A 32 Year Old Male Presents In ER With Sharp Chest Pain

A 32 years old man is brought to the emergency department with a complaint of sharp chest pain that began suddenly an hour ago. He says he was moving some furniture when this pain started. The pain is sharp and tearing in nature. It is located just behind the sternum and radiated to the back. He also complains of mild dizziness since the pain started. His past history includes a mirtal valve prolapse and he takes a low dose aspirin for this condition.

Physical Examination reveals:

• A tall, thin man looking very anxious and diaphoretic.
• On auscultation, lung fields are clear but a blowing late systolic murmur can be heard that radiates from the apex to the axilla. 
• Pulses are palpable and strong. 
• He has long arms and legs and long thin digits. 
• Neurological examination is normal. 

X-Ray Chest shows following appearance:

What is the Diagnosis?

.
.

.
Aortic Dissection In A Patient With Marfan Syndrome.

Case discussion.

Aortic Dissection Is condition in which a tear within the aorta extending from the intima to the media that creates a false lumen within the aorta.
Type A: involves the ascending aorta.
Type B: dissection that begins distal to the left subclavian atery.

Risk factors:

• Hypertension.
• Trauma 
• Coarctation of aorta
• Syphillis
• Ehler-Danlos syndrome
• Marfan syndrome

Clinical features:

Patient usually presents with:

• Tearing chest pain
• Dyspnea or syncope.
• BP may be normal or increased.
• Heart murmurs.

Diagnosis: 

 1. Chest X- ray shows superior mediastinal widening for ascending aorta dissections.

2. CT angiography and MRI are highly sensitive for detecting intimal tears and false lumens.

Management:

1. Stabilize high blood pressure with nitroprusside, calcium channel blockers or B- blockers.

2. Standard A dissections require emergent cardiothoracic surgery to repair or reconstruct the ascending aorta.

3. Standard B dissections may be treated medically unless rupture or occlusion develops.

4. life long B- blocker therapy and close cardiac monitoring is required. 

Marfan Syndrome is a genetic disorder that affects the body's connective tissues. Since connective tissue is present through out the body therefore it can affect any part of the body. Some features include:

• Tall and slender body.
• Disproportionately long arms, legs.fingers and toes.
• A forward protruding breast bone
• Heart murmurs
• Extreme nearsightedness
• Abnormally curved spine.
• Aortic dissection as a life threatening complication