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.

Abstract 15067: Incidence and Outcomes of Neonates With Congenital Heart Disease Complicated by Necrotizing Enterocolitis

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Pirouz Shamszad ◽  
Shaine A Morris ◽  
Deipanjan Nandi ◽  
Andrew T Costarino ◽  
Bradley S Marino ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Necrotizing Enterocolitis ◽  
Congenital Heart ◽  
Health Information System ◽  
Hospital Charge ◽  
Chromosomal Anomalies ◽  
Modifiable Factors ◽  
The Impact

Introduction: The management of neonates with congenital heart disease (CHD) may be complicated by necrotizing enterocolitis (NEC), however, there is limited multicenter data describing the incidence and outcomes of NEC in the CHD population. Objective: We aimed to assess the incidence and risk factors for the development of NEC in neonates with major CHD and the impact on survival. Methods: A retrospective cohort study of neonates with CHD was performed for all index hospitalizations of neonates (<28 days) with major CHD between 2004 and 2014 using the Pediatric Health Information System database. The diagnosis of NEC was determined by the presence of ICD-9 code 777.5x. The incidence of NEC was determined as were risk factors for the development of NEC. Mortality was the primary outcome measure. Results: Of 38770 neonates with major CHD, 1448 (3.6%) were diagnosed with NEC. The rate of NEC varied between 0-8% by hospital and was not associated with hospital volume (p=0.4). Among neonates with a single, major CHD diagnosis, the rate of NEC was 6% in hypoplastic left heart syndrome (HLHS), 6% in truncus arteriosus (TA) , 4% in tetralogy of Fallot (TOF), 3% in aortic arch obstruction (AO), and 2% in transposition of the great arteries (TGA); these diagnoses accounted for 47% of all NEC. Prematurity and chromosomal anomalies were independently associated with the diagnosis of NEC (p≤0.01 for both). Unadjusted mortality among neonates with NEC was 24% compared to 12% in neonates without NEC (OR 2.4, 95%CI 2.1-2.7). When evaluating changes in adjusted mortality associated with NEC by CHD diagnosis, TOF mortality increased from 8% to 16% (p<.01), TGA increased from 5% to 21% (p<0.01), AO increased from 6% to 20% (p<0.01), HLHS increased from 22% to 28% (p=.07), and TA decreased from 13% to 12% (p=0.7). Median LOS was higher in neonates with NEC than without NEC (54d [IQR 31-93] vs. 18d [IQR 9-34], p<0.01) as was median hospital charge ($600k [IQR 310k-1.1m] vs. $220k [IQR 100k-430k], p<0.01). Conclusions: The incidence of NEC among neonates with major CHD is highest in HLHS and TA. NEC is associated with significantly higher hospital mortality, LOS, and charges. Determining modifiable factors associated with NEC may allow for interventions to reduce morbidity in this population.

Abstract 18813: Outcomes of Patients with Congenital Heart Disease following Heart Transplantation: the Impact of Disease Type, Prior Thoracic Surgeries and End-Organ Dysfunction

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Matthew J Lewis ◽  
Jonathan Ginns ◽  
P. C Schulze ◽  
Matt Lippel ◽  
Paul Chai ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Organ Dysfunction ◽  
Congenital Heart ◽  
Fontan Operation ◽  
Pulmonary Atresia ◽  
Patient Specific ◽  
Increased Risk ◽  
The Impact

Introduction: Adults with congenital heart disease (ACHD) are at increased risk for early adverse outcomes following heart transplantation (Htx). Despite the need for improved risk stratification, small cohorts have constrained identification of patient-specific factors associated with poor prognosis. We hypothesized that type of CHD, number of sternotomies and prior end-organ dysfunction would be associated with an increased risk for mortality post-HTx. Methods: We performed a retrospective, observational cohort study of all patients with ACHD who underwent HTx at our institution from 1/1997 to 1/2014. The primary endpoint was death. Exposures of interest included type of CHD, number of sternotomies and hepatic dysfunction secondary to passive congestion, measured as high Models for End-Stage Liver Disease Excluding INR (MELD-XI) score. Results: 48 ACHD patients were followed (mean age at HTx: 36±1.7 years). Diagnoses included: Tetralogy of Fallot (TOF)/pulmonary atresia/double outlet right ventricle in 15 (31%), D-transposition of the great arteries in 10 (21 %), tricuspid atresia/double inlet left ventricle in 9 (19%), VSD/ASD in 4 (8%), heterotaxy in 3 (6%), congenitally corrected transposition of the great arteries in 2 (4%), and 5 (10%) with other diagnoses. Pre-transplant cardiac surgeries included: Fontan operation in 12 (25%), Mustard/Senning repair in 9 (19%), TOF repair in 5 (11%), Rastelli repair in 5 (11%), VSD/ASD closures in 3 (6%), other another congenital heart surgery in 8 (17%) and no prior cardiac surgery in 5 (11%). Over a median follow-up time of 3.9 years, 14 patients died (29%) and 10 (71%) deaths occurred within 30 days of HTx. Survival at 1 and 5 years was 77%. Deaths within 30 days of surgery were secondary to hemorrhage in 4 (40%), graft failure in 3 (30%) and multi-system organ failure in 3 (30%). By multivariate analysis, ≥3 sternotomies (HR 8.5, p=0.02), MELD-XI score >18 (HR 6.2, p=0.009) and heterotaxy (HR 9.8, p=0.01), were significant predictors of mortality. Conclusion: In our cohort of patients with CHD undergoing Htx, ≥3 sternotomies, MELD-XI score >18 and heterotaxy syndrome were significantly associated with death. These findings may be important in patient selection and timing of Htx in this 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.

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