Dopamine as a potential rescue therapy for refractory protein‐losing enteropathy in Fontan‐palliated patients

Friedland‐Little, Joshua M.; Gajarski, Robert J.; Schumacher, K.

doi:10.1111/petr.12925

Cited by 15 publications

(4 citation statements)

References 18 publications

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“…Finally, although information on management of failing Fontan patients has accumulated on a case-by-case basis 60) 61) 62) 63) 64) 65) 66) 67) 68) 69) 70) 71) 72) 73) and based on small randomized controlled trials, 74) 75) 76) 77) 78) 79) 80) this data cannot be generalized to the majority of Fontan patients, especially those with failing hemodynamics. In addition, we now realize that Fontan hemodynamics changes significantly over time, as patients develop 12) multiple end-organ interactions.…”

Section: Hemodynamics-based Management For Patients With Fontan Circumentioning

confidence: 99%

Where Is the “Optimal” Fontan Hemodynamics?

Ohuchi

2017

Korean Circ J

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Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with congenital heart disease is selected as a candidate for this type of circulation, the ultimate goals of therapy consist of 2 components. One is a smooth adjustment to the new circulation, and the other is long-term circulatory stabilization after adjustment. When either of these goals is not achieved, the patient is categorized as having “failed” Fontan circulation, and the prognosis is dismal. For the first goal of smooth adjustment, a lot of effort has been made to establish criteria for patient selection and intensive management immediately after the Fontan operation. For the second goal of long-term circulatory stabilization, there is limited evidence of successful strategies for long-term hemodynamic stabilization. Furthermore, there have been no data on optimal hemodynamics in Fontan circulation that could be used as a reference for patient management. Although small clinical trials and case reports are available, the results cannot be generalized to the majority of Fontan survivors. We recently reported the clinical and hemodynamic characteristics of early and late failing Fontan survivors and their association with all-cause mortality. This knowledge could provide insight into the complex Fontan pathophysiology and might help establish a management strategy for long-term hemodynamic stabilization.

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Section: Hemodynamics-based Management For Patients With Fontan Circumentioning

confidence: 99%

Where Is the “Optimal” Fontan Hemodynamics?

Ohuchi

2017

Korean Circ J

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“…As data are lacking, the use of chronic inotrope in this subset of patients is not recommended but is commonly and successfully used as an inpatient bridge to more definitive therapy like heart transplant [ 61 ]. In patients with protein-losing enteropathy (PLE), dopamine has been shown to be helpful in producing remission by its action on lymphatic receptors [ 62 ].…”

Section: Advanced Therapies For Heart Failurementioning

confidence: 99%

Contemporary Management of the Failing Fontan

Venkatesh,

Gao,

Abudayyeh

et al. 2024

JCM

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Adult patients with congenital heart disease have now surpassed the pediatric population due to advances in surgery and improved survival. One such complex congenital heart disease seen in adult patients is the Fontan circulation. These patients have complex physiology and are at risk for several complications, including thrombosis of the Fontan pathway, pulmonary vascular disease, heart failure, atrial arrhythmias, atrioventricular valve regurgitation, and protein-losing enteropathy. This review discusses the commonly encountered phenotypes of Fontan circulatory failure and their contemporary management.

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“…Pediatric heart failure guidelines recommend milrinone or dobutamine as first line therapy with the addition of epinephrine for refractory hypotension ( 6 ). In patients with PLE, dopamine therapy can be trialed, as there is evidence it may induce remission in patients with PLE possibly through effects on lymphatic receptors ( 124 ).…”

Section: Treatmentmentioning

confidence: 99%

Management of circulatory failure after Fontan surgery

2022

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With improvement in survival after Fontan surgery resulting in an increasing number of older survivors, there are more patients with a Fontan circulation experiencing circulatory failure each year. Fontan circulatory failure may have a number of underlying etiologies. Once Fontan failure manifests, prognosis is poor, with patient freedom from death or transplant at 10 years of only about 40%. Medical treatments used include traditional heart failure medications such as renin-angiotensin-aldosterone system blockers and beta-blockers, diuretics for symptomatic management, antiarrhythmics for rhythm control, and phosphodiesterase-5 inhibitors to decrease PVR and improve preload. These oral medical therapies are typically not very effective and have little data demonstrating benefit; if there are no surgical or catheter-based interventions to improve the Fontan circulation, patients with severe symptoms often require inotropic medications or mechanical circulatory support. Mechanical circulatory support benefits patients with ventricular dysfunction but may not be as useful in patients with other forms of Fontan failure. Transplant remains the definitive treatment for circulatory failure after Fontan, but patients with a Fontan circulation face many challenges both before and after transplant. There remains significant room and urgent need for improvement in the management and outcomes of patients with circulatory failure after Fontan surgery.

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Dopamine as a potential rescue therapy for refractory protein‐losing enteropathy in Fontan‐palliated patients

Cited by 15 publications

References 18 publications

Where Is the “Optimal” Fontan Hemodynamics?

Where Is the “Optimal” Fontan Hemodynamics?

Contemporary Management of the Failing Fontan

Management of circulatory failure after Fontan surgery

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