EHA Library - The official digital education library of European Hematology Association (EHA)

TRANSTHORACIC AORTIC VALVE IMPLANTATION (TAVI) IN HEMOPHILIA
Author(s): ,
Manuel Lopez
Affiliations:
Hematology Department,Hospital Alvaro Cunqueiro,Vigo,Spain
,
Elsa Lopez Ansoar
Affiliations:
Hematology Department,Hospital Alvaro Cunqueiro,Vigo,Spain
,
Agustin Nieto Vazquez
Affiliations:
Hematology Department,Hospital Alvaro Cunqueiro,Vigo,Spain
,
Oscar Dominguez Muñiz
Affiliations:
Hematology Department,Hospital Alvaro Cunqueiro,Vigo,Spain
Carmen Albo Lopez
Affiliations:
Hematology Department,Hospital Alvaro Cunqueiro,Vigo,Spain
EHA Library. Rodríguez Lopez M. 06/09/21; 324144; PB1464
Dr. Manuel Rodríguez Lopez
Dr. Manuel Rodríguez Lopez
Contributions
Abstract

Abstract: PB1464

Type: Publication Only

Session title: Bleeding disorders (congenital and acquired)

Background

The increase in life expectancy of hemophiliac patients leads to the appearance of health problems whose proper managment is not clearly defined. Cardiac Surgery presents a major hemostatic challenge for a multitude of reasons, particularly in patients suffering from congenital or acquired hemostatic disorders. Acknowledging that, evidence from the literature suggests that cardiac surgery can be performed safely in this patient population

Aims

We reported the first TAVI performed in our hospital in hemophiliac patient, the management during and after hospital discharge

Methods

Male, 74 years, mild hemophilia A (F8:C 9%, F8 chromogenic 5,4%) without inibitor AntiF8(< 0.6 Bethesda Units, uBT) . We not know the responsible mutation. In the past, he received treatment with different Concentrates of F8 (the last, ReFacto AF®). Previous surgeries: Total Hip Prostesis. CV Risk Factors: Arterial Hypertension (Medical treatment), Obesity, Diet-controlled hyperglycemia. He has a chronic liver disease due Virus C.


He had an Severe Aortic stenosis who needed surgical replacement and the chosen option was TAVI to reduce the surgical risks, among which bleeding

Results

30 minutes before the procedure, ReFacto AF® 50 IU / kg was administered; A 29 mm Corevalve Evolut® R prosthesis was implanted, for which access was required through both femoral and right jugular veins to place a temporary pacemaker, a procedure that required the use of Unfractionated Heparin (5000 IU) without complications. At 3 hours, a 2nd dose of ReFacto AF® of approx. 10 IU / Kg was administered and the plasma levels of F8 were monitored using the chromogenic technique (level 110%). 8 hours after the 1st administration, ReFacto AF® started every 8 hours to maintain a trough level of approx 80% for first 48 hours, then adjusted every 12 hours (trough level of 50%) up to 7 days, then 30% (Desmopressin and ReFacto AF, alternate days) until completing 12 days of treatment. Simultaneously, antithrombotic prophylaxis was started with Enoxaparin®  40 mg (from day +1 to discharge) and simple antiplatelet therapy (ASA 100 mg / day), which he currently maintains. Hospital dischage was at day +14.


The inhibitor was removed 15 days and 2 months after the procedure - according to the protocol of our service - resulting negative. The patient reports an improvement of his quality of life, compared with the previous one, with disappearance of associated symptoms and progressive increase of physical activity, more according to his age and general condition


 

Conclusion

We present a succesfully case of TAVI in mild haemophiliac patient. TAVI is a safe and effective option versus open heart surgery in high-risk patients as haemophiliac patients. A search in PuBmed® database, crossing 2 topics: Aortic Stenosis and Haemophilia, showed 5 citations, 1 for Haemophilia B and 4 Haemophilia A; just case reports, so we consider it interesting to communicate our experience in order to generate scientific evidence so as not only to recomend this therapeutic option as of choice in these patients but also to guide the performance of the procedure, in close collaboration with cardiologists and anesthetists

Keyword(s): Hemophilia A

Abstract: PB1464

Type: Publication Only

Session title: Bleeding disorders (congenital and acquired)

Background

The increase in life expectancy of hemophiliac patients leads to the appearance of health problems whose proper managment is not clearly defined. Cardiac Surgery presents a major hemostatic challenge for a multitude of reasons, particularly in patients suffering from congenital or acquired hemostatic disorders. Acknowledging that, evidence from the literature suggests that cardiac surgery can be performed safely in this patient population

Aims

We reported the first TAVI performed in our hospital in hemophiliac patient, the management during and after hospital discharge

Methods

Male, 74 years, mild hemophilia A (F8:C 9%, F8 chromogenic 5,4%) without inibitor AntiF8(< 0.6 Bethesda Units, uBT) . We not know the responsible mutation. In the past, he received treatment with different Concentrates of F8 (the last, ReFacto AF®). Previous surgeries: Total Hip Prostesis. CV Risk Factors: Arterial Hypertension (Medical treatment), Obesity, Diet-controlled hyperglycemia. He has a chronic liver disease due Virus C.


He had an Severe Aortic stenosis who needed surgical replacement and the chosen option was TAVI to reduce the surgical risks, among which bleeding

Results

30 minutes before the procedure, ReFacto AF® 50 IU / kg was administered; A 29 mm Corevalve Evolut® R prosthesis was implanted, for which access was required through both femoral and right jugular veins to place a temporary pacemaker, a procedure that required the use of Unfractionated Heparin (5000 IU) without complications. At 3 hours, a 2nd dose of ReFacto AF® of approx. 10 IU / Kg was administered and the plasma levels of F8 were monitored using the chromogenic technique (level 110%). 8 hours after the 1st administration, ReFacto AF® started every 8 hours to maintain a trough level of approx 80% for first 48 hours, then adjusted every 12 hours (trough level of 50%) up to 7 days, then 30% (Desmopressin and ReFacto AF, alternate days) until completing 12 days of treatment. Simultaneously, antithrombotic prophylaxis was started with Enoxaparin®  40 mg (from day +1 to discharge) and simple antiplatelet therapy (ASA 100 mg / day), which he currently maintains. Hospital dischage was at day +14.


The inhibitor was removed 15 days and 2 months after the procedure - according to the protocol of our service - resulting negative. The patient reports an improvement of his quality of life, compared with the previous one, with disappearance of associated symptoms and progressive increase of physical activity, more according to his age and general condition


 

Conclusion

We present a succesfully case of TAVI in mild haemophiliac patient. TAVI is a safe and effective option versus open heart surgery in high-risk patients as haemophiliac patients. A search in PuBmed® database, crossing 2 topics: Aortic Stenosis and Haemophilia, showed 5 citations, 1 for Haemophilia B and 4 Haemophilia A; just case reports, so we consider it interesting to communicate our experience in order to generate scientific evidence so as not only to recomend this therapeutic option as of choice in these patients but also to guide the performance of the procedure, in close collaboration with cardiologists and anesthetists

Keyword(s): Hemophilia A

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