Aortic dissection

Updated: January 1, 2024

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Background

Acute aortic dissection (AAD) is an infrequent, yet severe medical condition characterized by the separation of the layers of the aortic wall. The dissection occurs when a tear develops in the intimal layer, leading to the progression of the dissection, either in a proximal or retrograde direction, primarily caused by the entry of blood between the intima and media layers.

Unfortunately, acute aortic dissection is associated with a remarkably high mortality rate, with a majority of individuals succumbing to the condition even before reaching the emergency department. On the other hand, patients with chronic aortic dissection (lasting more than two weeks) have a slightly more favorable prognosis. 

Epidemiology

Aortic dissection is estimated to occur in approximately 2 to 3 cases per 100,000 people per year. The incidence increases with age, with the majority of cases occurring in individuals over 60 years old. Aortic dissection can affect individuals of all ages, but it is more commonly seen in older adults. The condition is slightly more prevalent in men than in women.

Connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome, increase the risk of aortic dissection. Aortic dissection may be associated with certain underlying conditions, such as bicuspid aortic valve, coarctation of the aorta, and previous heart surgery

Aortic dissection is associated with high mortality rates, particularly if not diagnosed and treated promptly. Mortality rates are influenced by factors such as the type of dissection, the presence of complications, and the timeliness of medical intervention. 

Anatomy

Pathophysiology

Aortic dissection is a life-threatening cardiovascular emergency characterized by the separation of the layers within the aortic wall. The pathophysiology of aortic dissection typically involves the initiation of a tear in the intimal layer of the aorta, allowing blood to enter and propagate within the vessel wall. The tear often occurs due to structural weaknesses in the aortic wall, which can be associated with conditions such as hypertension, connective tissue disorders (e.g., Marfan syndrome), or other underlying vascular diseases.

The high-pressure blood flow within the aorta then dissects or cleaves the layers apart, creating a false lumen alongside the true lumen. As the dissection extends, it can compromise blood flow to vital organs, leading to ischemia and potentially fatal complications. The location of the tear plays a critical role in determining the type of aortic dissection—Type A involves the ascending aorta, whereas Type B involves the descending aorta.

The separation of the layers can lead to the formation of a hematoma, expansion of the aorta, and potential rupture, posing a severe risk of mortality if not promptly diagnosed and treated. The complex interplay of hemodynamics, vascular anatomy, and underlying risk factors underscores the urgency of recognizing and managing aortic dissection to prevent further propagation and complications and ultimately to improve patient outcomes. 

Etiology

Intrinsic Factors: 

Hypertension: Chronic high blood pressure is a significant contributor to aortic dissection. Elevated blood pressure can lead to increased stress on the arterial walls, making them more susceptible to tearing. 

Atherosclerosis: The presence of atherosclerotic plaques in the aorta may compromise the integrity of the vessel wall and contribute to the development of aortic dissection. 

Connective Tissue Disorders: Conditions such as Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome are associated with genetic abnormalities affecting connective tissue, making the aorta more prone to dissection. 

Bicuspid Aortic Valve: Individuals with a bicuspid aortic valve, a congenital heart valve anomaly, have an increased risk of aortic dissection. 

Extrinsic Factors: 

  • Trauma: Severe trauma, such as an accident or a fall, can cause aortic dissection by directly injuring the aorta or indirectly increasing stress on the vessel walls. 
  • Iatrogenic Causes: Aortic dissection can be a complication of certain medical procedures, such as cardiac catheterization or aortic surgery. 
  • Pregnancy: Although rare, aortic dissection can occur during pregnancy, particularly in individuals with pre-existing risk factors.

Genetic Factors: 

  • There is evidence to suggest a genetic predisposition to aortic dissection, especially in cases where there is a family history of the condition. 

 

Genetics

Prognostic Factors

Type A dissections (involving the ascending aorta) are generally associated with a higher risk of mortality compared to Type B dissections (involving the descending aorta). Type A dissections often require emergency surgical intervention. Older age is generally associated with a higher risk of mortality.

Patients with good overall health may have a more favorable prognosis. Long-term prognosis also depends on ongoing medical management, lifestyle modifications, and regular follow-up to monitor the health of the aorta and manage any potential complications or recurrence. 

Clinical History

The signs, symptoms, onset, and clinical history of aortic dissection can vary depending on the location, extent, and severity of the dissection. A hallmark symptom is the abrupt onset of intense, sharp, or tearing pain, often described as the worst pain ever experienced. The pain can originate in the chest and may radiate to the back, neck, abdomen, or limbs.

Depending on the involvement of blood vessels supplying the brain, neurological symptoms such as confusion, difficulty speaking, or paralysis may occur. Aortic dissection often presents suddenly and without warning. The pain associated with aortic dissection is persistent and does not typically resolve spontaneously.

Individuals with a history of aortic aneurysms, aortic valve disorders, or previous cardiac surgeries may be at increased risk. In cases of traumatic aortic dissection, a history of recent trauma or injury may be relevant. In some instances, aortic dissection may have a chronic course with milder symptoms or vague discomfort. Pain is typically felt in the anterior chest or back, and it may radiate to the neck, jaw, or arms in Type A Dissection. Pain may be felt in the back or abdomen in Type B Dissection 

Physical Examination

A notable finding is a difference in blood pressure between the two arms. This discrepancy may be due to compromised blood flow to one of the subclavian arteries. A pulse deficit, where there is a difference between the heart rate and peripheral pulses, may be observed. This can be a sign of impaired blood flow to the peripheral arteries. Aortic regurgitation may occur if the dissection involves the aortic valve, leading to a diastolic murmur.

In cases where the dissection extends into the pericardium, signs of cardiac tamponade may be present, including muffled heart sounds, jugular venous distension, and hypotension. Depending on the involvement of blood vessels supplying the brain, neurological findings may include focal deficits, confusion, or altered mental status.

In cases where the dissection involves the abdominal aorta, diminished or absent pulses may be noted in the lower extremities. Abdominal pain or tenderness may be present, particularly if the dissection involves the abdominal aorta. If branches supplying the extremities are compromised, there may be signs of limb ischemia, such as coolness, pallor, or weakness. 

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Cardiac Tamponade 

Aortic Aneurysm 

Pulmonary Embolism 

Esophageal Rupture 

 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The treatment paradigm for aortic dissection involves a comprehensive and multidisciplinary approach aimed at stabilizing the patient, relieving symptoms, preventing complications, and addressing the underlying pathology. Aortic dissection requires prompt diagnosis and intervention. Initial management focuses on aggressive blood pressure control using intravenous beta-blockers, which help reduce shear stress on the aortic wall. Analgesics are also administered to manage pain.  

Postoperative care is crucial, with close monitoring for potential complications such as organ malperfusion, persistent hypertension, and the development of new dissection flaps. Long-term management involves ongoing surveillance through imaging studies to monitor the integrity of the repair and address any potential complications that may arise. Additionally, patients are often prescribed medications, such as beta-blockers and antihypertensives, to control blood pressure and reduce the risk of recurrent dissection. 

 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Surgical intervention is often necessary, and the choice between open surgery and endovascular repair depends on the location and extent of the dissection, as well as the patient’s overall health. Emergency surgery may involve replacing the affected segment of the aorta with a synthetic graft or repairing the torn layers of the aortic wall. Endovascular repair, using stent grafts, has become a less invasive alternative for certain cases, especially in patients who are not optimal candidates for open surgery. 

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

Aortic dissection

Updated : January 1, 2024

Mail Whatsapp PDF Image



Acute aortic dissection (AAD) is an infrequent, yet severe medical condition characterized by the separation of the layers of the aortic wall. The dissection occurs when a tear develops in the intimal layer, leading to the progression of the dissection, either in a proximal or retrograde direction, primarily caused by the entry of blood between the intima and media layers.

Unfortunately, acute aortic dissection is associated with a remarkably high mortality rate, with a majority of individuals succumbing to the condition even before reaching the emergency department. On the other hand, patients with chronic aortic dissection (lasting more than two weeks) have a slightly more favorable prognosis. 

Aortic dissection is estimated to occur in approximately 2 to 3 cases per 100,000 people per year. The incidence increases with age, with the majority of cases occurring in individuals over 60 years old. Aortic dissection can affect individuals of all ages, but it is more commonly seen in older adults. The condition is slightly more prevalent in men than in women.

Connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome, increase the risk of aortic dissection. Aortic dissection may be associated with certain underlying conditions, such as bicuspid aortic valve, coarctation of the aorta, and previous heart surgery

Aortic dissection is associated with high mortality rates, particularly if not diagnosed and treated promptly. Mortality rates are influenced by factors such as the type of dissection, the presence of complications, and the timeliness of medical intervention. 

Aortic dissection is a life-threatening cardiovascular emergency characterized by the separation of the layers within the aortic wall. The pathophysiology of aortic dissection typically involves the initiation of a tear in the intimal layer of the aorta, allowing blood to enter and propagate within the vessel wall. The tear often occurs due to structural weaknesses in the aortic wall, which can be associated with conditions such as hypertension, connective tissue disorders (e.g., Marfan syndrome), or other underlying vascular diseases.

The high-pressure blood flow within the aorta then dissects or cleaves the layers apart, creating a false lumen alongside the true lumen. As the dissection extends, it can compromise blood flow to vital organs, leading to ischemia and potentially fatal complications. The location of the tear plays a critical role in determining the type of aortic dissection—Type A involves the ascending aorta, whereas Type B involves the descending aorta.

The separation of the layers can lead to the formation of a hematoma, expansion of the aorta, and potential rupture, posing a severe risk of mortality if not promptly diagnosed and treated. The complex interplay of hemodynamics, vascular anatomy, and underlying risk factors underscores the urgency of recognizing and managing aortic dissection to prevent further propagation and complications and ultimately to improve patient outcomes. 

Intrinsic Factors: 

Hypertension: Chronic high blood pressure is a significant contributor to aortic dissection. Elevated blood pressure can lead to increased stress on the arterial walls, making them more susceptible to tearing. 

Atherosclerosis: The presence of atherosclerotic plaques in the aorta may compromise the integrity of the vessel wall and contribute to the development of aortic dissection. 

Connective Tissue Disorders: Conditions such as Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome are associated with genetic abnormalities affecting connective tissue, making the aorta more prone to dissection. 

Bicuspid Aortic Valve: Individuals with a bicuspid aortic valve, a congenital heart valve anomaly, have an increased risk of aortic dissection. 

Extrinsic Factors: 

  • Trauma: Severe trauma, such as an accident or a fall, can cause aortic dissection by directly injuring the aorta or indirectly increasing stress on the vessel walls. 
  • Iatrogenic Causes: Aortic dissection can be a complication of certain medical procedures, such as cardiac catheterization or aortic surgery. 
  • Pregnancy: Although rare, aortic dissection can occur during pregnancy, particularly in individuals with pre-existing risk factors.

Genetic Factors: 

  • There is evidence to suggest a genetic predisposition to aortic dissection, especially in cases where there is a family history of the condition. 

 

Type A dissections (involving the ascending aorta) are generally associated with a higher risk of mortality compared to Type B dissections (involving the descending aorta). Type A dissections often require emergency surgical intervention. Older age is generally associated with a higher risk of mortality.

Patients with good overall health may have a more favorable prognosis. Long-term prognosis also depends on ongoing medical management, lifestyle modifications, and regular follow-up to monitor the health of the aorta and manage any potential complications or recurrence. 

The signs, symptoms, onset, and clinical history of aortic dissection can vary depending on the location, extent, and severity of the dissection. A hallmark symptom is the abrupt onset of intense, sharp, or tearing pain, often described as the worst pain ever experienced. The pain can originate in the chest and may radiate to the back, neck, abdomen, or limbs.

Depending on the involvement of blood vessels supplying the brain, neurological symptoms such as confusion, difficulty speaking, or paralysis may occur. Aortic dissection often presents suddenly and without warning. The pain associated with aortic dissection is persistent and does not typically resolve spontaneously.

Individuals with a history of aortic aneurysms, aortic valve disorders, or previous cardiac surgeries may be at increased risk. In cases of traumatic aortic dissection, a history of recent trauma or injury may be relevant. In some instances, aortic dissection may have a chronic course with milder symptoms or vague discomfort. Pain is typically felt in the anterior chest or back, and it may radiate to the neck, jaw, or arms in Type A Dissection. Pain may be felt in the back or abdomen in Type B Dissection 

A notable finding is a difference in blood pressure between the two arms. This discrepancy may be due to compromised blood flow to one of the subclavian arteries. A pulse deficit, where there is a difference between the heart rate and peripheral pulses, may be observed. This can be a sign of impaired blood flow to the peripheral arteries. Aortic regurgitation may occur if the dissection involves the aortic valve, leading to a diastolic murmur.

In cases where the dissection extends into the pericardium, signs of cardiac tamponade may be present, including muffled heart sounds, jugular venous distension, and hypotension. Depending on the involvement of blood vessels supplying the brain, neurological findings may include focal deficits, confusion, or altered mental status.

In cases where the dissection involves the abdominal aorta, diminished or absent pulses may be noted in the lower extremities. Abdominal pain or tenderness may be present, particularly if the dissection involves the abdominal aorta. If branches supplying the extremities are compromised, there may be signs of limb ischemia, such as coolness, pallor, or weakness. 

Cardiac Tamponade 

Aortic Aneurysm 

Pulmonary Embolism 

Esophageal Rupture 

 

The treatment paradigm for aortic dissection involves a comprehensive and multidisciplinary approach aimed at stabilizing the patient, relieving symptoms, preventing complications, and addressing the underlying pathology. Aortic dissection requires prompt diagnosis and intervention. Initial management focuses on aggressive blood pressure control using intravenous beta-blockers, which help reduce shear stress on the aortic wall. Analgesics are also administered to manage pain.  

Postoperative care is crucial, with close monitoring for potential complications such as organ malperfusion, persistent hypertension, and the development of new dissection flaps. Long-term management involves ongoing surveillance through imaging studies to monitor the integrity of the repair and address any potential complications that may arise. Additionally, patients are often prescribed medications, such as beta-blockers and antihypertensives, to control blood pressure and reduce the risk of recurrent dissection. 

 

Surgical intervention is often necessary, and the choice between open surgery and endovascular repair depends on the location and extent of the dissection, as well as the patient’s overall health. Emergency surgery may involve replacing the affected segment of the aorta with a synthetic graft or repairing the torn layers of the aortic wall. Endovascular repair, using stent grafts, has become a less invasive alternative for certain cases, especially in patients who are not optimal candidates for open surgery. 

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