Failures of the Fontan System in Univentricular Hearts and Mortality Risk in Heart Transplantation: A Systematic Review and Meta-Analysis.
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ABSTRACT:
SUBMITTER: Marquez-Gonzalez H
PROVIDER: S-EPMC8709145 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:BackgroundIn patients with left heart failure, micro-RNAs (miRNAs) have been shown to be of diagnostic and prognostic value. The present study aims to identify those miRNAs in patients with univentricular heart (UVH) disease that may be associated with overt heart failure.MethodsA large panel of human miRNA arrays were used to determine miRNA expression profiles in the blood of 48 UVH patients and 32 healthy controls. For further selection, the most abundantly expressed miRNA arrays were related to clinical measures of heart failure and selected miRNAs validated by polymerase chain reaction were used for the prediction of overt heart failure and all-cause mortality.ResultsAccording to microarray analysis, 50 miRNAs were found to be significantly abundant in UVH patients of which miR-150-5p was best related to heart failure parameters. According to ROC analysis, NT-proBNP levels (AUC 0.940, 95% CI 0.873-1.000; p = 0.001), miR-150-5p (AUC 0.905, 95% CI 0.779-1.000; p = 0.001) and a higher NYHA class ? III (AUC 0.893, 95% CI 0.713-1.000; p = 0.002) were the 3 most significant predictors of overt heart failure. Using a combined biomarker model, AUC increased to 0.980 indicating an additive value of miR-150-5p. Moreover, in the multivariate analysis, a higher NYHA class ? III (p = 0.005) and miR-150-5p (p = 0.006) turned out to be independent predictors of overt heart failure.ConclusionIn patients with UVH, miR-150-5p is an independent predictor of overt heart failure and thus may be used in the risk assessment of these patients.
| S-EPMC6786722 | biostudies-literature
Project description:ObjectiveAfter univentricular Fontan conversion, systemic venous pressure serves as the sole driving force for transpulmonary blood flow. Consequently, systemic venous return is markedly altered and ventricular filling is subnormal. The mechanisms and time course of systemic adaptation to Fontan conversion are incompletely understood. We hypothesized that acute elevation in systemic venous pressure induces an adaptive response similar to conversion to a univentricular Fontan circulation.MethodsAdjustable vessel occluders were placed around the superior and inferior vena cavae in juvenile sheep. After 1-week recovery, occluders were tightened to acutely increase and maintain systemic venous pressure at 15 mm Hg (n = 6), simulating 1-stage Fontan conversion. Control animals (n = 4) received identical surgery, but venous pressure was not manipulated.ResultsCardiac index decreased significantly (3.9 ± 1.0 mL/min/m(2) to 2.7 ± 0.7 mL/min/m(2), P < .001) and then normalized to control at 2 weeks. Circulating blood volume increased (100 ± 9.4 mL/kg vs 85.5 ± 8.4 mL/kg, P = .034) as a persistent response. Cardiac reserve improved and was not different from control by week 3. Resting heart rate decreased in both groups. Oxygen extraction (arteriovenous oxygen difference) and neurohormonal mediators increased transiently and then normalized by week 2.ConclusionsAdaptation to global elevation in systemic venous pressure to Fontan levels is complete within 2 weeks. Increased blood volume and reduced heart rate are persistent responses. Increased oxygen extraction and neurohormonal up-regulation are temporary responses that normalize with recovery of cardiac output. With improved physiologic understanding of systemic adaptation to Fontan conversion, approaches to single-ventricle palliation can be more objectively assessed and optimized.
| S-EPMC2925053 | biostudies-literature
Project description:Aim: The present study aims to identify those microRNAs (miRNAs) in patients with univentricular heart (UVH) disease with and without Fontan palliation that may be associated with advanced liver fibrosis/cirrhosis. Materials and Methods: SurePrint™ 8 × 60K Human v21 miRNA arrays were used to determine the miRNA abundance profiles in the blood of 48 UVH patients with and without Fontan palliation and 32 matched healthy controls. The abundance levels of selected miRNAs have been validated by quantitative reverse transcription-polymerase chain reaction (RT-qPCR). Results: According to microarray analysis, 50 miRNAs were found to be significantly abundant in UVH patients of which miR-29b-3p and miR-29c-3p were significantly related to the model of end-stage liver disease (MELD)-Albumin and albumin-bilirubin (ALBI) score representing advanced liver fibrosis/cirrhosis. Relative expression levels of both miRNAs were significantly higher in patients with a higher collapsibility index representing venous hepatic congestion, a higher MELD-Albumin or ALBI score and incomplete or no Fontan palliation. In the logistic regression analysis, a MELD-Albumin score ? 11 or ALBI score > -2.6 were best predicted by total bilirubin (OR 6.630, P = 0.016), albumin (OR 0.424, P = 0.026), and miR-29c-3p (OR 33.060, P = 0.047). After adjustment to the status of Fontan palliation, however, no statistical significance of these parameters was found thus underlining the importance of palliation status on progression of liver fibrosis/ cirrhosis in UVH patients. Conclusions: In UVH patients with and without Fontan palliation, miR-29b-3p and miR-29c-3p seem to be markers of advanced liver fibrosis/cirrhosis and thus may be used in the risk assessment of these patients.
| S-EPMC7820747 | biostudies-literature
Project description:Background: In patients with left heart failure, microRNAs (miRNAs) have been shown to be of diagnostic and prognostic value. The present study aims to identify miRNAs in patients with univentricular heart (UVH) disease that may be predictive of overt heart failure. Methods: A large panel of human miRNA arrays were used to determine miRNA expression profiles in the blood of 48 UVH patients and 32 age and gender-matched healthy controls. For further selection, the most abundantly expressed miRNA arrays were related to clinical measures of heart failure and selected miRNAs validated by Reverse transcription quantitative polymerase chain reaction (RT-qPCR). These validated miRNAs were used for the prediction of overt heart failure and all-cause mortality. Results: According to microarray analysis, 50 miRNAs were found to be significantly abundant in UVH patients of which miR-150-5p was best related to heart failure parameters. According to ROC analysis, NT-proBNP levels (AUC 0.940, 95% CI 0.873-1.000; p=0.001), miR-150-5p expression levels (AUC 0.905, 95% CI 0.779-1.000; p=0.001) and a higher NYHA class ≥ III (AUC 0.893, 95% CI 0.713-1.000; p=0.002) were the 3 most significant predictors of overt heart failure. Using a combined biomarker model, AUC increased to 0.980 indicating an additive value of miR-150-5p. Moreover, in the multivariate analysis, a higher NYHA class ≥ III (p=0.005) and miR-150-5p (p=0.006) turned out to be independent predictors of overt heart failure. Conclusion: In patients with UVH, miR-150-5p has additive value to natriuretic peptides for the prediction of overt heart failure and thus may be used as additional biomarker for risk assessment in these patients.
2019-10-17 | GSE136547 | GEO
Project description:Background:The domino-donor operation occurs when a "conditioned" heart from the heart-lung transplant (HLT) recipient is transplanted into a separate heart transplant (HT) recipient. The purpose of this systematic review was to investigate the indications and outcomes associated with the domino procedure. Methods:An electronic search was performed to identify all prospective and retrospective studies on the domino procedure in the English literature. Eight studies reported 183 HLT recipients and 263 HT recipients who were included in the final analysis. Results:HLT indications included cystic fibrosis in 58% (95% CI: 27-84%) of recipients, primary pulmonary hypertension (PPH) in 17% (95% CI: 12-24%), bronchiectasis in 5% (95% CI: 3-10%), emphysema in 5% (95% CI: 0-45%), and Eisenmenger's syndrome in 4% (95% CI: 2-8%). HT indications included ischemic heart disease in 40% (95% CI: 33-47%), non-ischemic disease in 39% (95% CI: 25-56%), and re-transplantation in 10% (95% CI: 1-59%). The pooled mean pulmonary vascular resistance (PVR) in HT recipients was 3.05 Woods units (95% CI: 0.14-5.95). The overall mortality in the HLT group was 28% (95% CI: 18-41%) at an average follow-up of 15.68 months (95% CI: 0.82-30.54), and 35% (95% CI: 17-58%) in the HT group at an average follow-up of 37.26 months (95% CI: 6.68-67.84). Freedom from rejection in HT was 94% (95% CI: 75-99%) at 1 month, 77% (95% CI: 30-96%) at 6 months, and 41% (95% CI: 33-50%) at 1 year. Conclusions:The domino procedure appears to be a viable option in properly selected patients that can be performed safely with acceptable outcomes.
| S-EPMC7049558 | biostudies-literature
Project description:The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to hearts not amenable to biventricular repair, namely hearts with two ventricles unable to sustain separately pulmonary and systemic circulations in sequence. In the latter definition, even hearts with one hypoplastic ventricle are considered "functional" univentricular hearts. They include pulmonary/aortic atresia or severe stenosis with hypoplastic ventricle, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of right ventricular cavity. In this setting, the surgical repair is univentricular with "Fontan" operation, bypassing the ventricular mass. In other words, functionally univentricular heart is a condition in which, after surgery, only one ventricle sustain systemic circulation. Univentricular hearts (double inlet or absent AV connection) almost invariably show two ventricular chambers, one main and one accessory, which lacks an inlet portion. The latter is located posteriorly when morphologically left and anteriorly when morphologically right. As far as double inlet left ventricle, this is usually associated with discordant ventriculo-arterial (VA) connection (transposition of the great arteries) and all the blood flow to the aorta, which takes origin from the hypoplastic anterior right ventricle, is ventricular septal defect (bulbo-ventricular foramen) dependent. If restrictive, an aortic arch obstruction may be present. Double inlet left ventricle may be rarely associated with VA concordance (Holmes heart). As far as double inlet right ventricle with posterior hypoplastic left ventricular cavity, ventriculo-arterial connection is usually of double outlet type; thus the term double inlet-outlet right ventricle may be coined. Absent right or left AV connection may develop in the setting of both d- or l-loop, whatever the situs. In this condition, the contra-lateral patent AV valve may be either mitral or tricuspid in terms of morphology and the underlying ventricle (main chamber) either morphologically left or right. Establishing the loop, whatever right or left (also called right or left ventricular topology), is a fundamental step in the segmental-sequential analysis of congenital heart disease.
| S-EPMC4083454 | biostudies-other
Project description:To understand the attitudes of the Saudi population towards heart donation and transplantation. Methods: A survey using a questionnaire addressing attitudes towards organ transplantation and donation was conducted across 18 cities in Saudi Arabia between September 2015 and March 2016. Results: A total of 1250 respondents participated in the survey. Of these, approximately 91% agree with the concept of organ transplantation but approximately 17% do not agree with the concept of heart transplantation; 42.4% of whom reject heart transplants for religious reasons. Only 43.6% of respondents expressed a willingness to donate their heart and approximately 58% would consent to the donation of a relative's organ after death. A total of 59.7% of respondents believe that organ donation is regulated and 31.8% fear that the doctors will not try hard enough to save their lives if they consent to organ donation. Approximately 77% believe the heart is removed while the donor is alive; although, the same proportion of respondents thought they knew what brain death meant. Conclusion: In general, the Saudi population seem to accept the concept of transplantation and are willing to donate, but still hold some reservations towards heart donation.
| S-EPMC5556283 | biostudies-other
Project description: Not available
| S-EPMC10960831 | biostudies-literature
Project description:Objective:Patients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV). Methods:All 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX. Results:Among the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five ?years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6). Conclusion:SRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS.
| S-EPMC6196969 | biostudies-other
Project description:At 22 years following heart transplantation, a patient presented with incessant atrial flutter. During electrophysiologic study, 2 simultaneous atrial arrhythmias were mapped, 1 from the donor and 1 from the recipient's heart. High-density mapping allowed for rapid identification of electrically abnormal areas, which were successfully ablated, thus restoring sinus rhythm. (Level of Difficulty: Advanced.).
| S-EPMC8301501 | biostudies-literature