John Ritter’s death from an aortic dissection raises questions about the condition. Source: Baltimore Sun

While a California jury has cleared a cardiologist and a radiologist accused of negligence and liability in a malpractice lawsuit involving the death of actor John Ritter, aortic dissection remains a challenging diagnosis. Cardiology leaders need to pay close attention to this pathology in order to avoid costly litigation involving a misdiagnosis.

Aortic dissection “is not a common disorder, so it’s often not the first thing we [as cardiologists] consider when a patient presents,” said Clyde W. Yancy, MD, medical director and chief of heart transplantation at Baylor Heart and Vascular Institute in Dallas.

Yancy noted that his comments are not in reference to any specific cases. His comments to Cardiovascular Business News are about aortic dissection in general and the complications associated with its management.

An excellent way to identify a patient with an aortic dissection is by the intensity and the description of the pain. A classic description is the patient will feel “ripping, tearing, cutting or stabbing” in their chest or back, Yancy said.

While this symptom could potentially be confused for angina or coronary disease, Yancy said that a CT scan of the chest is the best method to identify aortic dissection. It can be done quickly and the contrast will identify the tear’s location.

The gold-standard has been the aortogram, but that is often harder to obtain compared to the CT, Yancy said. He noted that transthoracic MRI and echocardiography are also used to identify discontinuity in the aorta.

The symptoms for aortic dissection are fairly classic, as well as consistent, according to Yancy. There is a high acuity rate, and a high mortality rate, particularly with the initial presentation, and even in the long-term treatment, there can be some challenges.

“With fewer than about 10,000 cases a year, it’s important to know that once a dissection has occurred, the patient’s life can be in jeopardy, and particularly if it goes undiagnosed,” Yancy said.

Aortic dissections can have a congenital origin, those born with an inherent weakness of the blood vessels. The prototype at-risk patients are those with Marfan’s syndrome, which is often hereditary, Yancy said. As a result, cardiologists should pay particular attention to family history.

The other type of aortic dissection happens when a patient acquires a weakening of the aorta, which can occur with age and atherosclerosis, and can be exacerbated through high blood pressure.

Treatment depends on the location of the tear. For a type I/A dissection, the tear occurs at the aorta-ventricle junction or very close to the heart; this invariably requires surgical treatment—generally a composite root replacement that replaces the first segment of the aorta, the aortic valve and involves re-implantation of the coronary arteries. “It is not without risk,” Yancy said.

A type II/B dissection, which occurs in the distal or downstream aorta, usually happens in older patients, those with high blood pressure, diabetes, or who are at risk for atherosclerosis, Yancy said.
 
Distal dissection often can be treated by controlling blood pressure and changing lifestyle, Yancy said. The surgery for distal dissection is fairly high-risk because it potentially involves interrupting blood flow to the spine, which could result in paralysis with only the slightest error, he noted.

“Many times, we are reluctant to operate on patients with distal dissections because the complication rate is real, and if we can control the patient’s blood pressure, their kidney disease, and their hardening of the arteries, we can help these patients make it through; in fact, many will do as well with medical therapy as they might with surgery” Yancy said. 

Stent grafts that don’t require a surgical approach have been used to treat distal dissection, in order to help support the aorta’s weaker points. But for now, surgery is the best choice, Yancy noted.

An annual CT scan of high-risk patients will allow cardiologists to monitor the stability of blood vessels. It will also allow them to see whether gradual or smaller tears are occurring, because changes can be very subtle, according to Yancy, who recommends screening the family members of high-risk patients as well.

In the Ritter case, his wife Amy Yasbeck filed a lawsuit against Los Angeles-based cardiologist Joseph Lee, MD, and radiologist Matthew Lotysch, MD, seeking damages of $67 million. The jury decided in favor of the doctors, holding that they were not negligent or responsible for Ritter’s death.