Extracorporeal membrane oxygenation as a bridge to cardiac transplantation in a patient with cardiomyopathy and hemophilia A

Thiagarajan, Ravi R.; Roth, Stephen J.; Margossian, Steven; Mackie, Andrew S.; Neufeld, Ellis J.; Laussen, Peter C.; Forbess, Joseph M.; Blume, Elizabeth D.

doi:10.1007/s00134-003-1748-5

Cited by 13 publications

(4 citation statements)

References 12 publications

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“… 115 Not surprisingly, relative to similar procedures in the general population, cardiac operations in PWH incur substantially increased costs (primarily due to hemostatic treatments) and resource utilization. 115 , 123 , 126 Advance planning and involvement of a multidisciplinary team serves to optimize clinical outcomes while minimizing risk.…”

Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

“…Importantly, the accuracy of ACT monitoring is uncertain unless there are near-normal factor levels. 126 In addition, the utility and accuracy of ACT monitoring during CPB when using hemostatic coverage with bypassing agents is uncertain. For monitoring hemostatic therapy during cardiac surgery (with or without CPB), the measurement of PTT or factor levels is a viable option.…”

Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

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Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B

Ferraris

Boral

Cohen

et al. 2015

Cardiology in Review

104

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With advances in care, increasing numbers of people with hemophilia (PWH) achieve near-normal life expectancies and present with typical age-related cardiovascular conditions. Evidence-based guidelines for medical or surgical management of cardiovascular conditions in individuals with hemophilia are limited. Published recommendations exist for the management of some common cardiovascular conditions (eg, ischemic heart disease, atrial fibrillation), but identifying optimal strategies for anticoagulant or antithrombotic therapy constitutes the primary challenge of managing nonoperative cardiovascular disease (CVD) in PWH. In general, as long as factor concentrates or other hemostatic therapies maintain adequate hemostasis, the recommended medical and surgical management of CVD in PWH parallels that in individuals without hemophilia. The presence of factor inhibitors complicates hemophilia management. Published outcomes of CVD treatment in PWH are similar to those in the general population. Specific knowledge about factor replacement, factor inhibitors, and disease-specific treatment distinguishes the cardiovascular care of PWH from similar care of individuals without this rare bleeding disorder. Furthermore, a multidisciplinary approach incorporating a hematologist with an onsite coagulation laboratory, ideally associated with a hemophilia treatment center, is integral to the management of CVD in PWH.

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Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B

Ferraris

Boral

Cohen

et al. 2015

Cardiology in Review

104

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“…A review of the literature revealed case reports of heart transplant in HA patients were limited to a 63‐year‐old man 7 and four children ages 5, 12, 14, and 15 (Table S1). 8–11 Two patients required VAD and two patients were on ECMO, before and/or after the procedure. Interestingly, two patients developed heart failure secondary to cardiac infarcts/thromboses after receiving activated prothrombin complex concentrate 9,10 .…”

Section: Discussionmentioning

confidence: 99%

Balancing anticoagulation and hemostasis in an infant with severe hemophilia A during cardiac transplantation: Review of the literature and development of a surgical protocol

Regling,

Sehgal,

Hollon

et al. 2023

Pediatric Blood & Cancer

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Hemophilia A is a disorder resulting in a deficiency of clotting factor VIII that can lead to life‐threatening bleeding. Evidence‐based guidelines for surgical interventions like cardiac surgery are limited. Anticoagulation is necessary for cardiac bypass, thus risk of bleeding in a patient with hemophilia is increased and requires careful attention to maintain hemostasis. We report the first infant with severe hemophilia A and dilated cardiomyopathy who underwent successful cardiac transplantation, and review the literature on previous cardiac transplant cases in congenital hemophilia. To ensure safe and effective management, a multidisciplinary approach was used to develop the surgical protocol for transplant.

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“…Patients with multiple organ failure from any etiology, septic shock, burns, trauma, airway abnormalities requiring complicated surgical repair, and immunocompromised patients all represent categories of disease that would have precluded ECMO use in the past but have been successfully supported with ECMO in more recent years. [5][6][7][8][9][10][11][12] Neonates While neonates with respiratory failure have formed the largest group of ECLS patients, advancement in prenatal and perinatal care, coupled with therapies and techniques such as inhaled nitric oxide, surfactant replacement, and high-frequency ventilation, have reduced the need for ECLS in many neonates with respiratory failure. 13 Recognition and resolution of persistent pulmonary hypertension of the newborn with some of the therapies mentioned has decreased the need for ECLS in such patients.…”

Section: Patient Populations Treated With Eclsmentioning

confidence: 99%

Extracorporeal Life Support: Moving at the Speed of Light

Dalton

2011

Respir Care

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Extracorporeal life support (ECLS), or extracorporeal membrane oxygenation (ECMO) as it is alsoknown, has been used to support over 45,000 patients to date. Overall survival is 62%. After many years of no change in equipment and technology, there has been a recent flurry of new pumps, cannulas, and oxygenators available for ECLS use. While the impact of this new technology is not yet completely defined, initial results have found that these systems provide safe support with lower priming volumes and less bleeding complications. New cannulas are also available, some making it easier for venovenous support in patients, from infants through adults. The reported success of ECLS in patients with H1N1 during the 2009 -2010 epidemic and the improved survival of patients randomized to the ECMO arm of a recently completed adult study of respiratory failure have also brought ECLS into the spotlight much more than other years. Whether these developments will usher in a new era of ECLS expansion to a wider range of patients will require close consideration and observation. Other areas that need to be further refined include anticoagulation management, treatment of bleeding complications, learning to "nurse" patients in an awake state, such as is done in some European (and a few United States) centers, and neurodevelopmental outcome on a longterm basis.

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Extracorporeal membrane oxygenation as a bridge to cardiac transplantation in a patient with cardiomyopathy and hemophilia A

Abstract: We report the successful use of ECMO as a bridge to cardiac transplantation in a patient with hemophilia A and low-level factor VIII inhibitors.

Cited by 13 publications

References 12 publications

Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B

Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B

Balancing anticoagulation and hemostasis in an infant with severe hemophilia A during cardiac transplantation: Review of the literature and development of a surgical protocol

Extracorporeal Life Support: Moving at the Speed of Light

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