Dear Dr. Andersen,

I am a 50-year-old woman and my first cousin almost died from a rare heart condition, a ruptured thoracic aneurysm. While at a concert, she suddenly experienced a severe pain across her back. She knew something terrible was happening and had the good sense to have her husband take her to the lobby, where she fainted. Luckily, she was only one block away from the hospital and was in the ER in five minutes. It was a miracle she survived. She had not totally ruptured the aneurysm, according to the surgeons, which is why she did survive.
Should I be evaluated? What information should all of us know about how to respond to heart symptoms? Thanks so much,

Shirley



Illustration: Cleveland Clinic



Dear Shirley,

I am glad to hear your cousin sought immediate medical attention, that she was diagnosed correctly and that she is now okay. She is indeed lucky. Fifty percent of patients with a ruptured, or “dissecting,” aortic aneurysm die before reaching a hospital. And even if they reach a hospital, patients are often misdiagnosed. After the rupture, or “dissection,” occurs, there is a 1 to 2 percent mortality per hour in the first 24 hours, 75 percent die in the first two weeks, and 90 percent die in the first three months if not appropriately treated. This is not a commonly recognized condition, but public awareness has increased since the death of John Ritter.

The aorta is the biggest artery (blood vessel) in the body. It arises from the heart, arches in the chest (thorax) and extends down through the lower abdomen carrying blood to all parts of the body. Arterial walls are layered with muscle and connective tissue. An aortic aneurysm occurs when a portion of the muscular wall weakens, allowing the aorta to dilate or bulge. Such aneurysms are prone to dissection. This occurs when the inner layers of the arterial wall tear, allowing blood to flow into the tear separating the inner and outer layers, thus creating a “dissecting aneurysm.” If this occurs in the chest, it is called a thoracic aortic dissection.

Having a family history of an aortic aneurysm does predispose you. So yes, Shirley, you should be screened. Other risk factors include hypertension, coronary artery disease, smoking, blunt trauma to the chest, and genetic disorders such as the Marfan syndrome, Ehlers-Danlos syndrome and bicuspid aortic valve. The Marfan and Ehlers-Danlos syndromes have distinct genetic abnormalities which render the body’s connective tissue more “distensible,” or stretchable. People with these syndromes are very flexible; those with Marfan’s are additionally often tall with long extremities. Thoracic aortic aneurysms are more common in men, but there is some evidence to suggest they are more likely to rupture in women. Most patients have no symptoms until the aneurysm leaks or ruptures. The physical exam is most often normal. A chest CT scan is the best way to screen for this condition. Once an aneurysm is diagnosed, strict of control of blood pressure and avoidance of smoking are crucial interventions.

A class of medications called “beta-blockers” have been shown to slow the dilation of the aorta and are therefore the mainstay of therapy. A newer medication, losartan, shows promise in reversing the connective tissue abnormalities in some patients, but this is still considered investigational. Patients are monitored by cardiothoracic surgeons who have an expertise in this field, with chest scans every 6 to 12 months. Surgical intervention is recommended if the aorta dilates quickly (by 0.4 cm in one year) or to a maximum diameter of > 5.0 to 5.5 cm in the ascending aorta, the aorta closest to the heart, or > 6.0 cm in the descending aorta, which is further downstream. If severe leakiness of the aortic valve exists, surgical repair is recommended at a diameter of 4.5 cm.

Symptoms of a dissecting aortic aneurysm are often sudden. Patients frequently complain of a sharp or tearing pain in the chest or back, especially between the shoulder blades, similar to what your cousin experienced. The presentation, however, can be much less dramatic, making the diagnosis even more challenging. John Ritter’s main symptoms were nausea and sweating. Other symptoms include dizziness, a fast heart rate and shortness of breath.

As aforementioned, the condition is often misdiagnosed. Electrocardiograms and blood tests are often absolutely normal. An astute practitioner might notice a discrepancy which can occur between the blood pressure or pulses in the arms, or between the arms and legs. Imaging tests are needed to confirm the diagnosis. Sometimes it can be seen on a simple chest x-ray, but most often a CT scan or MRI of the chest, or an echocardiogram, is needed. Dissections that involve the ascending aorta must be treated with surgery. If they involve only the descending aorta, they are most often managed with medications alone.

Traditional surgical repair is done by removing the aneurismal portion of the aorta and replacing it with a Dacron graph. If the aortic valve is involved, it too may need to be replaced or repaired. Under investigation are stent-like devices called “endographs” that can be inserted through an artery in the leg and fed up into the thoracic aorta and deployed in the affected region. These devices have been approved and are currently being used for repairing abdominal aortic aneurysms.

The surgery to repair dissected aortic aneurysms is no walk in the park. Operative mortality across the board, for patients from 20 to 30 centers of aortic expertise listed in the international registry of “aortic dissection,” is traditionally 20 to 25 percent. At the Ronald O. Perelman Heart Institute, at the New York Presbyterian Hospital, Dr. Leonard Girardi specializes in thoracic aortas and leads the thoracic surgery team. He reports that over the past 11 years, the center has repaired over 210 dissections, with an operative mortality of approximately 4 percent. Clearly, screening, early detection and elective repair is preferred. Thank you, Shirley, for the increased awareness about this very important medical condition.

Assistant Professor of Medicine at the Weill Cornell Medical Center, Dr. Holly Andersen has additionally served as the Chief Medical Resident for the Department of Medicine, and Director of Education and Outreach for the Ronald O. Perelman Heart Institute at The New York Presbyterian Hospital. She currently serves on the board of directors for the Michael J. Fox Foundation for Parkinson’s Research, the President’s Council for the International Women’s Health Coalition, and the National Advisory Board for the Women’s Sports Foundation. She has also been an expert panelist on Internet webcasts and an on-air medical consultant to ABC World News Tonight, MTV, the CBS Evening News, NBC Evening News, The Early Show, the Fox Television Network, the Fox News Channel and the British Broadcasting Company. Dr. Andersen speaks extensively on preventive cardiology, and cardiovascular disease in athletes and in women.

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  • Patty Young May 23, 2009 at 8:37 pm

    Our nephew (one of five boys) was playing basket ball and told his brother he was going to the sidelines because he didn’t feel well. Daniel (30 years old and newly married) collapsed as he walked off the court. Despite immediate medical help, he was pronounced dead upon his arrival to the hospital. The autopsy showed he died of a similar aneurysm – an aortic aneurysm.

    Daniel’s brothers are currently getting tested because their physician said it was absolutely mandatory because they have a genetic vulnerability to this disease.

    No on could have saved Daniel that day. The doctors said he may have had some symptoms that he dismissed. We are still healing from our loss, but wanted to encourage everyone to listen to their body and to be aware of even the smallest changes. It is also just as important to know your medical history.
    A physician once said, “When you hear hoof beats, what do you think of? The common answer is horses. But a good diagnostician must always think of zebras!” Don’t forget about the zebras in your life and the lives of your loved ones.

    Reply