Abstract Number: PB1070
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: Dilated cardiomyopathy requires anticoagulation and support devices, ECMO and VAD, bridging to HT. Bleeding and thrombosis are common complications. Patients with SHA, make hemostatic management especially challenging.
Aims: To report the management of a pediatric SHA patient admitted with dilated cardiomyopathy: from initial anticoagulation, through V-A ECMO and VAD to HT.
Methods: Case report: 13 y.o. SHA patient without inhibitor, diagnosed with refractory dilated myocardiopathy. FVIII replacement and anticoagulation, from initial clinical treatment through implantation of ECMO and VAD, and final HT is reported.
FVIII measurement: one stage clotting assay (3 dilutions). UFH and LMWH monitoring by chromogenic AntiXa activity. Thromboelastometry (ROTEM) for intraoperative monitoring.
Results: FVIII Concentrate was initially administered on continuous infusion (levels 30-50IU/dL ) and UFH was started (antiXa 0.35-0.7U/mL). As he was stable, FVIII IV bolus/8 hours was given (aim 20-50IU/dL) and UFH replaced by LMWH (antiXa 0.5-0.1U/ml). After ECMO and VAD UFH was restarted. Prior to surgical procedures 2000U FVIII were administered, with additional 1000 U/2 hours during surgery to raise FVIII to 80-100UI/dL. Tranexamic acid was used during VAD and HT surgeries. Post CPB (cardiopulmonary bypass) blood loss was minimal.
APTT, FVIII, antiXa and ROTEM results in table 1. Correlation between ROTEM parameters and FVIII shown in figure 1. A good correlation between INTEM or HEPTEM CT and FVIII was seen, but all CT values were within normal range, implying a low sensitivity to detect 30 IU/dL FVIII.
The patient had a good clinical outcome, with a DVT (related to ECMO cannulation) as the only complication.
Conclusions: Assessing bleeding risk of every patient and setting adequate FVIII levels targets before anticoagulation and surgery, as well as evaluating bleeding and thrombotic risk related to support devices to manage anticoagulation by a multidisciplinary team, makes it possible to choose the best option for each patient.
[Correlation between ROTEM parameters and FVIII levels ]
| Type of Cx and stage | APTT (sec) | FVIII (U/dL) | PT (% activity | AntiXa (IU/mL) | CT INTEM (sec) | CT HEPTEM (sec) | CT HEPTEM /CT INTEM RATIO | CT EXTEM (sec) | |
| Reference or target range | 24-40 | 50-150 | 70-120 | 0.3-0.7 (UFH) | 120-220 | 120-220 | Heparin Absence >0.8 | 45- 79 | |
| Test 2 weeks pre Surgery | Pre-Infusion of FVIII | 38 | 39 | 97 | 176 | 56 | |||
| Test 2 weeks pre Surgery | Post-Infusion of FVIII | 37 | 80 | 97 | 127 | 66 | |||
| VAD CCV surgery | Basal | 0.42 | 320 | 217 | 0.68 | 75 | |||
| VAD CCV surgery | CPB | 253 | 90 | ||||||
| VAD CCV surgery | Post Protamine | 45 | 49 | 63 | 218 | 201 | 0.92 | 80 | |
| HT CCV surgery | Basal | 36 | 81 | 89 | 0.29 | 176 | 169 | 0.96 | 56 |
| HT CCV surgery | CPB | 179 | 89 | ||||||
| HT CCV surgery | Post Protamine | 34 | 102 | 73 | 152 | 145 | 0.95 | 66 | |
[APTT, FVIII, AntiXa and ROTEM parameters at VAD and HT surgeries]
To cite this abstract in AMA style:
Tisi Bana MF, Neme D, Cereigido C, Ferrero M, Barretta J, Osuna J, Napoli N, Martinuzzo M, Lopez M, Rossi P, Polidori C, Young G, Altuna D. Hemostatic Management of Severe Hemophilia A (SHA) Patient with Dilated Myocardiopathy through Extracorporeal Membrane Oxygenation (ECMO), Ventricular Assist Device (VAD) and Heart Transplant (HT) [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/hemostatic-management-of-severe-hemophilia-a-sha-patient-with-dilated-myocardiopathy-through-extracorporeal-membrane-oxygenation-ecmo-ventricular-assist-device-vad-and-heart-transplant-ht/. Accessed November 30, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/hemostatic-management-of-severe-hemophilia-a-sha-patient-with-dilated-myocardiopathy-through-extracorporeal-membrane-oxygenation-ecmo-ventricular-assist-device-vad-and-heart-transplant-ht/