(Press-News.org) When prenatal diagnosis detects the severe heart defect hypoplastic left heart syndrome (HLHS) in a fetus, a comprehensive prenatal evaluation is important to provide parents an accurate prognosis. In HLHS, one of the heart's pumping chambers is severely underdeveloped. However, say researchers, in two-thirds of cases, reconstructive surgery affords the infant an excellent chance of early survival.
Researchers from The Children's Hospital of Philadelphia report on five years of experience at that hospital, in a review of 240 fetuses diagnosed with HLHS from 2004 to 2009. Children's Hospital has some of the world's longest and most extensive experience in performing staged surgical repair of HLHS.
"Because we have offered this type of reconstructive heart surgery for over 25 years, our goal was to establish a benchmark for perinatal and early surgical outcome in the present era," said Jack Rychik, M.D., medical director of the Fetal Heart Program at Children's Hospital.
Rychik was the primary investigator of the study, published in the October issue of the journal Ultrasound in Obstretrics and Gynecology.
In HLHS, one of the most common forms of congenital heart disease diagnosed before birth, the underdeveloped left ventricle is unable to properly circulate blood. Over the past 25 years, surgeons at Children's Hospital of Philadelphia and elsewhere have developed and refined reconstructive surgery—currently a series of three planned procedures, beginning in the newborn period and extending to 1 ½ to 4 years of age. Although outcomes vary broadly worldwide, overall survival rates for children with HLHS have steadily improved.
In the current study the researchers classified 162 (68 percent) of the 240 fetuses as standard-risk, and 78 of them (32 percent) as high-risk. In high-risk cases, in addition to the severely underdeveloped left ventricle, the fetus also had genetic and chromosomal defects, prematurity, or other heart abnormalities.
Of the 240 fetuses diagnosed with HLHS, 185 newborns underwent the first stage of surgery, called the Norwood procedure, resulting in 155 survivors and 30 deaths. Within those overall figures, 93 percent of standard-risk cases survived the first operation, compared to 57 percent of high-risk cases.
The Children's Hospital of Philadelphia typically employs a staged surgical approach for fetuses diagnosed with HLHS, says Rychik, but at that center and elsewhere, some parents choose to terminate a pregnancy or to decline medical intervention at birth. The current study, he says, may provide clarity to families and caregivers in categorizing the degree of mortality risk from this condition.
"Surgical outcomes for HLHS are in-part related to patient volume, institutional experience, and the availability of dedicated resources," said Rychik. "However, we found a striking survival advantage for the standard-risk fetuses compared to the higher-risk cases. Two-thirds of fetuses with HLHS do not have a higher-risk form of the condition, and have a stronger chance of survival. After an initial prenatal diagnosis of HLHS, we strongly encourage families to receive a comprehensive evaluation including amniocentesis, so they may obtain a more accurate prognosis. In this way, families can have the best information during prenatal counseling by which to make their plans for the future of their fetus and newborn child."
"Our current research and clinical efforts are focused on improving the quality of life and long-term survival for this group of patients, including dedicated follow-up programs for the evaluation and treatment of cardiac and non-cardiac outcomes," said Gil Wernovsky, M.D., associate chief of cardiology and director of the NeuroCardiac Care Program at Children's Hospital. He added, "Many of these children will face life-long challenges as they mature, and we are committed to understanding and improving these on-going issues as our patients grow."
INFORMATION:
Dr. Rychik receives funding for his research work through the Robert and Delores Harrington Endowed Chair for Pediatric Cardiology at The Children's Hospital of Philadelphia.
Dr. Rychik's co-authors were Anita Szwast, M.D.; Shoba Natarajan, M.D.; Michael Quartermain, M.D.; Denise Donaghue, R.N. M.S.N.; Jill Combs, R.N., M.S.N.; J. William Gaynor, M.D.; Peter Gruber, M.D.; Thomas Spray, M.D.; Michael Bebbington, M.D.; and Mark Johnson, M.D.; all of The Children's Hospital of Philadelphia.
About The Children's Hospital of Philadelphia: The Children's Hospital of Philadelphia was founded in 1855 as the nation's first pediatric hospital. Through its long-standing commitment to providing exceptional patient care, training new generations of pediatric healthcare professionals and pioneering major research initiatives, Children's Hospital has fostered many discoveries that have benefited children worldwide. Its pediatric research program is among the largest in the country, ranking third in National Institutes of Health funding. In addition, its unique family-centered care and public service programs have brought the 460-bed hospital recognition as a leading advocate for children and adolescents. For more information, visit http://www.chop.edu.
END
COLUMBUS, Ohio – New research links three molecules to a critical tumor suppressor gene that is often turned off in multiple myeloma, a presently incurable cancer of the blood.
The findings might offer a new strategy for treating this disease and other blood cancers, according to researchers at The Ohio State University Comprehensive Cancer Center-Arthur G. James Cancer Hospital and Richard J. Solove Research Institute (OSUCCC – James) who led the study.
The silenced molecules are called miR-192, miR-194 and miR-215. All of them are microRNAs, a large class of molecules ...
CHAMPAIGN, Ill. – An obscure provision in the health care reform bill has the potential to seriously alter the long-term care landscape for older Americans, but it may not be as beneficial to retirees as it will be for near-retirees and successive generations of workers, new research by a University of Illinois elder law expert warns.
Richard L. Kaplan, an expert on federal taxes and retirement issues, says the new federal entitlement program, known as Community Living Assistance Services and Supports, or CLASS, shouldn't be viewed as a replacement for long-term care ...
SAN DIEGO -- Prostate cancer patients who receive intensity modulated radiation therapy (IMRT) are less apt to suffer serious gastrointestinal complications following their treatment than those who receive three-dimensional conformal radiotherapy (CRT), according to new research from the University of Pennsylvania School of Medicine. The study, which will be presented Nov. 1 at the 52nd Annual Meeting of the American Society for Radiation Oncology (ASTRO) in San Diego, found that men who were treated with IMRT had fewer serious bowel complications, including painful rectal ...
COLUMBIA, Mo. – Asian-American youth are one of the fastest growing populations in the United States. Although Asian Americans begin smoking later in life, they are more likely to smoke regularly and at a higher rate than other ethnic or racial groups, according to the Department of Health and Human Services. Now, a University of Missouri researcher is examining the unique differences in adolescent tobacco use among Asians and other groups to provide specific recommendations for prevention and treatment.
"Given the large number of addicted teenagers, tobacco control ...
EAST LANSING, Mich. — With big storms ripping across the Midwest, Bob Drost is hoping people are paying attention to the severe weather and tornado warnings.
Unfortunately, Drost, a doctoral student at Michigan State University, knows that many times those warnings are ignored, according to his research. "Only 63 percent understood that a warning is the most urgent National Weather Service statement during severe weather," he said.
Next week, Drost will present his research findings at the Geological Society of America's annual conference to fellow earth scientists ...
CHAPEL HILL – The method used to assess infertility in at-home tests might not be the best for identifying which women will have trouble getting pregnant, according to new research from the University of North Carolina at Chapel Hill School of Medicine.
The study found that the cutoffs used by such infertility tests, which measure levels of a molecule called follicle stimulating hormone or FSH, label many women as infertile who actually go on to have children naturally.
It also suggests that another hormone, called antimullerian hormone or AMH, could prove to be a much ...
Researchers at the University of Colorado School of Medicine and The Children's Hospital have taken a big step toward understanding what causes one of the most serious liver diseases in infants.
The disease is called biliary atresia, It blocks the bile ducts in young infants, through which bile, crucial for digestion, flows to the small intestine. The disease is rare – it strikes in about one in 10,000 births. But it's life-threatening.
"It is fatal if not treated quickly," says Cara Mack, MD, who led the CU research.
Surgical removal of the blocked main bile duct ...
Most babies are delivered head-first, but in about 4% of all deliveries babies are "born breech" ― with their buttocks or feet first. Doctors usually exercise caution and use caesarean sections (C-sections) as the delivery method of choice for such births, believing it safer for the baby. After a large-scale international study in 2000, C-sections became the near-universal choice for such births.
But now researchers at Tel Aviv University are saying that, under certain circumstances, traditional vaginal delivery for breech babies is not only safe for baby, but even ...
Berkeley, CA—A team of scientists at the U.S. Department of Energy's Lawrence Berkeley National Laboratory has detected six isotopes, never seen before, of the superheavy elements 104 through 114. Starting with the creation of a new isotope of the yet-to-be-named element 114, the researchers observed successive emissions of alpha particles that yielded new isotopes of copernicium (element 112), darmstadtium (element 110), hassium (element 108), seaborgium (element 106), and rutherfordium (element 104). Rutherfordium ended the chain when it decayed by spontaneous fission. ...
Type 1 diabetes (T1D), formerly known as juvenile diabetes, is a multifactorial disease of complex etiology characterized by the autoimmune destruction of pancreatic beta cells. In addition to genetic susceptibility, it is generally accepted that environmental factors play important roles in triggering disease, with virus infection having perhaps the strongest association. Multiple viral infections including cytomegalovirus, mumps, rubella, enteroviruses, and parvovirus have all been associated with human T1D. Indeed, the effects of diverse viruses in triggering T1D may ...