“With Love and Laughter, John Ritter”

Yesterday morning, actress Amy Yasbeck appeared on the Today show to promote her new book, “With Love and Laughter, John Ritter.” Yasbeck spoke with Meredith Vieira about her life with late husband John Ritter, star of “Three’s Company” and “8 Simple Rules for Dating My Teenage Daughter.” Ritter died in 2003 as a result of an undiagnosed aortic dissection. Since that time, Yasbeck has worked tirelessly to promote public awareness about this dangerous condition, founding the John Ritter Foundation for Aortic Health. Their mission is to promote knowledge of aortic aneurysm and dissection through research and education. Working with the TAD (Thoracic Aortic Disease) Coalition, the Ritter Foundation is working to increase public awareness of new Treatment Guidelines for Thoracic Aortic Disease released in March of 2010 from the American Heart Association (AHA) and the American College of Cardiology (ACC) as well as other interested groups. Part of this campaign includes what are now called the Ritter Rules, life-saving reminders to recognize, treat and prevent thoracic aortic dissection (see below).

What is aortic dissection?

The aorta is the main blood vessel that takes blood from the heart to the rest of the body. Like all blood vessels, its walls are made up of three layers:

• the inner lining (which is in contact with the blood) called the tunica intima (commonly called the intima), which is made up of endothelial cells- thin smooth cell which reduce friction through the blood vessels.
• the tunica media which is made up of smooth muscles and elastic tissues
• the tunica adventia, the outer layer of the blood vessel made of a more fibrous material.

Blood dissecting from the tunica intima to the tunica media

In aortic dissection, damage to the intima layer allows blood to dissect its way into the media layer. Over time, the wall of the blood vessel balloons out. This weakens the wall of the aorta, and if it reaches a critical point, the wall can rupture, leading to sudden death. The dissection can occur anywhere along the length of the aortic, either close to the heart (proximal dissection) or lower down in the abdomen (distal dissection). Aortic dissection occurs more frequently in men, and between the ages of 50-55 for proximal dissection, 60-70 years old for distal dissection. Factors that can increase the risk of aortic dissection include high blood pressure, an abnormal aortic heart valve (especially one called a bicupsid valve where the valve is made of two instead of the normal three leaflets), family history of aortic dissection, and certain genetic conditions, such as Marfan’s Disease and Turner’s Syndrome.

What are the Ritter Rules? (Reproduced here with permission from the TAD Coalition)

URGENCY: Thoracic aortic dissection is a medical emergency. The death rate increases 1% every hour the diagnosis and surgical repair are delayed.

PAIN: Severe pain is the #1 symptom. Seek immediate emergency medical care for a sudden onset of severe pain in the chest, stomach, back or neck. The pain is likely to be sharp, tearing, ripping, moving or so unlike any pain you have ever had that you feel something is very wrong.

MISDIAGNOSIS: Aortic dissection can mimic heart attack. Heart attacks are far more common than aortic dissection. But if a heart attack or other important diagnosis is not clearly and quickly established, then aortic dissection should be quickly considered and ruled out, particularly if a patient has a family history or features of a genetic syndrome that predisposes the patient to an aortic aneurysm or dissection.

IMAGING: Get the right scan to rule out aortic dissection. Only three types of imaging studies can identify aortic aneurysms and dissections: CT, MRI and transesophageal echocardiogram. A chest X-ray or EKG cannot rule out aortic dissection.

RISK FACTOR: Aortic dissections are often preceded by an enlargement of the first part of the aorta where it comes out of the heart, called an aortic aneurysm. If you have an aneurysm, you are at increased risk for an aortic dissection.

RISK FACTOR: A personal or family history of thoracic disease puts you at risk. If you or a family member is living with an aneurysm or if you have a family member who has had an aortic dissection, you are at an increased risk for thoracic aortic dissection. You and your other family members should be evaluated to determine if a predisposition for aortic aneurysm and dissection is running in the family.

RISK FACTOR: Certain genetic syndromes put you at risk. These genetic syndromes greatly increase your risk for thoracic aortic disease and a potentially fatal aortic dissection: Marfan syndrome, Loeys-Dietz syndrome, Turner syndrome and vascular Ehlers-Danlos syndrome.

RISK FACTOR: Bicuspid aortic valve disease puts you at risk. If you have a bicuspid aortic valve (two leaflets instead of the typical three), or have had a bicuspid aortic valve replaced, you need to be monitored for thoracic aortic disease.

TRIGGERS: Lifestyle and trauma can trigger aortic dissection. It is possible to trigger an aortic dissection through injury to the chest, extreme straining associated with body building, illicit drug abuse, poorly controlled high blood pressure or by discontinuing necessary blood pressure medications. Rarely, pregnancy can trigger an aortic dissection. However, women with aortic aneurysms and connective tissue disorders who are pregnant are at higher risk of aortic dissection during late pregnancy and delivery and should be carefully monitored by a cardiovascular specialist.

PREVENTION: Medical management is essential to preventing aortic dissection. If you have thoracic aortic disease, medical management that includes optimal blood pressure control, aortic imaging and genetic counseling is strongly recommended. Talk with your physician.

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