A penetrating aortic ulcer (PAU) is a deep sore in the multi-layered wall of the aorta, the body’s main artery. This large, cane-shaped blood vessel carries oxygen-rich blood from the heart to other vital organs and tissues. Often associated with hardening of the arteries (atherosclerosis), a PAU is a chronic condition that develops when plaque builds up in the aorta, eroding the aortic wall and breaking through its innermost layer (intima). This erosion can substantially weaken the aortic wall, potentially leading to a full perforation that allows blood to seep into the surrounding tissues.

An intramural hematoma (IMH) is an acute condition in which blood seeps through the aortic wall and collects between its inner (intimal) and outer (adventitial) layers. Unlike a PAU, an IMH does not perforate the aortic wall or create a separate channel for blood flow. Instead, the blood leaks from small vessels in the aortic wall (vaso vasorum) or from a damaged area of its intima.

Though relatively uncommon, both penetrating aortic ulcers and intramural hematomas can significantly impact heart health by compromising the structure and function of the aorta. A PAU can weaken the aortic wall, increasing the risk of life-threatening rupture or dissection. Similarly, an IMH can cause blood to pool within the aortic wall, straining the heart and reducing blood flow to vital organs and tissues throughout the body. Early diagnosis and treatment are essential to prevent severe complications, such as serious cardiovascular events.

What Are the Types of Penetrating Aortic Ulcers and Intramural Hematomas?

Penetrating aortic ulcers are classified based on their location within the aorta:

• Ascending PAU – Located in the chest cavity, the ascending aorta is the first segment of the aorta, which extends from the left ventricle of the heart and carries oxygen-rich blood upward, where it forms an arch and gives rise to the coronary arteries that supply blood to the heart muscle.
• Descending PAU (most common) – The descending aorta continues from the aortic arch and travels downward through the chest, supplying oxygenated blood to the ribs, esophagus, lungs and other structures in the thoracic region through smaller branches.
• Abdominal PAU – The abdominal aorta extends from the diaphragm to the pelvis, transporting oxygen-rich blood to the stomach, liver, pancreas, intestines, kidneys and other organs in the abdominal cavity. At its end, the abdominal aorta splits into the two common iliac arteries, which supply blood to the pelvis and legs.

Intramural hematomas affect the middle (medial) layer of the aorta but are classified based on their location in the aorta:

• Type A IMH – Though rare, a type A IMH is a life-threatening condition that occurs when blood leaks into the ascending aorta.
• Type B IMH – Generally less severe than a type A IMH, a type B IMH occurs in the descending aorta.

What Causes Penetrating Aortic Ulcers and Intramural Hematomas and What Are the Risk Factors?

PAUs are often linked to atherosclerosis, a progressive condition characterized by the accumulation of plaque on the intimal layer of arteries. Over time, plaque buildup can cause this normally smooth, elastic tissue to become thickened, brittle and more prone to damage. Eventually, atherosclerosis can compromise the strength of the aortic wall, eroding its intimal layer and forming an ulcer.

Risk factors for penetrating aortic ulcers include:

• Aging – Arterial walls naturally stiffen and weaken over time, increasing the likelihood of ulcer formation.
• Arterial dissection – A tear in the aortic wall can compromise its structure, contributing to PAU development.
• Diabetes – High blood sugar levels can damage blood vessels, elevating the risk of atherosclerosis.
• Family history of atherosclerosis – A genetic predisposition to plaque buildup can increase the risk of developing penetrating aortic ulcers.
• Fibromuscular dysplasia – Abnormal cell growth in the aortic wall can weaken the aorta and contribute to PAU formation.
• Heart disease – An underlying cardiovascular condition can increase the strain on the aorta, making it more prone to damage.
• High blood pressure (hypertension) – Increased pressure within the aorta can stress and damage its wall.
• High cholesterol – Excess cholesterol contributes to plaque buildup, a major factor in aortic ulcer formation.
• Inflammation of the arteries (Takayasu’s arteritis) – Chronic inflammation can weaken the arterial walls, increasing the risk of ulcers.
• Moyamoya disease – This rare condition causes narrowing of the arteries, which can contribute to vessel damage and ulcer formation.
• Smoking – Smoking damages the arterial walls and accelerates the progression of atherosclerosis, increasing the risk of PAUs.

IMHs usually result from the rupture of small blood vessels within the aortic wall, leading to blood pooling. Common contributing factors include hypertension, which increases stress on the aortic wall, and degenerative changes in the aorta due to aging or atherosclerosis. Trauma or connective tissue disorders may also play a role in some cases.

Risk factors for intramural hematomas include:

• Aortic aneurysm – Weakening of the aortic wall increases the likelihood of blood leakage between its layers.
• Aortic coarctation – Narrowing of the aorta can create abnormal pressure, contributing to wall stress and hematoma formation.
• Bicuspid aortic valve – This congenital condition can lead to abnormal blood flow and stress on the aortic wall.
• Chronic hypertension – Persistently high blood pressure places significant strain on the aortic wall, increasing the risk of hematomas.
• Cocaine use – Cocaine can cause a sudden spike in blood pressure, damaging the aortic wall and triggering hematomas.
• High-intensity weightlifting – Extreme physical exertion can cause spikes in blood pressure and lead to aortic wall injury.
• Marfan syndrome – This genetic disorder weakens connective tissues, making the aortic wall more prone to damage and hematomas.
• Pregnancy – Hormonal and physiological changes that occur during pregnancy can increase the risk of vascular complications, including intramural hematomas.
• Severe atherosclerosis – Plaque buildup can weaken the aortic wall, making it more susceptible to blood infiltration.
• Takayasu’s arteritis – Chronic inflammation of the aorta weakens its structure, increasing the risk of intramural hematomas.
• Turner syndrome – This genetic condition can cause vascular abnormalities, including weakened aortic walls.
• Traumatic chest injury – Physical trauma can directly damage the aortic wall, leading to blood leakage and hematoma formation.

What Are the Warning Signs and Symptoms of Penetrating Aortic Ulcers and Intramural Hematomas?

PAUs and IMHs can have similar warning signs, including:

• Difficulty breathing – Pressure on the structures surrounding the aorta can cause chest tightness and breathlessness.
• Dizziness and fainting – Disrupted blood flow can cause lightheadedness and syncope.
• Sudden blood pressure changes – Strain on the aorta can lead to changes in blood pressure.
• Severe discomfort – Depending on the part of the aorta affected by the PAU or IMH, sharp pain or a tearing sensation may be felt in the chest, back or abdomen.

Because these symptoms can mimic those of other heart and vascular conditions, prompt medical attention is essential to ensure an accurate diagnosis and appropriate treatment.

How Are Penetrating Aortic Ulcers and Intramural Hematomas Diagnosed?

The diagnostic process for both PAUs and IMHs usually begins with a medical history review and physical examination. During the exam, the physician will use a stethoscope to listen to the patient's heart, lungs and abdomen and check for abnormal heart rhythms (arrhythmias).

To visualize the aorta and check for tears and other damage, the physician may order a transesophageal echocardiography (TEE). This sensitive imaging technique uses an ultrasound probe inserted through the esophagus to gain a close-up view of the aorta. To further examine the aorta, the physician may order additional imaging tests, such as a magnetic resonance angiogram (MRA), computed tomography (CT) scan or aortic angiogram. Sometimes, a special dye is administered intravenously beforehand to improve the resulting image quality.

Before confirming a diagnosis of a penetrating aortic ulcer or intramural hematoma, the physician may also order laboratory tests, such as blood work, to rule out other conditions, such as a heart attack.

How Are Penetrating Aortic Ulcers and Intramural Hematomas Treated?

PAUs and IMHs are life-threatening conditions that often require immediate treatment, which may involve medication to stabilize the aorta, reduce stress on the aortic wall and prevent further damage and complications. Common approaches include:

• Antihypertensive therapy – ACE inhibitors, beta blockers or calcium channel blockers may be prescribed to maintain long-term blood pressure control, lower the resting heart rate and reduce strain on the aorta.
• Cholesterol control – Statins may be considered to slow the progression of atherosclerosis, a key contributing factor to the development of both PAUs and IMHs.
• Pain management – Analgesics can minimize discomfort caused by a PAU or IMH and also reduce stress, which may worsen the condition.

Medications can be particularly effective when a penetrating aortic ulcer or intramural hematoma is confined to the descending aorta. Periodic monitoring with imaging is essential to ensure the condition does not progress.

Surgery may be considered to address a PAU or IMH with a high risk of complications, such as rupture, dissection or rapid progression, or if the condition cannot be managed effectively with medication. Surgical treatment approaches may include:

• Endovascular repair (TEVAR) – During this minimally invasive procedure, a surgeon will use a catheter to place a stent graft inside the aorta to reinforce the weakened area and restore normal blood flow. This approach is a mainstay of treatment for PAUs and IMHs in the descending aorta.
• Open surgery – After making a large incision in the chest, the surgeon will replace the damaged portion of the aorta with a synthetic graft. This approach is often used to address PAUs and IMHs in the ascending aorta.

The choice of procedure will depend on the location, size and severity of the PAU or IMH as well as the patient’s overall health. Both surgical treatment options aim to prevent life-threatening complications.

What Are the Possible Complications and Long-Term Effects of Penetrating Aortic Ulcers and Intramural Hematomas?

If left untreated, PAUs and IMHs can lead to several serious complications. These include:

• Aortic dissection – A separation of the aortic wall layers can disrupt blood flow to organs and tissues throughout the body.
• Aortic rupture – A tear in the aortic wall can cause severe internal bleeding.
• Poor circulation – Inadequate blood supply to vital organs can lead to organ dysfunction or failure.
• Progression – A PAU can deepen or enlarge, while an IMH may evolve into a full aortic dissection.

The long-term effects of a penetrating aortic ulcer or intramural hematoma can vary depending on the severity of the condition and the patient’s response to treatment. Some patients may require regular imaging to monitor the condition, along with lifelong blood pressure and cholesterol management to help prevent recurrence or further damage.

How Can Penetrating Aortic Ulcers and Intramural Hematomas Be Prevented?

Effective strategies for preventing PAUs and IMHs focus on managing controllable risk factors that can weaken the aorta. Key steps include:

• Adopting a healthy lifestyle – Eating a balanced diet, exercising regularly and managing stress promote overall health, with a specific focus on supporting cardiovascular health.
• Controlling blood pressure – Maintaining healthy blood pressure levels through proper nutrition, moderate physical activity and/or prescribed medications can reduce the strain on the aortic wall.
• Having regular medical checkups – Proactively monitoring for underlying health conditions, such as hypertension and atherosclerosis, which significantly increase the risk of PAUs and IMHs, can lead to early detection and treatment.
• Managing cholesterol – Keeping cholesterol levels in check with a heart-healthy diet or prescribed statins can help prevent atherosclerosis, a primary risk factor for PAUs and IMHs.
• Quitting tobacco – Smoking cessation can help prevent further damage to blood vessels throughout the body, including the aorta.

Life After a Penetrating Aortic Ulcer or Intramural Hematoma

After being diagnosed with a PAU or IMH, most patients are advised to adhere to a long-term management plan to help prevent complications. This may include:

• Follow-up care – The patient will usually work closely with their physician, who can provide periodic health evaluations and adjust the treatment regimen as needed.
• Lifestyle changes – To achieve and maintain the best possible vascular health, the patient should follow a heart-healthy diet, exercise regularly, manage stress and quit smoking.
• Medications – To reduce strain on the aorta, the patient may be prescribed maintenance drugs to manage their blood pressure and/or cholesterol over the long run.
• Regular monitoring – The patient may receive periodic imaging tests, such as CT or MRI scans, to help their physician track the condition and detect any progression.

With proper management, many individuals with a penetrating aortic ulcer or intramural hematoma can lead a stable and healthy life while avoiding serious complications.

Benefit From World-Class Care at TGH

As a nationally recognized leader in cardiovascular care, Tampa General Hospital is dedicated to delivering top-tier medical services and achieving exceptional patient outcomes. Our Heart and Vascular Institute is an outstanding choice for patients seeking medical attention for a possible or confirmed penetrating aortic ulcer or intramural hematoma. Our cardiac imaging specialists have extensive experience in evaluating complex aortic conditions, ensuring each patient receives an accurate and timely diagnosis, which is the key to developing an effective treatment plan.

TGH is also proud to offer the latest medication-based and surgical treatment options for rare and multifaceted aortic conditions, including PAUs and IMHs. Our fellowship-trained cardiovascular surgeons are highly skilled in performing advanced minimally invasive procedures, such as TEVAR, helping to ensure a quick recovery and outstanding result.

If you would like to request a consultation with a specialist on our team, please contact us at (800) 844-3900 or email us at AorticDiseaseProgram@tgh.org.