AIMS: The Fontan operation is associated with significant long-term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan palliation. Our aim was to assess haemodynamic profiles for Fontan patients and evaluate their relationships to prognosis. METHODS AND RESULTS: Eighty-four adult Fontan patients without obstruction or significant valve disease undergoing cardiac catheterization for Fontan-related co-morbidities were divided into four different haemodynamic profiles based on normal/high Fontan pressures (FP) (< 15 or ≥ 15 mmHg) and low/normal cardiac index (CI) (< 2.5 or ≥ 2.5 L/min/m2 ). Fourteen patients had low CI/high FP (17%), 24 low CI/normal FP (29%), 20 normal CI/high FP (24%), and 26 normal CI/normal FP (31%). Demographic and clinical data were similar among groups except for a trend towards a higher prevalence of cirrhosis in the normal CI/high FP (50%) group. Systemic vascular afterload, assessed by systemic vascular resistance and effective arterial elastance, was higher in patients with low CI/high FP and low CI/normal FP as compared to normal CI profiles. Pulmonary vascular resistance indices were higher in patients with low CI/high FP than all other profiles. The normal CI/high FP haemodynamic profile was an independent predictor of mortality (hazard ratio 4.1, 95% confidence interval 1.2-11.5). Heterotaxy and protein-losing enteropathy were also predictors of mortality on the multivariate model. CONCLUSION: The proposed haemodynamic classification provides prognostic information in adults post-Fontan with patients in the normal CI/high FP profile having worse survival. Whether this classification can guide management in these patients deserves further investigation.
Abstract Objective The staged Fontan procedure has shown promising short-term outcomes in patients with single ventricles. However, Fontan-associated liver disease is a marked problem as patients age. The purpose of this study is to investigate the relationship between hemodynamics and liver fibrosis in patients undergoing the Fontan. Methods A total of 33 patients undergoing the Fontan with liver fibrosis were included in this study. Cardiac magnetic resonance and phase-contrast cardiac magneti...
INTRODUCTION: The Fontan procedure is the palliative procedure for single ventricle physiology. Pulmonary resistance plays a key role in the success of this operation. There are conflicting data concerning the impact of pulmonary vasodilators on survival and functional capacity among Fontan patients. AIM: The aim of this retrospective, single-center, nonrandomized study was to investigate the potential effect of pulmonary vasodilators on pulmonary vasculature in Fontan patients. METHOD: Twenty-s...
#2Heidi M. Connolly(Cincinnati Children's Hospital Medical Center)H-Index: 75
Last. Barry A. Borlaug(Cincinnati Children's Hospital Medical Center)H-Index: 76
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Background Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The objective was to determine whether the absence of subpulmonary ventricle in the Fontan circulation would make patients more vulnerable to even low-level elevations in PVRI, and when coupled with low cardiac index, this would identify patients at increased risk of F...
Background Elevated central venous pressure (CVP), low cardiac output, and mild hypoxia are common early and late after Fontan operations. However, the association of these characteristics with late mortality is unclear. We aimed to elucidate the hemodynamic determinants of mortality after Fontan operation. Method We evaluated early (group early; 0.5-5years postoperatively, n=387) and late (group late; ≥15years postoperatively, n=161) Fontan hemodynamics that included CVP (mm Hg), cardiac index ...
Abstract Objectives To evaluate the effects of endothelin inhibitors (ERAs) on hemodynamic and functional parameters in patients post-Fontan procedure with high pulmonary vascular resistance (PVR). Methods Among our cohort of patients with Fontan circulation, 8 children, 8 adolescents, and 8 adults had PVR ≥2 WU*m 2 . These patients were treated with ERAs (minors with bosentan, adults with macitentan) and reevaluated after 6 months. Pre- and posttreatment hemodynamic variables were assessed by c...
#2Lea F. Surrey(Children's Hospital of Philadelphia)H-Index: 14
Last. Jack Rychik(UPenn: University of Pennsylvania)H-Index: 69
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BackgroundCongestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. Methods and ResultsLiver biopsies and c...
Background In adult Fontan patients, ventricular or arterial dysfunction may impact homeostasis of the Fontan circulation and predispose to heart failure. We sought to characterize ventricular‐arterial (VA) properties in adult Fontan patients. Methods and Results Adult Fontan patients (n=170), including those with right (SRV, n=57) and left (SLV, n=113) dominant ventricular morphology, were compared to age, sex, and body size matched controls (n=170). Arterial function, load‐insensitive measures...
Background In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. Methods A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse...
Background There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation. Objectives This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic. Methods Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study. Results Overall, 10-, 20-, and 30-year survival for 1,052...
Background The Fontan circulation is a palliation for patients with a functionally univentricular heart. It is characterized by gradual attrition over time. An increase in pulmonary vascular resistance could be a key factor in the long-term failure of the Fontan circulation. In this study we aimed to identify pulmonary vascular remodeling in patients with a Fontan circulation. Methods Pulmonary vascular histomorphometric analysis and immunohistochemistry were performed in lung tissue obtained at...
INTRODUCTION Systemic ventricular end-diastolic pressure is an important haemodynamic variable in adult patients with Fontan circulation. Risk factors associated with elevated end-diastolic pressure have not been clearly identified in this population. METHODS All patients > 18 years with Fontan circulation who underwent cardiac catheterisation at our centre between 1/08 and 3/19 were included. Relevant patient variables were extracted. Univariate and multivariate general linear models were analy...
Abstract null null Background null The Fontan operation is a palliative procedure and a substantial number of patients eventually experiences late Fontan circulation failure. Previous concepts of Fontan failure implicate increasing pulmonary vascular resistance (PVR) as a key contributor to late circulatory failure. However, data to support this assumption are sparse. We sought to characterize longitudinal hemodynamic and echocardiographic findings in adult failing Fontan patients. null null nul...
Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous ...
Last. Kimberly Y. Lin(Children's Hospital of Philadelphia)H-Index: 12
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AIM To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant. METHODS In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan...
Portosystemic venous shunt (PSVS) is a vascular anomaly between the portal and systemic veins, resulting in several critical complications. Although PSVS is often associated with congenital heart diseases, the clinical association between Fontan circulation and PSVS has not been elucidated. This study aimed to investigate the clinical features of Fontan patients with PSVS. Two hundred thirteen patients who underwent Fontan procedure are being followed up at Adult Congenital Heart Disease clinic ...
Abstract Background Pulmonary artery wedge pressure (PAWP) is often elevated in patients with right-sided congenital heart disease (CHD), raising the possibility of coexisting left heart disease, but pressure-volume relationships in the left and right sides of the heart influence one another through interdependence, which may be amplified in patients with CHD. Methods We hypothesized that increases in PAWP in CHD patients would be more strongly related to ventricular interdependence as compared ...
Abstract Background Elevated pulmonary artery wedge pressure (PAWP) is the hallmark of left heart failure, and is responsible for heart failure symptoms and mortality. Although, PAWP typically correlates with right atrial pressure (RAP), it is primarily dependent on left heart myocardial properties and volume status. Since right heart disease can occur in the absence of left heart disease in patients with tetralogy of Fallot (TOF), we hypothesized that RAP was the primary determinant of PAWP in ...
#1James Monaco(CU: University of Colorado Boulder)H-Index: 1
#2Amber Khanna(CU: University of Colorado Boulder)H-Index: 8
Last. Prateeti Khazanie(CU: University of Colorado Boulder)H-Index: 1
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Advances in surgery and pediatric care over the past decades have achieved improved survival for children born with congenital heart disease (CHD) and have produced a large, growing population of patients with adult congenital heart disease (ACHD). Heart failure has emerged as the leading cause of death and a major cause of morbidity among the ACHD population, while as little evidence supports the efficacy of guideline-directed medical therapies in this population. It is increasingly important t...
Abstract Background Previous studies have described echocardiographic indices of right ventricular (RV) diastolic function in patients with tetralogy of Fallot (TOF) but these indices have not been validated against invasive hemodynamic data. The purpose of this study was to determine echocardiographic predictors of severe RV diastolic dysfunction, and the impact of severe RV diastolic dysfunction on transplant-free survival. Methods Cohort study of TOF patients that underwent non-simultaneous c...
AIMS: In univentricular hearts, selective lung vasodilators such as phosphodiesterase type 5 (PDE5) inhibitors would decrease pulmonary resistance and improve exercise tolerance. However, the level of evidence for the use of PDE5 inhibitors in patients with a single ventricle (SV) remains limited. We present the SV-INHIBITION study rationale, design, and methods. METHODS AND RESULTS: The SV-INHIBITION trial is a nationwide multicentre, randomized, double blind, placebo-controlled, Phase III stud...
