Objective: The effect of Hybrid stage 1 palliation for hypoplastic left heart syndrome on right ventricular function is unknown. We sought to compare right ventricular function in normal neonates and those with hypoplastic left heart syndrome before Hybrid palliation and to assess the effect of Hybrid palliation on right ventricular function, using the right ventricular myocardial performance index and the ratio of systolic and diastolic durations.
Methods: We carried out a retrospective review of echocardiographic data on 23 infants with hypoplastic left heart syndrome who underwent Hybrid palliation and 35 normal controls. Data were acquired before Hybrid and after Hybrid palliation - post 1, 0-4 days; post 2, 1 week; post 3, 2-3 weeks; post 4, 1-1.5 months following Hybrid palliation.
Results: Myocardial performance index and ratio of systolic and diastolic durations were higher in the pre-Hybrid hypoplastic left heart syndrome group (n=23) - 0.47±0.16 versus 0.25±0.07, p<0.001; 1.59±0.44 versus 1.09±0.14, p<0.0001 - compared with controls (n=35). There was no significant change in the myocardial performance index at any of the post-Hybrid time points. Ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid - post 3: 2.08±0.62 and post 4: 2.21±0.45 versus pre: 1.59±0.44, p=0.043 and 0.003. There were no significant differences in parameters between sub-groups of infants who died (n=10) and survivors (n=13).
Conclusions: Right ventricular myocardial performance index and ratio of systolic and diastolic durations were significantly higher in infants with hypoplastic left heart syndrome before intervention compared with controls. The ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid palliation. Our data suggest that infants with hypoplastic left heart syndrome have right ventricular dysfunction before intervention, which worsens over 2 weeks after Hybrid palliation.
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http://dx.doi.org/10.1017/S1047951115001419 | DOI Listing |
J Med Ultrasound
February 2024
Department of Obstetrics and Gynecology, Faculty of Medicine, Karadeniz Technical University, Ortahisar, Trabzon, Turkey.
Background: The effect of congenital cardiac malformation on fetal cerebral circulation has not been well known. This study aimed to compare the cerebral blood circulation of fetuses with congenital heart disease (CHD) with healthy fetuses.
Methods: This prospective cohort study included 37 pregnant women who presented to the gynecology and obstetrics department of department of Farabi Hospital, Faculty of Medicine, Karadeniz Technical University for anomaly screening in the second trimester.
Ann Thorac Surg Short Rep
September 2024
Biostatistics Unit, Department of Data Science, National Center for Child Health and Development, Tokyo, Japan.
Background: The primary treatment for hypoplastic left heart syndrome (HLHS) is the Fontan pathway, which entails performing the Glenn procedure. We hypothesized that the superior vena cava in patients with HLHS was short. As the length of the superior vena cava influences the Glenn procedure, we compared its length between patients with HLHS and those with other congenital heart diseases.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Section of Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital, New York, New York.
Among repairs for ventriculoarterial discordance, ventricular septal defect, and pulmonary stenosis, aortic root translocation (Nikaidoh operation) offers the most anatomic result. With a diminutive pulmonary annulus or hypoplastic left ventricular outflow tract, the distance gained posteriorly with aortic translocation is negligible. We developed the "hemi-Nikaidoh" procedure as an alternative.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan.
Undetected coronary anomalies at Norwood procedure are associated with poor prognosis due to inadequate myocardial protection. We report a case of anomalous origin of the right coronary artery from the main pulmonary artery trunk with hypoplastic left heart syndrome and aortic atresia. Although, during bilateral pulmonary artery banding as initial palliation, the proximity between the right coronary artery origin and the aortic root made a visual diagnosis difficult, it was diagnosed using computed tomography before the Norwood procedure.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
June 2024
Department of Cardiothoracic Surgery and Perfusion Services, The Heart Center, Nationwide Children's Hospital, Columbus, Ohio.
Background: Right ventricular (RV) failure after heart transplantation (HT) is common in those with pretransplantation elevated pulmonary vascular resistance (PVR). Mechanical circulatory support has been used as a bridge to recovery, with mixed outcomes. We describe a patient with failed single-ventricle palliation in whom severe RV failure developed after HT.
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