scholarly journals Longitudinal Assessment of Outcome From Prenatal Diagnosis Through Fontan Operation for Over 500 Fetuses With Single Ventricle‐Type Congenital Heart Disease: The Philadelphia Fetus‐to‐Fontan Cohort Study

2018 ◽  
Vol 7 (19) ◽  
Author(s):  
Michael Y. Liu ◽  
Benjamin Zielonka ◽  
Brian S. Snarr ◽  
Xuemei Zhang ◽  
J. William Gaynor ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Cohort Study ◽  
Heart Disease ◽  
Prenatal Diagnosis ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Longitudinal Assessment

scholarly journals Transplantation for Congenital Heart Disease: Focus on the Impact of Functionally Univentricular Versus Biventricular Circulation

2021 ◽  
Vol 12 (3) ◽  
pp. 352-359
Author(s):  
Kyle W. Riggs ◽  
John T. Broderick ◽  
Nina Price ◽  
Clifford Chin ◽  
Farhan Zafar ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Blood Type ◽  
Abo Blood Type ◽  
The Impact ◽  
Fontan Patients

Background: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases. Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched. Patients undergoing transplantation at a PHIS hospital from 2006 to 2017 were categorized as single ventricle or biventricular strategy based on their diagnoses and procedures in 90% of patients. When known, single ventricle patients were further analyzed by their palliative stage post-Glenn or post-Fontan (known in 31%). Results: A total of 1,517 CHD transplantations were identified, 67% with single ventricle strategy (1,016). Single ventricle, biventricular, and indeterminate patients had similar survival (log-rank P > .1). Risk factors for mortality in patients with CHD were extracorporeal membrane oxygenation (ECMO) support at transplant (hazard: 2.27), ABO blood type incompatibility (hazard: 1.61), African American recipient (hazard 1.42), and liver dysfunction (hazard 1.29). A total of 130 confirmed Fontan and 185 confirmed bidirectional Glenn patients underwent transplantation, each with survival equivalent to biventricular patients (log-rank P > .500). For Fontan patients, renal dysfunction (hazard: 5.40) and transplant <1 year after Fontan (hazard 2.82) were found to be associated with mortality. Conclusions: Single ventricle patients, as a group, experience similar outcomes as biventricular patients with CHD undergoing transplantation, and this extends to Fontan patients. Risk factors for mortality correlate with end-organ dysfunction as well as race and ABO blood type incompatibility in the CHD population.

scholarly journals Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction

2021 ◽  
Vol 8 (10) ◽  
pp. 117
Author(s):  
Liliana Chemello ◽  
Massimo Padalino ◽  
Chiara Zanon ◽  
Luisa Benvegnu’ ◽  
Roberta Biffanti ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Platelet Count ◽  
Heart Disease ◽  
Liver Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Transient Elastography ◽  
Fontan Operation ◽  
Liver Stiffness ◽  
Morbidity And Mortality

Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 ± 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan®). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (p < 0.05), larger hepatic and cava veins diameter (p < 0.05), worsened NYHA class (p < 0.05), abnormal lymphocytes (p < 0.01), platelet count (p < 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (p < 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (p < 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (p < 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD.

scholarly journals Down syndrome and congenital heart disease: perioperative planning and management

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Defects ◽  
Careful Consideration ◽  
Children With Down Syndrome ◽  
Perioperative Planning

AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

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