The fixed incidence of congenital heart defects and improved survival have resulted in increasing numbers of adults with congenital heart disease (CHD) who have undergone complex repairs and/or palliations. Eventually, there will be more adults with CHD than children. They will require cardiac surgical interventions associated with progression of their CHD or for age-related disease, such as coronary revascularization. During bypass, anatomical shunts may exist within or without the heart. Left-to-right shunts can result in dramatically lower systemic blood flow than pump flow due to 'steal', while pulmonary edema ensues due to excessive pulmonary flow. Right-to-left shunts carry risks of massive air embolism and double or triple venous cannulation may be necessary. Cannulation of composite reconstructed aortas may be difficult, risking dissection or aortic obstruction, and double arterial cannulation may be indicated. Aberrant coronary arterial and venous anatomy may .preclude adequate myocardial preservation with common techniques and can be complicated by aortic insufficiency. Valves and conduits may exhibit failure. Conventional monitoring, such as central venous oximetry, may be misleading. Monitoring, such as serial lactate measurement, near-infrared spectroscopy and transcranial Doppler blood velocity, offer advantages for such patients. The perfusionist needs to be aware of such conditions as much congenital aberrancy may present unexpectedly during cardiac surgery.
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http://dx.doi.org/10.1191/0267659106pf839oa | DOI Listing |
Pediatr Surg Int
December 2024
Department of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Introduction: This study investigates risk factors and surgical outcomes in pediatric patients with congenital heart defects (CHD) who develop ischemic colitis (IC). Previous research indicates a higher IC risk in very low birth weight neonates with CHD.
Methods: A retrospective analysis compared an IC-CHD group to a CHD-only group.
Pediatr Cardiol
December 2024
Division of Pediatric Cardiology, Benioff Children's Hospital, Department of Pediatrics, University of California, San Francisco (UCSF), San Francisco, CA, USA.
Unplanned admissions are associated with worse clinical outcomes and increased hospital resource utilization. We hypothesized that children with congenital heart disease (CHD) from lower-income neighborhoods have higher rates of unplanned hospital admissions and greater resource utilization. Utilizing the Kids' Inpatient Database (2016 and 2019), we included children under 21 years of age with CHD, excluding newborn hospitalizations.
View Article and Find Full Text PDFEur J Pediatr
December 2024
Department of Medical Genetics, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.
Unlabelled: The RASopathies are a group of disorders resulting from a germline variant in the genes encoding the Ras/mitogen-activated protein kinase pathway. These disorders include Noonan syndrome (NS), cardiofaciocutaneous syndrome (CFC), Costello syndrome (CS), Legius syndrome (LS), and neurofibromatosis type 1 (NF1), and have overlapping clinical features due to RAS/MAPK dysfunction. In this study, we aimed to describe the clinical and molecular features of patients exhibiting phenotypic manifestations consistent with RASopathies.
View Article and Find Full Text PDFAm J Transplant
December 2024
Department of Cardiovascular Surgery, Arkansas Children's Hospital, Little Rock, Arkansas. Electronic address:
Heart valve replacement in pediatric cardiothoracic surgery poses problems because conventional heart valve implants do not have the ability to grow. This mandates serial reoperations for implant exchanges until an adult size implant can fit. Partial heart transplantation eliminates these reoperations because the transplanted valves grow.
View Article and Find Full Text PDFInt J Cardiovasc Imaging
December 2024
Department of Cardiology, Fukuoka Children's Hospital, 5-1-1, Kashiiteriha, Higashi-Ku, Fukuoka City, Fukuoka, 813-0017, Japan.
Fontan-associated liver disease (FALD) may be caused by chronic liver congestion due to high central venous pressure (CVP). Recently, the usefulness of liver native T1 mapping in magnetic resonance imaging (MRI) in adulthood has been reported. To evaluate the usefulness of native liver T1 mapping in children with congenital heart disease (CHD), we investigated the utility of native liver T1 relaxation time (LT1) in pediatric Fontan patients in comparison to other CHDs.
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