About 30% of dilated cardiomyopathies are inherited. What modes of transmission are included?

1. Autosomal dominant
   
2. Autosomal recessive
   
3. X-linked
   
4. Mitochondrial
   
5. All of the above.

What is the most common mode of inheritance in dilated cardiomyopathy (DCM)?

1. Autosomal dominant
   
2. Autosomal recessive
   
3. X-linked
   
4. Mitochondrial

A 19-year-old patient has DCM with an ejection fraction (EF) of 10%, complete heart block, junctional escape rhythm with left bundle branch block (LBBB), and muscle weakness. What is the likely mutation in?

1. Lamin A/C gene
   
2. Troponin T gene
   
3. Desmin gene
   
4. Titin gene

Which of the following gene mutations can cause DCM?

1. Myosin heavy chain
   
2. Cardiac actin
   
3. Troponin T
   
4. Titin
   
5. Dystrophin
   
6. All of the above.

Which of the following is not a standard indication for anticoagulation in patients with dilated cardiomyopathy?

1. Male, 30 years, DCM, EF of 10%
   
2. Male, 30 years, DCM, EF of 25%, apical thrombus
   
3. Male, 30 years, DCM, EF is 20%, multiple transient ischemic attacks (TIAs), no thrombus seen in left ventricle or left atrium, aorta is clear, no patent foramen ovale
   
4. Male, 30 years, DCM, EF 35%, no left ventricular (LV) thrombus, no TIA or stroke, left ventricle is hypertrabeculated with noncompacted to compacted ratio of 2.5

Which of the following mutations may not cause hypertrophic cardiomyopathy (HCM)?

1. Myosin heavy chain
   
2. Myosin light chain
   
3. Troponin T
   
4. Desmin

Which of the following mutation can cause restrictive cardiomyopathy?

1. Myosin heavy chain
   
2. Cardiac actin
   
3. Troponin T
   
4. Troponin I

A 42-year-old man is diagnosed to have HCM with dynamic LV outflow tract (LVOT) obstruction. He has had two episodes of syncope, and maximum interventricular septal thickness is 30 mm. No genetic testing has been done. Which of the following statements is not accurate regarding screening?

1. It is appropriate to screen first-degree relatives with an echocardiogram
   
2. It is strongly recommended to screen first-degree relatives for high-risk genotypes
   
3. It is appropriate to perform a physical examination and an electrocardiogram (ECG) in first-degree relatives
   
4. It is appropriate to screen adult first-degree relatives every 5 years and children every 12–18 months.

Which of the following statements is inaccurate regarding genetic testing in HCM?

1. Evaluation of familial inheritance and genetic counseling is recommended as part of the assessment of patients with HCM
   
2. Patients who undergo genetic testing should also undergo counseling by someone knowledgeable in the genetics of cardiovascular disease so that results and their clinical significance can be appropriately reviewed with the patient
   
3. Genetic testing is reasonable in the index patient to facilitate the identification of first-degree family members at risk for developing HCM
   
4. Genetic testing is indicated in relatives when the index patient does not have a definitive pathogenic mutation as sporadic mutations may occur.

A 45-year-old man has HCM and found to have MHC gene mutation. The same mutation is found in one of his sons who is 20 years old. The son had a normal physical examination, ECG, and an echocardiogram. How frequently would you repeat an echocardiogram on this son?

1. No need to repeat
   
2. Every 12–18 months
   
3. Every 5 years
   
4. Only if symptoms occur or ECG becomes abnormal

Which of the following statements are accurate regarding performance of a 24-h Holter monitor in a patient with HCM?

1. Twenty-four-hour ambulatory (Holter) ECG monitoring is recommended in the initial evaluation of patients with HCM to detect ventricular tachycardia (VT) and identify patients who may be candidates for ICD therapy even if they have no palpitations or syncope
   
2. Twenty-four-hour ambulatory (Holter) ECG monitoring or event recording is recommended in patients with HCM who develop palpitations or lightheadedness
   
3. Twenty-four-hour ambulatory (Holter) ECG monitoring, repeated every 1–2 years, is reasonable in patients with HCM who have no previous evidence of VT to identify patients who may be candidates for implantable cardioverter-defibrillator (ICD) therapy
   
4. All of the above
   
5. None of the above

In a patient with known or suspected HCM, which of the following statements regarding echocardiography are accurate?

1. A transthoracic echocardiogram (TTE) is recommended in the initial evaluation of all patients with suspected HCM
   
2. A TTE is recommended as a component of the screening algorithm for family members of patients with HCM unless the family member is genotype negative in a family with known definitive mutations
   
3. Periodic (12–18 months) TTE screening is recommended for children of patients with HCM, starting by age 12 years or earlier if a growth spurt or signs of puberty are evident and/or when there are plans for engaging in intense competitive sports or there is a family history of sudden cardiac death (SCD)
   
4. All of the above

In a patient with known or suspected HCM, which of the following statements regarding cardiac magnetic resonance (CMR) imaging are accurate?

1. CMR imaging is indicated in patients with suspected HCM when echocardiography is inconclusive for diagnosis
   
2. CMR imaging is indicated in patients with known HCM when additional information that may have an impact on management or decision making regarding invasive management, such as magnitude and distribution of hypertrophy or anatomy of the mitral valve apparatus or papillary muscles, is not adequately defined with echocardiography
   
3. CMR imaging is reasonable in patients with HCM to define apical hypertrophy and/or aneurysm if echocardiography is inconclusive
   
4. In selected patients with known HCM, when SCD risk stratification is inconclusive after documentation of the conventional risk factors, CMR imaging with assessment of late gadolinium enhancement may be considered in resolving clinical decision making
   
5. All of the above.

Which of the following drugs may prolong survival in an asymptomatic HCM patient?

1. Beta blocker
   
2. Verapamil
   
3. Amiodarone
   
4. None of the above

Which of the following therapies are indicated in asymptomatic HCM patients with no outflow obstruction?

1. Beta blocker to reduce atrial fibrillation risk
   
2. Calcium channel blocker as positive lusiotropic agents to improve cardiac performance
   
3. Amiodarone to reduce risk of sudden death
   
4. None of the above
   
5. All of the above

A patient with hypertrophic obstructive cardiomyopathy (HOCM) has a resting LVOT gradient of 80 mmHg despite verapamil of 480 mg/day. His heart rate is 58 bpm, blood pressure (BP) 125/70 mmHg and he is still in class III symptoms and short of breath on minimal exertion. Which of the following would you do?

1. Add Nifedipine as a positive lusiotropic agent to improve LV diastolic function
   
2. Add small dose of frusemide to relieve pulmonary congestion
   
3. Consider dual-chamber pacer with short atrioventricular delay to cause asynchronous septal contraction and elimination of obstruction
   
4. Consider disopyramide as a negative inotropic agent

In a hypotensive patient with HOCM with high outflow gradient, in addition to intravenous fluids, which intravenous drug would be most useful?

1. Phenylephrine
   
2. Norepinephrine
   
3. Dopamine
   
4. Verapamil

Which of the following statements are accurate regarding septal reduction procedures in patients with HOCM?

1. Septal reduction therapy should be performed only by experienced operators in the context of a comprehensive HCM clinical program and only for the treatment of eligible patients with severe drug-refractory symptoms and LVOT obstruction
   
2. Surgical septal myectomy, when performed in experienced centers, can be beneficial and is the first consideration for the majority of eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction
   
3. When surgery is contraindicated or the risk is considered unacceptable because of serious comorbidities or advanced age, alcohol septal ablation, when performed in experienced centers, can be beneficial in eligible adult patients with HCM with LVOT obstruction and severe drug-refractory symptoms
   
4. All of the above

Which of the following markers may be associated with elevated SCD risk in HCM patients?

1. Family history of sudden death
   
2. Syncope
   
3. Nonsustained VT
   
4. Hypotension on exercise
   
5. LV wall thickness >30 mm
   
6. All of the above.

Which of the following markers may also be associated with elevated SCD risk in HCM patients?

1. Delayed enhancement on CMR imaging
   
2. Severe LVOT gradient
   
3. Double and compound mutations
   
4. All of the above
   
5. None of the above

Which of the following statements are accurate regarding use of an ICD for primary prevention in a patient with HCM?

1. An ICD can be useful in select patients with nonsustained VT (particularly those <30 years of age) in the presence of other SCD risk factors or modifiers
   
2. An ICD can be useful in select patients with HCM with an abnormal blood pressure response with exercise in the presence of other SCD risk factors or modifiers
   
3. It is reasonable to recommend an ICD for high-risk children with HCM, based on unexplained syncope, massive LV hypertrophy (LVH), or family history of SCD, after taking into account the relatively high complication rate of long-term ICD implantation
   
4. All of the above

A 42-year-old man presents with shortness of breath, tingling of fingers, vague abdominal pains, and blurred vision. He is hypertensive, creatinine is 2.4 mg/dL, and has corneal opacities. He has reduced touch sensations in hands and feet. An echocardiogram shows moderate LVH with normal wall motion. What is the likely diagnosis?

1. HCM
   
2. Hypertensive heart disease
   
3. Fabry disease
   
4. Hemochromatosis

A 52-year-old man presented with anginal sounding chest pain. The ECG showed increased voltage and deep T wave inversions V2 to V6. The cardiac enzymes were negative and the coronary angiogram was completely normal. What is the LV gram likely to show?

1. Apical akinesis
   
2. Severe LVH
   
3. Dyskinesis distal 2/3 of left ventricle
   
4. A spade-shaped left ventricle