Q1:
A
12-year-old boy wishes to participate in competitive sports. His father died
suddenly at age 28 years, and hypertrophic cardiomyopathy was found on autopsy.
Of
the following, the MOST helpful test for assessing the boy's risk is:
A. echocardiography
B. electrocardiography
C. exercise
myocardial perfusion scintigraphy
D. genetic
testing for myosin chain mutations
E. genetic
testing for troponin mutations
Answer
A
Exercise myocardial perfusion scanning may have
a role in risk stratification for patients who have hypertrophic cardiomyopathy
because exercise-related myocardial ischemia appears to be one of the
mechanisms for syncope, chest pain, and sudden death in affected patients.
However, perfusion scanning cannot be used to screen for the presence of
hypertrophic cardiomyopathy
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Q 2
Answer
A
The
dominant inheritance of many forms of hypertrophic cardiomyopathy is the
rationale for screening examinations of family members of patients who are
diagnosed with these disorders. Furthermore, a positive family history of
sudden death at a young age from hypertrophic cardiomyopathy, such as described
for the boy in the vignette, is a significant risk factor for premature or
sudden cardiac death in patients who have hypertrophic cardiomyopathy.
Echocardiography is the most commonly employed screening tool for individuals
who have such a positive history.
Electrocardiography
results may be very abnormal in adolescents or children who have hypertrophic
cardiomyopathy, but electrocardiographic findings may be normal until
echocardiography results are abnormal for a longer period of time.
-----
Q 2
A
12-year-old boy had tetralogy of Fallot repaired at age 2 years. Right
ventricular outflow obstruction was corrected with a patch across the pulmonary
valve annulus. Now he complains of exercise intolerance and fatigue.
Of
the following, the MOST likely echocardiographic finding in this patient is
significant:
A. atrial
left-to-right shunting
B. atrial
right-to-left shunting
C. pericardial
thickening
D. right
ventricular diastolic volume elevation
E. right
ventricular systolic pressure elevation
Answer
D
---------------------------
Q 3:
Answer
D
The
functional status of cases years after successful repair
of tetralogy of Fallot can be excellent. Nevertheless, significant problems
with right ventricular function may occur, the most serious of which is the
development of episodic rapid ventricular tachycardia arising from the right
ventricle. In some instances, sudden death has been attributed to this
complication.
Repaired tetralogy of Fallot invariably results in some degree of
either outflow obstruction to the right ventricle with right ventricular
hypertension or abnormal pulmonary valve incompetence resulting in a volume
overload to the right ventricle. During ventricular diastole, the normal right
ventricular filling through the tricuspid valve is augmented by regurgitation
of blood back into the right ventricle from the pulmonary arteries. Often there
is some element of both right ventricular pressure and volume elevation.
---------------------------
Q 3:
A
7-year-old girl presents with chorea and a new murmur of mild mitral valve
regurgitation.
Of
the following interventions, chronic disability is MOST likely to be prevented
by:
A. intramuscular
penicillin monthly
B. intravenous
penicillin for 6 weeks
C. oral
haloperidol for 6 weeks
D. oral
prednisone for 4 weeks
E. oral
salicylates for 6 weeks
Answer
A
Answer
A
The
most important therapeutic intervention for the patient who has rheumatic fever
is chronic antibiotic prophylaxis to prevent group A streptococcal pharyngitis,
which could lead to recurrence of acute rheumatic fever. Serious cardiac damage
is unusual after an initial attack of acute rheumatic fever, but it becomes
more likely with subsequent attacks.
Administration of intramuscular benzathine
penicillin every 21-28 days affords better protection against streptococcal
infection than any oral regimen, perhaps largely due to the ability to monitor
and ensure compliance with the regimen. Rheumatic fever prophylaxis must
continue at least into adult life after rheumatic heart disease has occurred.
Intravenous
penicillin for 4 to 6 weeks is a potential regimen for the treatment of
streptococcal bacterial endocarditis caused by the various Lancefield
nontypeable strains of Streptococcus collectively termed S viridans.
Haloperidol
has been used in some cases of Sydenham chorea to treat the involuntary
movements, but the neurologic sequelae of the chorea associated with rheumatic
fever should resolve. Moreover, treatment of the movement disorder has not been
shown to have any specific role in preventing permanent sequelae.
-----------------------
Q4
-----------------------
Q4
You
are seeing a 4-month-old male infant who has a history of Tetralogy of Fallot.
The infant underwent palliation with a systemic-to-pulmonary shunt
(Blalock-Taussig shunt) at 3 months of age because of persistent cyanosis. He
is scheduled for complete repair at 8 months of age. His mother wants to know
if he will have normal development after he undergoes complete repair and his
oxygen saturation levels are normal.
Of
the following, the MOST appropriate statement about this child’s
neurodevelopmental prognosis is that the child is likely to have
A.
delayed motor skills
B. normal
intellectual functioning
C.
profound hearing deficit
D.
profound speech delay
E. significant
behavioral difficulties
Answer
B
Although the large majority of patients who have Tetralogy of Fallot (TOF) have normal intellectual functioning, it has been reported that about 22% of patients score less than 80 on IQ testing, as compared with 2.8% of the general population. Potential risk factors include prolonged hypoxemia, congestive heart failure, and thromboembolic events. An additional important risk factor is the association of TOF and chromosome 22q11.2 microdeletion, which is found in over 15% of patients with TOF.
-----------
Q5 :
You are seeing a 10-year-old boy for a health supervision visit. His brother and sister accompany him and his mother to the visit. The mother reports that her husband recently underwent a heart transplant for a “thick heart.” As you explore the family history in more detail, you learn that the boy’s paternal uncle and grandfather both have been diagnosed with hypertrophic cardiomyopathy. The boy has never had chest pain, palpitation, shortness of breath, dizziness, or syncope. He participates in sports and activities without any problems. Findings on physical examination are within normal limits.
Of the following, the MOST appropriate next step is
A. cardiology referral for this patient
B. cardiology referral for this patient and all of his siblings
C. cardiology referral for this patient and his male siblings
D. electrocardiography and echocardiography, followed by cardiology referral if results of either are abnormal
E. genetic testing for the entire family
Answer
B
Hypertrophic cardiomyopathy (HCM). The latter typically is inherited via one of several autosomal dominant genes that encode for the constituents of the sarcomere.
The family described in the vignette carries the diagnosis of HCM, and the children each have a 50% chance of having inherited the HCM gene from their father. Current recommendations suggest regular evaluation (perhaps as often as yearly) of such children, ideally by a cardiologist, involving detailed history, physical examination, and diagnostic testing that includes electrocardiography and echocardiography. Because the HCM gene is inherited in an autosomal dominant pattern without gender preference, referring only the patient in the vignette or limiting evaluation to males in the family risks missing the diagnosis in some siblings.
Answer
B
Although the large majority of patients who have Tetralogy of Fallot (TOF) have normal intellectual functioning, it has been reported that about 22% of patients score less than 80 on IQ testing, as compared with 2.8% of the general population. Potential risk factors include prolonged hypoxemia, congestive heart failure, and thromboembolic events. An additional important risk factor is the association of TOF and chromosome 22q11.2 microdeletion, which is found in over 15% of patients with TOF.
-----------
Q5 :
You are seeing a 10-year-old boy for a health supervision visit. His brother and sister accompany him and his mother to the visit. The mother reports that her husband recently underwent a heart transplant for a “thick heart.” As you explore the family history in more detail, you learn that the boy’s paternal uncle and grandfather both have been diagnosed with hypertrophic cardiomyopathy. The boy has never had chest pain, palpitation, shortness of breath, dizziness, or syncope. He participates in sports and activities without any problems. Findings on physical examination are within normal limits.
Of the following, the MOST appropriate next step is
A. cardiology referral for this patient
B. cardiology referral for this patient and all of his siblings
C. cardiology referral for this patient and his male siblings
D. electrocardiography and echocardiography, followed by cardiology referral if results of either are abnormal
E. genetic testing for the entire family
Answer
B
Hypertrophic cardiomyopathy (HCM). The latter typically is inherited via one of several autosomal dominant genes that encode for the constituents of the sarcomere.
The family described in the vignette carries the diagnosis of HCM, and the children each have a 50% chance of having inherited the HCM gene from their father. Current recommendations suggest regular evaluation (perhaps as often as yearly) of such children, ideally by a cardiologist, involving detailed history, physical examination, and diagnostic testing that includes electrocardiography and echocardiography. Because the HCM gene is inherited in an autosomal dominant pattern without gender preference, referring only the patient in the vignette or limiting evaluation to males in the family risks missing the diagnosis in some siblings.
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