Failed Fontan heart transplant candidates with preserved vs impaired ventricular ejection: 2 distinct patient populations

Simpson, Kathleen E.; Cibulka, Nicole; Lee, Caroline K.; Huddleston, Charles H.; Canter, Charles E.

doi:10.1016/j.healun.2012.02.003

Cited by 40 publications

(41 citation statements)

References 5 publications

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“…The causative mechanism of myocardial fibrosis development is believed to be myofibroblast activation and subsequent collagen production (15). Chronic cyanosis, pulmonary vascular disease, allosensitization, multiple previous operations, and the potential for renal and hepatic failure in Fontan patients (31) can present challenges when ACHD patients develop end stage heart failure (16). Protein losing enteropathy (PLE) is also a significant challenge in patients with failed Fontan physiology (32).…”

Section: Mechanisms Of Late Ventricular Dysfunction In Achd Patients mentioning

confidence: 99%

“…That analysis was also flawed by the lack of distinction between UCHD patients with impaired or preserved ventricular function. It has been shown that post-Fontan patients with preserved systolic ventricular function have worse post-transplant outcomes (4,31). This finding is related to high incidence of PLE potentially as a result of chronic ventricular volume overload from aortopulmonary collaterals (52,53).…”

Section: Risk Factors For Poor Outcome Post-transplant In Achd Patientsmentioning

confidence: 99%

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Overview of adult congenital heart transplants

Bryant¹,

Morales²

2018

Ann. Cardiothorac. Surg.

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Transplantation for adult patients with congenital heart disease (ACHD) is a growing clinical endeavor in the transplant community. Understanding the results and defining potential high-risk patient subsets will allow optimization of patient outcomes. This review summarizes the scope of ACHD transplantation, the mechanisms of late ventricular dysfunction, the ACHD population at risk of developing heart failure, ranges between 14% and 39%. The majority of early mortality occurs in ACHD patients with univentricular congenital heart disease. However, there has been improvement in operative survival in more contemporary studies. In conclusion, the experience with cardiac transplantation for ACHD patients with end-stage heart failure is growing, and high-risk patient subsets have been defined. Significant strides have been made in developing evidence-based guidelines of indications for transplant, and the intraoperative management of complex reconstruction has evolved. With proper patient selection, more aggressive use of mechanical circulatory support, and earlier referral of patients with failing Fontan physiology, outcomes should continue to improve.

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Section: Mechanisms Of Late Ventricular Dysfunction In Achd Patients mentioning

confidence: 99%

Section: Risk Factors For Poor Outcome Post-transplant In Achd Patientsmentioning

confidence: 99%

Overview of adult congenital heart transplants

Bryant¹,

Morales²

2018

Ann. Cardiothorac. Surg.

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“…Thus, although multiple pharmacotherapeutic options and anatomic interventions have demonstrated some efficacy in temporizing PLE symptoms, no available therapy has been shown to be consistently curative except heart transplantation (HTx). [5][6][7][8] The 2006 Pediatric Heart Transplant Study (PHTS) evaluation of Fontan patients undergoing HTx found no difference in survival between PLE and non-PLE patients but only included 25 PLE patients in the cohort. 8 This study was limited by a small sample size and limited duration of follow-up.…”

mentioning

confidence: 99%

“…More recently, reports from larger centers, including PLE patients in the current era, found worsened survival in Fontan patients with preserved ventricular systolic function, most of whom had PLE. 5,9 This creates a more concerning picture for PLE patients after HTx.…”

mentioning

confidence: 99%

Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis

Schumacher

Gossett

Guleserian

et al. 2015

The Journal of Heart and Lung Transplantation

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“…5 Depiction of the hemodynamic profile of Fontan failure has been similar to traditional heart failure: elevated central venous pressure, pulmonary capillary wedge pressure, and systemic vascular resistance (SVR), with a low cardiac index. 1,6–8 However, clinical deterioration can occur in the absence of ventricular dysfunction, 3,9 suggesting that distinct mechanisms are contributive. Based on the growing evidence of liver pathology in Fontan patients over time, 10,11 we hypothesized that portal hypertension might play a signifi-cant role in failing Fontan pathophysiology.…”

mentioning

confidence: 99%

Hemodynamic Phenotype of the Failing Fontan in an Adult Population

Hebson

McCabe

Elder

et al. 2013

The American Journal of Cardiology

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Fontan failure can occur even with normal systolic ventricular function and often in the context of significant liver disease. We hypothesized that Fontan failure is hemodynamically distinct from traditional heart failure and characterized by low systemic vascular resistance (SVR) index and preserved cardiac index. Twenty-seven symptomatic adult Fontan (SAF) patients who underwent catheterization from 2001 to 2011 constituted our study group. Fifty-four predominantly asymptomatic pediatric Fontan (PF) patients who underwent catheterization during the same period were randomly selected to perform a control:case cohort analysis. Clinical comparisons were made between the 2 groups. The adults were more symptomatic than the PF cohort (New York Heart Association classes I and II or III and IV: 48% or 52% [SAF] vs 94% or 6% [PF], respectively, p <0.01). SAF versus PF mean catheterization findings were central venous pressure 18 ± 6 versus 14 ± 3 mm Hg (p <0.01), SVR index 1,680 ± 368 versus 1,960 ± 550 dyn s/cm5/m2 (p = 0.02), and cardiac index 2.7 ± 0.8 versus 2.8 ± 0.7 L/min/m2 (p = 0.25). By imaging, the SAF cohort demonstrated a greater incidence of abnormal liver texture changes (96% vs 75%, p = 0.04) and nodularity (77% vs 42%, p = 0.02). In conclusion, adult patients with failing Fontan circulation had a lower SVR index and similar cardiac index compared with the pediatric cohort. Liver disease in the adults was more advanced. Our data suggest that Fontan failure is a distinct circulatory derangement with hemodynamic features similar to portal hypertension, albeit with limited ability to augment cardiac output.

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Failed Fontan heart transplant candidates with preserved vs impaired ventricular ejection: 2 distinct patient populations

Cited by 40 publications

References 5 publications

Overview of adult congenital heart transplants

Overview of adult congenital heart transplants

Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis

Hemodynamic Phenotype of the Failing Fontan in an Adult Population

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