When ambulance workers in Yakima responded to a call to adjust a females internal cardiac defibrillator, they expected to see a 70-year-old woman. Instead, they found 7-year-old Hali Hentze.


The device was implanted when Hali underwent surgery at Sacred Heart Medical Center, in Spokane, last January to correct a rare congenital heart defect. She is one of the youngest patients in the region to have had a cardiac defibrillator installed, but a growing number of children here are receiving such devices, which are similar to pacemakers but send larger electrical shocks to the heart.


Niesha Gordon, Halis mother, says she first started noticing something was wrong a little over a year ago when her daughter fainted when she got scared or excited. Doctors in Yakima initially thought the episodes could be seizures, she says. When Dr. Chris Anderson, an electrophysiologist at Northwest Pediatric Cardiology at Sacred Heart Childrens Hospital, visited Yakima and looked at her reports, he suggested Hali come to Spokane for further tests.


After several exams at Sacred Heart, Anderson diagnosed Hali with Long QT Syndrome, a rare, genetic, and sometimes fatal disruption of the hearts electrical system that causes irregular heartbeat. Gordon says she, too, has been diagnosed with the disorder.


A lot of people dont know they have it until its too late, Gordon says. She was surprised that her young daughter needed the defibrillator.


Hali educates friends about her condition by carrying around a teddy bear with a replica mechanical defibrillator in its pocket. Since she underwent the $100,000 procedure, in which the defibrillator was placed surgically underneath the skin in a small slit in her side, she hasnt experienced any further problems, Gordon says.


Beth Dullanty, cardiac care coordinator for Northwest Pediatric Cardiology, says that when the first surgeries to correct congenital heart defects were performed more than 50 years ago, most children with heart ailments didnt live to adulthood. As research continued and technology advanced, though, survival rates and patient care have improved.


It still amazes me what we are able to do now, Dullanty says. Surgical repairs have gotten better within the past 15 years and electrophysiology in the past five years. Electrophysiology is the study of the electrical conduction system of the heart.


One early-detection technique that has been used in pediatric medicine for several years and now is coming of age at the Childrens Hospital is the fetal echocardiogram, Dullanty says. If doctors notice irregularities during their cardiac evaluation of ultrasound images of a fetus, further tests can be done using fetal echocardiograms to diagnose possible congenital heart problems.


When Kate Holland, of Spokane, was 16 weeks into her pregnancy, doctors told her that her baby had an abnormal heart rhythm. Last September, 10 days after her son, William, was born, the cardiac team at Sacred Heart Childrens Hospital performed open-heart surgery to patch a hole in Williams heart and insert an artificial pacemaker, Holland says.


William has recovered so well that you cant even tell there was anything wrong, Holland says. Since his operation, the only complications the 10-month-old has had with his pacemaker involve setting off metal detectors and needing to stay six inches away from cell phones, she says.


Causes remain unclear


Although detection has improved, health-care professionals dont know 97 percent of the time what causes congenital heart problems, because the heart forms 28 days after conception, before women even know that theyre pregnant, Dullanty says. A few maternal diseases, such as Lupus and diabetes, have been linked to fetal heart problems, she says.


While research still needs to be done on prevention, technological advancements in non-surgical outpatient procedures that have been performed for 15 years are expanding at the Childrens Hospital, Dullanty says.


Interventional cardiology has totally blossomed, she says.


Interventional cardiology involves inserting catheters into the cardiovascular system to repair heart problems without major surgery, she says. The number of open-heart surgeries at the hospital has decreased by around 30 percent from the number done at Sacred Heart Medical Center in 2000, when doctors began performing more interventional procedures instead to fix certain heart problems, she says. The cost of such procedures can range from $15,000 to $30,000, far less than the typical cost of an open-heart surgery.


Three-year-old Emma Watson, of Lewiston, Idaho, has undergone two open-heart surgeries, one-closed heart surgery, and more than 10 interventional heart catheterizations to repair her heart, says her mother, Patty Watson.


Emmas heart has only one functioning ventricle, which means the right side of her heart pumps all the blood through her body. Seven of Emmas procedures and operations have been done at the Childrens Hospital, Dullanty says.


One doctor here that performs such interventional procedures, pediatric cardiologist Dr. Carl Garabedian, says that higher detection rates and lower mortality rates can make it seem as though more children are developing heart problems than in the past. In reality, the percentage of the population with congenital heart disease remains the same, at about 1 percent, Garabedian says.


Were hearing about these cases more now because defibrillators are becoming more common among youth, and we have more success stories than we did in the past, Garabedian says. In the late 1980s, kids with congenital heart problems still were given little hope, but now early detection and correction of such problems greatly improve their chances for a longer, healthier life, he says.


Pre-natal diagnosis of heart disease is the best way to treat the problem, Garabedian says. Pregnant women with fetuses that have been found to have potential heart problems can be referred to a pediatric cardiologist to determine what needs to be done.


The sickest kids we see are newborns that are born with heart problems and are left untreated for a time, Garabedian says. Far too many kids have had ultrasounds that missed the boat, and then they come in here crashing.


Garabedian says some symptoms that suggest heart disease in children include poor weight gain, fast breathing, and cyanosis, a condition characterized by a bluish tint to the skin, when babies are born. Parents should see a childs pediatrician first if such symptoms occur.


In the case of older children and teenagers, signs include family members with a history of heart problems or sudden, unexplained deaths, and passing out during times of exertion, such as on a basketball court, Garabedian says.


Given the sudden and unexpected deaths often caused by congenital heart problems, all school gyms here should be equipped with automated external defibrillators, he says. In Milwaukee, Wis., where Garabedian attended medical school, the local business community contributed money to set up the $3,000 devices in the schools, and that has saved at least one persons life, he says.


Northwest Pediatric Cardiology, with five cardiologists who specialize in congenital heart disease, three cardiac surgeons, and a support staff, is the only pediatric cardiac center for surgery between Seattle and Minnesota, Garabedian says.


Last year alone, it worked with 1,800 new patients, Dullanty says. Northwest Pediatric Cardiology plans to change its name to the Northwest Center for Congenital Heart Disease on July 1, she says.