Disease of the Aorta

Disease of the Aorta Lindsey Cook, CNP Coordinator COACH: Central Ohio Adult Congenital Heart Program The Heart Center at Nationwide Children’s Hospi...
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Disease of the Aorta

Lindsey Cook, CNP Coordinator COACH: Central Ohio Adult Congenital Heart Program The Heart Center at Nationwide Children’s Hospital

Normal Heart

Aorta • The major artery that is responsible for supplying the body with oxygenated (red) blood from the heart. It is composed of various sections.


Aortic Root

•The beginning of the aorta that is attached to the heart. This gives rise to the right and left coronary arteries which supply the heart muscle with oxygen.

Henry Gray (1825–1861). Anatomy of the Human Body. 1918.

Ascending Aorta

• The beginning of the aorta (includes the aortic root). This portion extends from the heart to the aortic arch (or bend of the aorta).

Henry Gray (1825–1861). Anatomy of the Human Body. 1918.

Aortic Arch

•The aortic (or transverse) arch gives rise to arteries that supply oxygenated blood to the head and arms.

Henry Gray (1825–1861). Anatomy of the Human Body. 1918.

Descending Aorta

•The descending aorta (lower portion of the aorta) supplies blood to the lower body.

Henry Gray (1825–1861). Anatomy of the Human Body. 1918.

Layers of the Aorta • The walls of the aorta consist of three layers: • Tunica intima (inner layer) • Tunica media (middle layer) • Tunica adventitia (outer layer) • These layers are made of connective tissue and elastic fibers which allow the aorta to stretch www.marfan.org/nmf

Aortopathy • Definition: – Any disease of the aorta • Aortic enlargement • Aortic aneurysm • Aortic dissection

Associated Conditions • • • •

Bicuspid aortic valve Coarctation of the aorta Tetralogy of Fallot Connective tissue disorders – Marfan syndrome

• Turner syndrome • Single Ventricle (Fontan)

Bicuspid Aortic Valve Most common heart defect (1-2% of the population) •

• Affects twice as many males

Normal, trileaflet aortic valve

• Defect of the aortic valve where there are two valve leaflets instead of three Bicuspid aortic valve, closed and open

Bicuspid Aortic Valve Cardiac MRI

Bicuspid Aortic Valve Aortopathy • The aortic tissue is also abnormal (tunica media) • The aortic wall may become weak over time, leading to enlargement or aneurysm of the ascending aorta • In some cases, this can lead to aortic dissection (tearing)

Coarctation of the Aorta (CoA)

Coarctation of the Aorta Occurs in 6-8% of congenital heart disease Affects twice as many boys than girls May be associated with genetic syndromes (Turner’s syndrome in girls) May have other heart defects as well (bicuspid aortic valve)

Treatment OptionsSurgical Patch augmentation

Treatment OptionsSurgical End-to-end anastomosis

Treatment OptionsSurgical Subclavian flap repair

Coarctation of the Aorta

Treatment OptionsTranscatheter Balloon angioplasty

Coarctation of the Aorta Aortopathy • The aortic tissue is abnormal • May develop aortic enlargement of the ascending aorta (if associated with bicuspid aortic valve) • May develop aneurysms at previous surgical repair sites – Highest incidence after patch graft procedure – May be as high as 50% • Also reported with end-end anatomosis, subclavian patch, and interposition graft

Aortic Aneurysm • Definition- enlargement of the aorta more than 50% more than the normal size. • Involves all three layers of the aortic wall • Ascending aortic aneurysms are the most common • Most patients have no cardiac symptoms

Long Term Complications Aneurysm/Pseudoaneurysm 60% –


50% –


40% – 30% – 25% 22%

20% – 12.8%

10% –

6% 2.8%


< 20

20 - 39

> 39

Age (years) Oliver JM, et al. JACC 2004;44:1641-7

Long Term Complications Aortic aneurysm

COA - Aortic Aneurysm

Coarctation of the Aorta Aortic aneurysm

Long Term Complications Aortic Aneurysm

Long Term Complications Aortic Pseudoaneurysm Aortic angiogram/ICE catheter

Tetralogy of Fallot • Ventricular septal defect (VSD) • Pulmonary stenosis • Overriding of the aorta • Right ventricular hypertrophy

Tetralogy of Fallot Aortopathy • Involves the ascending aorta • Thought to be a result of increased blood flow to the aorta over a long period of time (particularly pulmonary atresia, history of a cardiac shunt) • Also related to abnormality of the medial layer of the aorta • Enlargement of the ascending aorta may lead to aortic regurgitation (leaking of the aortic valve)

Tetralogy of Fallot Aortic Enlargement

Connective Tissue Disorders Marfan Syndrome • •

• • • •

Genetic mutation that can be inherited Disorder of the connective tissue (tissue that holds parts of the body together and controls how the body grows) Affects multiple body systems, including the heart Results in significant enlargement of the aortic root May involve other parts of the aorta as well Progressive over time

Marfan Syndrome Aortic Enlargement Normal

Marfan Syndrome

Enlarged Aorta




LA Mitral Valve

Aortic Valve





Prolapsed Mitral Valve

Aortic Enlargement Over Time

Normal Aorta


Enlarged Aorta

Aortic Root Enlargement Echocardiogram

Graph of Aortic Diameters

Aortic Root Enlargement MRI

Additional Considerations in Aortopathy • Each of these also affect the aorta, resulting in less elasticity: – Increasing age – Pregnancy – High blood pressure

Treatment Aortic Enlargement • Medicines are sometimes used to lower blood pressure and decrease the forcefulness of the heartbeat – Beta blockers – Angiotensin receptor blockers (ARBs)

• The goal is to slow the rate of aortic growth

Aortic Dissection • Definition- a tear in the lining of the artery that allows blood to flow between the layers of the blood vessel but remains contained in the aorta • Rupture of an aortic dissection may lead to significant bleeding outside the layers of the aorta • Type A Dissection – Begins in the ascending aorta

• Type B Dissection – Begins in the descending aorta

Aortic Dissection

Aortic Dissection

Signs of Aortic Dissection • May depend on the area of dissection • Severe chest or back pain that occurs suddenly – May be sharp, stabbing, or ripping pain

• • •

Severe abdominal pain Extremely low blood pressure Shock (Fast pulse, sweating, nausea, vomiting, dizziness, fainting, weak or absent pulse)

Aortic Dissection CT Scan

Aortic Dissection CT Scan

Aortic Root Replacement Surgery for Aortic Aneurysm/Dissection Marfan Syndrome Aortic Graft

Prosthetic Valve

Aortic Root and Aortic Valve Replacement

Native Valve

Aortic Root Replacement with Valve Sparing

Regular Screening is Essential

Prevention and early identification of problems is key in staying healthy

What Routine Testing Can I Expect?

• •

Echocardiogram Blood pressure monitoring • Cardiac MRI • Cardiac CT scan

What can I do to stay healthy? Long-term clinic follow-up Routine cardiac studies, cholesterol labs Take medications as they are prescribed Be aware of cardiac symptoms & report them Avoid tobacco products Use alcohol only in moderation Weight control Healthy diet (low fat, cholesterol, sodium) Good blood pressure control Avoid dietary or medication sources of stimulants

What should I know about exercise? The best activities are aerobic: Walking Swimming Biking Water aerobics Low impact aerobics Isometric activities put more strain on the heart: Heavy weight lifting Push-ups Pull-ups/chin-ups Activities to the point of grunting or straining