ECG

d)

Exercise ECG

19.

A 28-year-old man with hypertrophic obstructive cardiomyopathy is investigated for palpitations. A 24 hour ECG reveals runs of non-sustained ventricular tachycardia. What is the most appropriate management?

a)

Implantable cardioverter defibrillator

b)

Sotalol

c)

Amiodarone

d)

Accessory pathway ablation

20.

A 45-year-old man with a background of hypertrophic cardiomyopathy (HOCM) attends for review at the cardiology clinic. During the examination, a fourth heart sound is heard.


Which of the following is a feature of this clinical finding?

a)

It coincides with the T wave of the ECG

b)

It occurs in early diastole

c)

It coincides with the P wave of the ECG

d)

It is a common finding in patients with atrial fibrillation

21.

A 23-year-old man with a family history of sudden cardiac death is diagnosed as having hypertrophic obstructive cardiomyopathy. Which one of the following is the strongest marker of poor prognosis?

a)

Septal wall thickness of > 30 mm

b)

Asymmetric hypertrophy

c)

Mitral regurgitation

d)

Systolic anterior motion of the anterior mitral valve leaflet

22.

A 17-year-old female presents with recurrent attacks of collapse. These episodes typically occur without warning and have occurred whilst she was running for a bus. There is no significant past medical history and the only family history of note is that her father died suddenly when he was 38-years-old. What is the likely cause?

a)

Vaso-vagal attacks

b)

Anxiety

c)

Epilepsy

d)

Cardiogenic syncope

23.

A 21-year-old keen sportsman collapses dead. Two other family members have died young in similar ircumstances.


Which of the following methods of inheritance is correct for this condition?

a)

Autosomal dominant

b)

Autosomal recessive

c)

X-linked recessive

d)

X-linked dominant

24.

The most common cause of restrictive cardiomyopathy in the UK is:

a)

Diabetes mellitus

b)

SLE

c)

Haemochromatosis

d)

Amyloidosis