Are You a Walking Time Bomb?

Some of you may have read a recent front-page article in The Wall Street Journal. It was titled “Medical Ignorance Contributes to Toll from Aortic Illness.” The reporters discussed how all too often, the medical community is incompetent when it comes to diagnosing aortic aneurysms; not enough physicians and care providers are sufficiently knowledgeable about this precarious problem. Countless autopsies, performed on individuals whose sudden deaths occurred after a bout of chest pain, have revealed that an aortic aneurysm was the true culprit, and not a heart attack.

This was the case with someone I loved deeply. I was devastated and grief stricken when I failed to resuscitate my own father on the floor of my childhood bedroom. Can you imagine, with all my training, to lose one of the people to whom I owed my life and education? An autopsy revealed that no one could have saved my dad that day; his aorta had ripped open suddenly. He was seventy-six years young. He was so bright and vital, we’d never dreamed anything like this could be wrong. But there he was. He denied any chest pain. He had an abrupt onset of weakness, dizziness, and leg paralysis. How many times have I asked myself if there were any earlier, telltale signs that his physicians, and I, may have missed? Though I’ve written of this event and warning signs before, it’s a subject I’d like to revisit and update.1

The recent unexpected loss of actor John Ritter, only fifty-four years old, was the alarm clock that reawakened my own feelings of loss and made me aware of just how many people continue to walk around with undiagnosed aneurysms, just like my dad. You may have caught some of the media interviews that followed Ritter’s death, during which doctors described dissection (a weakened portion of the aorta that begins to tear) as rare and undetectable. Hearing such comments was, quite frankly, unsettling for someone like me. In my professional life as an interventional cardiovascular specialist (that’s one who does invasive procedures), I’ve seen my fair share of thoracic aortic aneurysms in all age groups. Some are slow tears, when there is time for intervention if the correct diagnosis is made. Blood leaks between layers of the arterial wall but can be repaired. But sometimes, the tear happens too quickly and forcefully for it to be repaired.

My first experience with this catastrophic situation was with a twenty-five-year-old fellow whose aortic wall tore apart during a free-fall parachute jump. Although emergency surgery saved this young man, he later went on to die of a second dissection when he was only in his mid-thirties. When this happened, twenty-five years ago, we lacked the technology or knowledge to predict it.

I recall with deep sadness another case. A fifteen-year-old girl had passed out while she was working at a nursing home. She was at the top of her school class, bright, alert, and engaging. I thanked God that day that I was able to diagnose her symptoms very quickly in our community hospital’s emergency room. I had high hopes that she would be fine if we acted fast. She was resting comfortably when I called the closest heart surgery hospital to mobilize the open heart team. When the chief of cardiac surgery challenged my diagnosis, I was firm and confident and stood my ground. His team scrambled to the operating room as the ambulance pulled in to transport her there. But we lost our race against time. As I explained what was happening to her and her parents that day, she smiled in acceptance. She looked so peaceful. We would learn afterward that her aorta had clotted itself and stabilized at that point. A moment later, she sat up. Looking startled, she exclaimed, “Oh!” and died in my arms as a secondary tear ruptured her entire aorta. It will always be a painful memory.

Some of you may recall another case from my book Heartbreak and Heart Disease. I shared the story of an older gentleman in a similar emergency situation. He was sixty-four years old and had just experienced an episode of intense emotional distress. His aorta began to dissect during a fit of rage. By the time I met him in the emergency room, he was hanging onto life by a thread. He was in cardiogenic shock, with profoundly low blood pressure and respiratory depression. Fortunately for him and his family, a quick diagnosis and early surgical intervention ended happily.

So you must be asking yourself by now, how common are thoracic aortic aneurysms (TAAs)? As I mentioned, a large number of TAAs are not apparent until an autopsy is performed. TAAs are also found in people who have died of other causes, lurking like a potential time bomb, just ticking away until it explodes.

Epidemiology and Genetics of Thoracic Aortic Aneurysms

It’s imperative that you understand how common TAA can be and the hereditary risks involved so you can seek evaluation if you, like me, are at risk. You see, it’s actually been estimated that aortic aneurysm is the thirteenth most common cause of death in the United States. Every year, there are approximately 5.9 cases of TAA for every 100,000 people, and the average age at diagnosis is fifty-nine to sixty-nine.

Cases of thoracic aortic dissection in young people, like the two I have shared with you, are usually due to a genetic vulnerability. Individuals with a predisposition to TAA develop premature necrosis, or cell death, of the medial (middle) layer of their aorta. In this condition, a loss of elastic fibers and smooth muscle cells leads to the accelerated aging of the aorta, rendering the artery weak and vulnerable to dissection.

We also know that there is an association between aortic aneurysms and two uncommon medical disorders: Marfan’s and Turner syndromes. Even with this knowledge, patients who have these conditions still have undetected and undiagnosed TAAs. So if you have one of these conditions, have yourself checked for TAA risk.

More commonly, we see a phenomenon known as familial, nonsyndromic TAA in about 19 percent of patients who have it. In other words, it runs in their families but is not associated with another medical syndrome. These patients are younger at the time of diagnosis (mean age 56.8 years) than those with sporadic TAA (mean age 64.3 years).

In sporadic TAA, some factor has provoked an episode of aortic dissection, such as extremely high blood pressure—this may have been the cause for the sixty-four-year-old enraged gentleman I mentioned. Preexisting high blood pressure is a contributing factor that’s been observed in 73 percent of cases of familial nonsyndromic TAAs and 75 percent of sporadic cases.

Early Detection Is a Must If You’re at Risk

Remember, awareness can be curative for many conditions, and TAA is one of them. My dad’s brother, my dear uncle Ben, also endured the pain of a growing aneurysm for many years before he died last year of unrelated causes. But he knew he had one, and he monitored it closely. With our strong family history, I’ve advised all my siblings and cousins that they be checked and that we check our kids and grandkids.

For those of you with a family history of aortic aneurysm, it is absolutely mandatory that your children, and even grandchildren, be assessed for thoracic aortic aneurysm. This is where knowing your family history can be life saving. Remember, The Wall Street Journal article said it all. Most doctors won’t think of ruptured aortic aneurysm if you find yourself in the emergency room, so you may have to be the one to raise the red flag. People with TAA can look perfectly fine when their arterial blood gas is checked. The electrocardiogram won’t show any changes either, leaving physicians perplexed as to why there is so much pain. Many thoracic aneurysms won’t even show up on a routine chest X ray, and rarely, folks like my dad won’t have any chest pain at all. The leg paralysis, dizziness, and weakness, like my dad had, are also atypical symptoms.

A high-resolution computed tomography (CT) scan will, however, provide a clear image of the aortic pathology in TAA. I’m going to repeat my own CT scan soon. Magnetic resonance imaging (MRI) is also frequently used, particularly for people who have poor kidney function that contraindicates the injection of contrast material. MRI is especially useful for complex cases, where imaging from multiple angles is key to diagnosis. In this regard, MRI can give you vital information. If the definition of branch vessel anatomy is required, some physicians will also order a magnetic resonance angiography. Angiography is performed to evaluate the need for surgery.

Other simple diagnostic tools that can detect a large number of dissections—and even dilatation of aortic root—are the aortic ultrasound and the transesophageal echocardiography, where the definitive anatomy of the aortic valve and ascending aorta can be displayed. Again, the problem with aortic dissection is that most physicians don’t recognize it or even think about it. Once the aorta tears, death can happen within two hours, if not sooner. So the importance of a correct diagnosis cannot be overstated.

Think of a Zebra

Thirty years ago, my interns nicknamed me “Zebra.” Why? I had an expression that went like this: “When you hear the hoof beats, what do you think of? The common answer is horses. But a good diagnostician must always think of zebras,” the less obvious, unexpected condition. TAA is a zebra diagnosis.

So I advise you to get your own doctor thinking about zebras. If you have any family history of TAA, or any undiagnosed chest pain, plant the seed for your concern about an aortic aneurysm with your MD, PA, or nurse practitioner. Acute dissection is a situation that you do not want to find yourself facing. If they won’t cooperate, or reject the idea, stand firm and insist on testing.

And What If You Know You Have an Aortic Aneurysm?

If you’re one of the unfortunate individuals who are diagnosed with an aortic aneurysm, it’s important to understand the natural history of thoracic aortic aneurysms and how it applies to you. First of all, thoracic abdominal aneurysms grow an average of about 0.1 cm a year. So have checkups and diagnostic evaluations at least annually, and more often should your symptoms change. And you’ll also want to know at what point surgery is indicated and the risks of that surgery compared to the risk of not having it. In my uncle’s case, his risk of dying during surgery was excessively high, and so he endured the discomfort of a growing aneurysm that limited his lifestyle rather than run that risk. Let’s look at research that can help sway your decision.2

The Yale Experience

Here in my home state of Connecticut, a ten-year study was conducted at Yale University, where both my wife and I did some training. Yale developed a large database of patients with thoracic aneurysms and dissections. Specialized statistical methods were applied to an accumulated population of 1,600 people with TAA dissection and included 3,000 serial imaging studies and 3,000 patient years of follow-up. It is really good data.

The major cutoff points for natural complications of aortic aneurysm (rupture or dissection) were found to be 6 cm for the ascending aorta (which comes right out of the heart) and 7 cm for the descending one (the portion that leads through the diaphragm and into the abdomen). Yearly event rates suggest that anyone whose thoracic aorta reaches a 6-cm maximal diameter faces significant risks for a devastating cardiac event: a 3.6 percent rupture rate; a 3.7 percent dissect rate; and a 10.8 percent mortality rate.

Their reported surgical risk of death from aortic surgery of thoracic aneurysms was 2.5 percent for the ascending aorta and the aortic arch and 8 percent for the descending and abdominal aorta. In fact, the Yale report of a 2.5 percent for the ascending aorta is a conservative estimate. Most centers predict a 5 to 10 percent mortality rate for this population. Interestingly, genetic analysis in the Yale study revealed that 21 percent of patients with TAA have a first-family member with some arterial aneurysm. This is similar to previous data reporting a 19 percent occurrence rate.

My Surgical Guidelines

What does this all mean to you? On the basis of these findings, I set guidelines for my own surgical recommendations. In asymptomatic people, I suggest surgery only if your thoracic aneurysm is 5.5 cm or greater or your abdominal aorta measures 6.5 cm or more. If you are symptomatic with chest pain, or have a tear or dissection, then you must be operated on, regardless of the size of your aneurysm. Remember, anyone who has an aortic aneurysm, but doesn’t know it, is at risk for sudden cardiac death. You don’t want that to be the first sign that you have a problem. This large Yale trial is reaffirmation that anyone with a family history be evaluated.

I immediately thought of this disorder when my sister experienced severe chest pain, but imaging studies of her aorta showed that she did not have an aortic aneurysm. Who knows if the tragic and unexpected death of John Ritter as well as many others might have been prevented with early diagnosis and intervention? One thing is quite reassuring: we know that nonemergency surgical repair of aneurysms has clearly had a positive impact on the natural history of the disease.

The Wall Street Journal said it all. Many physicians may not consider this diagnosis, so your awareness of it can be life saving. We now have the science to easily diagnose TAA with simple, noninvasive imaging techniques, so we can treat it successfully.

Most importantly, if you do uncover this life-threatening condition in yourself or a loved one, please be vigilant about it. Wear a Medic Alert bracelet. If yours is a family history of thoracic aortic aneurysm—or even a sudden death for which a cause was not clearly defined at autopsy—you must be proactive and get evaluated. Should you recognize the symptoms, take yourself or any of your family members to an emergency room for a medical assessment, and remember to tell them about zebras.

Bibliography

Coady, M. A., R. R. Davies, M. Roberts, L. J. Goldstein, M. J. Rogalski, J. A. Rizzo, G. L. Hammond, G. S. Kopf, and J. A. Elefteriades, “Familial Patterns of Thoracic Aortic Aneurysms,” Archives of Surgery 134 (1999): 361–67.
Coady, M. A., J. A. Rizzo, L. J. Goldstein, and J. A. Elefteriades, “Natural History, Pathogenesis, and Etiology of Thoracic Aortic Aneurysms and Dissections,” Cardiology Clinics 17 (1999): 615–35.
Coady, M. A., J. A. Rizzo, G. L. Hammond, G. S. Kopf, and J. A. Elefteriades, “Surgical Intervention Criteria for Thoracic Aortic Aneurysms: A Study of Growth Rates and Complications,” Annals of Thoracic Surgery 67 (1999): 1922–26.
Elefteriades, J. A., “Natural History of Thoracic Aortic Aneurysms: Indications for Surgery, and Surgical versus Non-surgical Risks,” Annals of Thoracic Surgery 74 (2002): S1877–80.

** This article is one of 101 great articles that were published in 101 Great Ways to Improve Your Health. To get complete details on “101 Great Ways to Improve Your Health”, visit http://selfgrowth.com/healthbook3.html