Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease

PEDIATRICS ◽  
2017 ◽  
Vol 140 (Supplement 3) ◽  
pp. S226.1-S226
Author(s):  
Edith Schussler ◽  
Scott H. Sicherer
2021 ◽  
pp. 1-7
Author(s):  
Joshua M. Fisher ◽  
Sarah Badran ◽  
John T. Li ◽  
Jodie K. Votava-Smith ◽  
Patrick M. Sullivan

Abstract Objective To describe outcomes of acute coronavirus disease 2019 in paediatric and young adult patients with underlying cardiac disease and evaluate the association between cardiac risk factors and hospitalisation. Study design We conducted a retrospective single-institution review of patients with known cardiac disease and positive severe acute respiratory syndrome coronavirus 2 RT-PCR from 1 March, 2020 to 30 November, 2020. Extracardiac comorbidities and cardiac risk factors were compared between those admitted for coronavirus disease 2019 illness and the rest of the cohort using univariate analysis. Results Forty-two patients with a mean age of 7.7 ± 6.7 years were identified. Six were 18 years of age or more with the oldest being 22 years of age. Seventy-six percent were Hispanic. The most common cardiac diagnoses were repaired cyanotic (n = 7, 16.6%) and palliated single ventricle (n = 7, 16.6%) congenital heart disease. Fourteen patients (33.3%) had underlying syndromes or chromosomal anomalies, nine (21%) had chronic pulmonary disease and eight (19%) were immunosuppressed. Nineteen patients (47.6%) reported no symptoms. Sixteen (38.1%) reported only mild symptoms. Six patients (14.3%) were admitted to the hospital for acute coronavirus disease 2019 illness. Noncardiac comorbidities were associated with an increased risk of hospitalisation (p = 0.02), particularly chronic pulmonary disease (p = 0.01) and baseline supplemental oxygen requirement (p = 0.007). None of the single ventricle patients who tested positive required admission. Conclusions Hospitalisations for coronavirus disease 2019 were rare among children and young adults with underlying cardiac disease. Extracardiac comorbidities like pulmonary disease were associated with increased risk of hospitalisation while cardiac risk factors were not.


2011 ◽  
Vol 21 (5) ◽  
pp. 562-571 ◽  
Author(s):  
Hannah C. Glass ◽  
Chelsea Bowman ◽  
Vann Chau ◽  
Alisha Moosa ◽  
Adam L. Hersh ◽  
...  

AbstractMore than 60% of newborns with severe congenital cardiac disease develop perioperative brain injuries. Known risk factors include: pre-operative hypoxemia, cardiopulmonary bypass characteristics, and post-operative hypotension. Infection is an established risk factor for white matter injury in premature newborns. In this study, we examined term infants with congenital cardiac disease requiring surgical repair to determine whether infection is associated with white matter injury. Acquired infection was specified by site – bloodstream, pneumonia, or surgical site infection – according to strict definitions. Infection was present in 23 of 127 infants. Pre- and post-operative imaging was evaluated for acquired injury by a paediatric neuroradiologist. Overall, there was no difference in newly acquired post-operative white matter injury in infants with infection (30%), compared to those without (31%). When stratified by anatomy, infants with transposition of the great arteries, and bloodstream infection had an estimated doubling of risk of white matter injury that was not significant, whereas those with single ventricle anatomy had no apparent added risk. When considering only infants without stroke, the estimated association was higher, and became significant after adjusting for duration of inotrope therapy. In this study, nosocomial infection was not associated with white matter injury. Nonetheless, when controlling for risk factors, there was an association between bloodstream infection and white matter injury in selected sub-populations. Infection prevention may have the potential to mitigate long-term neurologic impairment as a consequence of white matter injury, which underscores the importance of attention to infection control for these patients.


2010 ◽  
Vol 21 (1) ◽  
pp. 39-45 ◽  
Author(s):  
Pierre Wauthy ◽  
Jacques Massaut ◽  
Ahmed Sanoussi ◽  
Hélène Demanet ◽  
Marielle Morissens ◽  
...  

AbstractThe number of adults with congenital cardiac disease continues to increase, and adult patients are now more numerous than paediatric patients. We sought to identify risk factors for perioperative death and report our results with surgical management of adult patients with congenital cardiac disease. We retrospectively analysed in-hospital data for 244 consecutive adult patients who underwent surgical treatment of congenital cardiac disease in our centre between January, 1998 and December, 2007. The mean patient age was 27.2 plus or minus 11.9 years, 29% were in functional class III or IV, and 25% were cyanosed. Of the patients, half were operated on for the first time. A total of 61% of patients underwent curative operations, 36% a reoperation after curative treatment, and 3% a palliative operation. Overall mortality was 4.9%. Predictive factors for hospital death were functional class, cyanosis, non-sinus rhythm, a history of only palliative previous operation(s), and an indication for palliative treatment. Functional class, cyanosis, type of initial congenital cardiac disease (single ventricle and double-outlet right ventricle), and only palliative previous operation were risk factors for prolonged intensive care stay (more than 48 hours). The surgical management of adult patients with congenital cardiac disease has improved during recent decades. These generally young patients, with a complex pathology, today present a low post-operative morbidity and mortality. Patients having undergone palliative surgery and reaching adulthood without curative treatment present with an increased risk of morbidity and mortality. Univentricular hearts and double-outlet right ventricles were associated with the highest morbidity.


2007 ◽  
Vol 17 (4) ◽  
pp. 360-371 ◽  
Author(s):  
Constancio Medrano ◽  
Luis Garcia-Guereta ◽  
Josefina Grueso ◽  
Beatriz Insa ◽  
Fernando Ballesteros ◽  
...  

AbstractObjectivesTo evaluate the rate of hospitalization for acute respiratory tract infection in children less than 24 months with haemodynamically significant congenital cardiac disease, and to describe associated risk factors, preventive measures, aetiology, and clinical course.Materials and methodsWe followed 760 subjects from October 2004 through April 2005 in an epidemiological, multicentric, observational, follow-up, prospective study involving 53 Spanish hospitals.ResultsOf our cohort, 79 patients (10.4%, 95% CI: 8.2%–12.6%) required a total of 105 admissions to hospital related to respiratory infections. The incidence rate was 21.4 new admissions per 1000 patients-months. Significant associated risk factors for hospitalization included, with odds ratios and 95% confidence intervals shown in parentheses: 22q11 deletion (8.2, 2.5–26.3), weight below the 10th centile (5.2, 1.6–17.4), previous respiratory disease (4.5, 2.3–8.6), incomplete immunoprophylaxis against respiratory syncytial virus (2.2, 1.2–3.9), trisomy 21 (2.1, 1.1–4.2), cardiopulmonary bypass (2.0, 1.1–3.4), and siblings aged less than 11 years old (1.7, 1.1–2.9). Bronchiolitis (51.4%), upper respiratory tract infections (25.7%), and pneumonia (20%) were the main diagnoses. An infectious agent was found in 37 cases (35.2%): respiratory syncytial virus in 25,Streptococcus pneumoniaein 5, and Haemophilus influenzae in 4. The odds ratio for hospitalization due to infection by the respiratory syncytial virus increases by 3.05 (95% CI: 2.14 to 4.35) in patients with incomplete prophylaxis. The median length of hospitalization was 7 days. In 18 patients (17.1%), the clinical course of respiratory infection was complicated and 2 died.ConclusionsHospital admissions for respiratory infection in young children with haemodynamically significant congenital cardiac disease are mainly associated with non-cardiac conditions, which may be genetic, malnutrition, or respiratory, and to cardiopulmonary bypass. Respiratory syncytial virus was the most commonly identified infectious agent. Incomplete immunoprophylaxis against the virus increased the risk of hospitalization.


2011 ◽  
Vol 140 ◽  
pp. 84-90
Author(s):  
Yu Qing Jiao ◽  
Geng Xu Zhou ◽  
Jian Ping Huang ◽  
Xiao Yang Hong ◽  
Xue Yong Yang ◽  
...  

Objective To assess risk factors of acute kidney injury in congenital cardiac disease following cardiac surgery with cardiopulmonary bypass in children. Methods A 50% postoperative creatinine increase was regarded as the criterion of acute kidney injury. 124 children aged 3 years or little undergoing cardiac surgery were divided into three groups: (1) negative AKI (-); (2) AKI (+) with an increase in postoperative creatinine from 150% to 200%; and (3) AKI (++) with an increase in postoperative creatinine by more than 200%. Demographics, and preoperative, intraoperative, and postoperative variables were evaluated for associations with AKI using univariate and multiple logistic regression analysis. Results Several variables, including mortality, preoperative albumin and creatinine levels, cardiopulmonary bypass duration, and postoperative creatinine levels, were significant differences among the three groups (P < 0.05). Multiple regression analysis demonstrated that three risk factors, age (OR, 0.962; 95% CI, 0.925 to 1.000; P = 0.046), intraoperative red blood cell transfusion (OR, 1.003; 95% CI, 1.001 to 1.005; P = 0.030), and cardiopulmonary bypass duration (OR, 1.024; 95% CI, 1.016 to 1.032; P = 0.000) were independently associated with AKI after cardiac surgery. Conclusions This study showed that younger age, incremental intraoperative red blood cell transfusion, and prolonged cardiopulmonary bypass duration were independently associated with acute kidney injury after cardiac surgery. The type of congenital cardiac disease should be included in the analysis of acute kidney injury.


2005 ◽  
Vol 15 (2) ◽  
pp. 229-231 ◽  
Author(s):  
Rebecca Sparkes ◽  
Judy Chernos ◽  
Franciscus Dicke

The DiGeorge, or velocardiofacial, syndrome has been aetiologically linked to heterozygous deletion of the q11.2 region of chromosome 22. It is the most common of the microdeletion syndromes, and is associated with malformations involving the ventricular outflow tracts. Duplication of the 22q11.2 region has also been reported, adding to a growing list of syndromes involving genomic deletion or duplication that cause disease by decreasing or increasing the gene dosage. We report two cases of congenital cardiac disease associated with microduplications of 22q11.2, and discuss the evidence to date for the potential clinical significance of this genetic defect.


2006 ◽  
Vol 16 (S3) ◽  
pp. 35-42 ◽  
Author(s):  
Matthew J. Gillespie ◽  
Bradley S. Marino ◽  
Meryl S. Cohen ◽  
Arianne E. Korteweg ◽  
Marjolijn Henket ◽  
...  

In contrast to older patients, children and young adults rarely have isolated disease of the systemic atrioventricular valve. Stenosis and/or regurgitation of the systemic atrioventricular valve, however, frequently coexist with complex congenital cardiac disease. In addition, most patients undergoing surgery on the systemic atrioventricular valve have had previous intracardiac repairs.


Author(s):  
Naresh Dhawan ◽  
Rohin Kumar ◽  
Reema Kumar Bhatt

Cardiac disease in pregnancy is a leading cause of maternal death in more so high-income countries. The armamentarium for winning this difficult battle involves shared decision-making with communication across the clinical team and the patient. There is limited clinical evidence concerning effective approaches to managing such complex care and moreover involvement of different specialists makes coordinated care challenging. Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1-2% of the population whereas a single ventricle is a rare congenital heart disease that accounts for less than 1% of all congenital heart diseases. We had two cases of pregnancy with bicuspid aortic valve in one case and the other with single ventricle. The involvement of multidisciplinary team involving cardiologist, cardiothoracic anaesthetist and fetal maternal medicine specialist resulted in good maternal and fetal outcome in both the cases.


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