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 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 Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Christine H. Attenhofer Jost ◽  
Dörthe Schmidt ◽  
Michael Huebler ◽  
Christian Balmer ◽  
Georg Noll ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Lung Transplantation ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Eisenmenger’S Syndrome ◽  
Number Of Patients ◽  
Increased Risk

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

scholarly journals Novel JAG1 Deletion Variant in Patient with Atypical Alagille Syndrome

2019 ◽  
Vol 20 (24) ◽  
pp. 6247 ◽  
Author(s):  
Emanuele Micaglio ◽  
Andreea Alina Andronache ◽  
Paola Carrera ◽  
Michelle M. Monasky ◽  
Emanuela T. Locati ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Neural Tube ◽  
Neural Tube Defect ◽  
Congenital Heart ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Alagille Syndrome ◽  
Collateral Arteries ◽  
Increased Risk

Alagille syndrome (AGS) is an autosomal-dominant disorder characterized by various degrees of abnormalities in the liver, heart, eyes, vertebrae, kidneys, face, vasculature, skeleton, and pancreas. This case report describes a newborn child exhibiting a congenital neural tube defect and peculiar craniofacial appearance characterized by a prominent forehead, deep-set eyes, bulbous nasal tip, and subtle upper lip. Just a few hours after birth, congenital heart disease was suspected for cyanosis and confirmed by heart evaluation. In particular, echocardiography indicated pulmonary atresia with ventricular septal defect with severe hypoplasia of the pulmonary branches (1.5 mm), large patent ductus arteriosus and several major aortopulmonary collateral arteries. Due to the association of peculiar craniofacial appearance and congenital heart disease, a form of Alagille syndrome was suspected. In addition, on the fifth day after birth, the patient developed jaundice, had acholic stools, and high levels of conjugated bilirubin and gamma-glutamyltransferase (GGT) were detected in the blood. Genetic testing revealed the novel variant c.802del in a single copy of the JAG1 gene. No variants in the NOTCH2 gene were detected. To the best of our knowledge, this is the first clinical description of a congenital neural tube defect in a molecularly confirmed Alagille patient. This work demonstrates a novel pathogenic heterozygous JAG1 mutation is associated with an atypical form of Alagille syndrome, suggesting an increased risk for neural tube defects compared to other Alagille patients.

scholarly journals Impact of COVID-19 in adult patients with congenital heart disease

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
I Cardoso ◽  
T B Branco ◽  
A Constante ◽  
J Martins ◽  
L Sousa ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Adult Patients ◽  
Transposition Of Great Arteries ◽  
Tertiary Centre ◽  
Increased Risk ◽  
Atrioventricular Canal Defect ◽  
The Impact

Abstract Background COVID-19 is responsible for a worldwide pandemic, causing more than 13 000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation. Aims To evaluate the impact of COVID-19 in adult patients with congenital heart disease in our tertiary centre Methods Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to February 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records. Results We identified 36 patients (pts) with COVID-19 infection. Symptoms were present in 31 (86%). The median age was 39 (32–49) years, 58% were females. Seven P (19%) had complex cyanotic disease; three (8%) Tetralogy of Fallot; three (8%) transposition of great arteries (one after Senning procedure and 2 after arterial switch); six (14%) right ventricle obstacle; two (8%) atrioventricular canal defect; four (11%) atrial septal defect; five (14%) ventricular septal defect; five (14%) aortic coarctation; two aortopathies (one submitted do David procedure); one subaortic stenosis; two (6%) had Eisenmenger syndrome. The majority (61%) of P had previous surgery and 58% were at New York Heart Association class of I. Mild symptoms were reported by 24 P (67%). Seven adults experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, although none required mechanical ventilation. One death was reported. There was a significant association between the gravity of CHD and hospitalizations (p=0.012). Conclusion Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome; the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status. FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals Impact of hepatopathy in pediatric patients after surgery for complex congenital heart disease

PLoS ONE ◽  
2021 ◽  
Vol 16 (3) ◽  
pp. e0248776
Author(s):  
Torben Kehl ◽  
Daniel Biermann ◽  
Andrea Briem-Richter ◽  
Gerhard Schoen ◽  
Jakob Olfe ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Organ Dysfunction ◽  
Congenital Heart ◽  
Heart Rhythm ◽  
Complex Congenital Heart Disease ◽  
Control Group ◽  
Heart Rhythm Disorders ◽  
The Impact

Patients undergoing complex pediatric cardiac surgery in early infancy are at risk of postoperative secondary end-organ dysfunction. The aim of this study was to determine specific risk factors promoting the development of peri- and postoperative hepatopathy after surgery for congenital heart disease. In this retrospective study, we identified 20 consecutive patients operated between 2011 and 2019 from our institutional cohort who developed significant postsurgical hepatic dysfunction. These patients were compared to a control group of 30 patients with comparable initial cardiac conditions and STS-EACTS risk score. Patients who developed hepatopathy in the intensive care unit have chronic cholestasis and decreased liver synthesis. The impact of postoperative hepatopathy on morbidity was marked. In six patients (30%), liver transplantation was executed as ultima ratio, and two (10%) were listed for liver transplantation. The overall mortality related to postoperative hepatopathy is high: We found nine patients (45%) having severe hepatopathy and mostly multiple organ dysfunction who died in the postoperative course. According to risk analysis, postoperative right and left heart dysfunction in combination with a postoperative anatomical residuum needing a re-operation or re-intervention in the postoperative period is associated with a high risk for the development of cardiac hepatopathy. Furthermore, postoperative complications (pleural effusion, heart rhythm disorders, etc.), postoperative infections, and the need for parenteral nutrition also raise the risk for cardiac hepatopathy. Further investigations are needed to reduce hepatic complications and improve the general prognosis of such complex patients.

Abstract 4553: Pre-Operative Brain Injury in Newborn Infants with Transposition of the Great Arteries Occurs at Similar Rates to other Complex Congenital Heart Disease, and is not Related to Balloon Atrial Septostomy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Julia Gunn ◽  
Lara Shekerdemian ◽  
Rod Hunt ◽  
Lee Coleman ◽  
Ayton Hope ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Brain Injury ◽  
Ischaemic Stroke ◽  
Congenital Heart ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Balloon Atrial Septostomy ◽  
Increased Risk ◽  
Atrial Septostomy

Introduction: Brain injury (BI) in the form of white matter injury (WMI) or ischaemic stroke, seen on magnetic resonance imaging (MRI), is common in neonates with congenital heart disease (CHD). It has previously been suggested that balloon atrial septostomy (BAS) may be an independent risk factor for pre-operative BI in neonates with transposition of the great arteries (TGA). Hypothesis : We hypothesised that: 1. The incidence of BI is similar in neonates with TGA and other severe CHD 2. The risk of pre-operative BI is not increased by BAS Methods: Neonates with TGA, hypoplastic left heart syndrome (HLHS) or pulmonary atresia (PA) were enrolled in a prospective study of BI related to CHD. Pre-operative brain MRI was performed in all patients. In those with TGA who underwent BAS, MRI occurred after septostomy. T1, T2 and diffusion-weighted images were obtained in axial and coronal planes. Scans were reviewed by blinded assessors, and were classified according to WMI (punctate lesions) or ischaemic stroke. Fisher’s Exact Test was used to compare rates of WMI and stroke in each patient group. Results: Sixty-six neonates (mean weight 3.4±0.5kg) were studied, of whom 44 had TGA, 13 had HLHS and 9 had PA. The median age at MRI was 7 days (range 1–31). Thirty-five of the neonates with TGA underwent BAS a median of 1 day (1–21) followed by MRI 7 days (2–12) later. BI was present in 17 (26%) patients. The table shows the incidence of BI in the sub-groups. Two neonates with a stroke also had WMI. Table showing MRI abnormalities in patient sub-groups. Neonates with TGA were not at increased risk of pre-operative BI compared with those with HLHS or PA (p=1.0). BAS did not increase the risk of pre-operative BI in neonates with TGA (p=1.0). Conclusions: Pre-operative BI occurs at a similar rate in neonates with TGA and other severe CHD. The risk of pre-operative BI in neonates with TGA is not increased by BAS.

The impact of medical interventions on admission characteristics in children with congenital heart disease and cardiomyopathy

2020 ◽  
pp. 1-8
Author(s):  
Rohit S. Loomba ◽  
Jacqueline Rausa ◽  
Vincent Dorsey ◽  
Ronald A. Bronicki ◽  
Enrique G. Villarreal ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Length Of Stay ◽  
Congenital Heart ◽  
Unit Length ◽  
Channel Blockers ◽  
Regression Analyses ◽  
Converting Enzyme Inhibitors ◽  
Medical Interventions ◽  
The Impact

Abstract Introduction: Children with congenital heart disease and cardiomyopathy are a unique patient population. Different therapies continue to be introduced with large practice variability and questionable outcomes. The purpose of this study is to determine the impact of various medications on intensive care unit length of stay, total length of stay, billed charges, and mortality for admissions with congenital heart disease and cardiomyopathy. Materials and methods: We identified admissions of paediatric patients with cardiomyopathy using the Pediatric Health Information System database. The admissions were then separated into two groups: those with and without inpatient mortality. Univariate analyses were conducted between the groups and the significant variables were entered as independent variables into the regression analyses. Results: A total of 10,376 admissions were included these analyses. Of these, 904 (8.7%) experienced mortality. Comparing patients who experienced mortality with those who did not, there was increased rate of acute kidney injury with an odds ratio (OR) of 5.0 [95% confidence interval (CI) 4.3 to 5.8, p < 0.01], cardiac arrest with an OR 7.5 (95% CI 6.3 to 9.0, p < 0.01), and heart transplant with an OR 0.3 (95% CI 0.2 to 0.4, p < 0.01). The medical interventions with benefit for all endpoints after multivariate regression analyses in this cohort are methylprednisolone, captopril, enalapril, furosemide, and amlodipine. Conclusions: Diuretics, steroids, angiotensin-converting enzyme inhibitors, calcium channel blockers, and beta blockers all appear to offer beneficial effects in paediatric cardiomyopathy admission outcomes. Specific agents within each group have varying effects.

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