Pediatr Transplantation 2015: 19: E93–E96
© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Pediatric Transplantation DOI: 10.1111/petr.12454
Refractory cardiogenic shock in a patient with b-thalassemia major requiring mechanical circulatory support: Case report and literature review Horne D, Conway J, Kantor PF, AlAklabi MM, Anand V, Bruce A, Garcia Guerra G, Rebeyka IM, Ross DB, Buchholz H. (2015) Refractory cardiogenic shock in a patient with b-thalassemia major requiring mechanical circulatory support: Case report and literature review. Pediatr Transplant, 19: E93–E96. DOI: 10.1111/petr.12454.
David Horne1, Jennifer Conway2, Paul F. Kantor2, Mohammed M. AlAklabi1, Vijay Anand3, Aisha Bruce4, Gonzalo Garcia Guerra3, Ivan M. Rebeyka1, David B. Ross1 and Holger Buchholz1 1
Abstract: Iron overload cardiomyopathy secondary to b-thalassemia major is a potentially reversible condition managed with chelation and medical hemodynamic support, as bridge-to-recovery or transplant. We describe our experience, and challenges faced, in a pediatric patient with iron overload cardiomyopathy secondary to b-thalassemia major, requiring biventricular MCS.
Division of Cardiac Surgery, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada, 2Department of Paediatric Cardiology, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada, 3Department of Critical Care, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada, 4Department of Haematology, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada Key words: cardiomyopathy – paediatrics – surgery – transition David Horne, MD, Division of Cardiac Surgery, Stollery Children’s Hospital, University of Alberta, 4A7.C Mazankowski Alberta Heart Institute 8440 112 Street, T6G 2B7 Edmonton, Alberta, Canada Tel.: 1 780-407-8033 Fax: 1 780-407-8033 E-mail: [email protected] Accepted for publication 6 February 2015
Background
Despite a recent consensus statement from the American Heart Association (1) addressing cardiovascular function and treatment of b-TM, there is paucity of literature describing refractory cardiac failure in patients with iron overload cardiomyopathy necessitating MCS. We present a unique pediatric case requiring Abbreviations: ARDS, acute respiratory distress syndrome; BiVAD, bi-ventricular assist devices; INTERMACS, Interagency Registry for Mechanically Assited Circulatory Support; LVAD, left ventricular assist device; MCS, mechanical circulatory support; RPM, revolutions per minute; RV, right ventricle; RVAD, right ventricular assist device; TAH, total artificial heart; VAD, ventricular assist devices; b-TM, b-thalassemia major.
long-term biventricular mechanical support, highlighting the challenges in this unique patient population. Case report
A 16-yr-old girl (31 kg, BSA = 1.2) with b-TM presented in cardiogenic shock with biventricular failure from iron overload. Despite high-dose inotropes and escalation to dual intravenous chelation therapy (desferrioxamine and deferiprone), she remained in refractory heart failure with symptoms and signs of low cardiac output (INTERMACS 2). One week following her presentation, ThoratecÒ (Thoratec Corp., Pleasanton, CA, USA) CentriMagÒ continuous flow BiVAD were implanted as a bridge-to-decision as her prognoE93
Horne et al.
sis for cardiac function was unclear. Post-operatively she had issues with bleeding and ongoing hemolysis needing frequent blood transfusions. She was supported with BiVAD for six wk with no echocardiographic evidence of improvement in either the left or right ventricular function. Therefore, a decision was made for implantation of a durable continuous flow LVAD (HeartMateÒ II) (Thoratec Corp., Pleasanton, CA, USA). At the time of implantation, we felt that the HeartMateÒ II would require less anticoagulation than comparable devices. The right heart was supported with ThoratecÒ CentriMagÒ connected to EXCORÒ, Berlin Heart, The Woodlands, TX, USA cannulas in the right atrium and pulmonary artery. Due to her small stature, we felt that there was not enough thoracic volume to accommodate two HeartWareÒ devices (Heartware Inc., Framingham, MA, USA). After two wk of support with the ThoratecÒ CentriMagÒ, the mode of right heart support was switched to a pulsatile EXCORÒ pump (60 cc chamber at 75/min providing a cardiac output of 2.25 L/min) (Fig. 1a). This strategy was implanted to allow for increased mobility and discharge from the intensive care unit. Adequate function of the HeartMateÒ II LVAD was achieved (calculated CO 4–6 L/min at that point in time), but resulted in the early onset (
© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Pediatric Transplantation DOI: 10.1111/petr.12454
Refractory cardiogenic shock in a patient with b-thalassemia major requiring mechanical circulatory support: Case report and literature review Horne D, Conway J, Kantor PF, AlAklabi MM, Anand V, Bruce A, Garcia Guerra G, Rebeyka IM, Ross DB, Buchholz H. (2015) Refractory cardiogenic shock in a patient with b-thalassemia major requiring mechanical circulatory support: Case report and literature review. Pediatr Transplant, 19: E93–E96. DOI: 10.1111/petr.12454.
David Horne1, Jennifer Conway2, Paul F. Kantor2, Mohammed M. AlAklabi1, Vijay Anand3, Aisha Bruce4, Gonzalo Garcia Guerra3, Ivan M. Rebeyka1, David B. Ross1 and Holger Buchholz1 1
Abstract: Iron overload cardiomyopathy secondary to b-thalassemia major is a potentially reversible condition managed with chelation and medical hemodynamic support, as bridge-to-recovery or transplant. We describe our experience, and challenges faced, in a pediatric patient with iron overload cardiomyopathy secondary to b-thalassemia major, requiring biventricular MCS.
Division of Cardiac Surgery, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada, 2Department of Paediatric Cardiology, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada, 3Department of Critical Care, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada, 4Department of Haematology, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada Key words: cardiomyopathy – paediatrics – surgery – transition David Horne, MD, Division of Cardiac Surgery, Stollery Children’s Hospital, University of Alberta, 4A7.C Mazankowski Alberta Heart Institute 8440 112 Street, T6G 2B7 Edmonton, Alberta, Canada Tel.: 1 780-407-8033 Fax: 1 780-407-8033 E-mail: [email protected] Accepted for publication 6 February 2015
Background
Despite a recent consensus statement from the American Heart Association (1) addressing cardiovascular function and treatment of b-TM, there is paucity of literature describing refractory cardiac failure in patients with iron overload cardiomyopathy necessitating MCS. We present a unique pediatric case requiring Abbreviations: ARDS, acute respiratory distress syndrome; BiVAD, bi-ventricular assist devices; INTERMACS, Interagency Registry for Mechanically Assited Circulatory Support; LVAD, left ventricular assist device; MCS, mechanical circulatory support; RPM, revolutions per minute; RV, right ventricle; RVAD, right ventricular assist device; TAH, total artificial heart; VAD, ventricular assist devices; b-TM, b-thalassemia major.
long-term biventricular mechanical support, highlighting the challenges in this unique patient population. Case report
A 16-yr-old girl (31 kg, BSA = 1.2) with b-TM presented in cardiogenic shock with biventricular failure from iron overload. Despite high-dose inotropes and escalation to dual intravenous chelation therapy (desferrioxamine and deferiprone), she remained in refractory heart failure with symptoms and signs of low cardiac output (INTERMACS 2). One week following her presentation, ThoratecÒ (Thoratec Corp., Pleasanton, CA, USA) CentriMagÒ continuous flow BiVAD were implanted as a bridge-to-decision as her prognoE93
Horne et al.
sis for cardiac function was unclear. Post-operatively she had issues with bleeding and ongoing hemolysis needing frequent blood transfusions. She was supported with BiVAD for six wk with no echocardiographic evidence of improvement in either the left or right ventricular function. Therefore, a decision was made for implantation of a durable continuous flow LVAD (HeartMateÒ II) (Thoratec Corp., Pleasanton, CA, USA). At the time of implantation, we felt that the HeartMateÒ II would require less anticoagulation than comparable devices. The right heart was supported with ThoratecÒ CentriMagÒ connected to EXCORÒ, Berlin Heart, The Woodlands, TX, USA cannulas in the right atrium and pulmonary artery. Due to her small stature, we felt that there was not enough thoracic volume to accommodate two HeartWareÒ devices (Heartware Inc., Framingham, MA, USA). After two wk of support with the ThoratecÒ CentriMagÒ, the mode of right heart support was switched to a pulsatile EXCORÒ pump (60 cc chamber at 75/min providing a cardiac output of 2.25 L/min) (Fig. 1a). This strategy was implanted to allow for increased mobility and discharge from the intensive care unit. Adequate function of the HeartMateÒ II LVAD was achieved (calculated CO 4–6 L/min at that point in time), but resulted in the early onset (
