Aortic Disorder

Aortic disorder is a serious condition affecting the body’s largest blood vessel, which carries oxygen-rich blood from the heart to all vital organs. When the aorta becomes damaged or diseased, it can quickly turn into a medical emergency that puts the entire body’s blood supply at risk.

Table of contents

• What is Aortic Disorder?
• Understanding the Aorta
• Types of Aortic Disorders
• Symptoms of Aortic Disorders
• Causes and Risk Factors
• Diagnosis
• Treatment Options
• Living with Aortic Disorder

What is Aortic Disorder?

Aortic disorder is broadly defined as conditions affecting the aorta, the main and largest artery in the human body that supplies oxygenated blood to all of the body’s vital organs^[1]. The aorta is the largest blood vessel in the body, carrying oxygen-rich blood from the heart to the rest of the body^[2]. When the aorta becomes diseased or damaged, the entire body’s blood supply can be jeopardized and can quickly transform into a serious medical emergency^[1].

Disorders and conditions that affect the aorta are called aortic diseases. It’s important to know that diseases of the aorta are serious and can be life-threatening^[2]. They happen when the walls of the aorta become weak and bulge, split, burst or block up^[2]. Aortic disease can occur when the aortic wall is weakened or damaged, the heart’s aortic valve narrows or doesn’t open fully, or there is a blockage in the aorta^[2].

Aortic disease can put the heart and the whole body’s blood supply at risk^[2]. Many aortic conditions do not cause symptoms until they become serious, making early detection and screening particularly important.

Understanding the Aorta

• Heart
• Chest (thorax)
• Abdomen
• Brain
• Arms
• Spinal cord
• Intestines
• Liver
• Stomach
• Kidneys
• Legs

The aorta starts at the root of the heart, where the artery is connected to the heart by the aortic valve (a structure that controls blood flow from the heart)^[1]. It hooks like a candy cane down the spine, sending blood to the brain, arms, spinal cord, visceral organs (intestines, liver, stomach), kidneys, and legs^[1].

The aorta is shaped like a candy cane and has four sections^[2]:

• Ascending aorta: This is where the aorta leaves the heart and goes up towards the neck, before curving^[2].
• Aortic arch: This is the curved part of the aorta between the ascending and descending aorta. Arteries taking blood to the head and arms branch off here^[2].
• Descending aorta: After the arch, the aorta extends down through the chest^[2].
• Abdominal aorta: The aorta continues below the ribs down to the groin^[2].

Parts of the aorta may be more than an inch wide. It has thick walls so that it doesn’t burst under the pressure of the blood that runs through it^[2]. The aorta is made of strong, flexible walls which bear the pressure of blood flowing from the heart^[6].

Types of Aortic Disorders

The main diseases of the aorta include aortic aneurysms, aortic valve disease, aortic dissection, and penetrating ulcer^[6]. Different conditions can affect different parts of the aorta.

Aortic Aneurysm

An aortic aneurysm is a “ballooning” of the aorta that, if expanded far enough, can potentially rupture^[1]. It occurs when the walls of the aorta begin to weaken, gradually lose their elasticity and expand or bulge outwards^[6]. Aortic bulges or swellings are called aneurysms^[2].

An aortic aneurysm can occur in either the chest region, commonly called a thoracic aneurysm, or the abdomen region, often referred to as an abdominal aortic aneurysm (AAA)^[1]. It can occur in any part of the aorta and would be classified as thoracic aortic aneurysm or abdominal aortic aneurysm depending on the part of the aorta affected^[6].

Importantly, 95% of aortic aneurysms do not cause any symptoms^[2]. Aneurysms are often found during screenings for other conditions^[2]. The larger an aneurysm is, the more likely it is to rupture^[2].

Thoracoabdominal Aortic Aneurysm

Thoracoabdominal Aortic Aneurysm (TAAA) is an enlargement or ballooning in the aorta, the main artery in the body that supplies blood to the rest of the body^[1]. TAAA occurs in the thoracic aorta, the upper section of the aorta close to the heart and chest, and extends into abdominal aorta^[1]. If left untreated, the aneurysm can rupture, leading to severe pain and lethal internal bleeding^[1].

Aortic Dissection

Aortic dissection occurs when the middle layer of the aortic wall splits creating a “false” channel for blood flow^[1]. When the inner layer of the aortic wall tears, it causes blood to flow between the other layers, separating them^[6]. This dissecting process weakens the aortic wall making it more prone to rupture^[1]. When rupture occurs, survival is often less than 10%^[1].

Aortic dissection can also lead to obstruction of any of the branches of the aorta and ultimate organ death^[1]. An aortic dissection occurring in the ascending aorta is termed Type A and is an emergency that usually requires surgical intervention, while an aortic dissection occurring in the descending aorta is termed Type B and may be managed without surgery by controlling blood pressure^[6].

Aortic Valve Disease

Aortic valve disease is a type of heart valve disease. In this condition, the valve between the lower left heart chamber and the body’s main artery doesn’t work properly^[3]. Aortic valve disease refers to any abnormal conditions or disorders affecting the aortic valve in the heart^[6]. The aortic valve helps keep blood flowing in the correct direction through the heart^[3]. A damaged or diseased aortic valve can affect blood flow to the rest of the heart and body^[3].

Aortic valve disease includes^[3]:

• Aortic valve stenosis: Each heart valve has flaps of tissue that open and close once per heartbeat. The flaps also are called cusps. Sometimes the aortic valve flaps become thick and stiff, or they connect together. These problems cause the valve opening to become narrow. The narrowed valve reduces or blocks blood flow from the heart to the rest of the body^[3]. In this condition, the aortic valve becomes constricted and fails to open fully, obstructing the outflow of blood from the heart^[6].
• Aortic valve regurgitation (aortic insufficiency): The aortic valve doesn’t close properly, causing blood to flow backward into the left heart chamber^[3]. In this condition, the aortic valve doesn’t function properly and the blood flows back to the heart instead of flowing to the rest of the body^[6].
• Bicuspid aortic valve: This is a common congenital heart defect marked by the presence of two cusps in the aortic valve instead of three cusps^[6].

Penetrating Ulcer

A penetrating ulcer, also termed penetrating atherosclerotic ulcer, occurs when the plaque in the aorta forms ulcers that penetrate the aortic wall^[6]. This is a rare condition that most commonly affects the descending aorta^[6]. It is seen in people with high blood pressure, atherosclerosis (hardening of the arteries), chest or back pain and those who smoke^[6].

Symptoms of Aortic Disorders

Mild forms of aortic diseases are typically asymptomatic, meaning they don’t cause any symptoms^[6]. Many aortic aneurysms do not cause symptoms—95% of aortic aneurysms do not cause any symptoms^[2]. However, as the condition progresses, some common symptoms would include^[6]:

• Chest, back or abdominal pain
• Difficulty swallowing
• Cough
• Pulsing sensation in your stomach

Sudden stabbing, radiating pain, fainting, difficulty breathing, and sometimes sudden weakness on one side of the body could suggest an aortic event^[1]. Clammy skin, nausea and vomiting, or even shock are also common accompanying symptoms^[1]. Aortic disease can also mirror symptoms of a heart attack, such as chest pain or jaw pain^[1].

The aorta spans from above the heart all the way down to below the navel, so symptoms of pain can occur at any place along the torso^[1]. If an aneurysm is large or growing fast it may press on other organs causing pain in the chest or belly, but nearly all aneurysms grow without causing any symptoms^[2].

If you develop an aortic tear causing dissection, you may experience sudden severe pain accompanied by a drop in your blood pressure, nausea, and dizziness^[6]. This is a medical emergency and you should seek medical treatment^[6].

Causes and Risk Factors

There are numerous conditions and diseases that can cause damage to the aorta^[6]. Although many risk factors for aortic aneurysms have been identified, the exact cause remains unknown^[1].

Age and Genetics

Age is a major factor in developing an aortic aneurysm^[2]. Aging causes the aorta to lose its elasticity^[6]. Deterioration from wear and tear, and the buildup of calcium in the valve’s leaflets (making them stiff and consequently less able to open or close properly) can cause aortic stenosis or insufficiency^[14]. Aortic valve disease is most common among older people^[14].

Genetics and family history of aortic disease contribute significantly to the risk of developing an aortic aneurysm or dissection^[1]. A family member or relative suffering from aortic disease increases risk^[6]. Several genetic conditions are also risk factors, such as Turner’s Syndrome, Marfan Syndrome, polycystic kidney disease, and other genetic abnormalities^[6].

Congenital Conditions

About 1 to 2% of babies are born with a bicuspid aortic valve (BAV)^[14]. Normally, the aortic valve has three leaflets, or cusps, but newborns who are born with a bicuspid valve only have two cusps^[14]. In some cases, the BAV is unable to open fully, leading to aortic stenosis. In other cases, however, the BAV cannot close completely, resulting in aortic insufficiency^[14]. The development of significant aortic stenosis tends to occur earlier in persons with congenital bicuspid aortic valves^[12].

Health Conditions

Health conditions that increase risk include^[6]:

• High blood pressure (hypertension)
• Hardening of the aortic walls (atherosclerosis)
• High cholesterol (hypercholesterolemia)
• Infections such as endocarditis (infection of the heart’s inner lining)

Several diseases and infections can cause aortic valve disease, including rheumatic fever, endocarditis, Marfan syndrome, syphilis, rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, and high blood pressure^[14]. Aortic dissection, which is a tear in the inner lining of the wall of the aorta, can affect the functioning of the aortic valve^[14].

Lifestyle Factors

Unhealthy lifestyle habits like smoking contribute to aortic disease risk^[6]. Smoking, obesity, drinking too much alcohol, and a sedentary lifestyle all contribute to high blood pressure^[17]. Chest or stomach injury can also lead to aortic problems^[6]. Trauma, such as chest trauma, can damage the aortic valve^[14].

Diagnosis

You will need to visit a heart specialist or cardiologist to diagnose aortic disease^[6]. To diagnose aortic valve disease, a healthcare professional examines you and asks questions about your symptoms and medical history^[3]. The health professional listens to your heart with a stethoscope^[3]. A whooshing sound, called a heart murmur, may be heard when listening to the heart^[3]. If you have aortic valve stenosis, a sound called a heart murmur may be heard^[8].

If doctors think you may have aortic disease, they use imaging tests to confirm the diagnosis^[13]. Your doctor will inquire about your symptoms and order a chest X-ray^[6]. You may also be asked to undergo a computed tomography (CT) scan, a magnetic resonance imaging (MRI) or an echocardiogram for confirmation of aortic disease^[6].

Diagnostic Tests

Tests to diagnose aortic disease may include^[8][9][13]:

• Echocardiogram: Sound waves are used to make pictures of the beating heart. An echocardiogram shows how blood flows through the heart and heart valves. It can tell how severe aortic valve stenosis is. The test also can show if the heart muscle is weakened. A standard echocardiogram is done from outside the body. An ultrasound device moves over the skin of the chest above the heart. If more details are needed about the heart, a transesophageal echocardiogram may be done. This type creates pictures of the heart from inside the body. The ultrasound device attaches to a tube that goes down the throat and into the esophagus.
• Electrocardiogram (ECG or EKG): This quick test records the electrical activity of the heart. It shows how the heart beats. Sticky patches with sensors on them go on the chest and sometimes the legs. Wires connect the patches to a computer, which displays or prints results.
• Chest X-ray: A chest X-ray shows the condition of the heart and lungs. It can show if the heart is bigger than usual, which can occur in aortic valve disease.
• Cardiac MRI: A cardiac MRI uses magnetic fields and radio waves to create detailed pictures of the heart. This test may be used to determine the severity of aortic valve disease.
• CT scan of the chest: A computed tomography scan combines X-rays to show images of the aorta.
• Abdominal Ultrasound: This test uses sound waves to see the part of the aorta that runs through the belly.
• Transesophageal echocardiogram (TEE): This test uses sound waves to get pictures of the heart and aorta in motion. An ultrasound probe is guided down the throat and placed close to the heart.
• Magnetic resonance angiogram (MRA): An MRA uses magnetic fields and radio wave energy to make images of the aorta and how blood is flowing through it.

Many types of aortic disease, including aortic aneurysms, are often found by chance when imaging tests are done for other conditions^[13]. Rapid, accurate diagnosis is crucial in care for aortic dissection^[13].

Treatment Options

The choice of treatment for aortic disease depends on its severity^[6]. Treatment may include monitoring, medicine, less invasive surgery, open aortic surgery, or open-heart surgery^[2]. Your treatment might include monitoring, lifestyle changes, medications or surgery^[13]. The right therapy for you will depend on your age and overall health, and your aneurysm’s size, type, location and rate of growth^[13].

Medication and Monitoring

For mild symptoms, you will be prescribed medications like calcium channel blockers or beta-blockers to minimize the risk of aortic dissection or rupture^[6]. If your aneurysm is small, doctors may recommend watching and waiting^[13]. You will have regular checkups and imaging tests to track your aneurysm’s size^[13].

Your doctor may suggest lifestyle changes such as quitting smoking or eating a heart-healthy diet^[13]. They may prescribe medicines to lower your blood pressure or cholesterol^[13].

Surgery and Procedures

For severe symptoms, you may be recommended an open or endovascular surgery^[6]. Surgery for aortic aneurysm involves replacing the damaged aorta with a graft^[6]. A stent could also be placed^[6].

Surgery is the best treatment if your aneurysm is large or growing fast^[13]. The goal is to keep surgery as minimally invasive as possible^[13]. Surgical approaches include:

• Open surgery: Doctors make a cut in your chest or abdomen to reach the aorta. They replace the damaged section with a graft: a tube made of synthetic material. Blood then flows through this long-lasting graft. This strengthens your aorta and is a lasting repair^[13].
• Endovascular aneurysm repair: These procedures use tiny instruments and advanced imaging equipment. That means there’s less pain and scarring^[20].

Aortic valve replacement is recommended for most symptomatic patients with evidence of significant aortic stenosis on echocardiography^[12]. Surgical valve replacement is the standard of care for patients at low to moderate surgical risk^[12]. Transcatheter aortic valve replacement may be considered in patients at high or prohibitive surgical risk^[12].

Living with Aortic Disorder

Once an aortic aneurysm develops, it is at risk of growing bigger^[17]. If you are diagnosed with an aortic aneurysm, your physician will want to see you regularly for imaging tests to ensure that the aneurysm is not growing too fast^[17]. It is very important for you to keep up with these health visits^[17].

You can help slow the growth of the aneurysm and prevent complications by paying close attention to other health problems you may have and living a healthy lifestyle^[17]. With close follow-up, good blood pressure control and a healthy lifestyle, many patients living with aortic aneurysms can do well and may not need an intervention^[17].

Managing Blood Pressure

The most important way you can slow the progress of an aneurysm is to control your blood pressure^[17]. If you have high blood pressure, the extra force pushes against the walls of the aneurysm causing it to expand^[17]. If you have been given medicine for your blood pressure, it is very important to take it exactly as instructed^[17]. Your doctor may also advise you not to lift heavy weights, which may cause sudden increases in your blood pressure^[17].

Lifestyle Changes

Smoking, obesity, drinking too much alcohol, and a sedentary lifestyle all contribute to high blood pressure^[17]. If you need to have surgery or a procedure to repair your aorta, your overall health would factor into your recovery^[17]. Therefore, it is important to^[17]:

• Keep a healthy weight or have a body-mass index (BMI) of less than 30
• Keep your blood pressure controlled
• Quit smoking, if you do. There are medications and counseling options available to help you stop
• Be physically active. You can do this by brisk walking or biking for at least 30 minutes on most days of the week
• Keep your alcohol intake to at most 1-2 drinks a day
• Reduce the amount of salt (sodium) you take into your body. Limit the amount of sodium you take in each day to less than 2,300 milligrams. You can track this by reading food labels. Many products now have low-salt versions, and there are also substitutes for table salt available in stores
• Adopt a healthy diet rich in fruits and vegetables, and low in cholesterol and saturated fats. This will help control your blood pressure as well as your cholesterol levels. It also will decrease the risk of aneurysm complications

Monitoring and Follow-Up

Patients should be educated about the importance of promptly reporting symptoms to their physicians^[12]. Watchful waiting is recommended for most asymptomatic patients^[12]. In asymptomatic patients, serial Doppler echocardiography is recommended every six to 12 months for severe aortic stenosis, every one to two years for moderate disease, and every three to five years for mild disease^[12].

Cardiology referral is recommended for all patients with symptomatic moderate and severe aortic stenosis, those with severe aortic stenosis without apparent symptoms, and those with left ventricular systolic dysfunction^[12]. Medical management of concurrent hypertension, atrial fibrillation, and coronary artery disease will lead to optimal outcomes^[12].