John Ritter’s Life-Saving Rules

            Just six days shy of his 55th birthday, John Ritter—the Emmy-award winning sitcom star—wasn’t feeling quite right.  Ritter, best known for playing the rakish Jack Tripper on the 70s/80s television classic “Three’s Company,” was rehearsing lines on the set of his latest show, “8 Simple Rules for Dating My Teenage Daughter.”  On the program, Ritter played the stern-but-loving father to a pair of teenage girls.  In real life, his youngest daughter was turning three that very day.

            What should have been a time for celebration quickly took a turn when Ritter began complaining of the classic symptoms of a heart attack: he was sweaty, nauseous and experiencing pain in his chest.  He was rushed to a nearby hospital where doctors confirmed the diagnosis and immediately began treatment.  There was just one issue: he wasn’t actually having a heart attack—a problem with the small arteries that feed the heart; he was having an aortic dissection—a problem with the biggest artery in the entire body. 

            Aortic dissections occur when the inner wall of the aorta becomes torn.  Like other arteries, the Aorta is essentially a hollow tube that carries blood away from the heart.  The walls of that tube are made up of three layers: an inner layer, a middle layer, and an outer layer.  When the inner layer rips, blood can get redirected into the tear itself, creating a pocket in between the inner layer and the middle layer.  Instead of going to vital organs like the brain, liver and kidneys, this blood is trapped within the aorta’s walls.  And as more blood gets trapped, the tear itself gets bigger, worsening the problem.  That can lead to a stroke (if not enough blood reaches the brain), damage to the heart’s valves (if the dissection extends to the area where the aorta comes off the heart), and most deadly of all, a complete tear through the entire wall of the aorta—also called an aortic rupture.

            What causes that tear in the first place?  Just like a rip in fabric, a tear in the aorta comes from underlying weakness.  That weakness may be there from the start, it may develop gradually over time, or it can happen very quickly.  For your aorta, inherent weakness can be due to genetic disorders; slow-developing weakness usually comes from high blood pressure (aka hypertension); and sudden damage comes from traumatic events, like a car accident.

            Whatever the cause, once a tear develops, pain usually follows.  Medical textbooks usually describe it as a severe chest pain that starts abruptly and is associated with a ripping sensation.  That’s in contrast to the typical description of heart attack chest pain, which is characterized by feelings of pressure and tightness.  In the real world, though, these symptoms are not always so distinctly different, making the diagnosis of aortic dissection challenging and occasionally leading to delayed treatment.

            In Ritter’s case, the proper treatment was delayed too long.  On September 11th, 2003, John Ritter passed away as surgeons worked to repair his dissection.  It was a tragic ending to a life devoted to laughter, but it would not be the end of John Ritter’s impact on the world.  Shortly after his passing, Ritter’s wife, the actress Amy Yasbeck, formed The John Ritter Foundation (JRF), a charity devoted to educating people about aortic dissection and providing support to those affected by the disease. 

            Through the JRF, a set of guidelines—the Ritter Rules—was established to help recognize, diagnose and prevent aortic dissections.  As seen on the JRF’s website, the Ritter Rules are:

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 . Some people report feeling that 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 diagnosis is not clearly and quickly established, then aortic dissection should be quickly considered and ruled out. This is especially important if a patient has a family history of thoracic aortic aneurysm/dissection 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. Note: 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 are 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 aneurysm and/or dissection. You and other family members should be evaluated to determine if a predisposition for aortic aneurysm and dissection is running in your family.

RISK FACTOR:

Certain genetic syndromes that affect connective tissue 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, vascular Ehlers-Danlos syndrome, and Turner 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  aneurysm and/or 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.

We remember John Ritter as a sitcom star. But the Ritter Rules may be his greatest legacy. 

For more on Aortic Dissection, visit Aortic Surgery Program site.

For more on The John Ritter Foundation, visit: http://johnritterfoundation.org/