October 27, 2010 — 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. A history of repair of HLHSResearchers 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 one 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, MD, 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 Obstetrics 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. New studyIn 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“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. 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, said Gil Wernovsky, MD, associate chief of cardiology and director of the Cardiac Kids Developmental Follow-up Program at Children’s Hospital. 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, MD; Shoba Natarajan, MD; Michael Quartermain, MD; Denise Donaghue, MSN, RN; Jill Combs, MSN, RN; J. William Gaynor, MD; Peter Gruber, MD; Thomas Spray, MD; Michael Bebbington, MD; and Mark Johnson, MD; all of The Children’s Hospital of Philadelphia. ContactJoey McCool Ryan, Department of Public Relations, 267-426-6070
Background—There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. Methods and Results—Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival (P<0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality (P=0.004 and P=0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions—Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon. |
What is the life expectancy of someone with hypoplastic left heart syndrome?
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