The Relentless Effects of the Fontan Paradox

Rychik, Jack

doi:10.1053/j.pcsu.2015.11.006

Cited by 143 publications

(147 citation statements)

References 30 publications

(25 reference statements)

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“…One of the biggest barriers for successful transplants in the Fontan population is the underlying and poorly understood impact of subclinical liver failure. Fontan‐associated liver disease is highly prevalent, as these patients are exposed to systemic venous hypertension, hepatic congestion, and low cardiac output leading to various stages of liver disease ranging from clinically silent cirrhosis to hepatocellular carcinoma . A recent study found that adults with Fontan palliation universally have biopsy‐proven hepatic fibrosis despite normal synthetic function .…”

Section: Discussionmentioning

confidence: 99%

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

et al. 2020

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Introduction Treatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait‐list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients. Methods Using the Nationwide Inpatient Sample database, we identified all adults aged at least 18 years old who underwent heart transplantation across U.S. hospitals from 2004 to 2014. We then identified those with specific ICD‐9 codes to include tricuspid atresia, hypoplastic left heart syndrome and common ventricle. Multivariate regression models were created to adjust for potential confounders. Results A total of 93 Fontan patients underwent heart transplant during the study time (0.5% of all heart transplants). Compared to non‐Fontan heart transplantations, Fontan patients were younger, with a higher incidence of liver disease and coagulopathy. Fontan patients receiving heart transplant had higher mortality during transplant hospitalization compared to non‐Fontan patients (26.3% vs 5.3% OR, 18.10, CI, 5.06‐65.0 P < .001). Extracorporeal membrane oxygenator (ECMO) usage and bleeding were also higher in the Fontan cohort with an OR of 5.30 (P = .016) and 5.32 (P = .015) for ECMO and bleeding, respectively. The remaining outcomes were similar for both cohorts. Conclusion Adults with Fontan palliation undergoing heart transplantation have exceptionally high inpatient mortality, which is nearly five times that of non‐Fontan heart transplant recipients, perhaps related to a delayed referral, surgical complexity, and coexistent, underrecognized liver failure.

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Section: Discussionmentioning

confidence: 99%

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

et al. 2020

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“…Within years of Fontan completion, almost all patients are noted to develop hepatic fibrosis with some evolving to cirrhosis [91]. Some rare cases of hepatocarcinomas have also been described [49,92].…”

Section: Liver and Renal Failurementioning

confidence: 99%

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

2016

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Universally fatal only four decades ago, the progress in the three-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. While the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low risk operations, the stage I Norwood procedure remains one of the highest risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals in excess of 90% for the Norwood. This traditional three-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomized 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years post-randomization. In addition to modifications to the Norwood procedure, the “hybrid procedure,” a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a “comprehensive” stage II, that combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to not only improve short-term survival, but potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also burden of disease, neurodevelopmental outcomes, psychosocial development and quality of life.

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“…[10] Ongoing physiological and psychosocial issues place a considerable burden on patients and their families with a significant reduction in quality of life. [11] Whilst there are expectations of improving outcomes,[12] the Fontan circulation remains a life-limiting condition with limited treatment options; it is the highest risk diagnosis for heart transplantation yet is the least amenable to mechanical circulatory support.…”

Section: Introductionmentioning

confidence: 99%

Research priorities in single-ventricle heart conditions: a United Kingdom national study

et al. 2018

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Objective To bring together stakeholders in the United Kingdom to establish national priorities for research in single ventricle heart conditions. Methods This study comprised two surveys and a workshop. The initial public online survey asked respondents for up to 3 questions they would like answered by research. Responses were classified as: unanswered, already answered, or unable to be answered by scientific research. In the follow-up survey, unanswered questions were divided into categories and respondents were asked to rank categories and questions by priority. A stakeholder workshop attended by patients, parents, healthcare professionals, researchers and charities was held to determine a final list of research priorities. Results 128 respondents posed 344 research questions, of which 271 were classified as unanswered, and after removing duplicates, 204 questions remained, divided into 20 categories. 56 (49.1%) respondents successfully ranked categories and questions in the second survey. 39 participants attended the workshop, drawing up a list of 30 research priorities across 9 priority categories: Associated co-morbidities, Brain & neurodevelopment, Exercise, Fontan failure, Heart function, Living with a single ventricle heart condition, Management of the well-functioning Fontan circulation, Surgery & perioperative care, and Transplantation, mechanical support & novel therapies. Conclusions Through a multi-stage process, we engaged a wide range of interested parties to establish a list of research priorities in single ventricle heart conditions. This provides a platform for clinicians, researchers and funders in the UK and elsewhere to address the most important questions and improve outcomes in these rare but high-impact congenital heart defects.

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The Relentless Effects of the Fontan Paradox

Cited by 143 publications

References 30 publications

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions

Research priorities in single-ventricle heart conditions: a United Kingdom national study

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