To save content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about saving content to .
To save content items to your Kindle, first ensure email@example.com
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant’s small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81.
Loeys–Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.
We report a case of two-month old with a functionally univentricular heart and parallel circulation who presented to the emergency department with Covid-19 and subsequently developed acute respiratory distress syndrome. The course of illness, clinical values, and laboratory markers are characterized in this report.
A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures.
Children with CHD who undergo cardiopulmonary bypass are at an increased risk of acute kidney injury. This study evaluated the association of end-organ specific injury plasma biomarkers for brain: glial fibrillary acidic protein and heart: Galectin 3, soluble suppression of tumorgenicity 2, and N-terminal pro b-type natriuretic peptide with acute kidney injury in children undergoing cardiopulmonary bypass.
Materials and Methods:
We enrolled consecutive children undergoing cardiac surgery with cardiopulmonary bypass. Blood samples were collected pre-bypass in the operating room and in the immediate post-operative period. Acute kidney injury was defined as a rise of serum creatinine ≥50% from pre-operative baseline within 7 days after surgery.
Overall, 162 children (mean age 4.05 years, sd 5.28 years) were enrolled. Post-operative acute kidney injury developed in 55 (34%) children. Post-operative plasma glial fibrillary acidic protein levels were significantly higher in patients with acute kidney injury (median 0.154 (inter-quartile range 0.059–0.31) ng/ml) compared to those without acute kidney injury (median 0.056 (inter-quartile range 0.001–0.125) ng/ml) (p = 0.043). After adjustment for age, weight, and The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category, each natural log increase in post-operative glial fibrillary acidic protein was significantly associated with a higher risk for subsequent acute kidney injury (adjusted odds ratio glial fibrillary acidic protein 1.25; 95% confidence interval 1.01–1.59). Pre/post-operative levels of galectin 3, soluble suppression of tumorgenicity 2, and N-terminal pro b-type natriuretic peptide did not significantly differ between patients with and without acute kidney injury.
Higher plasma glial fibrillary acidic protein levels measured in the immediate post-operative period were independently associated with subsequent acute kidney injury in children after cardiopulmonary bypass. Elevated glial fibrillary acidic protein likely reflects intraoperative brain injury which may occur in the context of acute kidney injury-associated end-organ dysfunction.
To evaluate the association between novel pre- and post-operative biomarker levels and 30-day unplanned readmission or mortality after paediatric congenital heart surgery.
Children aged 18 years or younger undergoing congenital heart surgery (n = 162) at Johns Hopkins Hospital from 2010 to 2014 were enrolled in the prospective cohort. Collected novel pre- and post-operative biomarkers include soluble suppression of tumorgenicity 2, galectin-3, N-terminal prohormone of brain natriuretic peptide, and glial fibrillary acidic protein. A model based on clinical variables from the Society of Thoracic Surgery database was developed and evaluated against two augmented models.
Unplanned readmission or mortality within 30 days of cardiac surgery occurred among 21 (13%) children. The clinical model augmented with pre-operative biomarkers demonstrated a statistically significant improvement over the clinical model alone with a receiver-operating characteristics curve of 0.754 (95% confidence interval: 0.65–0.86) compared to 0.617 (95% confidence interval: 0.47–0.76; p-value: 0.012). The clinical model augmented with pre- and post-operative biomarkers demonstrated a significant improvement over the clinical model alone, with a receiver-operating characteristics curve of 0.802 (95% confidence interval: 0.72–0.89; p-value: 0.003).
Novel biomarkers add significant predictive value when assessing the likelihood of unplanned readmission or mortality after paediatric congenital heart surgery. Further exploration of the utility of these novel biomarkers during the pre- or post-operative period to identify early risk of mortality or readmission will aid in determining the clinical utility and application of these biomarkers into routine risk assessment.
Aneurysms of the right atrium are rare in the paediatric population. We report a case of a foetal diagnosis of right atrial aneurysm with associated atrial tachycardia in foetal and postnatal life. Unique to our case are the findings of isolated pericardial effusion without hydrops fetalis and the development of aortic coarctation in postnatal life.
Background: Although some prior studies have provided evidence to question the historical belief that pulmonary vascular resistance index ⩾6 Wood Units×m2 should be a contraindication to heart transplantation in children, no national analyses specific to the modern area have addressed this question. Methods: Data were analysed for paediatric heart transplant recipients from 1 January, 2002 to 1 September, 2012 (n=699). The relationship between pulmonary vascular resistance and all-cause 30-day mortality was evaluated using univariate and multivariate analyses. Results: The 30-day mortality included 10 patients (1.43%), which is lower than in the previous analyses. Receiver operating curve analysis of pulmonary vascular resistance index as a predictor of mortality yielded a cut-off value of 3.37 Wood Units×m2, but the area under the curve and specificity of this threshold was weaker than in previous analyses. Whereas pulmonary vascular resistance index treated as a dichotomised variable was a significant predictor of mortality in univariate (odds ratio 4.92, 95% confidence interval 1.04–23.33, p=0.045) and multivariate (odds ratio 5.26, 95% confidence interval 1.07–25.80, p=0.041) analyses, pulmonary vascular resistance index treated as a continuous variable was not a significant predictor of mortality in univariate (p=0.12) or multivariate (p=0.11) analyses. Conclusions: The relationship between pulmonary vascular resistance and post-heart transplant mortality in children is less convincing in this analysis of a comprehensive, contemporary database than in previous series. This suggests the possibility that modern improvements in the management of post-transplant right ventricular dysfunction have mitigated the contribution of pulmonary hypertension to early mortality.
Cardiac surgery was revolutionized on November 29, 1944, when Eileen Saxon underwent the first systemic-to-pulmonary artery shunt at Johns Hopkins University in Baltimore, Maryland, United States of America. The systemic-to-pulmonary artery shunt was initially developed in the laboratory and then applied to patients through the unique collaboration of Vivien Thomas, Alfred Blalock, and Helen B. Taussig. This innovation was the first operation to successfully treat cyanotic cardiac disease. The history of the first operation to successfully treat cyanotic heart disease is an extraordinary history of courage, innovation, and scientific breakthrough. Just as striking is perhaps the ability of the protagonists of this story to overcome seemingly insurmountable barriers of racial and gender discrimination and revolutionize medicine.
Objective: To determine whether blood levels of the brain-specific biomarker glial fibrillary acidic protein rise during cardiopulmonary bypass for repair of congenital heart disease. Methods: This is a prospective observational pilot study to characterise the blood levels of glial fibrillary acidic protein during bypass. Children <21 years of age undergoing bypass for congenital heart disease at Johns Hopkins Hospital and Texas Children's Hospital were enrolled. Blood samples were collected during four phases: pre-bypass, cooling, re-warming, and post-bypass. Results: A total of 85 patients were enrolled between October, 2010 and May, 2011. The median age was 0.73 years (range 0.01–17). The median weight was 7.14 kilograms (range 2.2–86.5). Single ventricle anatomy was present in 18 patients (22%). Median glial fibrillary acidic protein values by phase were: pre-bypass: 0 ng/ml (range 0–0.35); cooling: 0.039 (0–0.68); re-warming: 0.165 (0–2.29); and post-bypass: 0.112 (0–0.97). There were significant elevations from pre-bypass to all subsequent stages, with the greatest increase during re-warming (p = 0.0001). Maximal levels were significantly related to younger age (p = 0.03), bypass time (p = 0.03), cross-clamp time (p = 0.047), and temperature nadir (0.04). Peak levels did not vary significantly in those with single ventricle anatomy versus two ventricle repairs. Conclusion: There are significant increases in glial fibrillary acidic protein levels in children undergoing cardiopulmonary bypass for repair of congenital heart disease. The highest values were seen during the re-warming phase. Elevations are significantly associated with younger age, bypass and cross-clamp times, and temperature nadir. Owing to the fact that glial fibrillary acidic protein is the most brain-specific biomarker identified to date, it may act as a rapid diagnostic marker of brain injury during cardiac surgery.
Repair of complex malformations that necessitate restoration of continuity between the right ventricle and the pulmonary arteries can now safely be performed with low morbidity and mortality. Major concerns still remain on the long-term outlook for these patients, and about the durability of the different prostheses used to restore that continuity, whether during initial correction or at the time of reintervention for failure of the conduit or pulmonary regurgitation. In this review, we discuss the salient morphologic features of the right ventricular outflow tract, and then focus on the indications for early and late intervention, current therapeutic options, and outcomes.
The use of a conduit of polytetrafluoroethylene placed between the right ventricle and the pulmonary arteries as source of pulmonary arterial supply during the first stage of palliation for the hypoplastic left heart syndrome has facilitated post-operative management and resulted in decreased mortality. We describe here the use of a cryopreserved saphenous vein inserted in reversed direction to create the connection between the right ventricle and the pulmonary arteries in a neonate with low birth weight undergoing the modified Norwood procedure.
Email your librarian or administrator to recommend adding this to your organisation's collection.