A 32-year-old man is brought to the emergency department complaining of sudden-onset,

excruciating chest and neck pain. He has no prior history of dyspnea, chest pain, or
lower-extremity edema. He is 6 feet, 5 inches tall with long extremities. His hand joints
show significant extensibility. Pectus carinatum deformity is present. Which of the
following additional findings is likely in this patient?

o A. Early diastolic murmur

o B. Fixed splitting of the second heart sound
o C. Opening snap
o D. Pericardia! friction rub
0 E. Pulsus parvus et tardus
o F. Third heart sound

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A 32-year-old man is brought to the emergency department complaining of sudden-onset,

excruciating chest and neck pain. He has no prior history of dyspnea, chest pain, or
lower-extremity edema. He is 6 feet, 5 inches tall with long extremities. His hand joints
show significant extensibility. Pectus carinatum deformity is present. Which of the
following additional findings is likely in this patient?

A. Early diastolic murmur [56%)

B. Fixed splitting of the second heart sound [4%)
C. Opening snap [15%)
D. Pericardia! friction rub [3%]
E. Pulsus parvus et tardus [17%)
'.J F. Third heart sound [5%)

. .
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Explanation: User ld

Clinical features of Marfan syndrome

• Arachnodactyly
Skeletal • 1 Upper-to-lower body segment ratio, 1 arm-to-height ratio
• Pectus deformity, scoliosis, or kyphosis
• Joint hypermobility

Ocular • Ectopia lentis

• Aortic dilation, regurgitation, or dissection

Cardiovascu lar
• Mitral valve prolapse

Pulmonary • Spontaneous pneumothorax from apical blebs

• Recurrent or incisional hernia

Skin
• Skin striae
@UWorld

This patient appears to have marfanoid habitus with chest and neck pain suggesting an

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• Recurrent or incisional hernia
Skin
• Skin striae
@UWorld

This patient appears to have marfanoid habitus with chest and neck pain suggesting an
acute aortic dissection. Marfan syndrome is an autosomal dominant disorder due to
mutations in the extracellular matrix protein fibrillin-1. Clinical manifestations include
cardiac (e.g., aortic dissection, mitral valve prolapse), skeletal (e.g. , disproportionately
long extremities, increased arm-span-to-height ratio), pulmonary (e.g., spontaneous
pneumothorax), and ocular (e.g., ectopia lentis) abnormalities.

All Marfan syndrome patients with acute chest pain require evaluation for acute aortic
dissection as nearly 50% of aortic dissections in patients age <40 are due to the
syndrome. Examination may also show >20 mm Hg systolic blood pressure variation
between arms. Patients with aortic dissection and/or progressive aortic root dilatation
can develop aortic regurgitation. Examination shows an early decrescendo diastolic
murmur heard best along the left sternal border at the third and fourth intercostal space
with the patient sitting up, leaning forward, and holding the breath after full expiration.

(Choice B) A wide and fixed splitting of the second heart sound is present in patients
with atrial septal defect. Holt-Oram (heart-hand) syndrome is associated with both upper-
limb defects (e.g. , deformities of the radius, carpal bones) and atrial septal defect.
(Choice C) Mitral stenosis usually presents with an opening snap in early diastole, loud
first heart sound, and a mid-diastolic murmur stemming from turbulent flow across the
atrioventricular valves. Mitral valve prolapse, not mitral stenosis, is frequently seen in
Marfan syndrome and causes a mid- to late-systolic murmur.

(Choice 0) Pericardia! friction rub is seen with pericarditis. However, Marfan syndrome
is not associated with pericarditis.
(Choice E) Pulsus parvus et tardus is an arterial pulse with decreased amplitude and
delayed peak. It is commonly seen in severe aortic stenosis, which is not usually
associated with Marfan syndrome.
(Choice F) Third heart sound is a low-frequency diastolic sound that is a normal finding
in healthy young adults and an abnormal finding in those with dilated ventricles. Mitral
valve prolapse resulting in regurgitation and heart failure can cause third heart
sound. However, this patient has no symptoms of heart failure.
Educational objective:
Aortic dissection is the most dangerous complication of Marfan syndrome. It typically
presents as tearing chest pain radiating to the back and neck and must be identified
immediately to decrease the risk of death. Aortic regurgitation is a complication of aortic

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long extremities, increased arm-span-to-height ratio), pulmonary (e.g., spontaneous
pneumothorax), and ocular (e.g., ectopia lentis) abnormalities.

All Marfan syndrome patients with acute chest pain require evaluation for acute aortic
dissection as nearly 50% of aortic dissections in patients age <40 are due to the
syndrome. Examination may also show >20 mm Hg systolic blood pressure vari ation
between arms. Patients with aortic dissection and/or progressive aortic root dilatation
can develop aortic regurgitation. Examination shows an early decrescendo diastolic
murmur heard best along the left sternal border at the third and fourth intercostal space
with the patient sitting up, leaning forward, and holding the breath after full expiration.

(Choice B) A wide and fixed splitting of the second heart sound is present in patients
with atrial septal defect. Holt-Oram (heart-hand} syndrome is associated with both upper-
limb defects (e.g., deformities of the radius, carpal bones) and atrial septal defect.

(Choice C) Mitral stenosis usually presents with an opening snap in early diastole, loud
first heart sound, and a mid-diastolic murmur stemming from turbulent flow across the
atrioventricular valves. Mitral valve prolapse, not mitral stenosis, is frequently seen in
Marfan syndrome and causes a mid- to late-systolic murmur.

(Choice 0 ) Pericardia! friction rub is seen with pericarditis. However, Marfan syndrome
is not associated with pericarditis.

(Choice E) Pulsus parvus et tardus is an arterial pulse with decreased amplitude and
delayed peak. It is commonly seen in severe aortic stenosis, which is not usually
associated with Marfan syndrome.

(Choice F) Third heart sound is a low-frequency diastolic sound that is a normal finding
in healthy young adults and an abnormal finding in those with dilated ventricles. Mitral
valve prolapse resulting in regurgitation and heart failure can cause third heart
sound. However, this patient has no symptoms of heart failure.

Educational obj ective:

Aortic dissection is the most dangerous complication of Marfan syndrome. It typically
presents as tearing chest pain radiating to the back and neck and must be identified
immediately to decrease the risk of death. Aortic regurgitation is a complication of aortic
dissection and presents with an early diastolic murmur.

References:
1. Therapy insight: aortic aneurysm and dissection in Marfan's syndrome.

Time Spent: 2 seconds Copyright © UWorld Last updated: [09/13/2016]

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Media Exhibit

=eatures of aortic dissection

Clinical features of aortic dissection

• Hypertension (most common)

Risk factors/
• Marfan syndrome
associations
• Cocaine use

• Severe, sharp, tearing chest or back pain

Clinical
features • >20 mm Hg variation in systolic blood
pressure between arms

• Stroke (carotid arteries)

• Acute aortic regurgitation (aortic valves)
• Horner syndrome (superior cervical
sympathetic ganglion)
Complications • Acute myocardial ischemia/infarction
(involved (coronary artery)
structure) • Pericardia! effusion/cardiac tamponade
(pericardia! cavity)
• Hemothorax (pleural cavity)
• Lower-extremity weakness or ischemia
(spinal or common iliac arteries)
• Abdominal pain (mesenteric artery)
©UWor1d

! Aortic dissection -- Ill Q

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