Challenges of Cardiac Transplantation Following the Fontan Procedure

Kirklin, James K.; Pearce, F. Bennett; Dabal, Robert J.; Carlo, Waldemar F.; Mauchley, David C.

doi:10.1177/2150135117714460

Cited by 12 publications

(18 citation statements)

References 36 publications

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“…This setting is characterised by a chronic state of reduced CO due to ventricle under filling, which will progressively increase end-diastolic pressures and deteriorate systemic venous congestion. 51 From the clinical point of view, a failing Fontan is characterised by low CO syndrome and multiorgan system failure. The most common symptoms in young patients are listed in Table 2.…”

Section: Right-sided Obstructionmentioning

confidence: 99%

Pathophysiology and clinical presentation of paediatric heart failure related to congenital heart disease

et al. 2021

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Congenital heart disease (CHD) and cardiomyopathies represent the two most important causes of paediatric heart failure (HF) in developed countries. We made a review of the literature on pathophysiology and clinical presentation of paediatric HF in children with CHD. Two main pathophysiologic models can be identified: the 'overcirculation failure', characterised by signs and symptoms of congestion or hypoperfusion, due respectively to volume or pressure overload, and the 'pump failure'.

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Section: Right-sided Obstructionmentioning

confidence: 99%

Pathophysiology and clinical presentation of paediatric heart failure related to congenital heart disease

et al. 2021

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“…In an increasing number of patients, the chronic elevated central venous pressure leads to serious complications and may finally result in a failing circulation with or without preserved ventricular function. 1 Nowadays, cTx remains the only therapeutic option in this situation. Only little information is available in the literature about the perioperative management during cTx in this dedicated group of patients.…”

Section: Introductionmentioning

confidence: 99%

Anesthesia for Pediatric Heart Transplantation: Are Patients With a Failing Hemi-Fontan- or Fontan-Physiology Different?

Müeller

Paul

Mann

et al. 2019

Semin Cardiothorac Vasc Anesth

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Background. The absolute number of patients presenting with failure of the hemi-Fontan or Fontan circulation for cardiac transplantation (cTx) will continuously rise. We aimed to analyze the intraoperative differences in patients undergoing cTx for a failing hemi-Fontan or Fontan circulation (group A) with those undergoing cTx for cardiomyopathy and congenital heart disease (group B). Methods. Data of patients undergoing cTx during a 10-year period were compared between group A and group B patients. Results. cTx was performed in 83 patients (group A, n = 21; group B, n = 62). Surgical times including median incision-suture time (549 minutes vs 386 minutes, P < .05) and median organ ischemia time (305 minutes vs 233 minutes, P < .05) were longer in group A patients. After weaning off cardiopulmonary bypass a higher median modified Vasoactive Inotropic Score (16 vs 10, P < .05) was necessary in group A patients. During surgery more fresh frozen plasma (44 mL/kg vs 20 mL/kg, P < .05), platelet concentrates (20 mL/kg vs 14 mL/kg, P < .05), and coagulation factor concentrates were given in group A patients. Mortality during the first 90 days after cTx was higher in group A (23.8% vs 6.5%, P < .05). Conclusion. Patients undergoing cTx for a failing hemi-Fontan or Fontan circulation are challenging. They require an intensive vasoactive and inotropic support; furthermore, special attention should be paid to the management of bleeding complications. cTx for this group of patients is associated with higher 90 days mortality.

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“…[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. [13] Consequently, there is a demand for research to understand the impact of living with this condition and improve outcomes for patients and families throughout their journey. The International Fontan Interest Group has been established as a collaborative initiative to improve outcomes and has called for engagement of stakeholders to direct research priorities.…”

Section: Introductionmentioning

confidence: 99%

“…Ongoing physiological and psychosocial issues place a considerable burden on patients and their families with a significant reduction in quality of life 11 . Although 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 13 . Consequently, there is a demand for research to understand the impact of living with this condition and improve outcomes for patients and families throughout their journey.…”

mentioning

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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Challenges of Cardiac Transplantation Following the Fontan Procedure

Cited by 12 publications

References 36 publications

Pathophysiology and clinical presentation of paediatric heart failure related to congenital heart disease

Pathophysiology and clinical presentation of paediatric heart failure related to congenital heart disease

Anesthesia for Pediatric Heart Transplantation: Are Patients With a Failing Hemi-Fontan- or Fontan-Physiology Different?

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

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