HEART BEATAugust 20, 2012, 7:46 p.m. ET
.Facing Lifesaving Heart Surgery, Twice
Surgeries to Save Babies Pose Problems Years Later; Adults In the Children's Ward.Article Video Comments (18) more in Health & Wellness
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By RON WINSLOW
Just a few decades ago, infants born with heart defects weren't expected to live past their teens. Improved surgery techniques have changed that, resulting in a new condition called Adult Congenital Heart Disease, and the need for cardiologists who can treat it. WSJ's Christina Tsuei reports.
.The first generation of patients to have historic, lifesaving heart surgery as newborns or children is now in their 20s and 30s and presenting doctors with a puzzle: What some thought were cures for serious heart defects are breaking down.
Symptoms can begin years or decades after the childhood operations to treat congenital defects, hitting unsuspecting patients with chest pain, shortness of breath or debilitating fatigue. Often patients need additional heart surgery to survive.
Surgery at Ages 11 and 27
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Tiffani Harrington with a staff member at Texas Children's Hospital in 1991.
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Ms. Harrington, with her son, born Friday, and daughter. She had a calcified valve replaced before her first pregnancy.
..Pediatric cardiologists and surgeons, though, aren't trained to manage adult health issues, nor are children's hospitals accustomed to treating them. Adult doctors are often stymied in diagnosing symptoms of patients whose hearts have been reconfigured by pediatric surgeons.
"These patients don't have a natural home" in the health-care system, says Charles D. Fraser Jr., surgeon-in-chief at Texas Children's Hospitals in Houston.
About 1.3 million Americans ages 21 and older—and perhaps as many as 3 million according to some estimates—are living with congenital heart defects, with 35,000 to 40,000 joining their ranks each year.
Patients are often unaware of potential problems or skip checkups that would allow doctors to track the condition of their repairs. By some estimates more than 50% of aren't getting regular heart care.
"This is a tsunami hitting the health-care system," says Amy Verstappen, president and CEO of the Adult Congenital Heart Association, a patient advocacy group.
Top centers treating adults with congenital heart disease vary in their approaches. Texas Children's, Boston Children's Hospital, and Cincinnati Children's Hospital treat adults at their institutions, while pediatric cardiac surgeon Jonathan Chen treats many of his adult patients at New York Presbyterian/Weill Cornell Medical Center, an adult center. The Hospital of the University of Pennsylvania and Children's Hospital of Philadelphia have a hybrid program intended to help patients ease the transition between adult and pediatric medicine.
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Charles Thornton before his second heart surgery at age 15.
.To Fix Broken Hearts
Patients Who Had Childhood Surgeries Discover Those Operations May Need an Update. Some Rough Estimates:
90% of children born each year with heart defects survive until adulthood.
40% of children with heart defects are at significant risk of needing operations later in their lives.
50% of adults living with congenital heart defects are not getting regular care, according to some estimates.
WSJ reporting
.Charles Thorton, of Lafayette, La., underwent surgery in 2004, at age 38, at Texas Children's Hospital, where his family had to get permission to bring in meals—otherwise he got apple sauce and baby food. The surgery was a major revision of earlier operations he underwent at ages 2 and 15 to treat a birth defect called tricuspid atresia.
Being treated in children's hospital brought back tough memories of his experiences as a child, his wife Ann, says. Mr. Thornton recalls the emotional response of parents he met whose children were there to fix their hearts. That he was there as an adult represented "hope for their kids," he says. "I felt I was giving something to them."
Hard data are scarce, but experts say more than 90% of the estimated 40,000 children born each year with heart defects now survive until adulthood, the reverse of the situation just a few decades ago.
Defects vary widely in severity and complexity. Roughly half of people born with them don't require surgery, says Dr. Chen, chief of pediatric cardiac surgery at New York Presbyterian/Weill Cornell. But about 80% of the rest are at significant risk of needing operations later in their lives, suggesting a need for frequent regular checkups to monitor their disease.
Surgery at Ages 5 and 37
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Susanna Jacobs as a child, her scar visible from an open-heart surgery to repair a congenital condition.
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Today, Ms. Jacobs, right, underwent surgery in July after feeling short of breath with a rapid heart beat. She didn't realize that as an adult she might need a follow-up operation
..Two Days in ER Limbo
Susanna Jacobs was a so-called "blue baby," a newborn with a blue tint to her skin, lips and fingernails due to insufficient oxygen in the blood. Ms. Jacobs of Philadelphia, now 37, was born with the condition tetralogy of Fallot—four defects that conspire to inhibit the flow of blood from the heart to the lungs.
In 1980 at age 5, she had an open-heart operation to repair the defect. She had regular checkups as a child and teenager but no heart problems. Doctors "never told me I would need any follow-up surgery," says Ms. Jacobs, who as an adult had a couple of heart checkups. Ms. Jacobs "exemplified the whole idea of being lost to care," says Yuli Kim, medical director of the Philadelphia Adult Congenital Heart Program, a joint-venture between Children's Hospital of Philadelphia and the Hospital at the University of Pennsylvania.
Last April, 32 years after her surgery, Ms. Jacobs was at her job as a patient services representative at a pediatric dental office when her heart suddenly started beating rapidly and she felt short of breath. When symptoms persisted a second day, she went to a local emergency room. Tests detected an irregular heartbeat, an enlarged heart and a leaky pulmonary valve. For two days, she says, she was neither admitted nor discharged. "They just didn't know what to do for me," she says.
She was transferred to the Hospital of the University of Pennsylvania, where her heartbeat was stabilized and she went home. After further evaluation by Dr. Kim and other experts, she underwent open-heart surgery in July to have her pulmonary valve replaced. The need for a second surgery came as a shock, says Ms. Jacobs. Now, she says she realizes that "I'm never going to be finished with issues of my heart."
Strong Enough for Pregnancy?
Tiffani Harrington, 32, was born with transposition of the great arteries: Vessels connected to her left ventricle, intended to deliver blood to the body, and her right ventricle, which pumps blood to the lungs, were reversed. She has been a patient at Texas Children's since she was five months old and had an open-heart "arterial switch" to repair the problem in 1991 when she was 11.
A checkup in 2007 turned up evidence that her pulmonary artery was calcified. She also mentioned that she hoped to have a family to Wayne Franklin, director of the adult congenital heart program at Texas Children's Hospital in Houston
About 40% of Dr. Franklin's practice are young women who are pregnant or who want to conceive. Pregnancy requires more work of the heart: Blood volume increases by 25%. And labor, with accompanying pain, fast heartbeat and hormonal changes, "that's the most dangerous and challenging time," Dr. Franklin says.
Some conditions associated with congenital heart defects, such as pulmonary hypertension, come with a mortality rate for the mother or infant as high as 50%, Dr. Franklin says. He counsels such women against having children.
He estimates about half his patients become pregnant—preferably after they take steps to reduce their risk. Ms. Harrington, of Katy, Texas, had her calcified valve replaced in late 2007. About two years later, she gave birth to a daughter, now 2½ years old.
Last year Ms. Harrington and her husband decided they'd like another baby. "We asked him if my heart was OK to handle another pregnancy," she says. "He gave us the greenlight."
She had her second baby Friday. A few days before the birth, she had a fetal echocardiogram, which indicated her new baby's heart was free of problems. The chance that a mother with a congenital heart defect will give birth to a child with one is about 8 in 100, or nearly 10 times that of women with normal hearts. (For men with a heart defect, the rate is about 4 in 100 births.)
"Thankfully both babies have perfect hearts," she says.
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JoAnn McGuinness with Jonathan Chen, who performed her 2009 heart surgery.
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JoAnn McGuinness with Denton Cooley, who performed her childhood heart surgery in 1972.
.Too Tired to Push a Grocery Cart
JoAnn McGuinness, now 48 years old, was a patient of Denton Cooley, one of the icons of open-heart surgery, who operated on her when she was 8 to repair defects associated with tetralogy of Fallot.
Her family flew from New Jersey to Texas in 1972, the year she finally reached 40 pounds, a weight thought necessary at the time to be strong enough to survive an open-heart operation.
Once the procedure was over, "I was perfect," she says. That is what her mother told her. After graduating from college, she began seeing an adult family doctor, but her mother made her promise to never see another cardiologist. "She told me all they want to do is cut you open."
But by age 32, soon after her mother died, she began having serious heart palpitations. She saw two cardiologists. Then the problem subsided. In early 2009, during a routine checkup with a new cardiologist, she learned more about tetralogy of Fallot—including the fact the hardly anyone born with the condition survived to her age. That led to more tests which revealed she had a leaky valve that caused her heart to work so hard she was nearly in heart failure, she says.
By that fall she would get so exhausted she couldn't push a grocery cart. She got an appointment with Dr. Chen at Weill/Cornell, who told her he knew exactly what to do. In November 2009, he replaced her valve at Weill/Cornell.
She's doing well with her new valve and says she realizes in retrospect that she lived through symptoms for years that she just chalked up to getting older.
Now, she wonders what lies ahead, as few people her age have gone down this road before. "It's hard to have people say they don't know what's going to happen," she says.
Write to Ron Winslow at ron.winslow@wsj.com
Corrections & Amplifications
Amy Verstappen is president and CEO of the Adult Congenital Heart Association. An earlier version of this article gave the organization's name incorrectly as the Adult Congenital Heart Disease Association, and also spelled tetralogy of Fallot, a congenital heart defect, incorrectly.
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