"We create a controlled heart attack deliberately in the abnormally thickened heart muscle so it will not get in the way."
Dr. Paul Ho
Kaiser chief of surgery
CRAIG T. KOJIMA / CKOJIMA@STARBULLETIN.COM
Cardiologists Cyril Leung, Stephen Chan and Paul Ho work as a team at Kaiser Permanente Hawaii.
Kaiser participates in cardiac study
A clinical trial for one of the treatments involves malformed heart chambers
Kaiser Permanente Hawaii surgeons are performing two new procedures to correct congenital heart defects that affect younger people.
Michael Johnson, 49, of Princeville, Kauai, and Kaylynn Kamae-Kaopuiki, 34, of Waimanalo were the first to benefit.
Johnson has a congenital disorder called hypertrophic cardiomyopathy, which causes excessive thickening of the heart muscle.
"There are well-documented relationships between having hypertrophic obstructive cardiomyopathy and sudden death in young people," said Dr. Paul Ho, Kaiser chief of surgery.
Instead of traditional open-heart surgery to shave the excessive tissue, Johnson's thickened heart muscle was thinned out by injecting a controlled amount of absolute alcohol.
The procedure, called percutaneous alcohol septal ablation, was performed May 13 by Dr. Cyril Leung, director of inpatient surgery.
Kamae-Kaopuiki's genetic disorder involved incomplete formation of the wall between two of the heart's four chambers, the right and left atriums.
The defect results in a pattern of abnormal blood flow that can lead to a stroke with no clear cause, the surgeons said.
Kaiser Permanente Hawaii is the only local hospital participating in a national clinical trial that involves implanting a device in the heart to close the "hole" in the septum.
Half of the patients selected for the national trial receive the device; the other half receive medical treatment.
Kamae-Kaopuiki was chosen at random for the implant, which was inserted Sept. 17, 2004. Ho and Dr. Stuart Pang, chief of neurology, are collaborating on the clinical trial.
Dr. Saibal Kar, a specialist in the procedures at Cedars Sinai Heart Center, assisted the Kaiser surgeons with the first cases.
Thickening of the heart muscle -- Johnson's condition -- is related to severe chest pain and shortness of breath, especially with exertion, and arrhythmia, "even to the point where there is risk for sudden cardiac death," Ho said.
The thickened muscle protrudes into the aortic valve and gets in the way of blood flow from the heart, he said. "It obstructs blood to such a point it causes a high-velocity jet of blood leaving the heart," which sucks part of the flimsy mitral valve into the path to increase the obstruction, he said.
Great exertion by someone with this condition can cause fainting or even death, Ho said.
With the new procedure, he said, "We create a controlled heart attack deliberately in the abnormally thickened heart muscle so it will not get in the way.
"The definition of heart attack," he pointed out, "is death of heart muscle."
Leung said incidence of hypertrophic cardiomyopathy is believed roughly to be one in 500 or 1,000 and can occur at any age.
Other heart conditions can be similar, but an echocardiogram usually will show a thickened muscle, he said.
"The main therapy in the past has always been, and still is, medication to alleviate the symptoms, and people sometimes are very responsive to therapy," Leung said.
However, many patients, even with the best medical therapy, will develop very debilitating symptoms that do not respond to medicine, he said. In these cases, open heart surgery has been the traditional treatment for 20 to 30 years, he said.
The percutaneous alcohol septal ablation procedure, first performed in Europe in the mid-1990s, is less invasive and has a success rate of more than 95 percent, he said.
A heart catheter is inserted to identify the thickened part of the heart muscle, and a controlled amount of alcohol is injected into the branch artery that goes directly to the area.
"We've got to know what we're doing," Leung said. "We can't give too much (alcohol) because then we're causing too much of a heart attack. We cannot not give enough so we're not doing anything for the patient."
The alcohol technique is not for everyone, Leung said, but it is less invasive and if it fails, open heart surgery or a permanent pacemaker can be considered, he said.
Discussing the clinical trial, Ho said about half of stroke victims under age 55 have been identified with the heart defect where the tissue is not fused between the left and right atrium.
"As it turns out, one in four of us has a normally formed structure, but it is not fused, not shut," Ho said.
Certain conditions, such as coughing, straining or bearing down, can create greater pressure on one side of the heart than the other, causing the flap to pop open, he said.
Blood clots in the leg, which are fairly common, could then go to the brain and cause a stroke, he said.
About 25 percent of people have un-fused septa, and half of those 18 to 60 who have unexplained strokes have the defect, Ho said.
However, he said, a cause and effect relationship has not been proved -- just observed as an association. The clinical trial is to determine whether mechanical closure of the two chambers or a blood thinner is the most effective treatment.
Seattle doctors who started the national trial have uncovered an unexpected benefit, Ho said.
A lot of patients said they had lifelong migraine headaches but they disappeared after the device (STARflex septal occlusion system) was implanted to close the separated septa, Ho said.
As a result, he said the Food and Drug Administration has just approved a clinical trial to study effects of the device to close the "hole" in the interatrial septum versus medical therapy on patients with migraines. Kaiser is applying to be a site.
Ho said the new procedures are creating greater understanding of some neurological disorders. "What were thought to be separate entities, migraine headaches, strokes or TIA, all may belong to the same spectrum or same disease.
"Perhaps tiny showering of blood clots or bubbles into the brain may cause migraines, and perhaps larger ones will cause TIA (transient ischemic attack) and bigger clots will cause stroke."
COURTESY OF MICHAEL JOHNSON
Heart doctors give man new lease on life
A lifelong athlete, Michael Johnson, 49, could not understand why he began to feel fatigued and had less stamina in his early 30s.
"I was thinking I'm getting old, I guess; that's part of the price."
Johnson, who owns a drape and blind company on Kauai, said he ran cross-country as a youth and has always been involved in water sports. But his stamina and ability for athletics gradually changed over 15 to 20 years with no specific problem to see the doctor about, he said.
His "journey of discovery" about his medical condition began with some bad ahi at a restaurant about five or six years ago, he said. He ended up in the hospital emergency room with an allergic reaction.
The ER doctor who listened to his heart said, "Wow, you need to have that looked at right away," he said.
He went to a cardiologist after that, but his arresting echocardiogram did not show a problem, he said. He got approval from his primary care doctors to go to Kaiser Permanente in Honolulu for another opinion, and they discovered right away that he had a bulge, a thickening of the heart muscle, in one area.
They began treating it with medication, and then he developed atrial fibrillation, a secondary problem, that also required medications, he said.
The doctors monitored that for about 2 1/2 years, and his condition was not getting any better, he said. "There would be days I'd walk six stairs, and I'd have to stop and stand against the wall to keep from passing out from lightheadedness and my muscles would hurt from lack of oxygen."
His doctors told him about a year ago that something had to be done and outlined the options, he said, adding, "These guys at Kaiser are really stellar to work with."
He educated himself about the procedures and "went through an emotional roller coaster" deciding between open heart surgery or a new procedure using an injection of alcohol to thin the thickened heart muscle.
After a lot of agonizing and consulting with the doctors, he decided on open heart surgery. Then it turned out he was not a good candidate for it, he said.
He left the hospital 2 1/2 days after alcohol was injected May 13 to correct his genetic heart defect and went to Bali, where his brother lives, three weeks later.
The first day, they surfed three hours and climbed up and down a cliff, he said. "I told my brother I haven't felt this good in 20 years."
He has since been to Fiji and said he has taken up kite boarding and golfing. "Now, I have a whole new outlook."
Trial device closes hole in heart chamber
Kaylynn Kamae-Kaopuiki of Waimanalo began feeling numb from her neck down to her toes in January last year.
"I wasn't sure what was going on with me," said Kamae-Kaopuiki, 34, division manager for the La-Z-Boy Furniture Galleries in Aiea.
She said her doctor did not know what was wrong with her, and she spent six months going back and forth to Kaiser for all kinds of tests.
An echocardiogram uncovered an opening in the wall of her heart.
She said the neurologist explained that a high percentage of people are born with such an opening because chambers of the heart do not close at birth and they do not know it.
"They can actually live with it, and that's OK, but they can have a stroke," she said.
When told she could be treated either with medication or have a device inserted to correct the defect in a randomized clinical trial at Kaiser Permanente, she said, "I was hoping to get the device," which she did.
Before the procedure was done Sept. 17, 2004, she was getting a lot of headaches, and the numbness never went away, she said.
"Sometimes it was more severe. It was always on the left side, and the headaches started getting worse, almost to migraine.
"It was kind of irritating," she said, but she continued to work.
The first day she became numb, her staff was urging her to go to the doctor, but she did not until the next day, she said.
She was put on a blood thinner to avoid a possible blood clot going through the opening of her heart and causing a fatal stroke.
She is still taking a blood thinner but said her headaches "pretty much went away ... and the tingling went away" after the procedure.
She was allowed to opt out of the two-year study if she chose to, "but since I was the first one to get it done, I wanted to see it through so they could get whatever data they can," she said.
It took about six months for tissues to grow around the implanted device, but "the closure is still intact and everything's good," she said.
Besides managing a retail furniture store, Kamae-Kaopuiki has three children, 15, 11 and 9, who "keep me busy." Her husband, Al, is a pipe fitter in construction.