Neonates with congenital heart disease
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Updated: Oct 3, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 17, 2014
1Harvard Medical School and Cardiac Intensive Care Unit, Children's Hospital, Boston, Massachusetts 02115, USA. peter.laussen@tch.harvard.edu
Insights
Early reparative surgery for congenital heart disease in newborns has improved outcomes. However, single-ventricle defects, like hypoplastic left heart syndrome, still present challenges in neonatal intensive care units.
Area of Science:
- Pediatric Cardiology
- Neonatal Surgery
- Critical Care Medicine
Background:
- Early reparative surgery for congenital heart disease (CHD) in neonates and infants is now standard practice.
- Advancements in cardiopulmonary bypass, surgical techniques, and perioperative management have reduced mortality and morbidity.
- While successful for two-ventricle repairs, early repair strategies face challenges in newborns with single-ventricle defects, particularly hypoplastic left heart syndrome (HLHS).
Purpose of the Study:
- To review recent literature (past 12 months) on the management of newborns with single-ventricle defects.
- To identify predictors of early and late outcomes and guide patient management strategies.
- To summarize emerging research on attenuating the inflammatory response to bypass and managing postoperative complications.
Main Methods:
- Literature review of articles published within the last 12 months.
- Synthesis of findings related to classification, prenatal diagnosis, treatment options, and outcome predictors for single-ventricle defects.
- Summary of research on inflammatory response, necrotizing enterocolitis, lactate as a perfusion index, markers of injury, and mechanical circulatory support.
Main Results:
- Significant progress has been made in early reparative surgery for CHD, extending to premature and low-birth-weight infants.
- Newborns with single-ventricle defects, especially HLHS, experience higher mortality compared to two-ventricle repairs.
- Research is focusing on identifying outcome predictors and strategies to mitigate adverse responses, including inflammation, necrotizing enterocolitis, and organ injury.
Conclusions:
- While early repair has transformed CHD care, single-ventricle defects remain a critical challenge in neonatal cardiac surgery.
- Continued research into predictive markers and management strategies is essential for improving outcomes in high-risk neonates.
- Addressing the heightened inflammatory response and specific complications like necrotizing enterocolitis is crucial for advancing neonatal cardiac critical care.
Abstract:
Early reparative surgery in neonates and infants with congenital heart disease, as opposed to initial palliation and later repair, is now commonplace. Changes to the conduct of cardiopulmonary bypass, timing of surgery and surgical techniques, and perioperative management substantially have reduced the postoperative mortality and morbidity for these patients. The success of this strategy of early reparative surgery now has been extended to the premature and low-birth-weight newborn, and, along with this, new challenges to postoperative care in the intensive care unit. However, the low mortality associated with two-ventricle repairs has not been the experience in newborns undergoing palliation for single-ventricle defects, in particular, hypoplastic left heart syndrome. A number of articles regarding management of newborns with single-ventricle defects have been published during the past 12 months, ranging from classification, prenatal diagnosis, treatment options, and predictors of both early and late outcome, which may provide a guide for patient management. As mortality has declined, there has been an increased emphasis on identifying indices that may predict outcome or morbidity both before and after surgery, along with possible strategies to attenuate adverse clinical responses. The inflammatory response to bypass is heightened in neonates and infants, and several reports have addressed possible techniques for attenuating the response. In addition, reports regarding the risk for necrotizing enterocolitis, the utility of lactate as an index of systemic perfusion, potential markers of myocardial and neurologic injury, and the use of mechanical support of the circulation in newborns with congenital heart disease are summarized.
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