scholarly journals A Decision Rule for Determining the Optimal Transplant Listing Window for Patients With a Fontan Physiology

2021 ◽  
Vol 6 (2) ◽  
pp. 238146832110574
Author(s):  
Laura Delaney
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Palliative Treatment ◽  
Early Mortality ◽  
Optimal Time ◽  
Optimal Timing ◽  
Cardiac Transplant ◽  
The Past ◽  
Fontan Physiology

The Fontan is a complex surgical procedure used as a palliative treatment for children with univentricular hearts. In the past, the mortality rate was high and the associated comorbidities as a result of the Fontan circulation were many. However, as research into the condition developed, better understanding has led to a massive reduction in early mortality and a rapidly increasing population of such patients surviving well into adulthood. This has led to a large surge in patients with congenital heart disease being referred for cardiac transplant assessment. According to research, listing these patients at the optimal time is the key to improving transplant outcomes. However, determining that optimal time is unclear and controversial. In this article, I address this issue by developing an optimal timing rule that accounts for the factors faced by specialist cardiologists in determining when transplant ought to be considered for this cohort of patients.

Changing Profile of Congenital Heart Disease

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 790-791
Author(s):  
D. WOODROW BENSON ◽  
James Moller ◽  
Donald C. Fyler ◽  
David E. Fixler
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Surgical Treatment ◽  
Congenital Heart ◽  
Considerable Change ◽  
Diagnostic Methods ◽  
Younger Patients ◽  
The Past ◽  
Steady Improvement ◽  
Complex Lesions

In the past 20 years, considerable change has occurred in the profile of congenital heart disease regarding both diagnosis and treatment. The profile has changed from older to younger patients; the essence of this change has been from pediatric to infant to neonatal cardiology. There has been a shift from palliative to more definitive surgical procedures and a change from invasive (cardiac catheterization) to noninvasive (echocardiography) diagnostic methods. The profile has changed from definitive surgical treatment limited to simple lesions to surgical treatment of the most complex lesions. There has been steady improvement in the recognition of cases of congenital heart disease. Of all children admitted to hospitals for treatment of congenital heart disease, the portion less than three days of age has increased from 24% in 1969 to 1972 to 33% in 1982 to 1986.

An Atlas of Congenital Heart Disease

PEDIATRICS ◽  
1963 ◽  
Vol 32 (6) ◽  
pp. 1077-1077
Author(s):  
NORMAN J. SISSMAN
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Clinical Management ◽  
Congenital Heart ◽  
Pathological Examination ◽  
Children's Hospital ◽  
The Past ◽  
Present Volume ◽  
Children’S Hospital

Despite the vast experience accumulated during the past decade with all aspects of the lives and deaths of patients with congenital heart disease, there is still room for the clarification and direction that careful, informed, imaginative analysis of the results of pathological examination of postmortem material can give to the clinical management of these patients. Dr. Sherman has made a notable contribution in this area with the present volume. The atlas was compiled from 503 specimens collected in the Museum of Congenital Heart Disease at the Children's Hospital in Pittsburgh since 1954.

scholarly journals Association of Insurance Status With Emergent Versus Nonemergent Hospital Encounters Among Adults With Congenital Heart Disease

2021 ◽  
Vol 10 (19) ◽  
Author(s):  
Anushree Agarwal ◽  
Michelle Gurvitz ◽  
Janet Myers ◽  
Sarthak Jain ◽  
Abigail M. Khan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Health Planning ◽  
Private Insurance ◽  
Insurance Status ◽  
The Past ◽  
Use Of Resources ◽  
Hospital Visits ◽  
The Relationship

Background Although the number of hospital visits has exponentially increased for adults with congenital heart disease (CHD) over the past few decades, the relationship between insurance status and hospital encounter type remains unknown. The purpose of this study was to evaluate the association between insurance status and emergent versus nonemergent encounters among adults with CHD ≥18 years old. Methods and Results We used California Office of Statewide Health Planning and Development Database from January 2005 to December 2015 to determine the trends of insurance status and encounters and the association of insurance status on encounter type among adults with CHD. A total 58 359 nonpregnancy encounters were identified in 6077 patients with CHD. From 2005 to 2015, the number of uninsured encounters decreased by 38%, whereas government insured encounters increased by 124% and private by 79%. Overall, there was a significantly higher proportion of emergent than nonemergent encounters associated with uninsured status (13.0% versus 1.8%; P <0.0001), whereas the proportion of nonemergent encounters associated with private insurance was higher than emergent encounters (35.8% versus 62.4%; P <0.0001). When individual patients with CHD became uninsured, they were ≈5 times more likely to experience an emergent encounter ( P <0.0001); upon changing from uninsured to insured, they were significantly less likely to have an emergent encounter ( P <0.001). After multivariate adjustment, uninsured status exhibited the highest odds of an emergent rather than nonemergent encounter compared with all other covariates (adjusted odds ratio, 9.20; 95% CI, 7.83–10.8; P <0.0001). Conclusions Efforts to enhance the ability to obtain and maintain insurance throughout the lifetime of patients with CHD might result in meaningful reductions in emergent encounters and a more efficient use of resources.

scholarly journals An updated SYSCILIA gold standard (SCGSv2) of known ciliary genes, revealing the vast progress that has been made in the cilia research field

2021 ◽  
Author(s):  
Suly Saray Villa Vasquez ◽  
John van Dam ◽  
Gabrielle Wheway
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Gold Standard ◽  
Congenital Heart ◽  
Developmental Disorders ◽  
Research Field ◽  
The Past ◽  
Health And Disease ◽  
Made In ◽  
Standard List

Cilia are microtubule-based organelles with important functions in motility and sensation. They contribute to a broad spectrum of developmental disorders called ciliopathies, and have recently been linked to common conditions such as cancers and congenital heart disease. There has been increasing interest in the biology of cilia and their contribution to disease over the past two decades. As a result, in 2013 we published a ‘Gold Standard’ list of genes confirmed to be associated with cilia. This was published as part of the SYSCILIA consortium systems biology study dissecting the contribution of cilia to human health and disease, and was named the Syscilia Gold Standard (SCGS). Since this publication, interest in cilia and understanding of their functions has continued to grow, and we now present an updated SCGS version 2. This includes an additional 383 genes, more than doubling the size of SCGSv1. We use this dataset to conduct a review of advances in understanding of cilia biology 2013-2021, and perspectives on the future of cilia research. We hope that this continues to be a useful resource for the cilia community.

Long-term outcomes

2018 ◽  
Author(s):  
Barbara Mulder ◽  
Berton Bouma ◽  
Michiel Winter
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Medical Therapy ◽  
Congenital Heart ◽  
Morbidity And Mortality ◽  
Western World ◽  
Corrective Surgery ◽  
Long Term Outcomes ◽  
The Past

Due to tremendous improvements in corrective surgery, and medical therapy, survival of patients with congenital heart disease has improved dramatically over the past decades, with an estimated 95% of such patients in the Western world currently reaching adulthood. Nonetheless, patients with congenital heart disease have decreased long-term outcomes in terms of morbidity and mortality compared to their healthy counterparts.

scholarly journals P766Catheter ablation in congenital heart disease in preparation to cardiac transplant: a single centre experience

EP Europace ◽  
2018 ◽  
Vol 20 (suppl_1) ◽  
pp. i132-i132
Author(s):  
I Cazzoli ◽  
C Gunturiz-Beltran ◽  
S Guarguagli ◽  
R Alonso-Gonzalez ◽  
S V Babu-Narayan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cardiac Transplant ◽  
Single Centre ◽  
Single Centre Experience

8 Improved early mortality fter modified-fontan operation for complex congenital heart disease

1993 ◽  
Vol 72 (5) ◽  
pp. 499 ◽  
Author(s):  
Frank Cetta ◽  
Douglas D. Mair ◽  
Robert H. Feldt ◽  
Francisco J. Puga ◽  
Hartzell V. Schaff ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Fontan Operation ◽  
Early Mortality ◽  
Complex Congenital Heart Disease ◽  
Modified Fontan Operation
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