Newswise — A hunch on the part of paramedics may have saved Richard Houchin's life when the inner lining of his aorta split apart a year ago. Maybe the Playa del Rey resident is alive today because he collapsed while standing with a business associate who quickly called 911, or because he ended up at Cedars-Sinai Medical Center in the care of Sharo Raissi, M.D., director of Thoracic Aortic Surgery, and a world-recognized specialist in the thoracic aorta.
"A lot of people think that I was very lucky. We always correct that and say it was totally a miracle that I'm still here," says Richard, 63, who marvels at the series of events that took place Feb. 4, 2004, and acknowledges the prayers offered on his behalf by family, friends and members of his church.
He and his wife, Mary, who own rental property and real estate, had just eaten lunch at a local restaurant with a contractor who was going to do some work for them. Mary headed home while Richard and the contractor went to one of the couple's warehouses down the block.
"We were just standing there by my car, talking. He was getting ready to leave. In probably another 30 seconds, he would have left," Richard recalls. But his clear recollections stop there.
"Brian, my throat is burning," he reportedly blurted as he collapsed.
When paramedics arrived and contacted a medical center by radio for consultation, the emergency room staff suspected a heart attack and recommended medications to increase Richard's blood pressure and thin his blood. The paramedics, however, suspected a dissecting aortic aneurysm, which would require exactly the opposite treatment. They transported him to the closest hospital for more thorough evaluation.
The aorta, the largest artery in the body, is responsible for conducting oxygen-rich blood from the heart to arteries feeding all areas of the body. The section in the chest " the thoracic aorta " rises from the heart (ascending aorta) and curves (aortic arch) before descending into the abdominal cavity. The wall of the aorta consists of three layers " a thin inner layer, a thicker, elastic middle layer, and a thin outer layer. At any point along its length, a weakness in the connective tissue of the wall can cause an aneurysm, a bulge.
If the inner layer tears and separates from the outer layer " called dissection " blood passes into the middle layer, disrupting circulation within the aorta but not escaping into the body. If the thin outer layer then ruptures, massive internal bleeding occurs instantly and there is almost no chance of survival. The ascending aorta is especially vulnerable because it has little support around it and the volume of blood being pumped from the heart is at its highest.
In any case, emergency intervention is essential, and an accurate diagnosis is critical. "If a patient receives the wrong diagnosis, they may get the wrong treatment and end up going for the wrong procedure," says Raissi, adding that the chance of surviving a thoracic aortic dissection without treatment is estimated at five to 10 percent.
"A patient may be given blood thinners (anticoagulants), and then be taken for a coronary angiogram, which can be extremely dangerous for a freshly dissected aorta. Any manipulation with guide wires and catheters in an aorta that is barely holding on with a very thin layer is dangerous because all you have to do is make a hole in that thin outer layer and the patient will almost certainly die."
Fortunately for Richard Houchin, the local hospital proceeded quickly but with caution. Mary, a registered nurse, says the care was "wonderful," but as the seriousness of Richard's condition became increasingly clear, she wondered if a small community hospital would be equipped to provide the level of care he needed. After an afternoon of tests, the doctors made their diagnosis and decision. Richard had a tear in a large ascending aneurysm that extended all the way down the descending aorta, and he would be transferred to Cedars-Sinai for emergency surgery.
"When I heard Cedars-Sinai, I said, 'Oh, wow.' That was a big relief to me," says Mary, who has remained familiar with the reputations of area hospitals even though she is not working in the nursing field.
Although surgeons at many hospitals in the United States perform aortic surgery, Cedars-Sinai is one of only a few in the country that offer specialized thoracic aortic surgery expertise. "This level of expertise is becoming increasingly available throughout the world," Raissi notes, "but until just a few years ago, only about a dozen aortic surgery specialty programs existed in the world."
From 8:30 to 11:30 the night Richard was transferred to Cedars-Sinai, a surgical team directed by Raissi replaced the entire ascending aorta with a Dacron® graft. Dacron® is flexible, strong and durable enough to exceed the human life span. And because it is completely compatible with body tissue, there is no risk of rejection or calcification.
Raissi also repaired the aortic valve " and found a clue to the reason for Richard's aortic dissection. Although the valve's leaflets were healthy and functioning well, there were only two (bicuspid) instead of the normal three. Bicuspid aortic valve disease is one of several disorders that greatly increase risk of aortic aneurysm and dissection.
For 24 minutes of the three-hour operation, Raissi used a technique called total circulatory arrest with hypothermia. By lowering the patient's body temperature to 11 to 12 degrees Celsius (about 52 degrees Fahrenheit), circulation can be completely stopped because virtually all body function ceases. Although 40 minutes is considered the safe limit, Raissi typically can accomplish the most critical part of an operation to remove part of the aorta and connect a graft to the aortic arch in about 20 to 25 minutes.
Aortic repair is considered one of the most difficult surgical procedures in the chest, but years of experience and innovation have produced dramatically improved patient outcomes. Refinement of surgical techniques and appropriate blood pressure control peri-operatively have immediate and longer term benefits. Patients recover well and are not subject to subsequent problems, avoiding additional costly, complex surgeries.
"During surgery, we perform more extensive removal of the dissected aortic tissue and use shorter periods of total circulatory arrest. In conjunction with better protection of the heart, in our hands this ultimately results in an operative mortality well below five percent for Type A dissections, such as Richard's," says Raissi. Type A dissection always involves the ascending aorta. It is life-threatening and treated as a surgical emergency.
"Generally, emergency surgery for aortic dissection has an average mortality of 50 percent, but in expert aortic surgery centers this has been lowered to single digits," Raissi adds, noting that another strong indicator of progress is length of hospital stay. "Not infrequently, our elective aortic surgery patients leave the hospital in three days."
An ascending aortic dissection, such as Richard's, can have widespread consequences because blood destined for nearly all areas of the body passes through the ascending aorta before branching into other arteries. When paramedics arrived after Richard's collapse, they noted that he was unable to move his left side. Aortic dissection had diminished the blood flow to the right side of his brain, causing a stroke. The disruption of blood flow also temporarily damaged his kidneys " a common result of dissection along the lower portion of the aorta.
But after 13 days of post-surgery care, Richard transitioned to the inpatient unit of the Acute Rehabilitation Center at Cedars-Sinai, where he remained until March 1. Thanks to months of work through Cedars-Sinai Outpatient Physical and Occupational Therapy Services and a fierce determination from the beginning to function with minimal assistance, he has regained the better part of his life. He even is able to run again, going five miles just a few days before the first anniversary of his surgery. Part of his left hand and his left foot remain numb, and he feels slightly off balance when he isn't walking or running. Even so, he notes, no one looking at him would ever know he suffered a stroke.
Until the moment he collapsed at the warehouse, he had no indication that his aorta was ready to split. Mary and Christine noticed in December that he appeared pale and his eyes were dull instead of their typical bright blue, but they assumed he needed nothing more than rest. From all appearances, he was in good health " his heart sounded good with no murmurs and his lungs were fine. "For somebody to be able to run a half-marathon, you have to be in pretty good health," Mary points out.
But genetic tendencies, aneurysms, and aortic valves are not visible to the naked eye. Bicuspid aortic valve disease is inherited, and Richard now suspects that his father's sudden death was caused by an aortic dissection or rupture. "My dad was 57 when he died. They found him dead. I think he had a bicuspid valve but, of course, back then, everything was called a heart attack," he says.
Today, Richard and Mary are among a group of patients and family members who hope to spare others the pain, fear, uncertainty and loss that aortic dissection inevitably brings about. "I survived this but we just think it's a miracle that I survived," says Richard, adding that while aortic disease is estimated to kill more Americans than AIDS, few people seem to know about it.
The great need for an organized affiliation of patients, families, and medical professionals was the impetus for the creation of the nonprofit Bicuspid Aortic Foundation (www.bicuspidfoundation.com), according to Arlys Velebir, who chairs the Foundation's Board of Directors. "Based on the available medical literature, there may be 300,000 Americans with this disease who will experience aortic dissection, and this does not include the many others who have other forms of connective tissue disorders that lead to dissection. Unfortunately, all too often dissection leads to death," Velebir says. "Richard's survival is miraculous and his desire to help others through the Foundation is a great inspiration."
One of only four hospitals in California whose nurses have been honored with the prestigious Magnet designation, Cedars-Sinai Medical Center is one of the largest nonprofit academic medical centers in the Western United States. For 17 consecutive years, it has been named Los Angeles' most preferred hospital for all health needs in an independent survey of area residents. Cedars-Sinai is internationally renowned for its diagnostic and treatment capabilities and its broad spectrum of programs and services, as well as breakthroughs in biomedical research and superlative medical education. It ranks among the top 10 non-university hospitals in the nation for its research activities and was recently fully accredited by the Association for the Accreditation of Human Research Protection Programs, Inc. (AAHRPP). Additional information is available at http://www.cedars-sinai.edu.
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